Plasma cell disorder

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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
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
AL 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

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  17. "Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com".

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