Plasma cell disorder: Difference between revisions

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| style="background:#F5F5F5;" align="center" + |N/A
| style="background:#F5F5F5;" align="center" + |N/A
| style="background:#F5F5F5;" align="center" + |<10%
| style="background:#F5F5F5;" align="center" + |<10%
| style="background:#F5F5F5;" align="center" + |No end-organ
| style="background:#F5F5F5;" align="center" + |
* No end-organ
damage
damage
|-
|-
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Increase in involved light chain concentration
Increase in involved light chain concentration
| style="background:#F5F5F5;" align="center" + |<10%
| style="background:#F5F5F5;" align="center" + |<10%
| style="background:#F5F5F5;" align="center" + |No end-organ damage
| style="background:#F5F5F5;" align="center" + |
* No end-organ damage
|-
|-
| style="background:#DCDCDC;" align="center" + |[[Multiple myeloma classification|Active symptomatic Multiple myeloma]]
| style="background:#DCDCDC;" align="center" + |[[Multiple myeloma classification|Active symptomatic Multiple myeloma]]
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=== Monoclonal gammopathies of undetermined significance (MGUS) ===
=== Monoclonal gammopathies of undetermined significance (MGUS) ===
* Monoclonal gammopathy of undetermined significance is a condition in which a low or non-quantifiable level of a [[monoclonal]] [[paraprotein]] is detected in the blood by means of [[protein electrophoresis]].<ref name="pmid16628189">{{cite journal |vauthors=Jego G, Bataille R, Geffroy-Luseau A, Descamps G, Pellat-Deceunynck C |title=Pathogen-associated molecular patterns are growth and survival factors for human myeloma cells through Toll-like receptors |journal=Leukemia |volume=20 |issue=6 |pages=1130–7 |date=June 2006 |pmid=16628189 |doi=10.1038/sj.leu.2404226 |url=}}</ref><ref name="pmid19181642">{{cite journal |vauthors=Dinarello CA |title=Targeting the pathogenic role of interleukin 1{beta} in the progression of smoldering/indolent myeloma to active disease |journal=Mayo Clin. Proc. |volume=84 |issue=2 |pages=105–7 |date=February 2009 |pmid=19181642 |pmc=2664579 |doi=10.4065/84.2.105 |url=}}</ref><ref name="pmid23233640">{{cite journal |vauthors=Merlini G, Palladini G |title=Differential diagnosis of monoclonal gammopathy of undetermined significance |journal=Hematology Am Soc Hematol Educ Program |volume=2012 |issue= |pages=595–603 |date=2012 |pmid=23233640 |doi=10.1182/asheducation-2012.1.595 |url=}}</ref>
* [[Monoclonal gammopathy of undetermined significance]] is a condition in which a low or non-quantifiable level of a [[monoclonal]] [[paraprotein]] is detected in the blood by means of [[protein electrophoresis]].<ref name="pmid16628189">{{cite journal |vauthors=Jego G, Bataille R, Geffroy-Luseau A, Descamps G, Pellat-Deceunynck C |title=Pathogen-associated molecular patterns are growth and survival factors for human myeloma cells through Toll-like receptors |journal=Leukemia |volume=20 |issue=6 |pages=1130–7 |date=June 2006 |pmid=16628189 |doi=10.1038/sj.leu.2404226 |url=}}</ref><ref name="pmid19181642">{{cite journal |vauthors=Dinarello CA |title=Targeting the pathogenic role of interleukin 1{beta} in the progression of smoldering/indolent myeloma to active disease |journal=Mayo Clin. Proc. |volume=84 |issue=2 |pages=105–7 |date=February 2009 |pmid=19181642 |pmc=2664579 |doi=10.4065/84.2.105 |url=}}</ref><ref name="pmid23233640">{{cite journal |vauthors=Merlini G, Palladini G |title=Differential diagnosis of monoclonal gammopathy of undetermined significance |journal=Hematology Am Soc Hematol Educ Program |volume=2012 |issue= |pages=595–603 |date=2012 |pmid=23233640 |doi=10.1182/asheducation-2012.1.595 |url=}}</ref>
* In addition, some patients develop a [[polyneuropathy]] (damage to peripheral nerves) or other problems related to the secreted antibody. MGUS is distinct from [[multiple myeloma]].
* In addition, some patients develop a [[polyneuropathy]]<nowiki/>or other problems related to the secreted antibody.
* MGUS is a premalignant condition and is distinct from [[multiple myeloma]].
* Pathologically, the lesion in [[Monoclonal gammopathy of undetermined significance]] is in fact very similar to that in [[multiple myeloma]].
* Pathologically, the lesion in [[Monoclonal gammopathy of undetermined significance]] is in fact very similar to that in [[multiple myeloma]].
For more information about Monoclonal gammopathies of undetermined significance  '''[[Monoclonal gammopathies of undetermined significance |click here]]'''
For more information about Monoclonal gammopathies of undetermined significance  '''[[Monoclonal gammopathies of undetermined significance |click here]]'''
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===== Symptomatic [[multiple myeloma]] =====
===== Symptomatic [[multiple myeloma]] =====
* People with multiple myeloma with symptoms are categorized to have active multiple myeloma and will exibit any of the following features.<ref name="SergentanisZagouri2015">{{cite journal|last1=Sergentanis|first1=Theodoros N.|last2=Zagouri|first2=Flora|last3=Tsilimidos|first3=Gerasimos|last4=Tsagianni|first4=Anastasia|last5=Tseliou|first5=Melina|last6=Dimopoulos|first6=Meletios A.|last7=Psaltopoulou|first7=Theodora|title=Risk Factors for Multiple Myeloma: A Systematic Review of Meta-Analyses|journal=Clinical Lymphoma Myeloma and Leukemia|volume=15|issue=10|year=2015|pages=563–577.e3|issn=21522650|doi=10.1016/j.clml.2015.06.003}}</ref>
* People with [[Multiple myeloma classification|multiple myeloma]] with symptoms are categorized to have [[Multiple myeloma Classification|active multiple myeloma]] and will exibit any of the following features.<ref name="SergentanisZagouri2015">{{cite journal|last1=Sergentanis|first1=Theodoros N.|last2=Zagouri|first2=Flora|last3=Tsilimidos|first3=Gerasimos|last4=Tsagianni|first4=Anastasia|last5=Tseliou|first5=Melina|last6=Dimopoulos|first6=Meletios A.|last7=Psaltopoulou|first7=Theodora|title=Risk Factors for Multiple Myeloma: A Systematic Review of Meta-Analyses|journal=Clinical Lymphoma Myeloma and Leukemia|volume=15|issue=10|year=2015|pages=563–577.e3|issn=21522650|doi=10.1016/j.clml.2015.06.003}}</ref>
** M protein in blood or urine
** [[M protein]] in blood or urine
** Bone marrow plasma cells constitute more than 10% of the blood cells
** [[Bone marrow cells|Bone marrow]] [[plasma cells]] constitute more than 10% of the [[blood cells]]
** Presence of solitary plasmacytoma in bone
** Presence of solitary [[plasmacytoma]] in bone
** ≥1 myeloma-defining event
** ≥1 myeloma-defining event
** CRAB features ( explained above)
** CRAB features ( explained above)
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===== Smoldering multiple myeloma =====
===== Smoldering multiple myeloma =====
* It is asymptomatic type of multiple myeloma<ref name="pmid27291302">{{cite journal |vauthors=Rajkumar SV |title=Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=7 |pages=719–34 |date=July 2016 |pmid=27291302 |pmc=5291298 |doi=10.1002/ajh.24402 |url=}}</ref>
* It is asymptomatic type of [[Multiple myeloma classification|multiple myeloma]]<ref name="pmid27291302">{{cite journal |vauthors=Rajkumar SV |title=Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=7 |pages=719–34 |date=July 2016 |pmid=27291302 |pmc=5291298 |doi=10.1002/ajh.24402 |url=}}</ref>
* Shows presence of M-spike that quantitates > 3 g/dl on [[protein electrophoresis]]
* Shows presence of M-spike that quantitates > 3 g/dl on [[protein electrophoresis]]


