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Glossary


Conditions related to multiple myeloma

Plasma cell disorders that are related to multiple myeloma include:

  • primary amyloidosis
  • heavy chain disease

Primary amyloidosis

Primary amyloidosis (also known as AL amyloidosis) is an uncommon disorder of the plasma cells that is related to multiple myeloma. Primary amyloidosis could also be associated with other B cell cancers, such as various types of lymphoma and chronic lymphocytic leukemia (CLL).

 

The light chains of the immunoglobulin, and other substances in the blood, produce a sticky protein called amyloid. Amyloid builds up in organs and interferes with their function. It may affect the:

  • heart
  • gastrointestinal tract
  • kidney
  • nerves

 

About 10%–15% of people with multiple myeloma will develop amyloidosis. Treatment for amyloidosis is similar to treatment for multiple myeloma. It targets the abnormal plasma cells.

  • Chemotherapy is given with melphalan (Alkeran, L-PAM) and prednisone (Deltasone, Winpred, Apo-Prednisone, Novo-Prednisone) for people with symptoms caused by the amyloid deposits.
  • Radiation may prevent amyloid from building up in larger deposits.

Heavy chain disease

Heavy chain disease is not usually associated with multiple myeloma. It occurs with other blood cancers, such as chronic lymphocytic leukemia (CLL) and lymphoplasmacytic lymphoma. Sometimes people with heavy chain disease develop plasma cell leukemia or a lymphoma that resembles an aggressive myeloma (called anaplastic myeloma) in the later stages of the disease.

 

In heavy chain disease, a genetic defect causes plasma cells to produce incomplete immunoglobulin molecules that only have the heavy chain of the immunoglobulin. There are 3 types of heavy chain disease, which are classified according to the type of heavy chain.

IgA heavy chain disease

  • IgA heavy chain disease is also known as immunoproliferative small intestinal disease (IPSID) or Mediterranean lymphoma. It is a type of mucosa-associated lymphatic tissue (MALT) lymphoma.
  • IgA heavy chain disease most often affects people of Middle Eastern or Mediterranean ancestry.
  • It affects the gastrointestinal tract and symptoms include chronic diarrhea, poor uptake of nutrients and abdominal pain.
  • Experts believe it is caused by long-term stimulation of the immune system by bacteria.
  • Treatment is usually antibiotic drugs. Chemotherapy drugs, such as cyclophosphamide (Cytoxan, Procytox), melphalan and prednisone, may also be used.

IgG heavy chain disease

  • Signs and symptoms of IgG heavy chain disease include:
    • enlarged lymph nodes
    • enlarged liver and spleen
    • weakness
    • weight loss
    • fever
    • anemia
    • a related autoimmune condition (for example, rheumatoid arthritis, lupus erythematosus)
  • Treatment is usually given only when symptoms appear. Chemotherapy drugs, such as cyclophosphamide, prednisone, vincristine (Oncovin), chlorambucil (Leukeran) or doxorubicin (Adriamycin) may be used.

IgM heavy chain disease

  • IgM heavy chain disease is the rarest heavy chain disease.
  • It is often related to chronic lymphocytic leukemia (CLL) and follows the same disease progression.
  • Signs and symptoms of IgM heavy chain disease include:
    • an enlarged liver and spleen
    • enlarged lymph nodes
    • weight loss
    • fever
    • anemia
    • recurrent infections
  • Treatment includes chemotherapy with chlorambucil (Leukeran), melphalan, and cyclophosphamide or sometimes fludarabine (Fludara).
  • IgM heavy chain disease is treated as CLL if CLL has also been diagnosed.

References

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