四肢末端壊死を伴ったT細胞リンパ腫(Tγ)の1例

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A 75 year old housewife was admitted to our hospital because of generalized itchy eruptions which had been present for about 3 years. On admission, physical examination revealed generalized eruptions and necrosis in the bilateral fingertips. Spleen and superficial lymph nodes were not enlarged. Her leukocyte count was 38, 300/cmm with 88% abnormal leukocytes. These abnormal cells showed T cell characteristics, except blastoid transformation with PHA and ConA and had IgG-Fc receptors. Lymphangiography revealed enlargement of deep lymph nodes. Transmission electron microscopy of abnormal cells showed cerebriform nuclei with homogenous chromatin pattern that is similar to Sézary cells. Abnormal cell infiltration was found in the biopsied specimen of the skin. Combined chemotherapy with vincristine, procarbazine and cyclophosphamide was performed with effect. The patient, however, died of pneumonia on the 55th day of hospitalization. Autopsy revealed cytomegalo virus inclusion bodies in the lung, adrenal and ovarium. Abnormal cell infiltration was not found in the skin, lymph nodes or bone marrow. This case was diagnosed as T cell lymphoma based on the following : 1) no systemic involvement of superficial lymph nodes, 2) bone marrow invasion was significant, 3) eruptions were not identical to those of Sézary syndrome, 4) abnormal cells wer Fcr R<SUP>+</SUP>T cells. Although some differences in the clinical picture are present between T cell lymphoma, adult T cell leukemia, Sézary syndrome and mycosis fungoides, they belong to the same entity, that is T cell malignancy. Skin effects of various types have been reported in many cases of T cell malignancy. But, necrosis of the fingertips is rare in T cell malignancy. Fingertip necrosis may be caused by the infiltration of vessels and/or of surrounding tissue of fingertips with abnormal T cells.

四肢末端壊死を伴ったT細胞リンパ腫(Tγ)の1例

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