* Presence of bone marrow plasma cell burden of > 10% but < 60%
* Presence of [[Bone marrow cells|bone marrow plasma cell]] burden of > 10% but < 60%


* Absence of end-organ damage such as anemia, [[hypercalcemia]], renal dysfunction, or [[Osseous|osseous lesions]]
* Absence of end-organ damage such as [[anemia]], [[hypercalcemia]], renal dysfunction, or [[Osseous|osseous lesions]]


* Patients with smoldering (asymptomatic) [[Multiple myeloma diagnostic criteria|Multiple myeloma]] are managed by observation and undergoing follow up tests every 3 to 6 months
* Patients with smoldering (asymptomatic) [[Multiple myeloma diagnostic criteria|Multiple myeloma]] are managed by observation and undergoing follow up tests every 3 to 6 months
* There is high risk of developing active multiple myeloma.
* There is high risk of developing [[Multiple myeloma Classification|active multiple myeloma.]]


===== Plasma-cell leukemia =====
===== Plasma-cell leukemia =====
* Charecterized by large number of plasma cells circulating in the blood<ref name="Rajkumar2016" />
* Charecterized by large number of [[plasma cells]] circulating in the blood<ref name="Rajkumar2016" />
* Rare condition, can develop in to most aggressive form of multiple myeloma.
* Rare condition, can develop in to most aggressive form of [[Multiple myeloma Classification|multiple myeloma]].
* Treatment options for plasma cell leukemia is chemotherapy or stem cell transplant.
* Treatment options for [[plasma cell leukemia]] is [[chemotherapy]] or [[Hematopoietic stem cell transplantation|stem cell transplant]].


