Rare Case Report of Adult T-Cell Leukemia / Lymphoma (ATLL), in the City of Macau- Rio Grande Do Norte; Brazil

Introduction:Adult T-cell leukemia / lymphoma (ATLL) is a rare type of malignant haemopathy with the Human T-Cell Lymphotropic Virus type I (HTLV-1) as its etiologic agent. The clinical picture includes skin lesions, hypercalcemia, elevated lactate dehydrogenase (LDH), adenomegaly, myelopathy and leukocytosis with atypical TCD4 lymphocytes.Objective:To report a case of a patient from the city of Macau-RN, with ATLL, presenting skin lesions, weight loss (> 13%), leukocytosis and supraclavicular lymph node enlargement. Case Description: Male patient, bricklayer, 67-year-old, hypertensive, was admitted to a hospital in Natal-RN, with severe itching, diffuse exanthematic skin lesions for 13 days, adynamia, associated with progressive, weight loss (P: 73Kg, 63Kg -13.6%), hyporexia, feverish (37.8 ° C), insidious and progressive drowsiness. Physical examination showed palpable lymphadenomegaly in the right supraclavicular chain, 1 cm, mobile and painless. Painful abdomen on deep palpation in the right hypochondrium and left iliac fossa, moderate hepatosplenomegaly. The blood count showed leukocytosis with the presence of atypical lymphocytes with convoluted nucleus (Flow-Cell), Creatinine (6.81), urea 349, hyperuricemia (14.9 mg / dL), hypercalcemia (12.8), LDH (723), Alkaline phosphatase (1388) GGT (183). Abdominal, kidney and urinary tract ultrasound revealed acute pancreatitis with signs of saponification (steatonecrosis of peripancreatic fat) and bilateral acute parenchymal nephropathy, respectively. The transthoracic echocardiogram identified aortic and mitral valve sclerosis. Immunophenotyping by flow cytometry found lymphoproliferative disease of mature TCD4 + cells, and the diagnosis of ATLL was confirmed by the serological reagent for HTLV-1. From then on, adequate treatment for ATLL was started with reversion of the clinical period, currently being followed up on an outpatient basis.Conclusions:The multiprofessional performance with accurate clinical investigation associated and specific laboratory exams such as immunophenotyping by flow cytometry and serological tests are essential for the early diagnosis and treatment of ATLL, a rare entity in the state of Rio Grande do Norte., Brazil and that high mortality when not diagnosed and treated early.