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Revision as of 16:41, 12 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).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

Malignant monoclonal gammopathies

Multiple myeloma

Symptomatic multiple myeloma
  • People with multiple myeloma with symptoms are categorized to have active multiple myeloma and will exibit any of the following features.
    • 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[1]
  • Presence of bone marrow plasma cell burden of > 10% but < 60%
  • 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
  • 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
  • Type of multiple myeloma with less amount of M proteins secretion in blood or urine.
  • M protein is not detected by protein electrophoresis
  • Bone marrow exibit myeloma cells.
  • Osteolytic bone lesions are seen on xrays.
IgD myeloma
  • 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.
Osteosclerotic myeloma
  • It is a rare disorder affecting multiple systems of the body
  • It is called POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes
  • Treatment option for osteoclastic myeloma include:
    • Chemotherapy
    • Radiation therapy
    • Stem cell transplant
Solitary plasmacytoma of bone
  • Plasmacytoma is collection of abnormal plasma cells forming a singl e tumor.[2]
  • 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
  • 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 click here

Malignant lymphoproliferative disorders

Malignant lymphoma

For more information about lymphoma click here

Chronic lymphocytic leukemia

For more information about chronic lymphocytic leukemia click here

Heavy-chain diseases

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

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

References

  1. 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.

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