===== Non-secretory myeloma =====
===== Non-secretory myeloma =====
* Type of multiple myeloma with less amount of M proteins secretion in blood or urine.<ref name="Rajkumar2016" />
* Type of [[Multiple myeloma classification|multiple myeloma]] with less amount of [[M protein|M proteins]] secretion in [[blood]] or urine.<ref name="Rajkumar2016" />
* M protein is not detected by protein electrophoresis
* M protein is not detected by [[serum protein electrophoresis]]
* Bone marrow exibit myeloma cells.
* [[Bone marrow]] exibit [[Myeloma|myeloma cells]].
* Osteolytic bone lesions are seen on xrays.
* Osteolytic bone lesions are seen on xrays.


===== IgD myeloma =====
===== IgD myeloma =====
* IgD type constitues 2% of multiple myeloma<ref name="Rajkumar2016" />
* IgD type constitues 2% of [[Multiple myeloma Classification|multiple myeloma]]<ref name="Rajkumar2016" />
* IgD multiple myeloma has similar signs and symptoms as other types of multiple myeloma.
* IgD [[Multiple myeloma classification|multiple myeloma]] has similar signs and symptoms as other types of [[Multiple myeloma classification|multiple myeloma]].


* IgD myeloma mostly affect people of younger age.
* IgD myeloma mostly affect people of younger age.
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* It is a rare disorder affecting multiple systems of the body<ref name="Rajkumar2016">{{cite journal|last1=Rajkumar|first1=S. Vincent|title=Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management|journal=American Journal of Hematology|volume=91|issue=7|year=2016|pages=719–734|issn=03618609|doi=10.1002/ajh.24402}}</ref>
* It is a rare disorder affecting multiple systems of the body<ref name="Rajkumar2016">{{cite journal|last1=Rajkumar|first1=S. Vincent|title=Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management|journal=American Journal of Hematology|volume=91|issue=7|year=2016|pages=719–734|issn=03618609|doi=10.1002/ajh.24402}}</ref>


* It is called POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes
* It is called POEMS syndrome: [[polyneuropathy]], [[Organomegaly|organomegaly,]] [[endocrinopathy]], monoclonal protein, skin changes
* Treatment option for osteoclastic myeloma include:
* Treatment option for osteoclastic myeloma include:
** Chemotherapy
** [[Chemotherapy]]
** Radiation therapy
** [[Radiation therapy]]
** Stem cell transplant
** [[Hematopoietic stem cell transplantation|Stem cell transplant]]


===== Solitary plasmacytoma of bone =====
===== Solitary plasmacytoma of bone =====
* Plasmacytoma is collection of abnormal plasma cells forming a singl e tumor.<ref name="CaersPaiva2018">{{cite journal|last1=Caers|first1=J.|last2=Paiva|first2=B.|last3=Zamagni|first3=E.|last4=Leleu|first4=X.|last5=Bladé|first5=J.|last6=Kristinsson|first6=S. Y.|last7=Touzeau|first7=C.|last8=Abildgaard|first8=N.|last9=Terpos|first9=E.|last10=Heusschen|first10=R.|last11=Ocio|first11=E.|last12=Delforge|first12=M.|last13=Sezer|first13=O.|last14=Beksac|first14=M.|last15=Ludwig|first15=H.|last16=Merlini|first16=G.|last17=Moreau|first17=P.|last18=Zweegman|first18=S.|last19=Engelhardt|first19=M.|last20=Rosiñol|first20=L.|title=Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel|journal=Journal of Hematology & Oncology|volume=11|issue=1|year=2018|issn=1756-8722|doi=10.1186/s13045-017-0549-1}}</ref>
* [[Plasmacytoma]] is collection of abnormal [[plasma cells]] forming a singl e tumor.<ref name="CaersPaiva2018">{{cite journal|last1=Caers|first1=J.|last2=Paiva|first2=B.|last3=Zamagni|first3=E.|last4=Leleu|first4=X.|last5=Bladé|first5=J.|last6=Kristinsson|first6=S. Y.|last7=Touzeau|first7=C.|last8=Abildgaard|first8=N.|last9=Terpos|first9=E.|last10=Heusschen|first10=R.|last11=Ocio|first11=E.|last12=Delforge|first12=M.|last13=Sezer|first13=O.|last14=Beksac|first14=M.|last15=Ludwig|first15=H.|last16=Merlini|first16=G.|last17=Moreau|first17=P.|last18=Zweegman|first18=S.|last19=Engelhardt|first19=M.|last20=Rosiñol|first20=L.|title=Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel|journal=Journal of Hematology & Oncology|volume=11|issue=1|year=2018|issn=1756-8722|doi=10.1186/s13045-017-0549-1}}</ref>
* Solitary plasmacytoma is occurence of single bone tumor made up of myeloma cells.
* [[Plasmacytoma|Solitary plasmacytoma]] is occurence of single bone [[tumor]] made up of myeloma cells.
* Xray shows an osteolytic lesion at the site of the tumor.
* Xray shows an osteolytic lesion at the site of the [[Tumor|tumor.]]
* Bone marrow plasma population remains less than 10%
* [[Bone marrow]] plasma population remains less than 10%
* One third of patients with solitary plasmacytoma will develop multiple myeloma.
* One third of patients with solitary [[plasmacytoma]] will develop [[Multiple myeloma classification|multiple myeloma]].


