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).The plasma-cell disorders are characterized by the proliferation of a single clone of plasma cells that produces a homogeneous monoclonal (M) protein. these disorders have been defined by the International Myeloma Working Group.1 In 2006.

Classification

Monoclonal gammopathies of undetermined significance (MGUS)

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

Malignant monoclonal gammopathies

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

Chronic lymphocytic leukemia

Heavy-chain diseases (HCDs)

    • γHCD
    • αHCD
    • μHCD

Cryoglobulinemia

Primary amyloidosis (AL)

Differential Diagnosis

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

damage

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

Abnormal ratio (<0.26 or >1.65)

Increase in involved light chain concentration

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

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

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

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

Plasma cell disorders

Monoclonal gammopathies of undetermined significance (MGUS)

For more information about Monoclonal gammopathies of undetermined significance click here

Multiple myeloma

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
  • Plasmacytoma is collection of abnormal plasma cells forming a single tumor.
  • Solitary plasmacytoma is occurence of single bone tumor made up of myeloma cells.
  • Xray 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

For more information about Multiple myeloma click here

Waldenstrom macroglobulinemia

For more information about Waldenström's macroglobulinemia click here

Chronic lymphocytic leukemia

For more information about chronic lymphocytic leukemia click here

Heavy-chain diseases

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

γHCD

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

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