Plasma cell disorder: Difference between revisions

	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

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Latest revision as of 11:52, 19 July 2022

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nazia Fuad M.D., Anmol Pitliya, M.B.B.S. M.D.[2]

Synonyms and keywords: Plasma cell dyscrasia

Overview

Plasma cell disorders are a diverse type of blood disorders characterized by proliferation of a single clone of plasma cells that produces a homogeneous monoclonal (M) protein. These monoclonal paraprotein are seen 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 or 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). These disorders have been defined by the International Myeloma Working Group in 2006.

Classification

Plasma cell disorder

Primary amyloidosis

Malignant monoclonal gammopathy

Chronic lymphocytic leukemia

Heavy chain diseases (HCD)

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

IgD myeloma

Osteosclerotic myeloma

Solitary plasmacytoma of bone

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%	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 Nephrotic syndrome Restrictive cardiomyopathy Peripheral neuropathy Hepatomegaly with elevated liver enzymes Macroglossia Purpura and an unexplained bleeding diathesis
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)

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.^[1]^[2]^[3]
In addition, some patients develop a polyneuropathy 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.

For more information about monoclonal gammopathies of undetermined significance click here.

Malignant monoclonal gammopathies

Multiple myeloma

Symptomatic multiple myeloma

People with multiple myeloma with symptoms are categorized to have active multiple myeloma and will exhibit any of the following features:^[4]
- M protein in blood or urine
- Bone marrow plasma cells constitute more than 10% of the blood cells
- Presence of solitary plasmacytoma in bone
- ≥ 1 myeloma-defining event
- CRAB features ( explained above)
- Osteolytic lesions on bone x-ray

Patients with active multiple myeloma usually require treatment to prevent progression of disease which can lead to death.

Smoldering multiple myeloma

It is asymptomatic type of multiple myeloma.^[5]
Shows presence of M-spike that quantitates > 3 g/dl on protein electrophoresis.

Presence of bone marrow plasma cell burden of > 10% but < 60%.

Absence of end-organ damage such as anemia, hypercalcemia, renal dysfunction, or osseous lesions.

Patients with smoldering (asymptomatic) 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.

Plasma-cell leukemia

Characterized by large number of plasma cells circulating in the blood.^[6]
Rare condition, can develop in to most aggressive form of multiple myeloma.
Can occur as a secondary type in a patient with multiple myeloma.
Treatment options for plasma cell leukemia is chemotherapy or stem cell transplant.

Non-secretory myeloma

Type of multiple myeloma with less amount of M proteins secretion in blood or urine.^[6]
M protein is not detected by serum protein electrophoresis.
Bone marrow exibit myeloma cells.
Osteolytic bone lesions are seen on X-ray.

IgD myeloma

IgD type constitues 2% of multiple myeloma.^[6]
IgD multiple myeloma has similar signs and symptoms as other types of multiple myeloma.

IgD myeloma mostly affect people of younger age.

Osteosclerotic myeloma

It is a rare disorder affecting multiple systems of the body.^[6]

It is called POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes.
Treatment option for osteoclastic myeloma include:

Solitary plasmacytoma of bone

Plasmacytoma is collection of abnormal plasma cells forming a single tumor.^[7]
Solitary plasmacytoma is occurrence of single bone tumor made up of myeloma cells.
X-ray shows an osteolytic lesion at the site of the tumor.
Bone marrow plasma population remains less than 10%.
One third of patients with solitary plasmacytoma will develop multiple myeloma.

Extramedullary plasmacytoma

It is developed outside the bone marrow in soft tissues of the body^[6]
Most commonly seen in throat, paranasal sinuses, nasal cavity, larynx, GI tract, breast, and brain.
Diagnosis is confirmed by biopsy of the tumor.
X-rays and bone marrow biopsy is normal.
Treatment is done with either radiation therapy or surgery.

For more information about multiple myeloma, click here.

Malignant lymphoproliferative disorders

Waldenstrom macroglobulinemia

Waldenstrom macroglobulinemia is a cancer involving lymphocytes.^[8]
The main attributing antibody is IgM.
It is a type of lymphoproliferative disease.
It shares clinical characteristics with the indolent non-Hodgkin lymphomas.
Waldenstrom macroglobulinemia represents 1% of all hematological cancers.
Common causes of Waldenström's macroglobulinemia include genetic, environmental, and autoimmune factors.
Common risk factors in the development of Waldenström's macroglobulinemia are monoclonal gammopathies of undetermined significance.

For more information about Waldenström's macroglobulinemia, click here.

Malignant lymphoma

Type of cancer that originates in lymphocytes.^[9]
Also called hematological neoplasms.
Primary types include Hodgkin lymphoma and non-Hodgkin lymphoma.

For more information about malignant lymphoma, click here.

