Primary amyloidosis differential diagnosis: Difference between revisions

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==Differentiating Primary amyloidosis from Other Diseases==
==Differentiating Primary amyloidosis from Other Diseases==
{|
{|
! colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Organ System Involvement}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Differential Diagnosis}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
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! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Therapy}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Therapy}}
|-
|-
| colspan="2" rowspan="6" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''P = Polyneuropathy'''''
| rowspan="6" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Polyneuropathy'''''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''POEMS syndrome (Demyelinating)'''<ref name="pmid8608236">{{cite journal |vauthors=Gherardi RK, Bélec L, Soubrier M, Malapert D, Zuber M, Viard JP, Intrator L, Degos JD, Authier FJ |title=Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome |journal=Blood |volume=87 |issue=4 |pages=1458–65 |date=February 1996 |pmid=8608236 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''POEMS syndrome (Demyelinating)'''
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* [[Monoclonal]] [[plasma cell]] proliferation  
* [[Monoclonal]] [[plasma cell]] proliferation  
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* [[Areflexia]]
* [[Areflexia]]
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*Increased number of [[Platelet|thrombocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased number of [[Platelet|thrombocytes]]
*Increased number of [[Red blood cell|erythrocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased number of [[Red blood cell|erythrocytes]]<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref><ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref><ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Elevated [[Cerebrospinal fluid|cerebrospinal fluid (CSF)]] [[protein]] content<ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref>
*Elevated [[Cerebrospinal fluid|cerebrospinal fluid (CSF)]] [[protein]] content<ref name="pmid6248720">{{cite journal |vauthors=Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL |title=Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature |journal=Medicine (Baltimore) |volume=59 |issue=4 |pages=311–22 |date=July 1980 |pmid=6248720 |doi= |url=}}</ref>
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*High levels of [[Immunoglobulin G|IgG]] lambda or [[Immunoglobulin A|IgA]] lambda [[M protein|M-protein]] in the [[serum]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*High levels of [[Immunoglobulin G|IgG]] lambda or [[Immunoglobulin A|IgA]] lambda [[M protein|M-protein]] in the [[serum]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased number of [[Plasma cell|plasma cells]] in the [[bone marrow]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased number of [[Plasma cell|plasma cells]] in the [[bone marrow]]<ref name="pmid6315993">{{cite journal |vauthors=Takatsuki K, Sanada I |title=Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases |journal=Jpn. J. Clin. Oncol. |volume=13 |issue=3 |pages=543–55 |date=September 1983 |pmid=6315993 |doi= |url=}}</ref>
*Increased serum [[Vascular endothelial growth factor|VEGF]] level<ref name="pmid19289745">{{cite journal |vauthors=Nobile-Orazio E, Terenghi F, Giannotta C, Gallia F, Nozza A |title=Serum VEGF levels in POEMS syndrome and in immune-mediated neuropathies |journal=Neurology |volume=72 |issue=11 |pages=1024–6 |date=March 2009 |pmid=19289745 |doi=10.1212/01.wnl.0000344569.13496.ff |url=}}</ref>
*Increased serum [[Vascular endothelial growth factor|VEGF]] level
*Elevated levels of antitiroglobulin [[antibody]] and antithyroid peroxydase [[antibody]]<ref name="pmid25888197">{{cite journal |vauthors=Güneş HN, Bilecenoğlu NT, Şener U, Yoldaş TK |title=POEMS syndrome with peripheral and central nervous system demyelination: case report |journal=Neurologist |volume=19 |issue=4 |pages=101–3 |date=April 2015 |pmid=25888197 |doi=10.1097/NRL.0000000000000017 |url=}}</ref>
*Elevated levels of antitiroglobulin [[antibody]] and antithyroid peroxydase [[antibody]]
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* [[POEMS syndrome diagnostic criteria|International Myeloma Working Group (IMWG) clinical and laboratory diagnostic criteria]]<ref name="urlIMWG | International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma">{{cite web |url=http://imwg.myeloma.org/international-myeloma-working-group-imwg-criteria-for-the-diagnosis-of-multiple-myeloma/ |title=IMWG &#124; International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma |format= |work= |accessdate=}}</ref>