===== Extramedullary plasmacytoma =====
===== Extramedullary plasmacytoma =====
* It is develped outside the bone marrow in soft tissues of the body<ref name="Rajkumar2016" />
* It is develped outside the bone marrow in soft tissues of the body<ref name="Rajkumar2016" />
* Most commonly seen in throat,paranasal sinuses, nasal cavity, larynx,GI tract, breast and brain.
* Most commonly seen in throat,[[paranasal sinuses]], [[nasal cavity]], [[larynx]], [[Gastrointestinal tract|GI tract,]] [[breast]] and [[brain]].
* Diagnosis is confirmed by biopsy of the tumor.
* Diagnosis is confirmed by [[biopsy]] of the [[Tumor|tumor.]]
* X-rays and  bone marrow biopsy is normal.
* X-rays and  [[bone marrow]] [[biopsy]] is normal.
* Treatment is done with either radiation therapy or surgry.
* Treatment is done with either [[radiation therapy]] or surgery.
For more information about Multiple myeloma '''[[Multiple myeloma |click here]]'''
For more information about Multiple myeloma '''[[Multiple myeloma |click here]]'''


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=== Heavy-chain diseases ===
=== Heavy-chain diseases ===
Heavy chain diseases are plasma cell neoplasias, featuring overproduction of immunoglobulin heavy chains.
Heavy chain diseases are [[Neoplasias|plasma cell neoplasias]], featuring overproduction of [[Immunoglobulin|immunoglobulin heavy chains]].<ref name="MunshiCabot2008">{{cite journal|last1=Munshi|first1=Nikhil C.|last2=Cabot|first2=Richard C.|last3=Harris|first3=Nancy Lee|last4=Shepard|first4=Jo-Anne O.|last5=Rosenberg|first5=Eric S.|last6=Cort|first6=Alice M.|last7=Ebeling|first7=Sally H.|last8=Peters|first8=Christine C.|last9=Digumarthy|first9=Subba|last10=Rahemtullah|first10=Aliyah|title=Case 13-2008|journal=New England Journal of Medicine|volume=358|issue=17|year=2008|pages=1838–1848|issn=0028-4793|doi=10.1056/NEJMcpc0800959}}</ref>


==== γHCD ====
==== γHCD ====
Gamma chain or IgG heavy chain disease
Gamma chain or IgG heavy chain disease
* Primarily seen in elderly men but can occur in children.
* Primarily seen in elderly men but can occur in children.
* High levels of IgG with reduction of normal immunoglobulin level
* High levels of IgG with reduction of normal [[immunoglobulin]] level
* Lymphadenopathy, hepatosplenomegaly and recurrent infections are common features
* [[Lymphadenopathy|Lymphadenopath]]<nowiki/>y, [[hepatosplenomegaly]] and recurrent infections are common features
* Vincristine, corticosteroids and radiation therapy may produce remission.
* [[Vincristine]], [[Corticosteroid|corticosteroids]] and [[radiation therapy]] may produce [[Remission (medicine)|remission]].


==== αHCD ====
==== αHCD ====
Alpha chain or IgA heavy chain
Alpha chain or IgA heavy chain
* Appears between age 10-30 as an immune response to a microorganism
* Appears between age 10-30 as an [[immune]] response to a [[microorganism]]
* Patients present with diffuse abdominal lymphoma and malabsorption.
* Patients present with diffuse abdominal [[lymphoma]] and [[malabsorption]].
* Course is variable, some patients die in 1-2 yrs, others go in to remission lasting for many years.
* Course is variable, some patients die in 1-2 yrs, others go in to [[Remission (medicine)|remission]] lasting for many years.
* Serum protein electrophoresis detect increased α & β fraction.
* [[Serum protein electrophoresis]] detect increased α & β fraction.
* Treatment is corticosteroids, cytotoxic drugs and broadspectrum antibiotics
* Treatment is [[Corticosteroid|corticosteroids]], [[cytotoxic drugs]] and broadspectrum antibiotics


