A rare case of adult T cell leukemia/lymphoma: clinicopathological correlation with autopsy confirmation.

Adult T cell leukaemia/lymphoma (ATLL) is a rare T lymphoproliferative disorder which is etiologically linked with human T cell lymphotropic virus type-1 (HTLV-1). HTLV-1 is endemic in Japan, Caribbean and Africa. The highest incidence of ATLL is in Japan although sporadic cases have been reported elsewhere in the world. We describe a case of ATLL with an unusual presentation with clinic-pathological correlation and autopsy confirmation. A 56 year old male was referred to Command Hospital (Southern Command) for an incidental finding of lymphocytosis on a routine Hemogram. Clinical examination did not reveal hepatosplenomegaly, lymphadenopathy, jaundice or skin lesions. Laboratory investigations showed lymphocytosis with predominance of atypical lymphomonocytoid cells. Immunophenotyping of the bone marrow mononuclear cells showed positivity for CD45, CD2, CD3, CD4, CD5 and negative for CD7, CD8, CD13, CD33, CD19, which is characteristic of ATLL phenotype. Clonality was confirmed by PCR for TCR gene rearrangement on post mortem tissue. He succumbed to his illness after 40 days of initial presentation and 16 days of being diagnosed as ATLL. Here, we discuss the pathogenesis and characteristics of ATLL with clinico-pathological correlation and autopsy confirmation.