Hematology and Oncology Book

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MacroglobulinemiaAka: Waldenstrom's Macroglobulinemia, Primary Macroglobulinemia

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  1. Pathophysiology
    1. Lymphoproliferative Malignancy
      1. Proliferation of plasma cells and Lymphocytes
    2. Similar to Multiple Myeloma
    3. Produces a large monoclonal IgM protein
  2. Epidemiology
    1. Older men more frequently affected
    2. Hereditary component
  3. Symptoms
    1. Weakness
    2. Fatigue
    3. Oronasal bleeding
    4. Blurred vision or decreased Visual Acuity (33%)
    5. No Bone pain
      1. Contrast with Multiple Myeloma
    6. Hyperviscosity results in neurologic symptoms
      1. Dizziness or Vertigo
      2. Headache
      3. Nystagmus
      4. Paresthesias
      5. Decreased Level of Consciousness (Somnolence to Coma)
    7. Sudden Deafness
    8. Progressive spinal muscular atrophy
  4. Signs
    1. Pallor
    2. Splenomegaly
    3. Hepatomegaly
    4. Peripheral Lymphadenopathy
    5. Retinal lesions
      1. Retinal Hemorrhage
      2. Retinal Exudate
      3. Venous congestion
    6. Peripheral Neuropathy
    7. Pulmonary Disease
      1. Infiltrates or Pleural Effusion
  5. Labs
    1. Complete Blood Count
      1. Normocytic normochromic Anemia
    2. Peripheral Smear
      1. Rouleau formation
    3. Erythrocyte Sedimentation Rate markedly elevated
    4. Bone Marrow Biopsy
      1. Hypocellular
      2. Extensive Infiltrations by lymphoid and plasma cells
      3. Increased Mast Cell numbers
    5. Immunoelectrophoresis
      1. Monoclonal IgM Protein >3 g/dl
  6. Differential Diagnosis
    1. Multiple Myeloma
    2. Chronic Lymphocytic Leukemia (CLL)
    3. Lymphoma
  7. Management
    1. Suppression of Lymphocyte and Plasma Cell proliferation
      1. Chlorambucil
      2. Cyclophosphamide
      3. Combination chemotherapeutic regimens
    2. Hyperviscosity
      1. Plasmapheresis with cell separator
  8. Prognosis
    1. Five Year median survival after diagnosis

Waldenstrom Macroglobulinemia (C0024419)

Definition (MSH)A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity.
Definition (CSP)plasma cell dyscrasia resembling leukemia, with cells of lymphocytic, plasmacytic, or intermediate morphology, that secrete an IgM monoclonal component.
Definition (NCI)A rare cancer of the lymph cells that causes the body to produce abnormal levels of plasma cells (plasmacytosis) and lymphocytes (lymphocytosis) in the bone marrow. Waldenstrom's macroglobulinemia may also cause a decrease in the number of red blood cells (anemia) and enlargement of the liver (hepatomegaly), spleen (splenomegaly), or glands (adenopathy).
Definition (NCI)(MAK-ro-GLOB-u-li-NE-me-uh) A condition in which the blood contains high levels of large proteins and is too thick to flow through small blood vessels. One type is Waldenstrom's macroglobulinemia, which is a type of cancer.
Definition (NCI)A clonal neoplasm of small B-lymphocytes, lymphoplasmacytoid cells, and plasma cells involving the bone marrow, lymph nodes, and the spleen. The majority of patients have a serum IgM paraprotein. Lymphoplasmacytic lymphoma/ Waldenstrom's Macroglobulinemia is a relatively rare condition accounting for approximately 2% of hematologic malignancies. Symptoms include: weakness and fatigue, hemorrhagic manifestations, weight loss and visual disturbances. Physical findings include: hepatomegaly, splenomegaly, fundic changes, adenopathy, neurologic abnormalities and purpura. The most common laboratory finding is anemia. Treatment includes: chemotherapy (alkylating agents, nucleoside analogues) radiotherapy and plasmapheresis. Bone marrow transplant is being investigated. Asymptomatic patients may be monitored without treatment until complications occur.
ConceptsNeoplastic Process (T191)
ICD9273.3
Englishlymphoplasmacytic lymphoma, Lymphoplasmacytic Lymphoma/Waldenstrom Macroglobulinemia, Lymphoplasmacytic Lymphoma/Waldenstrom's Macroglobulinemia, lymphoplasmacytoid lymphoma, Lymphoplasmacytoid Lymphomas, Macroglobulinaemia, Macroglobulinemia, Primary macroglobulinaemia, Primary Macroglobulinemia, Waldenström macroglobulinaemia, Waldenström macroglobulinemia, Waldenstrom macroglobulinaemia, Waldenstrom macroglobulinemia, Waldenstrom's macroglob'naemia, Waldenstrom's macroglobulinaemia, Waldenstrom's macroglobulinemia, Waldenstroms Macroglobulinemia
Spanishmacroglobulinemia, macroglobulinemia de Waldenström, macroglobulinemia de Waldenstrom, macroglobulinemia primaria
CreditsDerived from the NIH UMLS (Unified Medical Language System)



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