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia arises from pre-follicular center B cells, normally involved in immunoglobulins production.^[10]
Development of chronic lymphocytic leukemia is the result of multiple genetic mutations that promote both malignant leukemic proliferation and apoptotic resistance of mature B cells.
Chronic lymphocytic leukemia must be differentiated from hairy cell leukaemia, prolymphocytic leukaemia, follicular lymphoma, and mantle cell lymphoma.
Prognosis is generally good, and the 5-year survival rate of patients with chronic lymphocytic leukemia is approximately 81.7%.
The mainstay of therapy for symptomatic chronic lymphocytic leukemia patients is immunochemotherapy.

For more information about chronic lymphocytic leukemia, click here

Heavy-chain diseases (HCD)

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

γHCD

Also known as gamma chain or IgG heavy chain disease.
- Primarily seen in elderly men but can occur in children.
- High levels of IgG with reduction of normal immunoglobulin level.
- Lymphadenopathy, hepatosplenomegaly, and recurrent infections are common features.
- Vincristine, corticosteroids and radiation therapy may produce remission.

αHCD

Also known as alpha chain or IgA heavy chain.
- Appears between age 10-30 as an immune response to a microorganism.
- 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.
- Serum protein electrophoresis detect increased α & β fraction.
- Treatment is corticosteroids, cytotoxic drugs and broadspectrum antibiotics

μHCD

Also known as mu chain or IGM heavy chain disease.
- Mainly affects individuals > 50 yrs.
- Spleen, liver and abdominal lymph nodes involvement is more common than peripheral lymphadenopathy.
- Serum protein electrophoresis exibit hypogammaglobulinemia.
- Vacuolated plasma cells are pathognomic on bone marrow exam.
- Treatment consists of alkylating agents and corticosteroids.

Cryoglobulinemia

Cryoglobulinemia is the presence of high amount of heavy globulins (e.g. IgM) in the bloodstream which thicken on exposure to cold.^[12]^[13]^[14]^[15]
Cryoglobulins are circulating immunoglobulins or 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.
This leads to the triad of palpable purpura, arthralgias and peripheral neuropathy.
Common causes of cryoglobulinemia are primarily hematologic, oncologic, and rheumatic

For more information on cryoglobulinemia, click here.

Primary amyloidosis

The "AL" refers to amyloid light chain.^[16]^[17]
- AL amyloidosis is the most common form of systemic amyloidosis in the US.
- Occurs in 5 to 15% of people with multiple myeloma.
- Treatment can involve application of chemotherapy similar to that used in multiple myeloma

For more information on primary amyloidosis, click here.

References

↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ ^6.0 ^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.
↑ 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.
↑ 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.
↑ Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6.
↑ 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.
↑ 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.
↑ 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.
↑ Suszek D, Majdan M (2018). "[Cryoglobulins and cryoglobulinemic vasculitis]". Wiad Lek. 71 (1 pt 1): 59–63. PMID 29558353.
↑ Blank N, Lorenz HM (2016). "[Cryoglobulinemic vasculitis]". Z Rheumatol. 75 (3): 303–15. doi:10.1007/s00393-016-0076-4. PMID 27034078.
↑ 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.
↑ 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)
↑ "Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com".

Template:WH Template:WS

[pmid16628189-1] 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.

[pmid19181642-2] 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.

[pmid23233640-3] 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.

[SergentanisZagouri2015-4] 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.

[pmid27291302-5] 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.

[Rajkumar2016-6] 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.

[CaersPaiva2018-7] 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.

[pmid22773606-8] 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.

[isbn92-832-2411-6-9] Pathology and Genetics of Haemo (World Health Organization Classification of Tumours S.). Oxford Univ Pr. ISBN 92-832-2411-6.

[Hallek2015-10] 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.

[MunshiCabot2008-11] 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.

[pmid16026843-12] 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.

[pmid29558353-13] Suszek D, Majdan M (2018). "[Cryoglobulins and cryoglobulinemic vasculitis]". Wiad Lek. 71 (1 pt 1): 59–63. PMID 29558353.

[pmid27034078-14] Blank N, Lorenz HM (2016). "[Cryoglobulinemic vasculitis]". Z Rheumatol. 75 (3): 303–15. doi:10.1007/s00393-016-0076-4. PMID 27034078.

[pmid10787003-15] 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.

[pmid15198347-16] 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)

[urlAmyloidosis_Causes,_Diagnosis,_Symptoms,_and_Treatment_on_MedicineNet.com-17] "Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com".