* [[POEMS syndrome diagnostic criteria|International Myeloma Working Group (IMWG) clinical and laboratory diagnostic criteria]]
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|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Metabolic syndrome|Metabolic Syndrome]] (Axonal pathology)'''<ref name="pmid25897354">{{cite journal |vauthors=Schreiber AK, Nones CF, Reis RC, Chichorro JG, Cunha JM |title=Diabetic neuropathic pain: Physiopathology and treatment |journal=World J Diabetes |volume=6 |issue=3 |pages=432–44 |date=April 2015 |pmid=25897354 |doi=10.4239/wjd.v6.i3.432 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Metabolic syndrome|Metabolic Syndrome]] (Axonal pathology)'''
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* [[Diabetes mellitus]]
* [[Diabetes mellitus]]
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* [[Monofilament|Monofilament testing]]
* [[Monofilament|Monofilament testing]]
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* [[Fasting blood sugar|Fasting blood sugar level]] greater than equal to 126 mg/dl on 2 separate occasions<ref name="pmid20042775">{{cite journal |vauthors= |title=Diagnosis and classification of diabetes mellitus |journal=Diabetes Care |volume=33 Suppl 1 |issue= |pages=S62–9 |date=January 2010 |pmid=20042775 |doi=10.2337/dc10-S062 |url=}}</ref>
* [[Fasting blood sugar|Fasting blood sugar level]] greater than equal to 126 mg/dl on 2 separate occasions
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* [[Diabetes mellitus medical therapy|Anti-diabetic therapy]]
* [[Diabetes mellitus medical therapy|Anti-diabetic therapy]]
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* [[Foot care]]
* [[Foot care]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Vitamin deficiencies|Vitamin Deficiencies]] (Axonal Pathology)'''<ref name="pmid28129784">{{cite journal |vauthors=Ekabe CJ, Kehbila J, Abanda MH, Kadia BM, Sama CB, Monekosso GL |title=Vitamin B12 deficiency neuropathy; a rare diagnosis in young adults: a case report |journal=BMC Res Notes |volume=10 |issue=1 |pages=72 |date=January 2017 |pmid=28129784 |doi=10.1186/s13104-017-2393-3 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Vitamin deficiencies|Vitamin Deficiencies]] (Axonal Pathology)'''
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* [[Vitamin B12 deficiency]] (Decreased [[S-Adenosyl methionine|S-adenosyl methionine]])<ref name="pmid2738712">{{cite journal |vauthors=Doi T, Kawata T, Tadano N, Iijima T, Maekawa A |title=Effect of vitamin B12 deficiency on S-adenosylmethionine metabolism in rats |journal=J. Nutr. Sci. Vitaminol. |volume=35 |issue=1 |pages=1–9 |date=February 1989 |pmid=2738712 |doi= |url=}}</ref>
* [[Vitamin B12 deficiency]] (Decreased [[S-Adenosyl methionine|S-adenosyl methionine]])
* [[Thiamine deficiency|Vitamin B1 deficiency]]
* [[Thiamine deficiency|Vitamin B1 deficiency]]
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*
*
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* [[Anemia]] ([[Megaloblastic Anemias|megaloblastic]] in case of [[Vitamin B12 deficiency|B12 deficiency]])<ref name="pmid23262189">{{cite journal |vauthors=Berg RL, Shaw GR |title=Laboratory evaluation for vitamin B12 deficiency: the case for cascade testing |journal=Clin Med Res |volume=11 |issue=1 |pages=7–15 |date=February 2013 |pmid=23262189 |doi=10.3121/cmr.2012.1112 |url=}}</ref>
* [[Anemia]] ([[Megaloblastic Anemias|megaloblastic]] in case of [[Vitamin B12 deficiency|B12 deficiency]])
* Decreased [[serum]] [[Vitamin B12]] levels (< 200 pg/ml)
* Decreased [[serum]] [[Vitamin B12]] levels (< 200 pg/ml)
* [[Methylmalonic acidemia|Elevated methylmalonic acid]]
* [[Methylmalonic acidemia|Elevated methylmalonic acid]]
*  
*  
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* [[Serum]] [[Vitamin B12]] levels<ref name="pmid2339684">{{cite journal |vauthors=Lindenbaum J, Savage DG, Stabler SP, Allen RH |title=Diagnosis of cobalamin deficiency: II. Relative sensitivities of serum cobalamin, methylmalonic acid, and total homocysteine concentrations |journal=Am. J. Hematol. |volume=34 |issue=2 |pages=99–107 |date=June 1990 |pmid=2339684 |doi= |url=}}</ref>
* [[Serum]] [[Vitamin B12]] levels
* [[Methylmalonic acid|Methylmalonic acid levels]]
* [[Methylmalonic acid|Methylmalonic acid levels]]
* [[Intrinsic factor|Intrinsic factor antibodies]]<ref name="pmid232621892">{{cite journal |vauthors=Berg RL, Shaw GR |title=Laboratory evaluation for vitamin B12 deficiency: the case for cascade testing |journal=Clin Med Res |volume=11 |issue=1 |pages=7–15 |date=February 2013 |pmid=23262189 |pmc=3573090 |doi=10.3121/cmr.2012.1112 |url=}}</ref>
* [[Intrinsic factor|Intrinsic factor antibodies]]


