216 Part III • White Cell Disorders

Summary Points

• Lymphoproliferative disorders comprise the B and T lymphocytes in which there is a clonal malignant proliferation of either cell subset.

• Chronic lymphocytic leukemia (CLL) is a clonal proliferation of B lymphocytes that is seen in older patients and often discovered by accident.

• CLL shows an accumulation of mature lymphocytes in the bone marrow and eventually the lymph nodes, spleen, and peripheral blood.

• The white counts in CLL are extremely elevated and the M:E ratio is 10 to 20:1.

• Immune function is compromised in CLL, and 10% to 30% of individuals may experience autoimmune hemolytic anemia.

• Hairy cell leukemia (HCL) is a rare B-cell malignancy in which the cells have a lymphoid appearance but hair-like projections in the cytoplasm.

• Pancytopenia, splenomegaly, and dry tap are the key features of HCL.

• Sezary syndrome is the blood equivalent of cutaneous T-cell lymphoma that presents with a convoluted, cerebriform, ovoid nucleus.

• Multiple myeloma is a disorder of plasma cells that leads to a monoclonal gammopathy, bone involvement, and pancytopenia.

• Most of the abnormal proteins are an accumulation of IgG, which may lead to a hyperviscosity and rouleaux in the peripheral smear.

• Serum calcium is elevated in MM patients due to bone loss and increased distribution of calcium in the peripheral circulation.

• Bence-Jones protein may be seen in individuals with MM.

• Plasma cell leukemia is a complication of MM in which mature plasma cells are seen in increasing numbers in the peripheral circulation.

• Waldenstrom's macroglobulinemia is a rare disorder of plasma cells in which IgM is overproduced.

• Many of the symptoms of Waldenstrom's macroglobulinemia are related to hyperviscosity of the plasma, which accounts for coagulation abnormalities, rouleaux formation, and bleeding or thrombotic complications.

• Plasmapheresis, the therapeutic removal of plasma, may be used as a treatment to decrease the amount of abnormal IgM protein.

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