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@@ Line 2: / Line 2: @@
 {{Plasma cell disorder}}
-{{CMG}};  {{AE}} {{N.F}}
+{{CMG}};  {{AE}} {{N.F}}, {{Anmol}}
+{{SK}} Plasma cell dyscrasia
 ==[[Plasma cell disorder overview|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 classification|monoclonal gammopathy of undetermined significance]] (MGUS)[[Multiple myeloma|, multiple myeloma]] (MM), [[lymphoplasmacytic lymphoma]]/ [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]]  (LPL/WM), [[Lymphoproliferative disorders|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.
+Plasma cell disorders are a diverse type of blood disorders characterized by [[proliferation]] of a single clone of [[plasma cells]] that produces a [[homogeneous]] monoclonal (M) protein. These monoclonal paraprotein are seen 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 classification|monoclonal gammopathy of undetermined significance]] (MGUS)[[Multiple myeloma|, multiple myeloma]] (MM), [[lymphoplasmacytic lymphoma]] or [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]]  (LPL/WM), [[Lymphoproliferative disorders|lymphoproliferative disorders,]] smoldering multiple myeloma (SMM); solitary or extramedullary plasmacytoma,  [[amyloidosis]], and [[POEMS syndrome]] ([[polyneuropathy]], [[Organomegaly|organomegaly,]] [[endocrinopathy]], monoclonal protein, and skin changes). These disorders have been defined by the International Myeloma Working Group in 2006.
 ==[[Plasma cell disorder classification|Classification]]==
@@ Line 14: / Line 14: @@
 {{family tree | | | | | | | | | | | | A01 | | | | | | | | | | | | | | A01=Plasma cell disorder}}
 {{family tree | |,|-|-|-|v|-|-|-|v|-|-|^|-|-|v|-|-|-|v|-|-|-|.| | | }}
-{{Family tree | B01 | | B02 | | B03 | | | | B04 | | B05 | | B06 | | B01=[[Monoclonal gammopathy of unknown significance]] (MGUS)|B02=Malignant monoclonal gammopathy|B03=[[Chronic lymphocytic leukemia]] |B04=Heavy chain diseases
+{{Family tree | B01 | | B02 | | B03 | | | | B04 | | B05 | | B06 | | B01=[[Primary amyloidosis]]|B02=[[Plasma cell disorder#Malignant monoclonal gammopathies|Malignant monoclonal gammopathy]]|B03=[[Chronic lymphocytic leukemia]] |B04=[[plasma cell disorder#Heavy-chain diseases|Heavy chain diseases (HCD)]]| B05=[[Cryoglobulinemia]] | B06=[[Monoclonal gammopathy of undetermined significance]] (MGUS)}}
-γHCD
+{{Family tree | | | | | |!| | | | | | | | | |!| | | | | | | |!| | | }}
-αHCD
+{{Family tree | | |,|-|-|^|-|.| | | |,|-|-|-|+|-|-|-|.| | | |!| | | }}
-μHCD | B05=[[Cryoglobulinemia]] | B06= [[Primary amyloidosis]]}}
+{{Family tree | | C01 | | | C02 | | C03 | | C04 | | C05 | | |!| | | |C01=[[Multiple myeloma]]|C02=[[Plasma cell disorder#Malignant lymphoproliferative disorders|Malignant lymphoproliferative disorders]]|C03=[[Plasma cell disorder#γHCD|γHCD]]|C04=[[Plasma cell disorder#αHCD|αHCD]]|C05=[[Plasma cell disorder#μHCD|μHCD]]}}
-{{Family tree | | | | | |!| | | | | | | | | | | | | | | | }}
+{{family tree | | |!| | | | |!| | | | | | | | | | | | | | | |!| | | }}
-{{Family tree | |,|-|-|-|^|-|-|-|-|-|-|-|.| | | | | | }}
+{{family tree | | |!| | | | |!| | | | | |,|-|-|-|v|-|-|-|v|-|^|-|.|}}
-{{Family tree | | C01 | | | | | | | | | C02 | | | | | | | | | | | | |C01=[[Multiple myeloma]]|C02=[[Malignant lymphoproliferative disorders]]}}
+{{family tree | | |!| | |,|-|^|-|.| | | F01 | | F02 | | F03 | | F04 | |F01= [[Benign]]<br>(IgG, IgA, IgD, IgM, and,<br>rarely, free light chains)|F02=Associated [[Neoplasm|neoplasms]]<br>or other diseases not known to<br>produce monoclonal proteins|F03=Biclonal and triclonal<br>[[Gammopathy|gammopathies]]|F04= Idiopathic<br>(Bence Jones<br>proteinuria)}}
-{{family tree | | |!| | | | | | | | | | |!| | | | | | | | | }}
+{{family tree | | |!| | D01 | | D02 | | | | | | | | | | | | | | | | |D01=[[Waldenstrom macroglobulinemia]] | D02=[[Malignant lymphoma]]}}
-{{family tree | | |!| | | | | | | | |,|-|^|-|-|.| | | | | | }}
+{{family tree | | |!| | | | | | | | | | | | | | | | | | | | | | | | | | | | }}
-{{family tree | | |!| | | | | | | | D01 | | D02 | | | | | | | | D01=[[Waldenstrom macroglobulinemia]] | D02=[[Malignant lymphoma]]}}
+{{family tree | |,|^|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.|}}
-{{family tree | | |!| | | | | | | | | | | | | | | | | | | | }}
