Plasma cell disorder: Difference between revisions

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== Plasma cell disorders ==
== 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>
*<nowiki/>In addition, some patients develop a [[polyneuropathy]]<nowiki/>or other problems related to the secreted antibody.
*<nowiki/>In addition, some patients develop a [[polyneuropathy]]<nowiki/>or other problems related to the secreted antibody.
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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]]'''


=== Malignant monoclonal gammopathies ===
== Malignant monoclonal gammopathies ==


==== '''Multiple myeloma''' ====
=== '''Multiple myeloma''' ===


===== ''Symptomatic multiple myeloma'' =====
==== Symptomatic multiple myeloma ====
* 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>
* 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
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* Patients with active [[Multiple myeloma diagnostic criteria|Multiple myeloma]] usually require treatment to prevent progression of disease which can lead to death.
* Patients with active [[Multiple myeloma diagnostic criteria|Multiple myeloma]] usually require treatment to prevent progression of disease which can lead to death.


===== ''Smoldering multiple myeloma'' =====
==== Smoldering multiple myeloma ====
* 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>
* 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]]
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* There is high risk of developing [[Multiple myeloma Classification|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 Classification|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 [[Hematopoietic stem cell transplantation|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 classification|multiple myeloma]] with less amount of [[M protein|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 [[serum protein electrophoresis]]
* M protein is not detected by [[serum protein electrophoresis]]
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* 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 Classification|multiple myeloma]]<ref name="Rajkumar2016" />
* IgD type constitues 2% of [[Multiple myeloma Classification|multiple myeloma]]<ref name="Rajkumar2016" />
* IgD [[Multiple myeloma classification|multiple myeloma]] has similar signs and symptoms as other types of [[Multiple myeloma classification|multiple myeloma]].
* IgD [[Multiple myeloma classification|multiple myeloma]] has similar signs and symptoms as other types of [[Multiple myeloma classification|multiple myeloma]].
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* IgD myeloma mostly affect people of younger age.
* IgD myeloma mostly affect people of younger age.


===== ''Osteosclerotic myeloma'' =====
==== Osteosclerotic myeloma ====
* 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>


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** [[Hematopoietic stem cell transplantation|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>
* [[Plasmacytoma|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.
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* One third of patients with solitary [[plasmacytoma]] will develop [[Multiple myeloma classification|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]], [[Gastrointestinal tract|GI tract,]] [[breast]] and [[brain]].
* Most commonly seen in throat,[[paranasal sinuses]], [[nasal cavity]], [[larynx]], [[Gastrointestinal tract|GI tract,]] [[breast]] and [[brain]].
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For more information about Multiple myeloma '''[[Multiple myeloma |click here]]'''
For more information about Multiple myeloma '''[[Multiple myeloma |click here]]'''


==== Malignant lymphoproliferative disorders ====
=== Malignant lymphoproliferative disorders ===
* Waldenstrom macroglobulinemia<ref name="pmid22773606">{{cite journal |vauthors=Braggio E, Philipsborn C, Novak A, Hodge L, Ansell S, Fonseca R |title=Molecular pathogenesis of Waldenstrom's macroglobulinemia |journal=Haematologica |volume=97 |issue=9 |pages=1281–90 |date=September 2012 |pmid=22773606 |pmc=3436227 |doi=10.3324/haematol.2012.068478 |url=}}</ref>  
==== Waldenstrom macroglobulinemia ====
** [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] is a cancer involving  [[lymphocytes]].
* [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] is a cancer involving  [[lymphocytes]].<ref name="pmid22773606">{{cite journal |vauthors=Braggio E, Philipsborn C, Novak A, Hodge L, Ansell S, Fonseca R |title=Molecular pathogenesis of Waldenstrom's macroglobulinemia |journal=Haematologica |volume=97 |issue=9 |pages=1281–90 |date=September 2012 |pmid=22773606 |pmc=3436227 |doi=10.3324/haematol.2012.068478 |url=}}</ref>  
** The main attributing [[antibody]] is [[IgM]].
* The main attributing [[antibody]] is [[IgM]].
** It is a type of [[lymphoproliferative disease]],
* It is a type of [[lymphoproliferative disease]],
** It shares clinical characteristics with the indolent [[Non-Hodgkin lymphoma|non-Hodgkin lymphomas]].
* It shares clinical characteristics with the indolent [[Non-Hodgkin lymphoma|non-Hodgkin lymphomas]].
** [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] represents 1% of all hematological cancers
* [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] represents 1% of all hematological cancers
** Common causes of [[Waldenström's macroglobulinemia]] include [[genetic]], environmental, and [[autoimmune]] factors.
* Common causes of [[Waldenström's macroglobulinemia]] include [[genetic]], environmental, and [[autoimmune]] factors.
** Common [[risk factors]] in the development of [[Waldenström's macroglobulinemia]] are [[Monoclonal gammopathy of undetermined significance]]. For more information about Waldenström's macroglobulinemia  '''[[Waldenström's macroglobulinemia |click here]]'''  
* Common [[risk factors]] in the development of [[Waldenström's macroglobulinemia]] are [[Monoclonal gammopathy of undetermined significance]].  
 
