[Peripheral T cell lymphoma (immunoblastic type, HTLV-1 negative) associated with aganglionosis of the intestine]

A 56-year-old female was hospitalized in 1992, because of fever and lymphadenopathy. She had ileus due to aganglionosis of the intestine diagnosed in 1990. Blood tests disclosed anemia and thrombocytopenia. Peripheral blood smear demonstrated 69.5% abnormal cells and a T-cell origin was demonstrated on cell marker study by flow cytometry. Immunological examinations, including serum HTLV-1 antibody were negative. Lymph node biopsy revealed malignant lymphoma; diffuse large, immunoblastic. The clonality of the T-cell malignancy was shown by detecting rearrangement of T-cell receptor beta gene. A diagnosis of PTCL, high grade, immunoblastic, was made. The patient received various chemotherapeutic agents and achieved a short remission, then died 5 months after the diagnosis. The classification of T-cell lymphoma was established by Suchi and the Kiel classification. Many ATL/L cases have been reported in Japan before the above classification was affirmed, although there have been few reports of ATL/L-like diseases in which serum HTLV-1 antibodies were negative. The case reported here was HTLV-1 negative, although the patient was diagnosed as ATL/L-like disease, because the results of various laboratory examinations were similar to these of HTLV-1 positive cases. Characteristics of this case included a female patient and leukemic clinical features, because the gender preponderance of PTCL, high grade, immunoblastic is male and the clinical features of the disease are not usually leukemic. It was also characteristic that PTCL coexisted with aganglionosis of the intestine. Basic disturbances in the latter condition would be similar to aganglionic megacolon in which a possible aberrant immune mechanism was shown.(ABSTRACT TRUNCATED AT 250 WORDS)