+{{family tree | E01 | | E02 | | E03 | | E04 | | E05 | | E06 | | E07 | | E08 | | E01=[[Plasma cell disorder#Symptomatic multiple myeloma|Symptomatic multiple myeloma]] | E02=[[Plasma cell disorder#Smoldering multiple myeloma|Smoldering multiple myeloma]] | E03=[[Plasma cell disorder#Plasma-cell leukemia|Plasma-cell leukemia]]|E04=[[Plasma cell disorder#Non-secretory myeloma|Non-secretory myeloma]] |E05=[[Plasma cell disorder#IgD myeloma|IgD myeloma]] | E06=[[Plasma cell disorder#Osteosclerotic myeloma|Osteosclerotic myeloma]] | E07=[[Plasma cell disorder#Solitary plasmacytoma of bone|Solitary plasmacytoma of bone]] |E08=[[Plasma cell disorder#Extramedullary plasmacytoma|Extramedullary plasmacytoma]]}}
-{{family tree | |,|^|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-| |-|-|v|-|-|-|v|-|-|-|.| | | | | | | | | | |}}
-{{family tree | E01 | | E02 | | E03 | | E04 | | E05 | | | E06 | | E07  | | E01=[[Symptomatic multiple myeloma]] | E02=[[Smoldering multiple myeloma]] | E03=[[Plasma-cell leukemia]]|E04=Non-secretory myeloma | E05=[[Solitary plasmacytoma]] of bone | E06=[[Osteosclerotic myeloma]] | E07=[[Extramedullary plasmacytoma]]}}
 {{Family tree/end}}
+==[[Plasma cell disorder| Differential Diagnosis]]==
-=== 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'''
-** [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]]
-** [[Malignant lymphoma]]
-=== Chronic lymphocytic leukemia ===
-=== Heavy-chain diseases (HCDs) ===
-** γHCD
-** αHCD
-** μHCD
-=== Cryoglobulinemia ===
-=== Primary amyloidosis (AL) ===
-== [[Plasma cell disorder| Differential Diagnosis]] ==
 {| class="wikitable"
 |+
@@ Line 96: / Line 62: @@
 | style="background:#F5F5F5;" align="center" + |N/A
 | style="background:#F5F5F5;" align="center" + |<10%
-| style="background:#F5F5F5;" align="center" + |No end-organ
+| style="background:#F5F5F5;" align="left" + |
-damage
+*No end-organ damage
 |-
 | style="background:#DCDCDC;" align="center" + |Smoldering MM
@@ Line 108: / Line 74: @@
 | style="background:#F5F5F5;" align="center" + |N/A
 | style="background:#F5F5F5;" align="center" + |10-60%
-| style="background:#F5F5F5;" align="center" + |
+| style="background:#F5F5F5;" align="left" + |
-* No myeloma-defining event
+*No myeloma-defining event
-* No  CRAB features
+*No  CRAB features
 |-
 | style="background:#DCDCDC;" align="center" + |[[Monoclonal gammopathy of undetermined significance|Light chain MGUS]]
@@ Line 119: / Line 85: @@
 | style="background:#F5F5F5;" align="center" + |−
 | style="background:#F5F5F5;" align="center" + |<500 mg/24 hrs (urine)
-| style="background:#F5F5F5;" align="center" + |Free kappa or lambda light chain
+| style="background:#F5F5F5;" align="center" + |Free kappa or lambda light chain<br>Abnormal ratio (<0.26 or >1.65)<br>Increase in involved light chain concentration
-Abnormal ratio (<0.26 or >1.65)
-Increase in involved light chain concentration
 | style="background:#F5F5F5;" align="center" + |<10%
-| style="background:#F5F5F5;" align="center" + |No end-organ damage
+| style="background:#F5F5F5;" align="left" + |
+*No end-organ damage
 |-
 | style="background:#DCDCDC;" align="center" + |[[Multiple myeloma classification|Active symptomatic Multiple myeloma]]
@@ Line 135: / Line 99: @@
 | style="background:#F5F5F5;" align="center" + |>100
 | style="background:#F5F5F5;" align="center" + |>60%
-| style="background:#F5F5F5;" align="center" + |
+| style="background:#F5F5F5;" align="left" + |
-* ≥1 myeloma-defining event
+*≥1 myeloma-defining event
+*CRAB features
-* CRAB features
 |-
 | style="background:#DCDCDC;" align="center" + |[[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]]
@@ Line 149: / Line 112: @@
 | style="background:#F5F5F5;" align="center" + |N/A
 | style="background:#F5F5F5;" align="center" + |>10%
-| style="background:#F5F5F5;" align="center" + |
+| style="background:#F5F5F5;" align="left" + |
-* Evidence of organ/tissue damage.
+*Evidence of organ/tissue damage
+*[[Anemia]]
-* Anemia,
+*[[Hepatosplenomegaly]]
-* Hepatosplenomegaly
 |-
 | style="background:#DCDCDC;" align="center" + |Solitary Plasmacytoma
@@ Line 163: / Line 125: @@
 | style="background:#F5F5F5;" align="center" + |<3mg/dl
 | style="background:#F5F5F5;" align="center" + |Abnormal in 47% cases
 | style="background:#F5F5F5;" align="center" + |Normal
-| style="background:#F5F5F5;" align="center" + |
+| style="background:#F5F5F5;" align="left" + |
-* Solitory bone lesion due to plasma cell tumor
+*Solitory bone lesion due to [[plasma cell]] tumor
+*Preserved levels of uninvolved [[immunoglobulins]]
-* Preserved levels of uninvolved immunoglobulins
+*No [[anemia]], [[hypercalcemia]] or [[renal disease]]