==== μHCD ====
==== μHCD ====
MU chain or IGM heavy chain disease  
MU chain or IGM heavy chain disease  
* Mainly affects individuals > 50 yrs
* Mainly affects individuals > 50 yrs
* Spleen, liver and abdominal lymph nodes involvement is more common than peripheral lymphadenopathy
* [[Spleen]], [[liver]] and abdominal [[lymph nodes]] involvement is more common than peripheral [[lymphadenopathy]]
* Serum protein electrophoresis exibit hypogammaglobulinemia.
* [[Serum]] [[protein electrophoresis]] exibit [[hypogammaglobulinemia]].
* Vacuolated plasma cells are pathognomic on bone marrow exam.
* Vacuolated [[Plasma cell|plasma cells]] are pathognomic on [[bone marrow]] exam.
* Treatment consists of alkylating agents and corticosteroids.
* Treatment consists of [[Alkylating agent|alkylating agents]] and [[Corticosteroid|corticosteroids]].


=== Cryoglobulinemia ===
=== Cryoglobulinemia ===
* [[Cryoglobulinemia]] is the presence of high amount of heavy [[Globulin|globulins]] (e.g. [[IgM]]) in the [[bloodstream]] which thicken on exposure to cold.<ref name="pmid16026843">{{cite journal| author=Scotto G, Cibelli DC, Saracino A, Prato R, Palumbo E, Fazio V et al.| title=Cryoglobulinemia in subjects with HCV infection alone, HIV infection and HCV/HIV coinfection. | journal=J Infect | year= 2006 | volume= 52 | issue= 4 | pages= 294-9 | pmid=16026843 | doi=10.1016/j.jinf.2005.05.025 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16026843  }} </ref><ref name="pmid29558353">{{cite journal| author=Suszek D, Majdan M| title=[Cryoglobulins and cryoglobulinemic vasculitis]. | journal=Wiad Lek | year= 2018 | volume= 71 | issue= 1 pt 1 | pages= 59-63 | pmid=29558353 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29558353  }} </ref><ref name="pmid27034078">{{cite journal| author=Blank N, Lorenz HM| title=[Cryoglobulinemic vasculitis]. | journal=Z Rheumatol | year= 2016 | volume= 75 | issue= 3 | pages= 303-15 | pmid=27034078 | doi=10.1007/s00393-016-0076-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27034078  }} </ref><ref name="pmid10787003">{{cite journal| author=Ramos-Casals M, Trejo O, García-Carrasco M, Cervera R, Font J| title=Mixed cryoglobulinemia: new concepts. | journal=Lupus | year= 2000 | volume= 9 | issue= 2 | pages= 83-91 | pmid=10787003 | doi=10.1191/096120300678828127 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10787003  }} </ref>
* [[Cryoglobulinemia]] is the presence of high amount of heavy [[Globulin|globulins]] (e.g. [[IgM]]) in the [[bloodstream]] which thicken on exposure to cold.<ref name="pmid16026843">{{cite journal| author=Scotto G, Cibelli DC, Saracino A, Prato R, Palumbo E, Fazio V et al.| title=Cryoglobulinemia in subjects with HCV infection alone, HIV infection and HCV/HIV coinfection. | journal=J Infect | year= 2006 | volume= 52 | issue= 4 | pages= 294-9 | pmid=16026843 | doi=10.1016/j.jinf.2005.05.025 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16026843  }} </ref><ref name="pmid29558353">{{cite journal| author=Suszek D, Majdan M| title=[Cryoglobulins and cryoglobulinemic vasculitis]. | journal=Wiad Lek | year= 2018 | volume= 71 | issue= 1 pt 1 | pages= 59-63 | pmid=29558353 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29558353  }} </ref><ref name="pmid27034078">{{cite journal| author=Blank N, Lorenz HM| title=[Cryoglobulinemic vasculitis]. | journal=Z Rheumatol | year= 2016 | volume= 75 | issue= 3 | pages= 303-15 | pmid=27034078 | doi=10.1007/s00393-016-0076-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27034078  }} </ref><ref name="pmid10787003">{{cite journal| author=Ramos-Casals M, Trejo O, García-Carrasco M, Cervera R, Font J| title=Mixed cryoglobulinemia: new concepts. | journal=Lupus | year= 2000 | volume= 9 | issue= 2 | pages= 83-91 | pmid=10787003 | doi=10.1191/096120300678828127 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10787003  }} </ref>
* Cryoglobulins are circulating [[immunoglobulins]] or [[Protein|proteins]] that become insoluble at less than 4 degrees Celsius.  
* Cryoglobulins are circulating [[immunoglobulins]] or [[Protein|proteins]] that become insoluble at less than 4 degrees Celsius.  
* Cryoglobulinemia can lead to a medium-sized vessel [[vasculitis]] due to vascular deposition of circulating [[immune complexes]].  
* [[Cryoglobulinemia]] can lead to a medium-sized vessel [[vasculitis]] due to vascular deposition of circulating [[immune complexes]].  
* This leads to the triad of palpable [[purpura]], [[Arthralgia|arthralgias]] and [[peripheral neuropathy]]
* This leads to the triad of palpable [[purpura]], [[Arthralgia|arthralgias]] and [[peripheral neuropathy]]
* Common causes of cryoglobulinemia are primarily hematologic, oncologic, and rheumatic
* Common causes of[[Cryoglobulinemia]] are primarily [[hematologic]], [[Oncology|oncologic]], and [[rheumatic]]
For more information on Cryoglobulinemia [[Cryoglobulinemia| click here]]
For more information on Cryoglobulinemia [[Cryoglobulinemia| click here]]


