title: An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs'-Negative Hemolytic Anemia.
abstract: Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδ T-cell receptor (TCR), but recently, a few cases have been shown to express the αβ TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs'-negative hemolytic anemia. Diagnosis proved challenging due to an unusual blastoid morphology with the absence of typical intrasinusoidal distribution of tumor cells in the bone marrow. This unique case adds to the growing list of this rare subtype of T-cell lymphomas, which warrant urgent attention due to the lack of effective treatment options and dismal prognosis.
authors.affiliation: Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Feryal A
authors.initials: FA
authors.lastname: Ibrahim
authors.affiliation: Department of Laboratory Medicine and Pathology, Weill Cornell Medical College in Qatar, Education City, Doha, Qatar.
authors.forename: Vignesh
authors.initials: V
authors.lastname: Shanmugam
authors.affiliation: Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Aliaa
authors.initials: A
authors.lastname: Amer
authors.affiliation: Department of Hematology and Medical Oncology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Halima
authors.initials: H
authors.lastname: El-Omri
authors.affiliation: Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Ahmad
authors.initials: A
authors.lastname: Al-Sabbagh
authors.affiliation: Department of Hematology and Medical Oncology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Ruba Y
authors.initials: RY
authors.lastname: Taha
authors.affiliation: Department of Laboratory Medicine and Pathology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
authors.forename: Dina S
authors.initials: DS
authors.lastname: Soliman
keywords: Coombs’-negative hemolytic anemia; bone marrow biopsy; diagnosis; hepatosplenic αβ T-cell lymphoma; pathology; prognosis