-* No anemia, hypercalcemia or renal disease
 |-
 | style="background:#DCDCDC;" align="center" + |[[AL amyloidosis|Primary amyloidosis]]
@@ Line 178: / Line 138: @@
 | style="background:#F5F5F5;" align="center" + |−
 | style="background:#F5F5F5;" align="center" + |<3md/dl
 | style="background:#F5F5F5;" align="center" + |[[Light chain|Light chains]] of [[Immunoglobulin|immunoglobulines]]
 | style="background:#F5F5F5;" align="center" + |<10%
-| style="background:#F5F5F5;" align="center" + |
+| style="background:#F5F5F5;" align="left" + |
-* No bone lesions,
+*No bone lesions
+*[[Nephrotic syndrome]]
+*[[Restrictive cardiomyopathy]]
+*[[Peripheral neuropathy]]
+*[[Hepatomegaly]] with elevated [[liver enzymes]]
+*[[Macroglossia]]
+*[[Purpura]] and an unexplained [[Hemorrhagic diathesis|bleeding diathesis]]
+|-
+| colspan="10" style="background:#DCDCDC;" align="left" + |
+*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, '''a'''nemia 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)==
+*[[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.
+*MGUS is a premalignant condition and is distinct fr<nowiki/>om [[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]].'''
+==Malignant monoclonal gammopathies==
+==='''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 exhibit 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
+**[[Bone marrow cells|Bone marrow]] [[plasma cells]] constitute more than 10% of the [[blood cells]]
+**Presence of solitary [[plasmacytoma]] in bone
+**≥ 1 myeloma-defining event
+**CRAB features ( explained above)
+**Osteolytic lesions on bone x-ray
+*Patients with active [[multiple myeloma]] usually require treatment to prevent progression of disease which can lead to death.
+====Smoldering multiple myeloma====
-* [[Nephrotic syndrome]]
+*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>
-* [[Restrictive cardiomyopathy]]
+*Shows presence of M-spike that quantitates > 3 g/dl on [[protein electrophoresis]].
-* [[Peripheral neuropathy]]
-* [[Hepatomegaly]] with elevated [[liver enzymes]]
+*Presence of [[Bone marrow cells|bone marrow plasma cell]] burden of > 10% but < 60%.
-* [[Macroglossia]]
-* [[Purpura]] and an unexplained [[Hemorrhagic diathesis|bleeding diathesis]]
+*Absence of end-organ damage such as [[anemia]], [[hypercalcemia]], [[Renal dysfunction|renal dysfunction,]] or [[Osseous|osseous lesions]].
-|}
-[[Category:Projects]]
+*Patients with smoldering (asymptomatic) [[multiple myeloma]] are managed by observation and undergoing follow up tests every 3 to 6 months.
-[[Category:Help]]
+*There is high risk of developing active [[multiple myeloma]].
+====Plasma-cell leukemia====
+*Characterized 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]].
+*Can occur as a secondary type in a patient with multiple myeloma.
+*Treatment options for [[plasma cell leukemia]] is [[chemotherapy]] or [[Hematopoietic stem cell transplantation|stem cell transplant]].
+====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" />
+*M protein is not detected by [[serum protein electrophoresis]].
+*[[Bone marrow]] exibit [[Myeloma|myeloma cells]].
+*Osteolytic bone lesions are seen on X-ray.
+====IgD myeloma====
+*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 myeloma mostly affect people of younger age.
+====Osteosclerotic myeloma====
-Myeloma defining events: >60% clonal plasma cells on B.M exam; serum involved:uninvolved FLC ratio >100; >1 focal lesion on MRI >5mm
+*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>
-CRAB features: elevated calcium >11mg/dl, renal insufficiency, '''a'''nemia 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.
+*It is called POEMS syndrome: [[polyneuropathy]], [[Organomegaly|organomegaly,]] [[endocrinopathy]], monoclonal protein, skin changes.
+*Treatment option for osteoclastic myeloma include:
+**[[Chemotherapy]]
+**[[Radiation therapy]]
+**[[Hematopoietic stem cell transplantation|Stem cell transplant]]
-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.
+====Solitary plasmacytoma of bone====
-== Plasma cell disorders ==
+*[[Plasmacytoma]] is collection of abnormal [[plasma cells]] forming a single 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 occurrence of single bone [[tumor]] made up of myeloma cells.
+*X-ray shows an osteolytic lesion at the site of the [[Tumor|tumor.]]
+*[[Bone marrow]] plasma population remains less than 10%.
+*One third of patients with solitary [[plasmacytoma]] will develop [[Multiple myeloma classification|multiple myeloma]].