=== Primary amyloidosis ===
=== Primary amyloidosis ===
The "AL" refers to [[amyloid]] light chain.<ref name="pmid15198347">{{cite journal |author=Gertz MA |title=The classification and typing of amyloid deposits |journal=Am. J. Clin. Pathol. |volume=121 |issue=6 |pages=787–9 |year=2004 |month=June |pmid=15198347 |doi=10.1309/TR4L-GLVR-JKAM-V5QT |url=http://ajcp.metapress.com/openurl.asp?genre=article&issn=0002-9173&volume=121&issue=6&spage=787}}</ref><ref name="urlAmyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com">{{cite web |url=http://www.medicinenet.com/amyloidosis/article.htm |title=Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com |format= |work= |accessdate=}}</ref>
The "AL" refers to [[amyloid]] [[light chain]].<ref name="pmid15198347">{{cite journal |author=Gertz MA |title=The classification and typing of amyloid deposits |journal=Am. J. Clin. Pathol. |volume=121 |issue=6 |pages=787–9 |year=2004 |month=June |pmid=15198347 |doi=10.1309/TR4L-GLVR-JKAM-V5QT |url=http://ajcp.metapress.com/openurl.asp?genre=article&issn=0002-9173&volume=121&issue=6&spage=787}}</ref><ref name="urlAmyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com">{{cite web |url=http://www.medicinenet.com/amyloidosis/article.htm |title=Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com |format= |work= |accessdate=}}</ref>


* AL amyloidosis is the most common form of systemic [[amyloidosis]] in the US.
* [[AL amyloidosis]] is the most common form of systemic [[amyloidosis]] in the US.
* Occurs in 5 to 15% of people with [[multiple myeloma]].
* Occurs in 5 to 15% of people with [[multiple myeloma]].
* Treatment can involve application of [[chemotherapy]] similar to that used in multiple myeloma
* Treatment can involve application of [[chemotherapy]] similar to that used in [[Multiple myeloma Classification|multiple myeloma]]
For more information on amyloidosis [[ amyloidosis | click here]]
For more information on amyloidosis [[ amyloidosis | click here]]


Revision as of 15:21, 13 September 2018


Plasma cell disorders

Overview

Classification

Monoclonal gammopathy of undetermined significance (MGUS)
Malignant monoclonal gammopathies
Multiple myeloma
Malignant lymphoproliferative disorders
Chronic lymphocytic leukemia
Heavy-chain diseases
Cryoglobulinemia
Primary amyloidosis

Differentiating Plasma Cell Disorder

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


Overview

Plasma cell disorders are a diverse type of blood disorders characterized by the presence of a monoclonal paraprotein in the serum or urine. Monoclonal plasma cells are present in the bone marrow or, rarely, in other tissues. Plasma cell disorders include monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM), lymphoplasmacytic lymphoma/ Waldenstrom macroglobulinemia (LPL/WM), lymphoproliferative disorders, smoldering multiple myeloma (SMM); solitary or extramedullary plasmacytoma, amyloidosis, and POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes). Plasma-cell disorders are characterized by the proliferation of a single clone of plasma cells that produces a homogeneous monoclonal (M) protein. These disorders have been defined by the International Myeloma Working Group.1 In 2006.

