This is very rare; only a small fraction (~2%) of persons infected with HTLV-I go on to develop the disease. Some HTLV-I-infected pts develop spastic paraplegia from spinal cord involvement without developing cancer. The characteristic clinical syndrome of ATL includes high white count without severe anemia or thrombocytopenia, skin infiltration, hepatomegaly, pulmonary infiltrates, meningeal involvement, and opportunistic infections. The tumor cells are CD4+ T cells with cloven hoof- or flower-shaped nuclei. Hypercalcemia occurs in nearly all pts and is related to cytokines produced by the tumor cells.
Treatment: Adult T Cell Leukemia/Lymphoma Aggressive therapy is associated with serious toxicity related to the underlying immunodeficiency. Glucocorticoids relieve hypercalcemia. The tumor is responsive to therapy, but responses are generally short lived. Zidovudine and IFN may be palliative in some pts. |
Section 6. Hematology and Oncology