A 34-year-old woman of HTLV-I carrier with T-PLL, whose quality of life improved and survival was prolonged after
splenectomy, is described. The patient had marked
splenomegaly, generalized
lymphadenopathy and marked proliferation of abnormal lymphocytes in the peripheral blood with an irregular nucleus, deeply basophilic cytoplasm and a single prominent nucleolus, which were positive for CD2, CD3, CD5, CD7, CD4 and CD8. Although the patient had serum antibody against HTLV-I, HTLV-I proviral
DNA integration was not detected. She was diagnosed as an HTLV-I carrier with T-PLL and received
combination chemotherapy and 15.1 Gy splenic irradiation. However, the generalized
lymphadenopathy and
splenomegaly did not improve. The patient underwent
splenectomy to palliate abdominal distension and
hypersplenism. After the operation, her symptoms improved dramatically and within a week her
hemoglobin concentration and platelet count normalized. She was discharged from hospital two weeks after the
splenectomy, however 11 months later, she relapsed and despite treatment with
chemotherapy and
alpha-interferon, she died two months after the second admission. Autopsy findings revealed that PLL cells had invaded the bone marrow, lymph nodes, liver, lungs, kidneys, uterus, ovaries and adrenal glands.