Classification

 
 
 
 
 
 
 
 
 
 
 
Plasma cell disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Monoclonal gammopathy of unknown significance (MGUS)
 
Malignant monoclonal gammopathy
 
Chronic lymphocytic leukemia
 
 
 
Heavy chain diseases

γHCD αHCD

μHCD
 
Cryoglobulinemia
 
Primary amyloidosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Multiple myeloma
 
 
 
 
 
 
 
 
Malignant lymphoproliferative disorders
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Waldenstrom macroglobulinemia
 
Malignant lymphoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Symptomatic multiple myeloma
 
Smoldering multiple myeloma
 
Plasma-cell leukemia
 
Non-secretory myeloma
 
Solitary plasmacytoma of bone
 
 
Osteosclerotic myeloma
 
Extramedullary plasmacytoma
 


Monoclonal gammopathies of undetermined significance (MGUS)

  • Benign (IgG, IgA, IgD, IgM, and, rarely, free light chains)
  • Associated neoplasms or other diseases not known to produce monoclonal proteins
  • Biclonal and triclonal gammopathies
  • Idiopathic (Bence Jones proteinuria)

Malignant monoclonal gammopathies

  • Multiple myeloma (IgG, IgA, IgD, IgE, and free light chains)
    • Symptomatic multiple myeloma
    • Smoldering multiple myeloma
    • Plasma-cell leukemia
    • Non-secretory myeloma
    • IgD myeloma
    • POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (osteosclerotic myeloma)
    • Solitary plasmacytoma of bone
    • Extramedullary plasmacytoma
  • Malignant lymphoproliferative disorders

Chronic lymphocytic leukemia

Heavy-chain diseases (HCDs)

    • γHCD
    • αHCD
    • μHCD

Cryoglobulinemia

Primary amyloidosis (AL)

Differential Diagnosis

Disease IgM IgG IgA IgE IgD Monoclonal Ig level SFLC Bone marrow plasma cells Other criteria
IgM MGUS + − − − − < 3gm/dl N/A <10%
  • No end-organ damage
Non igM MGUS + − + − − < 3gm/dl N/A <10%
  • No end-organ

damage

Smoldering MM − + + − − > 3gm/dl N/A 10-60%
  • No myeloma-defining event
  • No CRAB features
Light chain MGUS − − − − − <500 mg/24 hrs (urine) Free kappa or lambda light chain

Abnormal ratio (<0.26 or >1.65)

Increase in involved light chain concentration

<10%
  • No end-organ damage
Active symptomatic Multiple myeloma − + + + + >3gm/dl >100 >60%
  • ≥1 myeloma-defining event
  • CRAB features
Waldenstrom macroglobulinemia + − − − − Variable N/A >10%
  • Evidence of organ/tissue damage.
  • Anemia,
  • Hepatosplenomegaly
Solitary Plasmacytoma + − − − − <3mg/dl Abnormal in 47% cases Normal
  • Solitory bone lesion due to plasma cell tumor
  • Preserved levels of uninvolved immunoglobulins
  • No anemia, hypercalcemia or renal disease
Primary amyloidosis − − − − − <3md/dl Light chains of immunoglobulines <10%
  • No bone lesions,

Myeloma defining events: >60% clonal plasma cells on B.M exam; serum involved:uninvolved FLC ratio >100; >1 focal lesion on MRI >5mm

CRAB features: elevated calcium >11mg/dl, renal insufficiency, anemia Hb <10 g/dL , bone disease ≥1 lytic lesions on skeletal radiography, CT, or PET-CT , SFLC: serum free light chains, kappa and lambda immunoglobulin light chains.

The normal κ:λ ratio is 0.26 to 1.65 (17,18). A κ:λ ratio of <0.26 strongly suggests the presence of a of plasma cells that are producing clonal λ free light chains. Ratio >1.65 suggests production of clonal κ free light chains.

Plasma cell disorders

Monoclonal gammopathies of undetermined significance (MGUS)

For more information about Monoclonal gammopathies of undetermined significance click here

Malignant monoclonal gammopathies

Multiple myeloma

Symptomatic multiple myeloma
  • Patients with active Multiple myeloma usually require treatment to prevent progression of disease which can lead to death.
Smoldering multiple myeloma
Plasma-cell leukemia
Non-secretory myeloma
IgD myeloma
  • IgD myeloma mostly affect people of younger age.
Osteosclerotic myeloma
  • It is a rare disorder affecting multiple systems of the body[6]
Solitary plasmacytoma of bone
Extramedullary plasmacytoma

For more information about Multiple myeloma click here

Malignant lymphoproliferative disorders

Malignant lymphoma

For more information about lymphoma click here

Chronic lymphocytic leukemia

For more information about chronic lymphocytic leukemia click here

Heavy-chain diseases

Heavy chain diseases are plasma cell neoplasias, featuring overproduction of immunoglobulin heavy chains.[11]

γHCD

Gamma chain or IgG heavy chain disease

αHCD

Alpha chain or IgA heavy chain

μHCD

MU chain or IGM heavy chain disease

Cryoglobulinemia

For more information on Cryoglobulinemia click here

Primary amyloidosis

The "AL" refers to amyloid light chain.[16][17]