*
*
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* [[Vitamin B12]] supplement ([[parenteral]])<ref name="pmid12086562">{{cite journal |vauthors=Lane LA, Rojas-Fernandez C |title=Treatment of vitamin b(12)-deficiency anemia: oral versus parenteral therapy |journal=Ann Pharmacother |volume=36 |issue=7-8 |pages=1268–72 |date=2002 |pmid=12086562 |doi=10.1345/aph.1A122 |url=}}</ref>
* [[Vitamin B12]] supplement ([[parenteral]])
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Guillain-Barré syndrome|Guillain-Barre Syndrome]] (Demyelinating)'''<ref name="pmid117249122">{{cite journal |vauthors=Winer JB |title=Guillain Barré syndrome |journal=MP, Mol. Pathol. |volume=54 |issue=6 |pages=381–5 |date=December 2001 |pmid=11724912 |pmc=1187127 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Guillain-Barré syndrome|Guillain-Barre Syndrome]] (Demyelinating)'''
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* [[Anti-ganglioside antibodies|Anti-ganglioside]] and anti-[[myelin]] antibodies<ref name="pmid7486873">{{cite journal |vauthors=Rees JH, Gregson NA, Hughes RA |title=Anti-ganglioside GM1 antibodies in Guillain-Barré syndrome and their relationship to Campylobacter jejuni infection |journal=Ann. Neurol. |volume=38 |issue=5 |pages=809–16 |date=November 1995 |pmid=7486873 |doi=10.1002/ana.410380516 |url=}}</ref>
* [[Anti-ganglioside antibodies|Anti-ganglioside]] and anti-[[myelin]] antibodies
* [[Viral]] infections:<ref name="pmid3404161">{{cite journal |vauthors=Winer JB, Hughes RA, Anderson MJ, Jones DM, Kangro H, Watkins RP |title=A prospective study of acute idiopathic neuropathy. II. Antecedent events |journal=J. Neurol. Neurosurg. Psychiatry |volume=51 |issue=5 |pages=613–8 |date=May 1988 |pmid=3404161 |pmc=1033063 |doi= |url=}}</ref>
* [[Viral]] infections:
** [[Epstein Barr virus]]
**[[Epstein Barr virus]]
** [[Human Immunodeficiency Virus (HIV)|HIV]]
** [[Human Immunodeficiency Virus (HIV)|HIV]]
** [[Cytomegalovirus]]
** [[Cytomegalovirus]]
** [[Varicella Zoster Virus|Varicella Zoster virus]]
** [[Varicella Zoster Virus|Varicella Zoster virus]]
* [[Bacterial]] infections:<ref name="pmid16969154">{{cite journal |vauthors=Yuki N, Koga M |title=Bacterial infections in Guillain-Barré and Fisher syndromes |journal=Curr. Opin. Neurol. |volume=19 |issue=5 |pages=451–7 |date=October 2006 |pmid=16969154 |doi=10.1097/01.wco.0000245367.36576.e9 |url=}}</ref>
* [[Bacterial]] infections:
** [[Campylobacter]] infection
**[[Campylobacter]] infection
** [[Mycoplasma pneumoniae]]
** [[Mycoplasma pneumoniae]]
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* Delayed F waves<ref name="pmid666277">{{cite journal |vauthors=Kimura J |title=Proximal versus distal slowing of motor nerve conduction velocity in the Guillain-Barré syndrome |journal=Ann. Neurol. |volume=3 |issue=4 |pages=344–50 |date=April 1978 |pmid=666277 |doi=10.1002/ana.410030412 |url=}}</ref>
* Delayed F waves
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* Clinical diagnostic criteria (progressive weakness of more than two [[limbs]], [[areflexia]], and progression for no more than four weeks)<ref name="pmid11724912">{{cite journal |vauthors=Winer JB |title=Guillain Barré syndrome |journal=MP, Mol. Pathol. |volume=54 |issue=6 |pages=381–5 |date=December 2001 |pmid=11724912 |pmc=1187127 |doi= |url=}}</ref>
* Clinical diagnostic criteria (progressive weakness of more than two [[limbs]], [[areflexia]], and progression for no more than four weeks)
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* [[Intravenous]] [[immunoglobulins]]<ref name="pmid9014908">{{cite journal |vauthors= |title=Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group |journal=Lancet |volume=349 |issue=9047 |pages=225–30 |date=January 1997 |pmid=9014908 |doi= |url=}}</ref>
* [[Intravenous]] [[immunoglobulins]]
* [[Plasma]] exchange<ref name="pmid4022342">{{cite journal |vauthors= |title=Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group |journal=Neurology |volume=35 |issue=8 |pages=1096–104 |date=August 1985 |pmid=4022342 |doi= |url=}}</ref>
* [[Plasma]] exchange
* [[Mechanical ventilation|Respiratory support]]
* [[Mechanical ventilation|Respiratory support]]
* [[DVT]]/[[PE]] [[prevention]]
* [[DVT]]/[[PE]] [[prevention]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic inflammatory demyelinating polyneuropathy|Chronic Inflammatory Demyelinating Polyneuropathy]] (CIDP) (Mixed axonal and demyelinatiing)'''<ref name="urlChronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria | JAMA Neurology | JAMA Network">{{cite web |url=https://jamanetwork.com/journals/jamaneurology/article-abstract/589258 |title=Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Characteristics, Course, and Recommendations for Diagnostic Criteria &#124; JAMA Neurology &#124; JAMA Network |format= |work= |accessdate=}}</ref><ref name="urlonlinelibrary.wiley.com2">{{cite web |url=https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1468-1331.2009.02930.x |title=onlinelibrary.wiley.com |format= |work= |accessdate=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic inflammatory demyelinating polyneuropathy|Chronic Inflammatory Demyelinating Polyneuropathy]] (CIDP) (Mixed axonal and demyelinatiing)'''
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* Abnormal [[immune response]] (both [[Immunoglobulin G|IgG]] based [[Humoral immunity|humoral]] and [[Cell mediated immunity|T-Cell mediated]]) response to unknown [[antigen]] (possible culprits include [[myelin]] [[proteins]] P0, P2 and PMP22)<ref name="pmid2440998">{{cite journal |vauthors=Milner P, Lovelidge CA, Taylor WA, Hughes RA |title=P0 myelin protein produces experimental allergic neuritis in Lewis rats |journal=J. Neurol. Sci. |volume=79 |issue=3 |pages=275–85 |date=July 1987 |pmid=2440998 |doi= |url=}}</ref><ref name="pmid10713353">{{cite journal |vauthors=Gabriel CM, Gregson NA, Hughes RA |title=Anti-PMP22 antibodies in patients with inflammatory neuropathy |journal=J. Neuroimmunol. |volume=104 |issue=2 |pages=139–46 |date=May 2000 |pmid=10713353 |doi= |url=}}</ref><ref name="pmid10025777">{{cite journal |vauthors=Bouchard C, Lacroix C, Planté V, Adams D, Chedru F, Guglielmi JM, Said G |title=Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy |journal=Neurology |volume=52 |issue=3 |pages=498–503 |date=February 1999 |pmid=10025777 |doi= |url=}}</ref>
* Abnormal [[immune response]] (both [[Immunoglobulin G|IgG]] based [[Humoral immunity|humoral]] and [[Cell mediated immunity|T-Cell mediated]]) response to unknown [[antigen]] (possible culprits include [[myelin]] [[proteins]] P0, P2 and PMP22)


