HepatospenicT-cell Lymphoma (HSTL) is rare & an aggressive type of extranodal lymphoma,
the disease represent 1-2% of all peripheral T-cell lymphoma. Its incidence
might be underestimated, because the disease may mimic other conditions &
the diagnosis is sometimes difficult to be established. It has been seen in
patients receiving long-term immunosuppressive therapy, following AML or EBV
positive lymphoproliferative disorders or during pregnancy. Several cases were
reported recently in patients with crohns disease treated with azathioprine
& anti-tumor necrosis factor agent infliximab. We present here peculiar
scenario of young male adult known case of sickle cell disease patient whose
been diagnosed with gammadelta-T-cell lymphoma, after quite long history of
unexplained organomegaly & fever.
HSTLis rare, and the world health organization prefers the term hepatosplenic
T-cell lymphoma in the current WHO classification of tumors of haematopoietic
& lymphoid tissues. The disease occurs mainly in young adults presenting
with hepatomegaly, splenomegaly but without lymphadenopathy. Most patients have
B-symptoms & cytopenias. In fact bone marrow involvement is nearly always
present. Neoplastic cells infiltrate & expand the bone marrow sinuses, a
feature that is highly characteristic & thus a useful diagnostic criterion,
irrespective of their gammadelta or alphabeta phenotype, HSTL shows a clonal
rearrangement of TCRG gene. Recently gene expression profiling studies have
shown that the HSTL signature was distinct & characterized by over
expression of gene encoding KIRs molecules. In conventional cytogenetic &
FISH studies, 50-80% were characterized by the presence of an isochromsome
7q this is occasionally the sole karyotypic abnormality suggesting
that it plays a role in pathogenesis, in addition to trisomy 8 & loss of
chromosome Y. The major differential diagnoses include aggressive NK-cell
lymphoma/leukemia, T-Cell large granular lymphocytic leukemia & other
gammadelta T-cell lymphomas.
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