For more information about Waldenström's macroglobulinemia  '''[[Waldenström's macroglobulinemia |click here]]'''
* Malignant lymphoma


** Type of [[cancer]] that originates in [[Lymphocyte|lymphocytes]]<ref name="isbn92-832-2411-6">{{cite book |author= |title=Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.) |publisher=Oxford Univ Pr |location= |year= |pages= |isbn=92-832-2411-6 |oclc= |doi=}}</ref>
==== Malignant lymphoma ====
** Also called [[Hematological malignancy|hematological neoplasms]].  
* Type of [[cancer]] that originates in [[Lymphocyte|lymphocytes]]<ref name="isbn92-832-2411-6">{{cite book |author= |title=Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.) |publisher=Oxford Univ Pr |location= |year= |pages= |isbn=92-832-2411-6 |oclc= |doi=}}</ref>
** Main types are Hodgkin lymphoma and Non-Hodgkin lymphoma
* Also called [[Hematological malignancy|hematological neoplasms]].  
* Main types are Hodgkin lymphoma and Non-Hodgkin lymphoma
For more information about lymphoma '''[[lymphoma |click here]]'''
For more information about lymphoma '''[[lymphoma |click here]]'''


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For more information about chronic lymphocytic leukemia '''[[ chronic lymphocytic leukemia|click here]]'''
For more information about chronic lymphocytic leukemia '''[[ chronic lymphocytic leukemia|click here]]'''


=== Heavy-chain diseases ===
== Heavy-chain diseases ==
* 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>
* 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


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** [[Vincristine]], [[Corticosteroid|corticosteroids]] and [[radiation therapy]] may produce [[Remission (medicine)|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


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** Treatment is [[Corticosteroid|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  


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** Treatment consists of [[Alkylating agent|alkylating agents]] and [[Corticosteroid|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.  
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For more information on Cryoglobulinemia [[Cryoglobulinemia| click here]]
For more information on Cryoglobulinemia [[Cryoglobulinemia| click here]]


=== AL Primary amyloidosis ===
== AL 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>


Revision as of 20:06, 17 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 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
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary amyloidosis
 
Malignant monoclonal gammopathy
 
Chronic lymphocytic leukemia
 
 
 
Heavy chain diseases
 
Cryoglobulinemia
 
Monoclonal gammopathy of undetermined significance (MGUS)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Multiple myeloma
 
 
Malignant lymphoproliferative disorders
 
γHCD
 
αHCD
 
μHCD
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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)
 
 
 
 
 
 
 
 
 
 
 
 
Waldenstrom macroglobulinemia
 
Malignant lymphoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Symptomatic multiple myeloma
 
Smoldering multiple myeloma
 
Plasma-cell leukemia
 
Non-secretory myeloma
 
Solitary plasmacytoma of bone
 
 
Osteosclerotic myeloma
 
Extramedullary plasmacytoma
 

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%
Solitary Plasmacytoma + − − − − <3mg/dl Abnormal in 47% cases Normal
Primary amyloidosis − − − − − <3md/dl Light chains of immunoglobulines <10%
  • 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.

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

Waldenstrom macroglobulinemia

For more information about Waldenström's macroglobulinemia click here

Malignant lymphoma

For more information about lymphoma click here

Chronic lymphocytic leukemia

For more information about chronic lymphocytic leukemia click here

Heavy-chain diseases

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

AL Primary amyloidosis

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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