*
*
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* [[Areflexia]]
* [[Areflexia]]
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* Elevated [[CSF]] [[protein]] ([[oligoclonal bands]] with normal [[White blood cells|WBCs]])<ref name="pmid11558784">{{cite journal |vauthors=Yan WX, Archelos JJ, Hartung HP, Pollard JD |title=P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy |journal=Ann. Neurol. |volume=50 |issue=3 |pages=286–92 |date=September 2001 |pmid=11558784 |doi= |url=}}</ref>
* Elevated [[CSF]] [[protein]] ([[oligoclonal bands]] with normal [[White blood cells|WBCs]])
* Slowed [[Nerve conduction study|motor nerve conduction velocities]]<ref name="pmid2757528">{{cite journal |vauthors=Barohn RJ, Kissel JT, Warmolts JR, Mendell JR |title=Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria |journal=Arch. Neurol. |volume=46 |issue=8 |pages=878–84 |date=August 1989 |pmid=2757528 |doi= |url=}}</ref>
* Slowed [[Nerve conduction study|motor nerve conduction velocities]]
* Prolonged distal [[Motor skill|motor]] latencies (period between F wave and initial stimulation)
* Prolonged distal [[Motor skill|motor]] latencies (period between F wave and initial stimulation)
* Delayed F wave latencies (recorded from the [[feet]], hence called "F" waves)<ref name="pmid27575282">{{cite journal |vauthors=Barohn RJ, Kissel JT, Warmolts JR, Mendell JR |title=Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria |journal=Arch. Neurol. |volume=46 |issue=8 |pages=878–84 |date=August 1989 |pmid=2757528 |doi= |url=}}</ref>
* Delayed F wave latencies (recorded from the [[feet]], hence called "F" waves)
* [[MRI]] contrast enhancement and enlargement of [[Vertebra|T2]] [[spinal segments]]<ref name="pmid23564314">{{cite journal |vauthors=Dimachkie MM, Barohn RJ |title=Chronic inflammatory demyelinating polyneuropathy |journal=Curr Treat Options Neurol |volume=15 |issue=3 |pages=350–66 |date=June 2013 |pmid=23564314 |pmc=3987657 |doi=10.1007/s11940-013-0229-6 |url=}}</ref>
* [[MRI]] contrast enhancement and enlargement of [[Vertebra|T2]] [[spinal segments]]
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* EFNS/PNS criteria<ref name="urlonlinelibrary.wiley.com">{{cite web |url=https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1468-1331.2009.02930.x |title=onlinelibrary.wiley.com |format= |work= |accessdate=}}</ref>
* EFNS/PNS criteria
* Koski criteria<ref name="pmid19091330">{{cite journal |vauthors=Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, Gorson K, Hahn AF, Hughes RA, Katz J, Lewis RA, Parry GJ, van Doorn P, Cornblath DR |title=Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy |journal=J. Neurol. Sci. |volume=277 |issue=1-2 |pages=1–8 |date=February 2009 |pmid=19091330 |doi=10.1016/j.jns.2008.11.015 |url=}}</ref>
* Koski criteria
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Corticosteroids]]
* [[Corticosteroids]]
Line 152: Line 152:
* [[Immunosupressive drug|Immunosupressants]] ([[Alemtuzumab|Alemtuzemab]] [[Azathioprine]] [[Cyclophosphamide]] [[Cyclosporine|Cyclosporin]] [[Etanercept]] [[Interferon-alpha]])
* [[Immunosupressive drug|Immunosupressants]] ([[Alemtuzumab|Alemtuzemab]] [[Azathioprine]] [[Cyclophosphamide]] [[Cyclosporine|Cyclosporin]] [[Etanercept]] [[Interferon-alpha]])
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multifocal motor neuropathy|Multifocal Motor Neuropathy]]'''<ref name="RobbinsLawson2018">{{cite journal|last1=Robbins|first1=Nathaniel M|last2=Lawson|first2=Victoria|title=The Potential Misdiagnosis of Multifocal Motor Neuropathy as Amyotrophic Lateral Sclerosis—A Case Series|journal=US Neurology|volume=14|issue=2|year=2018|pages=102|issn=1758-4000|doi=10.17925/USN.2018.14.2.102}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multifocal motor neuropathy|Multifocal Motor Neuropathy]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Immune response|Abnormal immune response]] ([[Anti-ganglioside antibodies|Anti ganglioside]] [[Anti-ganglioside antibodies|GM-1]] [[IgM]] [[antibodies]])
* [[Immune response|Abnormal immune response]] ([[Anti-ganglioside antibodies|Anti ganglioside]] [[Anti-ganglioside antibodies|GM-1]] [[IgM]] [[antibodies]])
Line 163: Line 163:
*  
*  
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* Clinical criteria (EFNS/PNS):<ref name="pmid21199100">{{cite journal |vauthors= |title=European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision |journal=J. Peripher. Nerv. Syst. |volume=15 |issue=4 |pages=295–301 |date=December 2010 |pmid=21199100 |doi=10.1111/j.1529-8027.2010.00290.x |url=}}</ref>
* Clinical criteria (EFNS/PNS):
** Slowly progressive or step-wise progressive, focal, asymmetric  [[limb]] weakness; i.e., [[Motor skill|motor]] involvement in the [[motor nerve]]  distribution of at least two nerves for  > 1 month.
** Slowly progressive or step-wise progressive, focal, asymmetric  [[limb]] weakness; i.e., [[Motor skill|motor]] involvement in the [[motor nerve]]  distribution of at least two nerves for  > 1 month.
** No objective [[Sensory system|sensory]]  abnormalities except  for minor vibration  sense abnormalities in  the [[lower limbs]]
** No objective [[Sensory system|sensory]]  abnormalities except  for minor vibration  sense abnormalities in  the [[lower limbs]]
Line 173: Line 173:
* [[Rituximab]]
* [[Rituximab]]
|-
|-
| colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Organ System Involvement'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Organ System Involvement'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
Line 181: Line 181:
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
|-
|-
| colspan="2" rowspan="7" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''O = Organomegaly (Hepatosplenomegaly and Lymphadenopathy)'''''
| rowspan="7" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Organomegaly (Hepatosplenomegaly and Lymphadenopathy)'''''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Malaria]]'''<ref name="pmid22708041">{{cite journal |vauthors=Bartoloni A, Zammarchi L |title=Clinical aspects of uncomplicated and severe malaria |journal=Mediterr J Hematol Infect Dis |volume=4 |issue=1 |pages=e2012026 |date=2012 |pmid=22708041 |doi=10.4084/MJHID.2012.026 |url=}}</ref><ref name="pmid19488414">{{cite journal |vauthors=Tangpukdee N, Duangdee C, Wilairatana P, Krudsood S |title=Malaria diagnosis: a brief review |journal=Korean J. Parasitol. |volume=47 |issue=2 |pages=93–102 |date=June 2009 |pmid=19488414 |doi=10.3347/kjp.2009.47.2.93 |url=}}</ref><ref name="pmid8703186">{{cite journal |vauthors=White NJ |title=The treatment of malaria |journal=N. Engl. J. Med. |volume=335 |issue=11 |pages=800–6 |date=September 1996 |pmid=8703186 |doi=10.1056/NEJM199609123351107 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Malaria]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Plasmodium falciparum]]
* [[Plasmodium falciparum]]
Line 220: Line 220:
** [[Atovaquone-Proguanil|Atovaquone plus proguanil]]
** [[Atovaquone-Proguanil|Atovaquone plus proguanil]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Kala-azar]]'''<ref name="pmid10798038">{{cite journal |vauthors=Aggarwal P, Handa R, Singh S, Wali JP |title=Kala-azar--new developments in diagnosis and treatment |journal=Indian J Pediatr |volume=66 |issue=1 |pages=63–71 |date=1999 |pmid=10798038 |doi= |url=}}</ref><ref name="pmid28649370">{{cite journal |vauthors=Torres-Guerrero E, Quintanilla-Cedillo MR, Ruiz-Esmenjaud J, Arenas R |title=Leishmaniasis: a review |journal=F1000Res |volume=6 |issue= |pages=750 |date=2017 |pmid=28649370 |doi=10.12688/f1000research.11120.1 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Kala-azar]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Leishmaniasis|Leshmania donovani]]
* [[Leishmaniasis|Leshmania donovani]]
Line 245: Line 245:
* [[Pentamidine]]
* [[Pentamidine]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Hepatitis|Infective Hepatitis]]'''<ref name="pmid19399811">{{cite journal |vauthors=Liang TJ |title=Hepatitis B: the virus and disease |journal=Hepatology |volume=49 |issue=5 Suppl |pages=S13–21 |date=May 2009 |pmid=19399811 |pmc=2809016 |doi=10.1002/hep.22881 |url=}}</ref><ref name="pmid26052383">{{cite journal |vauthors=Li HC, Lo SY |title=Hepatitis C virus: Virology, diagnosis and treatment |journal=World J Hepatol |volume=7 |issue=10 |pages=1377–89 |date=June 2015 |pmid=26052383 |pmc=4450201 |doi=10.4254/wjh.v7.i10.1377 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Hepatitis|Infective Hepatitis]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Hepatitis A virus|Hepatitis A virus (HAV)]]
* [[Hepatitis A virus|Hepatitis A virus (HAV)]]
Line 282: Line 282:
* [[Antivirals|Nucleotide analogs]]
* [[Antivirals|Nucleotide analogs]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic myelogenous leukemia|Chronic Myelogenous Leukemia]] (CML)'''<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19</ref><ref name="pmid24729196">{{cite journal |vauthors=Jabbour E, Kantarjian H |title=Chronic myeloid leukemia: 2014 update on diagnosis, monitoring, and management |journal=Am. J. Hematol. |volume=89 |issue=5 |pages=547–56 |date=May 2014 |pmid=24729196 |doi=10.1002/ajh.23691 |url=}}</ref><ref name="pmid26434969">{{cite journal |vauthors=Thompson PA, Kantarjian HM, Cortes JE |title=Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015 |journal=Mayo Clin. Proc. |volume=90 |issue=10 |pages=1440–54 |date=October 2015 |pmid=26434969 |pmc=5656269 |doi=10.1016/j.mayocp.2015.08.010 |url=}}</ref><ref name="pmid10428738">{{cite journal |vauthors=Faderl S, Talpaz M, Estrov Z, Kantarjian HM |title=Chronic myelogenous leukemia: biology and therapy |journal=Ann. Intern. Med. |volume=131 |issue=3 |pages=207–19 |date=August 1999 |pmid=10428738 |doi= |url=}}</ref><ref name="cancer.ca">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia-chronic-myelogenous-cml/treatment/chronic/?region=ab</ref><ref name="Tefferi">{{cite journal|title=Classification, diagnosis and management of myeloproliferative disorders in the JAK2V617F era|author=Tefferi A|journal=Hematology Am Soc Hematol Educ Program|date=2006|pages=240-245|pmid=17124067}}</ref><ref name="cancer.ca2">Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia-chronic-myelogenous-cml/signs-and-symptoms/?region=ab</ref><ref name="pmid264349692">{{cite journal |vauthors=Thompson PA, Kantarjian HM, Cortes JE |title=Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015 |journal=Mayo Clin. Proc. |volume=90 |issue=10 |pages=1440–54 |date=October 2015 |pmid=26434969 |pmc=5656269 |doi=10.1016/j.mayocp.2015.08.010 |url=}}</ref><ref name="pmid11550277">{{cite journal |vauthors=Wang YL, Bagg A, Pear W, Nowell PC, Hess JL |title=Chronic myelogenous leukemia: laboratory diagnosis and monitoring |journal=Genes Chromosomes Cancer |volume=32 |issue=2 |pages=97–111 |date=October 2001 |pmid=11550277 |doi= |url=}}</ref><ref name="pmid264349692">{{cite journal |vauthors=Thompson PA, Kantarjian HM, Cortes JE |title=Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015 |journal=Mayo Clin. Proc. |volume=90 |issue=10 |pages=1440–54 |date=October 2015 |pmid=26434969 |pmc=5656269 |doi=10.1016/j.mayocp.2015.08.010 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Chronic myelogenous leukemia|Chronic Myelogenous Leukemia]] (CML)'''<ref name="pmid264349692">{{cite journal |vauthors=Thompson PA, Kantarjian HM, Cortes JE |title=Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015 |journal=Mayo Clin. Proc. |volume=90 |issue=10 |pages=1440–54 |date=October 2015 |pmid=26434969 |pmc=5656269 |doi=10.1016/j.mayocp.2015.08.010 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Philadelphia chromosome|BCR/ABL gene]] fusion product due to [[Chromosomal translocation|translocation]] [[mutation]] [[Philadelphia chromosome|t(9;22)]](q34;q11)<ref name="pmid23147546">{{cite journal |vauthors=Zahra K, Ben Fredj W, Ben Youssef Y, Zaghouani H, Chebchoub I, Zaier M, Badreddine S, Braham N, Sennana H, Khelif A |title=Chronic myeloid leukemia as a secondary malignancy after lymphoma in a child. A case report and review of the literature |journal=Onkologie |volume=35 |issue=11 |pages=690–3 |date=2012 |pmid=23147546 |doi=10.1159/000343952 |url=}}</ref>
* [[Philadelphia chromosome|BCR/ABL gene]] fusion product due to [[Chromosomal translocation|translocation]] [[mutation]] [[Philadelphia chromosome|t(9;22)]](q34;q11)
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Fever]]
* [[Fever]]
Line 349: Line 349:
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Primary amyloidosis|Primary (AL) Amyloidosis]]'''<ref name="pmid25378951">{{cite journal |vauthors=Real de Asúa D, Costa R, Galván JM, Filigheddu MT, Trujillo D, Cadiñanos J |title=Systemic AA amyloidosis: epidemiology, diagnosis, and management |journal=Clin Epidemiol |volume=6 |issue= |pages=369–77 |date=2014 |pmid=25378951 |pmc=4218891 |doi=10.2147/CLEP.S39981 |url=}}</ref><ref name="pmid24998818">{{cite journal |vauthors=Misumi Y, Ando Y |title=[Classification of amyloidosis] |language=Japanese |journal=Brain Nerve |volume=66 |issue=7 |pages=731–7 |date=July 2014 |pmid=24998818 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Primary amyloidosis|Primary (AL) Amyloidosis]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* Aggregation and deposition of [[immunoglobulin light chains]] that are usually produced by [[plasma cell]] clones<ref name="pmid21360109">{{cite journal |vauthors=Bilginer Y, Akpolat T, Ozen S |title=Renal amyloidosis in children |journal=Pediatr. Nephrol. |volume=26 |issue=8 |pages=1215–27 |date=August 2011 |pmid=21360109 |pmc=3119800 |doi=10.1007/s00467-011-1797-x |url=}}</ref><ref name="pmid28134587">{{cite journal |vauthors=Khoor A, Colby TV |title=Amyloidosis of the Lung |journal=Arch. Pathol. Lab. Med. |volume=141 |issue=2 |pages=247–254 |date=February 2017 |pmid=28134587 |doi=10.5858/arpa.2016-0102-RA |url=}}</ref>