-=== Monoclonal gammopathies of undetermined significance (MGUS) ===
+====Extramedullary plasmacytoma====
-* 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]].
-* 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]].
-* 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]]'''
-=== Malignant monoclonal gammopathies ===
+*It is developed 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]].
+*Diagnosis is confirmed by [[biopsy]] of the [[Tumor|tumor.]]
+*X-rays and [[bone marrow]] [[biopsy]] is normal.
+*Treatment is done with either [[radiation therapy]] or surgery.
-==== '''Multiple myeloma''' ====
+For more information about [[multiple myeloma]], '''[[Multiple myeloma |click here]].'''
-===== Symptomatic [[multiple myeloma]] =====
+===Malignant lymphoproliferative disorders===
-* People with multiple myeloma with symptoms are categorized to have active multiple myeloma and will exibit any of the following features.
+====Waldenstrom macroglobulinemia====
-** M protein in blood or urine
-** Bone marrow plasma cells constitute more than 10% of the blood cells
-** Presence of solitary plasmacytoma in bone
-** ≥1 myeloma-defining event
-** CRAB features ( explained above)
-** Osteolytic lesions on bone x-ray
-* Patients with active [[Multiple myeloma diagnostic criteria|Multiple myeloma]] usually require treatment to prevent progression of disease which can lead to death.
+*[[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]].
+*It is a type of [[lymphoproliferative disease]].
+*It shares clinical characteristics with the indolent [[Non-Hodgkin lymphoma|non-Hodgkin lymphomas]].
+*[[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] represents 1% of all hematological cancers.
+*Common causes of [[Waldenström's macroglobulinemia]] include [[genetic]], environmental, and [[autoimmune]] factors.
+*Common [[risk factors]] in the development of [[Waldenström's macroglobulinemia]] are [[monoclonal gammopathies of undetermined significance]].
-===== Smoldering multiple myeloma =====
+For more information about [[Waldenström's macroglobulinemia]], '''[[Waldenström's macroglobulinemia |click here]].'''
-* 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>
-* Shows presence of M-spike that quantitates > 3 g/dl on [[protein electrophoresis]]
-* Presence of bone marrow plasma cell burden of > 10% but < 60%
+====Malignant lymphoma====
-* Absence of end-organ damage such as anemia, [[hypercalcemia]], renal dysfunction, or [[Osseous|osseous lesions]]
+*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>
+*Also called [[Hematological malignancy|hematological neoplasms]].
+*Primary types include [[Hodgkin lymphoma]] and [[non-Hodgkin lymphoma]].
-* Patients with smoldering (asymptomatic) [[Multiple myeloma diagnostic criteria|Multiple myeloma]] are managed by observation and undergoing follow up tests every 3 to 6 months
+For more information about [[malignant lymphoma]], '''[[lymphoma |click here]].'''
-* There is high risk of developing active multiple myeloma.
-===== Plasma-cell leukemia =====
+===Chronic lymphocytic leukemia===
-* Charecterized by large number of plasma cells circulating in the blood
-* Rare condition, can develop in to most aggressive form of multiple myeloma.
-* Treatment options for plasma cell leukemia is chemotherapy or stem cell transplant.
-===== Non-secretory myeloma =====
+*[[Chronic lymphocytic leukemia]] arises from pre-follicular center [[B cell|B cells]], normally involved in [[Immunoglobulin|immunoglobulins]] production.<ref name="Hallek2015">{{cite journal|last1=Hallek|first1=Michael|title=Chronic lymphocytic leukemia: 2015 Update on diagnosis, risk stratification, and treatment|journal=American Journal of Hematology|volume=90|issue=5|year=2015|pages=446–460|issn=03618609|doi=10.1002/ajh.23979}}</ref>
-* Type of multiple myeloma with less amount of M proteins secretion in blood or urine.
+*Development of [[chronic lymphocytic leukemia]] is the result of multiple [[Genetic mutation|genetic mutations]] that promote both [[malignant]] leukemic proliferation and [[apoptotic]] resistance of mature [[B cells]].
-* M protein is not detected by protein electrophoresis
+*[[Chronic lymphocytic leukemia]] must be differentiated from [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].
-* Bone marrow exibit myeloma cells.
+*[[Prognosis]] is generally good, and the 5-year survival rate of patients with [[chronic lymphocytic leukemia]] is approximately 81.7%.
