title: Clinicopathological features of hepatosplenic T cell lymphoma: a single centre experience from India.
abstract: In a first series from India, we report 9 cases of hepatosplenic T cell lymphoma (HSTCL) seen in 23 months accounting for 4.2% of all mature T-non-Hodgkin lymphomas (NHLs) in our institution. All patients presented with organomegaly, cytopenias and had evidence of bone marrow involvement. The tumor cells had a blastic (55%) morphology with predominantly intrasinusoidal (33.3%) or intrasinusoidal with an additional interstitial component (33.3%). On flow cytometry, the classical phenotype (CD3+, CD7+, CD4-, CD8-, CD5-, CD56+/-) was seen only in 4 patients. Unusual variations included CD45 (overexpression), CD7 (dim expression), CD3 (overexpression, heterogeneous and dim), CD2 (overexpression), CD5 (heterogeneous), CD8 (heterogeneous or dim or overexpression) and aberrant expression of CD19. Fluoresvent in situ hybridisation (FISH) and karyotyping was abnormal in 5 out of 7 patients evaluated. All of the 5 cases showed abnormalities in chromosome 7 (ring chromosome or isochromosome 7q). Five patients died of disease and related complications in a span of 1-3 months after diagnosis whereas 4 were alive at their last follow up out of which 2 had documented a relapse. In our series, HSTCL was characterized by typical clinical and variable immunophenotypic features and a dismal clinical outcome.
authors.affiliation: Department of Haematology, Christian Medical College, Vellore, India.
authors.forename: Nikhil
authors.initials: N
authors.lastname: Patkar
authors.forename: Sheila
authors.initials: S
authors.lastname: Nair
authors.forename: Ansu Abu
authors.initials: AA
authors.lastname: Alex
authors.forename: Mayur
authors.initials: M
authors.lastname: Parihar
authors.forename: Marie Therese
authors.initials: MT
authors.lastname: Manipadam
authors.forename: Neeraj
authors.initials: N
authors.lastname: Arora
authors.forename: Rayaz
authors.initials: R
authors.lastname: Ahmed
authors.forename: Aby
authors.initials: A
authors.lastname: Abraham
authors.forename: Biju
authors.initials: B
authors.lastname: George
authors.forename: Auro
authors.initials: A
authors.lastname: Viswabandya
authors.forename: Vivi
authors.initials: V
authors.lastname: Srivastava
authors.forename: Alok
authors.initials: A
authors.lastname: Srivastava
authors.forename: Vikram
authors.initials: V
authors.lastname: Mathews
mesh_terms: D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D002869:Chromosome Aberrations; D002897:Chromosomes, Human, Pair 7 / Q000235:genetics; D017809:Fatal Outcome; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D007194:India; D008113:Liver Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D013160:Splenic Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D055815:Young Adult
chemical_list: D015703:Antigens, CD