* Aggregation and deposition of [[immunoglobulin light chains]] that are usually produced by [[plasma cell]] clones
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Nephrotic syndrome]] ([[peripheral edema]])
* [[Nephrotic syndrome]] ([[peripheral edema]])
Line 365: Line 365:
* Congo red staining
* Congo red staining
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* Melphalan-prednisone/dexamethasone<ref name="pmid15070667">{{cite journal |vauthors=Palladini G, Perfetti V, Obici L, Caccialanza R, Semino A, Adami F, Cavallero G, Rustichelli R, Virga G, Merlini G |title=Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation |journal=Blood |volume=103 |issue=8 |pages=2936–8 |date=April 2004 |pmid=15070667 |doi=10.1182/blood-2003-08-2788 |url=}}</ref>
* Melphalan-prednisone/dexamethasone
* Dexamethasone plus Cyclophosphamide-thalidomide <ref name="pmid16990593">{{cite journal |vauthors=Wechalekar AD, Goodman HJ, Lachmann HJ, Offer M, Hawkins PN, Gillmore JD |title=Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis |journal=Blood |volume=109 |issue=2 |pages=457–64 |date=January 2007 |pmid=16990593 |doi=10.1182/blood-2006-07-035352 |url=}}</ref>
* Dexamethasone plus Cyclophosphamide-thalidomide  
* Stem cell transplantation<ref name="pmid14734330">{{cite journal |vauthors=Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, Anderson JJ, O'Hara C, Finn KT, Libbey CA, Wiesman J, Quillen K, Swan N, Wright DG |title=High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study |journal=Ann. Intern. Med. |volume=140 |issue=2 |pages=85–93 |date=January 2004 |pmid=14734330 |doi= |url=}}</ref>
* Stem cell transplantation
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Gaucher's disease|Gaucher's Disease]]'''<ref name="pmid28218669">{{cite journal |vauthors=Stirnemann J, Belmatoug N, Camou F, Serratrice C, Froissart R, Caillaud C, Levade T, Astudillo L, Serratrice J, Brassier A, Rose C, Billette de Villemeur T, Berger MG |title=A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments |journal=Int J Mol Sci |volume=18 |issue=2 |pages= |date=February 2017 |pmid=28218669 |doi=10.3390/ijms18020441 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Gaucher's disease|Gaucher's Disease]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[GBA (gene)|GBA gene]] [[mutation]]
* [[GBA (gene)|GBA gene]] [[mutation]]
Line 395: Line 395:
* [[Blood transfusion]]
* [[Blood transfusion]]
|-
|-
| colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF| |'''Organ System Involvement'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF| |'''Organ System Involvement'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
Line 403: Line 403:
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
|-
|-
| rowspan="7" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''E = Endocrinopathy (Hypogonadism, Hypothyroidism, Hypopituitarism)'''''
| rowspan="7" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Cardiac Failure'''''
| rowspan="2" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Hypogonadism<ref name="pmid16985909">{{cite journal |vauthors=Carnegie C |title=Diagnosis of hypogonadism: clinical assessments and laboratory tests |journal=Rev Urol |volume=6 Suppl 6 |issue= |pages=S3–8 |date=2004 |pmid=16985909 |pmc=1472884 |doi= |url=}}</ref><ref name="pmid20518947">{{cite journal |vauthors=Dandona P, Rosenberg MT |title=A practical guide to male hypogonadism in the primary care setting |journal=Int. J. Clin. Pract. |volume=64 |issue=6 |pages=682–96 |date=May 2010 |pmid=20518947 |doi=10.1111/j.1742-1241.2010.02355.x |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Hypogonadism|Primary Hypogonadism]] (Hypergonadotrophic)'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Klinefelter's syndrome]]
*  
* [[Myotonic dystrophy]]
* [[Germ cell tumor|Sertoli-cell tumor]]
* [[Enzymatic]] defects
* [[Orchitis|Viral orchitis]] ([[mumps]])
* [[Cryptorchidism]]
* [[Autoimmune polyendocrine syndrome|Polyglandular autoimmune syndrome]]
* [[Testicular trauma]] and [[Testicular tumor|tumors]]
* [[Polycystic ovary syndrome|Polycystic ovarian syndrome]]
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Fatigue]]
* [[Decreased libido]]
* Delayed [[secondary sexual characteristics]]:
** Absent/scant [[pubic hair]]
** [[Impotence]]
** [[Erectile dysfunction]]
** [[Amenorrhea]]
** Delayed [[breast]] development
** Small [[testicular]] size
* Decreased [[muscle mass]]
* [[Osteoporosis]]
* [[Depression]]
* [[Infertility]]
* [[Irritability]]
* [[Mood swings]]
* Pot belly
* [[Weight gain]]
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased [[Sex hormones|sex hormone]] levels:
**  
** Decreased [[testosterone]]
** Decreased [[dihydrotestosterone]]
** Decreased [[estrogen]]
* Increased [[gonadotropins]]:
** Increased [[Luteinizing hormone|LH]]
** Increased [[FSH]]
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* Serum [[Sex hormone|sex hormone levels]]
*  
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Hormone replacement therapy]]
*  
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Hypogonadism|Secondary Hypogonadism]] (Hypogonadotrophic)'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Panhypopituitarism]]
*  
* [[Hyperprolactinemia]]
* Isolated [[gonadotropin deficiency]]
* [[Kallman syndrome|Kallman's syndrome]]
* Congenital
* [[Prader-Willi syndrome]]
* [[Hemochromatosis]]
* [[Obesity|Massive obesity]]
* [[Malnutrition]]
*  
*  
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased [[Sex hormones|sex hormone]] levels:
**  
** Decreased [[testosterone]]
**  
** Decreased [[dihydrotestosterone]]
** Decreased [[estrogen]]
* Decreased [[gonadotropins]]:
** Decreased [[LH]]
** Decreased [[FSH]]
|-
|-
| rowspan="3" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Hypothyroidism<ref name="pmid20596261">{{cite journal |vauthors=Kostoglou-Athanassiou I, Ntalles K |title=Hypothyroidism - new aspects of an old disease |journal=Hippokratia |volume=14 |issue=2 |pages=82–7 |date=April 2010 |pmid=20596261 |doi= |url=}}</ref><ref name="pmid24275187">{{cite journal |vauthors=Koulouri O, Moran C, Halsall D, Chatterjee K, Gurnell M |title=Pitfalls in the measurement and interpretation of thyroid function tests |journal=Best Pract. Res. Clin. Endocrinol. Metab. |volume=27 |issue=6 |pages=745–62 |date=December 2013 |pmid=24275187 |doi=10.1016/j.beem.2013.10.003 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Primary hypothyroidism|Primary Hypothyroidism]]
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Congenital hypothyroidism]]
*  
* Autoimmune ([[Hashimoto's thyroiditis|Hashimoto's]]) thyroiditis
* Resistance to [[TSH]]
| rowspan="3" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="3" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Fatigue]]
*  
* Cold intolerance
* Decreased [[sweating]]
* [[Hypothermia]]
* Coarse skin
* [[Weight gain]]
 