-* Osteolytic bone lesions are seen on xrays.
+*The mainstay of therapy for [[symptomatic]] [[chronic lymphocytic leukemia]] patients is immunochemotherapy.
-===== IgD myeloma =====
+For more information about [[chronic lymphocytic leukemia]], '''[[ chronic lymphocytic leukemia|click here]]'''
-* IgD type constitues 2% of multiple myeloma
-* IgD multiple myeloma has similar signs and symptoms as other types of multiple myeloma.
-* IgD myeloma mostly affect people of younger age.
+==Heavy-chain diseases (HCD)==
-===== Osteosclerotic myeloma =====
+*Heavy chain diseases are [[plasma cell]] [[neoplasia]] 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>
-* It is a rare disorder affecting multiple systems of the body
-* It is called POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes
+===γHCD===
-* Treatment option for osteoclastic myeloma include:
-** Chemotherapy
-** Radiation therapy
-** Stem cell transplant
-===== Solitary plasmacytoma of bone =====
+*Also known as gamma chain or IgG heavy chain disease.
-* 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>
+**Primarily seen in elderly men but can occur in children.
-* Solitary plasmacytoma is occurence of single bone tumor made up of myeloma cells.
+**High levels of IgG with reduction of normal [[immunoglobulin]] level.
-* Xray shows an osteolytic lesion at the site of the tumor.
+**[[Lymphadenopathy|Lymphadenopath]]<nowiki/>y, [[hepatosplenomegaly]], and recurrent infections are common features.
-* Bone marrow plasma population remains less than 10%
+**[[Vincristine]], [[Corticosteroid|corticosteroids]] and [[radiation therapy]] may produce [[Remission (medicine)|remission]].
-* One third of patients with solitary plasmacytoma will develop multiple myeloma.
-===== Extramedullary plasmacytoma =====
+===αHCD===
-* It is develped outside the bone marrow in soft tissues of the body
-* Most commonly seen in throat,paranasal sinuses, nasal cavity, larynx,GI tract, breast and brain.
-* Diagnosis is confirmed by biopsy of the tumor.
-* X-rays and  bone marrow biopsy is normal.
-* Treatment is done with either radiation therapy or surgry.
-For more information about Multiple myeloma '''[[Multiple myeloma |click here]]'''
-==== '''Malignant lymphoproliferative disorders''' ====
+*Also known as alpha chain or IgA heavy chain.
-* [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]]
+**Appears between age 10-30 as an [[immune]] response to a [[microorganism]].
-** [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] is a cancer involving  [[lymphocytes]].
+**Patients present with diffuse abdominal [[lymphoma]] and [[malabsorption]].
-** The main attributing [[antibody]] is [[IgM]].
+**Course is variable, some patients die in 1-2 yrs, others go in to [[Remission (medicine)|remission]] lasting for many years.
-** It is a type of [[lymphoproliferative disease]],
+**[[Serum protein electrophoresis]] detect increased α & β fraction.
-** It shares clinical characteristics with the indolent [[Non-Hodgkin lymphoma|non-Hodgkin lymphomas]].
+**Treatment is [[Corticosteroid|corticosteroids]], [[cytotoxic drugs]] and broadspectrum antibiotics
-** [[Waldenström's macroglobulinemia|Waldenstrom macroglobulinemia]] represents 1% of all hematological cancers
-** 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]]'''
-====== Malignant lymphoma ======
+===μHCD===
-* Type of [[cancer]] that originates in [[Lymphocyte|lymphocytes]]
-* Also called [[Hematological malignancy|hematological neoplasms]].
-* Main types are Hodgkin lymphoma and Non-Hodgkin lymphoma
-For more information about lymphoma '''[[lymphoma |click here]]'''
-=== Chronic lymphocytic leukemia ===
+*Also known as mu chain or IGM heavy chain disease.
-* [[Chronic lymphocytic leukemia]] arises from pre-follicular center [[B cell|B cells]], normally involved in [[Immunoglobulin|immunoglobulins]] production.
+**Mainly affects individuals > 50 yrs.
-* Development of [[chronic lymphocytic leukemia]] is the result of multiple [[Genetic mutation|genetic mutations]] that promote both [[malignant]] leukemic proliferation and [[apoptotic]] resistance of mature [[B cells]].
+**[[Spleen]], [[liver]] and abdominal [[lymph nodes]] involvement is more common than peripheral [[lymphadenopathy]].
-* [[Chronic lymphocytic leukemia]] must be differentiated from [[hairy cell leukaemia]], prolymphocytic leukaemia, [[follicular lymphoma]], and [[mantle cell lymphoma]].
+**[[Serum]] [[protein electrophoresis]] exibit [[hypogammaglobulinemia]].
