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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|Differential Diagnosis}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Causes}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF| Clinical Features}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Laboratory Findings}}
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|Gold Standard Test}}
! 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'''''
| 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: #F5F5F5;" |
* [[Monoclonal]] [[plasma cell]] proliferation
* Cytokine storm (IL-1, IL-6, IL-12, TNF alpha, VEGF)
| style="padding: 5px 5px; background: #F5F5F5;" |
* Symmetrical, ascending chronic progressive [[polyneuropathy]] with both [[Sensory system|sensory]] (pin-prick and vibration) and [[Motor skill|motor]] disability ([[Motor skill|motor]] > [[sensory]])
* Generalized/extermity [[pain]]
* [[Areflexia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*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 [[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>
*Increased number of [[White blood cells|leukocytes]]<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 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>
*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>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[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>
| style="padding: 5px 5px; background: #F5F5F5;" |
|-
| 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: #F5F5F5;" |
* [[Diabetes mellitus]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Symmetric sensorimotor distal polyneuropathy
* Asymmetric proximal neuropathy
* [[Oculomotor nerve palsy|3rd nerve palsy]]
* [[Carpal tunnel syndrome|Carpel tunnel syndrome]]
* [[Autonomic neuropathy]]
* "Glove and stocking" type pain
* [[Muscle wasting]]
* [[Hammer toe|Hammer toes]]
* [[Polyuria]]
* [[Polydipsia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Uncontrolled [[hyperglycemia]]
* Slowed [[Nerve conduction study|nerve conduction]]
* [[Small fiber peripheral neuropathy|Small fiber dysfunction]]
* [[Monofilament|Monofilament testing]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[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>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Diabetes mellitus medical therapy|Anti-diabetic therapy]]
* [[Gabapentin]]
* [[Carbamazepine]]
* [[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: #F5F5F5;" |
* [[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>
* [[Thiamine deficiency|Vitamin B1 deficiency]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Primarily [[sensory]] deficits
* Vibration and [[proprioception]] affected
* [[Gait abnormality|Gait abnormalities]]
* [[Cognitive impairment]]
* [[Irritability]]
* [[Glossitis]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[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>
* Decreased [[serum]] [[Vitamin B12]] levels (< 200 pg/ml)
* [[Methylmalonic acidemia|Elevated methylmalonic acid]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[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>
* [[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>
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[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>
|-
| 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: #F5F5F5;" |
* [[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>
* [[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>
** [[Epstein Barr virus]]
** [[Human Immunodeficiency Virus (HIV)|HIV]]
** [[Cytomegalovirus]]
** [[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>
** [[Campylobacter]] infection
** [[Mycoplasma pneumoniae]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Rapid onset and quick progression
* Progression stops after 2-3 weeks
* Bilateral ascending [[Paraesthesia|paraesthesias]] and [[paralysis]] (generalized)
* [[Muscle weakness|Weakness]]
* [[Ataxia]]
* [[Areflexia]]
* No fever
* 4 sub-types:
**[[Acute inflammatory demyelinating polyneuropathy]]
**[[Acute motor axonal neuropathy]]
**Acute motor and sensory axonal neuropathy
**[[Miller Fisher syndrome]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* 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>
| style="padding: 5px 5px; background: #F5F5F5;" |
* 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>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[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>
* [[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>
* [[Mechanical ventilation|Respiratory support]]
* [[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: #F5F5F5;" |
* 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>
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* Slow onset and gradual progression
* [[Relapse|Relapsing]] and remitting course
* Symmetrical [[Proximal muscle weakness|proximal]] and [[Distal muscle weakness|distal motor]] and [[Sensory system|sensory weakness]] (legs>arms)
* [[Foot drop]]
* [[Numbness]], [[tingling]] and [[pain]]
* [[Areflexia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* 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>
* 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>
* 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>
* [[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>
| 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>
* 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>
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Corticosteroids]]
* [[Intravenous immunoglobulin]] ([[IVIG]])
* [[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: #F5F5F5;" |
* [[Immune response|Abnormal immune response]] ([[Anti-ganglioside antibodies|Anti ganglioside]] [[Anti-ganglioside antibodies|GM-1]] [[IgM]] [[antibodies]])
| style="padding: 5px 5px; background: #F5F5F5;" |
* Progressive, asymmetric, distal and upper [[Limb (anatomy)|limb]] predominant weakness
* No significant [[sensory]] abnormalities
* [[Areflexia]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Elevated [[CSF]] [[protein]]
*
| 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>
** 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]]
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Intravenous immunoglobulin|Intravenous immunoglobulins]]
* [[Cyclophosphamide]]
* [[Rituximab]]
|-
! 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}}

Revision as of 03:35, 30 October 2019

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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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