For more information on amyloidosis click here

References

  1. ↑ Jego G, Bataille R, Geffroy-Luseau A, Descamps G, Pellat-Deceunynck C (June 2006). "Pathogen-associated molecular patterns are growth and survival factors for human myeloma cells through Toll-like receptors". Leukemia. 20 (6): 1130–7. doi:10.1038/sj.leu.2404226. PMID 16628189.
  2. ↑ Dinarello CA (February 2009). "Targeting the pathogenic role of interleukin 1{beta} in the progression of smoldering/indolent myeloma to active disease". Mayo Clin. Proc. 84 (2): 105–7. doi:10.4065/84.2.105. PMC 2664579. PMID 19181642.
  3. ↑ Merlini G, Palladini G (2012). "Differential diagnosis of monoclonal gammopathy of undetermined significance". Hematology Am Soc Hematol Educ Program. 2012: 595–603. doi:10.1182/asheducation-2012.1.595. PMID 23233640.
  4. ↑ Sergentanis, Theodoros N.; Zagouri, Flora; Tsilimidos, Gerasimos; Tsagianni, Anastasia; Tseliou, Melina; Dimopoulos, Meletios A.; Psaltopoulou, Theodora (2015). "Risk Factors for Multiple Myeloma: A Systematic Review of Meta-Analyses". Clinical Lymphoma Myeloma and Leukemia. 15 (10): 563–577.e3. doi:10.1016/j.clml.2015.06.003. ISSN 2152-2650.
  5. ↑ Rajkumar SV (July 2016). "Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (7): 719–34. doi:10.1002/ajh.24402. PMC 5291298. PMID 27291302.
  6. ↑ 6.0 6.1 6.2 6.3 6.4 Rajkumar, S. Vincent (2016). "Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 91 (7): 719–734. doi:10.1002/ajh.24402. ISSN 0361-8609.
  7. ↑ Caers, J.; Paiva, B.; Zamagni, E.; Leleu, X.; Bladé, J.; Kristinsson, S. Y.; Touzeau, C.; Abildgaard, N.; Terpos, E.; Heusschen, R.; Ocio, E.; Delforge, M.; Sezer, O.; Beksac, M.; Ludwig, H.; Merlini, G.; Moreau, P.; Zweegman, S.; Engelhardt, M.; Rosiñol, L. (2018). "Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel". Journal of Hematology & Oncology. 11 (1). doi:10.1186/s13045-017-0549-1. ISSN 1756-8722.
  8. ↑ Braggio E, Philipsborn C, Novak A, Hodge L, Ansell S, Fonseca R (September 2012). "Molecular pathogenesis of Waldenstrom's macroglobulinemia". Haematologica. 97 (9): 1281–90. doi:10.3324/haematol.2012.068478. PMC 3436227. PMID 22773606.
  9. ↑ Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6.
  10. ↑ Hallek, Michael (2015). "Chronic lymphocytic leukemia: 2015 Update on diagnosis, risk stratification, and treatment". American Journal of Hematology. 90 (5): 446–460. doi:10.1002/ajh.23979. ISSN 0361-8609.
  11. ↑ Munshi, Nikhil C.; Cabot, Richard C.; Harris, Nancy Lee; Shepard, Jo-Anne O.; Rosenberg, Eric S.; Cort, Alice M.; Ebeling, Sally H.; Peters, Christine C.; Digumarthy, Subba; Rahemtullah, Aliyah (2008). "Case 13-2008". New England Journal of Medicine. 358 (17): 1838–1848. doi:10.1056/NEJMcpc0800959. ISSN 0028-4793.
  12. ↑ Scotto G, Cibelli DC, Saracino A, Prato R, Palumbo E, Fazio V; et al. (2006). "Cryoglobulinemia in subjects with HCV infection alone, HIV infection and HCV/HIV coinfection". J Infect. 52 (4): 294–9. doi:10.1016/j.jinf.2005.05.025. PMID 16026843.
  13. ↑ Suszek D, Majdan M (2018). "[Cryoglobulins and cryoglobulinemic vasculitis]". Wiad Lek. 71 (1 pt 1): 59–63. PMID 29558353.
  14. ↑ Blank N, Lorenz HM (2016). "[Cryoglobulinemic vasculitis]". Z Rheumatol. 75 (3): 303–15. doi:10.1007/s00393-016-0076-4. PMID 27034078.
  15. ↑ Ramos-Casals M, Trejo O, García-Carrasco M, Cervera R, Font J (2000). "Mixed cryoglobulinemia: new concepts". Lupus. 9 (2): 83–91. doi:10.1191/096120300678828127. PMID 10787003.
  16. ↑ Gertz MA (2004). "The classification and typing of amyloid deposits". Am. J. Clin. Pathol. 121 (6): 787–9. doi:10.1309/TR4L-GLVR-JKAM-V5QT. PMID 15198347. Unknown parameter |month= ignored (help)
  17. ↑ "Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com".

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