-* Prognosis is generally good, and the 5-year survival rate of patients with [[chronic lymphocytic leukemia]] is approximately 81.7%.
+**Vacuolated [[Plasma cell|plasma cells]] are pathognomic on [[bone marrow]] exam.
-* The mainstay of therapy for symptomatic [[chronic lymphocytic leukemia]] patients is immunochemotherapy.
+**Treatment consists of [[Alkylating agent|alkylating agents]] and [[Corticosteroid|corticosteroids]].
-For more information about chronic lymphocytic leukemia '''[[ chronic lymphocytic leukemia|click here]]'''
-=== Heavy-chain diseases ===
+==Cryoglobulinemia==
-Heavy chain diseases are plasma cell neoplasias, featuring overproduction of immunoglobulin heavy chains.
-==== γHCD ====
+*[[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>
-Gamma chain or IgG heavy chain disease
+*Cryoglobulins are circulating [[immunoglobulins]] or [[Protein|proteins]] that become insoluble at less than 4 degrees celsius.
-* Primarily seen in elderly men but can occur in children.
+*[[Cryoglobulinemia]] can lead to a medium-sized vessel [[vasculitis]] due to vascular deposition of circulating [[immune complexes]].
-* High levels of IgG with reduction of normal immunoglobulin level
+*This leads to the triad of palpable [[purpura]], [[Arthralgia|arthralgias]] and [[peripheral neuropathy]].
-* Lymphadenopathy, hepatosplenomegaly and recurrent infections are common features
+*Common causes of [[Cryoglobulinemia CT|cryoglobulinemia]] are primarily [[hematologic]], [[Oncology|oncologic]], and [[rheumatic]]
-* Vincristine, corticosteroids and radiation therapy may produce remission.
-==== αHCD ====
+For more information on [[cryoglobulinemia]], [[Cryoglobulinaemia|'''click here''']].
-Alpha chain or IgA heavy chain
-* Appears between age 10-30 as an immune response to a microorganism
-* 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.
-* Serum protein electrophoresis detect increased α & β fraction.
-* Treatment is corticosteroids, cytotoxic drugs and broadspectrum antibiotics
-==== μHCD ====
+==Primary amyloidosis==
-MU chain or IGM heavy chain disease
-* Mainly affects individuals > 50 yrs
-* Spleen, liver and abdominal lymph nodes involvement is more common than peripheral lymphadenopathy
-* Serum protein electrophoresis exibit hypogammaglobulinemia.
-* Vacuolated plasma cells are pathognomic on bone marrow exam.
-* Treatment consists of alkylating agents and corticosteroids.
-=== Cryoglobulinemia ===
+*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>
-* [[Cryoglobulinemia]] is the presence of high amount of heavy [[Globulin|globulins]] (e.g. [[IgM]]) in the [[bloodstream]] which thicken on exposure to cold.
+**[[AL amyloidosis]] is the most common form of systemic [[amyloidosis]] in the US.
-* Cryoglobulins are circulating [[immunoglobulins]] or [[Protein|proteins]] that become insoluble at less than 4 degrees Celsius.
+**Occurs in 5 to 15% of people with [[multiple myeloma]].
-* Cryoglobulinemia can lead to a medium-sized vessel [[vasculitis]] due to vascular deposition of circulating [[immune complexes]].
+**Treatment can involve application of [[chemotherapy]] similar to that used in [[Multiple myeloma Classification|multiple myeloma]]
-* This leads to the triad of palpable [[purpura]], [[Arthralgia|arthralgias]] and [[peripheral neuropathy]]
-* Common causes of cryoglobulinemia are primarily hematologic, oncologic, and rheumatic
-For more information on Cryoglobulinemia [[Cryoglobulinemia| click here]]
-=== Primary amyloidosis ===
+For more information on primary amyloidosis, '''[[Primary amyloidosis|click here]].'''
 ==References==
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+[[Category:Medicine]]
+[[Category:Hematology]]
+[[Category:Oncology]]

Plasma cell disorder: Difference between revisions

Latest revision as of 11:52, 19 July 2022

Overview

Classification

Differential Diagnosis

Monoclonal gammopathies of undetermined significance (MGUS)

Malignant monoclonal gammopathies

Multiple myeloma

Symptomatic multiple myeloma

Smoldering multiple myeloma

Plasma-cell leukemia

Non-secretory myeloma

IgD myeloma

Osteosclerotic myeloma

Solitary plasmacytoma of bone

Extramedullary plasmacytoma

Malignant lymphoproliferative disorders

Waldenstrom macroglobulinemia

Malignant lymphoma

Chronic lymphocytic leukemia

Heavy-chain diseases (HCD)

γHCD

αHCD

μHCD

Cryoglobulinemia

Primary amyloidosis

References

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