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Hepatosplenic T cell lymphoma

Author: Ira Miller, MD, PhD, 05/29/2018
Category: Lymphoma: Mature T and NK cell lymphoproliferations > Mature T-cell Lymphomas > Gastro-intestinal T-cell lymphomas > Hepatosplenic T-cell Lymphoma
Published Date: 05/29/2018

A 48 year old woman presented with fatigue. The blood showed WBC of 0.45K/mcL with severe neutropenia (0.12k/mcL), Hb 8.2g/dL, and platelets 91K/mcL. Her spleen was palpable 6 cm below the left costal margin. The marrow was hypercellular with erythroid hyperplasia and a mainly sinuoidal infiltrate of CD3+ cells that were negative for granzyme B. By flow cytometry, the cells (82% of the lymphocytes) were TCRgamma/delta+, positive for CD2, CD3, CD7, CD16, CD56, and CD4negCD8neg. The karyotype was  normal, and FISH studies did not show any abnormality of chromosome 7 The aspirate smear shows clusters of abnormal medium sized lymphocytes with slightly open chromatin and a moderate amount of agranular light blue cytoplasm with blebbing. The patient was started on treatment with intensive chemotherapy with a plan for allogeneic bone marrow transplantation.