A 46-year-old black female patient was referred to the Hematological Unit of the University Hospital Prof. Edgard Santos, in Bahia, Brazil in January 2012 with confirmed HTLV-1 infection (ELISA positive confirmed by Western blot) and a history of cervical lymphadenomegaly in the previous month. She had had a diagnosis of pulmonary tuberculosis three months before in another center and was on rifampicin and isoniazid. Her blood tests taken in the previous 20 months showed lymphocytosis varying from 10,000 to 13,700 lymphocytes/mm3. Physical examination revealed cervical and retroauricular lymphadenomegaly. No skin lesions were observed. Blood examination showed leukocytosis with mild lymphocytosis (5940 lymphocytes/mm3). The blood levels of calcium, urea, and albumin were within the normal limits but the LDH was slightly elevated. Flow cytometry of peripheral blood revealed an expression of CD3, CD4, CD5, TCRalphabeta and CD2 with co-expression of CD25. The lymphoid cells were negative for CD7, CD8, TCRgammadelta, and for NK cell and B-cell markers. Serology for HIV was negative. Cervical and thoracic computed tomography (CT) showed cervical and thoracic enlarged lymph nodes and infiltration in the left lung; abdominal CT was normal. A lymph node biopsy was performed and a diagnosis of Hodgkin-like ATLL was given, clinically corresponding to a favorable chronic ATLL. It was decided that the treatment for ATLL should begin after Tb control, considering the high toxicity of the combined therapy and the fact that favorable chronic ATLL constitutes an indolent form of ATLL.
ATLL treatment was initiated six months after admission with zidovudine and interferon-alpha but due to important side-effects the drugs were discontinued and the patient began to be treated with six cycles of standard CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) with partial response. After 14 months of Tb treatment, she was considered cured with no acid-alcohol resistant bacilli in sputum smears and negative culture for Mycobacterium tuberculosis. The lymphocytosis reappeared after the end of chemotherapy and persisted until September 2013 varying from 4600 to 8000 lymphocytes/mm3. At this time, CT revealed enlarged cervical, thoracic and abdominal lymph nodes. Elevation of LDH, 2.5 times the upper normal limit, bilateral pleural effusion, slight peritoneal effusion and paresthesia and unilateral palpebral ptosis were also observed. She received two more salvage regimen of chemotherapy with partial response and died at home four months later. Pathologic findings - The lymph node exhibited diffuse infiltration of medium-sized atypical lymphocytes. Scattered in this background, HRS-like cells were seen, isolated or in small clusters (Fig. 1A). The medium-sized lymphocytes expressed CD3, CD4, CD5, CD25 (Fig. 1B), CD45RO (UCHL-1) and were CD8 negative. A marked reduction of CD7 expression was observed. The HRS-like cells were positive for CD30 (Fig. 1C), CS1-4 (Fig. 1D), CD79a, and focally for Bcl6 and CD20. The proliferative index as determined by Ki-67 immunostaining was 50%, especially in the giant cells. Both cell types were negative for CD10, CD15, CD21, CD23, and PD1.