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Clinicopathological features of hepatosplenic T cell lymphoma: a single centre experience from India.
- Source :
-
Leukemia & lymphoma [Leuk Lymphoma] 2012 Apr; Vol. 53 (4), pp. 609-15. Date of Electronic Publication: 2011 Dec 05. - Publication Year :
- 2012
-
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.
- Subjects :
- Adolescent
Adult
Antigens, CD metabolism
Chromosome Aberrations
Chromosomes, Human, Pair 7 genetics
Fatal Outcome
Female
Flow Cytometry
Humans
Immunophenotyping
In Situ Hybridization, Fluorescence
India
Liver Neoplasms genetics
Liver Neoplasms metabolism
Lymphoma, T-Cell genetics
Lymphoma, T-Cell metabolism
Male
Middle Aged
Splenic Neoplasms genetics
Splenic Neoplasms metabolism
Young Adult
Liver Neoplasms pathology
Lymphoma, T-Cell pathology
Splenic Neoplasms pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1029-2403
- Volume :
- 53
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Leukemia & lymphoma
- Publication Type :
- Academic Journal
- Accession number :
- 21916832
- Full Text :
- https://doi.org/10.3109/10428194.2011.622421