* Puffiness
* [[Hair loss]]
* [[Constipation]]
* [[Fever]] ([[thyroiditis]])
 
* [[Hoarseness]]
* [[Goiter]]
* Fullness in the throat and neck
 
* [[Depression]]
* [[Emotional lability]]
* [[Attention deficit]]
 
* [[Macroglossia]]
* [[Obstructive sleep apnea]]
* [[Paresthesia]]
* Nerve entrapment syndromes ([[carpal tunnel syndrome]])
* [[Blurred vision]] (central hypothyroidism)
* [[Ataxia]]
* [[Myxedema coma]] (with [[Edema|non-pitting edema]])
* [[Cardiomegaly]]
* [[Pericardial effusion]]
* [[Ascites]]
* [[Hyperlipidemia]]
* [[Galactorrhea]]
* [[Infertility]]
 
| style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased [[Triiodothyronine|T3]] and [[Thyroxine|T4]]
* Increased [[Thyroid-stimulating hormone|TSH]]
| rowspan="3" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="3" style="padding: 5px 5px; background: #F5F5F5;" |
* Serum [[Triiodothyronine|T3]], [[Thyroxine|T4]], [[Thyroid-stimulating hormone|TSH]] levels
*  
| rowspan="3" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Hormone replacement therapy]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Secondary hypothyroidism|Secondary Hypothyroidism]]
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Pituitary gland|Pituitary]] mass lesions, especially [[pituitary adenomas]]
*  
* [[Brain cyst|Brain cysts]] and [[Brain abscesses|abscesses]]
* [[Meningiomas]]
* [[Dysgerminoma|Dysgerminomas]]
* [[Metastatic tumor|Metastatic tumors]]
* [[Craniopharyngiomas]]
* [[Pituitary apoplexy]]
* [[Sheehan syndrome]] ([[postpartum]] pituitary necrosis)
* Idiopathic isolated [[Thyroid-stimulating hormone|TSH]] deficiency
* [[Lymphocytic hypophysitis|Lymphocytic]] or granulomatous hypophysitis
| style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased [[Triiodothyronine|T3]] and [[T4]]
* Decreased [[TSH]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |[[Tertiary hypothyroidism|Tertiary Hypothyroidism]]
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Hemochromatosis]]
*  
* [[Histiocytosis]]
* Developmental abnormalities
* [[Internal carotid artery|Internal carotid aneurysms]]
* Idiopathic isolated [[Thyrotropin-releasing hormone|TRH]] deficiency
| style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased [[Triiodothyronine|T3]] and [[Thyroxine|T4]]
* Decreased [[TRH]]
* Decreased [[TSH]]
|-
|-
| rowspan="2" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Hypopituitarism<ref name="pmid26790380">{{cite journal |vauthors=Kim SY |title=Diagnosis and Treatment of Hypopituitarism |journal=Endocrinol Metab (Seoul) |volume=30 |issue=4 |pages=443–55 |date=December 2015 |pmid=26790380 |doi=10.3803/EnM.2015.30.4.443 |url=}}</ref><ref name="pmid16597813">{{cite journal |vauthors=Prabhakar VK, Shalet SM |title=Aetiology, diagnosis, and management of hypopituitarism in adult life |journal=Postgrad Med J |volume=82 |issue=966 |pages=259–66 |date=April 2006 |pmid=16597813 |doi=10.1136/pgmj.2005.039768 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Congenital'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Idiopathic]]
*[[Idiopathic|I]]
* Anatomic lesion in [[sella turcica]] ([[Rathke's pouch|Rathke's cyst]])
* [[CNS]] malformations:
** Septo-optic-[[dysplasia]]
** [[Kallmann syndrome]]
** [[Pituitary stalk]] interruption syndrome
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Headache]]
*  
* [[Nausea]] and [[vomiting]]
* [[Visual impairment]]
* Fatigue
* [[Cold]] intolerance
* [[Hypotension]]
* [[Dizziness]]
* Weight loss
 
* [[Hypopituitarism history and symptoms|Features of hormonal deficiencies]]
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased FSH, LH
*  
* Decreased TSH
* Decreased ACTH
* Decreased GH
* Decreased ADH
* Decreased oxytocin
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* Serum hormone levels produced by pituitary
*  
| rowspan="2" style="padding: 5px 5px; background: #F5F5F5;" |
* Hormone replacement therapy
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''Acquired'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
* Posterior [[pituitary tumor]]: [[astrocytoma]], [[ganglioneuroma]]
*  
* [[Metastatic]]: [[Breast cancer|breast]], [[Lung cancer|lungs]], [[Colorectal cancer|colon]], [[Prostate Cancer|prostate]]
* Peripituitary lesions: [[Craniopharyngioma]], [[meningioma]], [[chordoma]], [[optic nerve glioma]]
* Transsphenoidal or transcranial surgery
* [[Radiation therapy|Radiation]]
* [[Traumatic brain injury]]
* [[Sheehan's syndrome]]
* [[Pituitary apoplexy]]
* [[Meningitis]]
* [[Hypophysitis]]
* Lymphoma
|-
|-
| colspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Organ System Involvement'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Organ System Involvement'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Causes'''}}
Line 599: Line 460:
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
| style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Therapy'''}}
|-
|-
| colspan="2" rowspan="4" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''M = M-protein ( Hematological Abnormality/Plasma Cell Dyscrasias)'''''
| rowspan="4" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''''Plasma Cell Dyscrasias'''''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multiple myeloma]]'''<ref name="urlMultiple Myeloma | NEJM">{{cite web |url=https://www.nejm.org/doi/full/10.1056/NEJMra1011442 |title=Multiple Myeloma &#124; NEJM |format= |work= |accessdate=}}</ref><ref name="pmid27557302">{{cite journal |vauthors=Palumbo A, Chanan-Khan A, Weisel K, Nooka AK, Masszi T, Beksac M, Spicka I, Hungria V, Munder M, Mateos MV, Mark TM, Qi M, Schecter J, Amin H, Qin X, Deraedt W, Ahmadi T, Spencer A, Sonneveld P |title=Daratumumab, Bortezomib, and Dexamethasone for Multiple Myeloma |journal=N. Engl. J. Med. |volume=375 |issue=8 |pages=754–66 |date=August 2016 |pmid=27557302 |doi=10.1056/NEJMoa1606038 |url=}}</ref><ref name="pmid26763514">{{cite journal |vauthors=Rajkumar SV, Kumar S |title=Multiple Myeloma: Diagnosis and Treatment |journal=Mayo Clin. Proc. |volume=91 |issue=1 |pages=101–19 |date=January 2016 |pmid=26763514 |pmc=5223450 |doi=10.1016/j.mayocp.2015.11.007 |url=}}</ref>
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Multiple myeloma]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Chromosomal aberration|Chromosomal aberrations]] or other [[Genetics|genetic]] insults
*[[Chromosomal aberration|Chromosomal aberrations]] or other [[Genetics|genetic]] insults
Line 637: Line 498:
*[[Autologous bone marrow transplantation|Autologous stem cell transplantation]]
*[[Autologous bone marrow transplantation|Autologous stem cell transplantation]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Monoclonal gammopathy of undetermined significance]] ([[MGUS]])<ref name="pmid16938117">{{cite journal |vauthors=Kyle RA, Rajkumar SV |title=Monoclonal gammopathy of undetermined significance |journal=Br. J. Haematol. |volume=134 |issue=6 |pages=573–89 |date=September 2006 |pmid=16938117 |doi=10.1111/j.1365-2141.2006.06235.x |url=}}</ref>'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Monoclonal gammopathy of undetermined significance]] ([[MGUS]])'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Chromosomal aberration|Chromosomal aberrations]] or other [[Genetics|genetic]] insults
*[[Chromosomal aberration|Chromosomal aberrations]] or other [[Genetics|genetic]] insults
Line 699: Line 560:
* Radiotherapy
* Radiotherapy
|-
|-
| colspan="2" rowspan="3" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Bone Lesions
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Skin Changes
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Osteoporosis]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Imbalance between [[bone resorption]] and [[bone]] formation
*Preceded by [[osteopenia]]
*Decreased bone mineral density
| style="padding: 5px 5px; background: #F5F5F5;" |
*Acute musculoskletal pain if [[bone fractures|fractures]] develop
*Severe decrease in [[bone mineral density|BMD]] on [[Dual energy X-ray absorptiometry|dual-energy X-ray absorptiometry]] ([[DEXA scan|DEXA]]) test
*T score less than -2.5 on [[DEXA scan|DEXA]] scan
| style="padding: 5px 5px; background: #F5F5F5;" |
* Normal serum calcium, phosphate, alkaline phosphatase and parathyroid hormone levels
* Decreased bone mass
 
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* T score less than -2.5 on [[DEXA scan|DEXA]] scan
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Calcium]] and [[vitamin D]] supplementation
*[[Bisphosphonate|Bisphosphonates]]
*Weight-bearing exercise
*[[Teriparatide]]
*[[RANKL|RANK ligand]] inhibitors ([[denosumab]])
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Osteomalacia]]'''<ref name="pmid15520146">{{cite journal |vauthors=Allen SC, Raut S |title=Biochemical recovery time scales in elderly patients with osteomalacia |journal=J R Soc Med |volume=97 |issue=11 |pages=527–30 |date=November 2004 |pmid=15520146 |doi=10.1258/jrsm.97.11.527 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" |
*Inadequate [[mineralization of bone]]
*Deficiency of [[vitamin D]] [[calcium]], or [[phosphorus]]
*[[Renal tubular acidosis]]
*[[Malabsorption]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*Diffuse [[bone]] pain, fatigue, and [[fractures]]
*Low [[bone mineral density|bone mineral density (BMD)]]
*[[Hypocalcemia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Decreased serum calcium
* Decreased serum phosphate
* Increased serum alkaline phosphatase
* Increased serum parathyroid hormone levels
| style="padding: 5px 5px; background: #F5F5F5;" |
* Bone biopsy (increased osteoid and decreased calcification)
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Vitamin D3]] supplementation
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Osteogenesis imperfecta]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Mutations]] in ''[[COL1A1]]'' or ''[[COL1A2]]''
*Impaired [[Type I collagen|type I collagen synthesis]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Short stature]], [[scoliosis]], and propensity for [[Bone fracture|fractures]]
*Blue discoloration of [[sclera]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Normal serum calcium
* Normal serum phosphate
* Increased serum alkaline phosphatase
| style="padding: 5px 5px; background: #F5F5F5;" |
* DNA analysis
* Collagen analysis
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Bisphosphonates]]
*[[Physical therapy]]
*Surgical fixation of brittle [[bones]]
*Genetic counseling for offspring
|-
| colspan="2" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Skin Changes
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Scurvy]]'''
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |'''[[Scurvy]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
Line 781: Line 578:
*Citrus fruits
*Citrus fruits
|}
|}
<references />

Revision as of 03:25, 30 October 2019

Primary amyloidosis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Differentiating Primary amyloidosis from Other Diseases

Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
Polyneuropathy POEMS syndrome (Demyelinating)
Metabolic Syndrome (Axonal pathology)
Vitamin Deficiencies (Axonal Pathology)
Guillain-Barre Syndrome (Demyelinating)
  • Delayed F waves
  • Clinical diagnostic criteria (progressive weakness of more than two limbs, areflexia, and progression for no more than four weeks)
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)
  • EFNS/PNS criteria
  • Koski criteria
Multifocal Motor Neuropathy
  • Progressive, asymmetric, distal and upper limb predominant weakness
  • No significant sensory abnormalities
  • Areflexia
  • Clinical criteria (EFNS/PNS):
    • Slowly progressive or step-wise progressive, focal, asymmetric limb weakness; i.e., motor involvement in the motor nerve distribution of at least two nerves for > 1 month.
    • No objective sensory abnormalities except for minor vibration sense abnormalities in the lower limbs
Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Organomegaly (Hepatosplenomegaly and Lymphadenopathy) Malaria
Kala-azar
Infective Hepatitis
Chronic Myelogenous Leukemia (CML)[4]
Lymphoma
Primary (AL) Amyloidosis
  • Typical green birefringence under polarized light after Congo red staining (appears in red under normal light)
  • Congo red staining
  • Melphalan-prednisone/dexamethasone
  • Dexamethasone plus Cyclophosphamide-thalidomide
  • Stem cell transplantation
Gaucher's Disease
Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Cardiac Failure
Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Plasma Cell Dyscrasias Multiple myeloma
  • Anemia
  • Thrombocytopenia
  • Leukopenia
  • Decreased albumin (reversed albumin:globulin ratio)
  • Increased serum creatinine, urea
  • Hypercalcemia
  • Elevated ESR
  • Normal-low alkaline phosphatase
  • RBC rouleaux formation
  • Bence-Jones proteins in urine
  • Clonal plasma cells on bone marrow exam greater than equal to 10%

AND

  • Any one of the following:
    • Evidence of end-organ damage
    • Hypercalcemia (>11 mg/dl)
    • Renal insufficiency
    • Anemia (Hb < 10 mg/dl)
    • Bone lesions
    • Greater than 1 lesions on MRI
Monoclonal gammopathy of undetermined significance (MGUS)
  • Serum M protein (IgG or IgA) <3g/dl

AND

  • Clonal bone marrow plasma cells < 10%

AND

  • No end-organ damage
  • Observation
Asymptomatic Plasma Cell Myeloma

(Smoldering and Indolent plasma cell myeloma)

  • Serum M protein (IgG or IgA greater than equal to 3 g/dl

OR

  • Urinary M protein greater than equal to 500 mg/24 h

AND/OR

  • Clonal bone marrow plasma cells 10-60%

AND

  • No end-organ damage
  • Observation
Plasmacytoma
  • On biopsy:
    • Solitary infiltrate of clonal plasma cells in bone (SBP) or soft tissue (EMP).
    • No evidence of infiltration by clonal plasma cells.
  • Negative skeletal survey plus MRI/CT spine and pelvis except for the solitary lesion.
  • Lack of hypercalcemia, renal insuffieciency, anemia, multiple bone lesions which would suggest MM
  • Diagnosis of exclusion
  • Radiotherapy
Skin Changes Scurvy
  1. ↑ Nozza A (2017). "POEMS SYNDROME: an Update". Mediterr J Hematol Infect Dis. 9 (1): e2017051. doi:10.4084/MJHID.2017.051. PMC 5584767. PMID 28894560.
  2. ↑ 2.0 2.1 Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (July 1980). "Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature". Medicine (Baltimore). 59 (4): 311–22. PMID 6248720.
  3. ↑ 3.0 3.1 3.2 3.3 Takatsuki K, Sanada I (September 1983). "Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases". Jpn. J. Clin. Oncol. 13 (3): 543–55. PMID 6315993.
  4. ↑ Thompson PA, Kantarjian HM, Cortes JE (October 2015). "Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015". Mayo Clin. Proc. 90 (10): 1440–54. doi:10.1016/j.mayocp.2015.08.010. PMC 5656269. PMID 26434969.
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