Hepatosplenic T-cell lymphoma (HSTCL)

Identity

ICD-Topo	C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho	9716/3 Hepatosplenic T-cell lymphoma
Atlas_Id	2099
Other names	Hepatosplenic Gamma delta T-cell lymphoma
	Diffuse small cleaved cell lymphoma, unclassified (WF classification)
	Pleomorphic small cell lymphoma, medium size cell (HTLV-1 negative) (Kiel classification)

Clinics and Pathology

Phenotype / cell stem origin	This lymphoma entity originates from T-lymphocytes expressing the Gamma Delta subunits of the TCR. Rarely, cases expressing the Alpha Beta subunits were reported.
Epidemiology	Young males are affected predominantly.
Clinics	The disease presents with hepatosplenomegaly, in the absence of lymphadenopathy. Bone marrow involvement and cytopenias are frequently encountered (Cooke et al., 1996).
Pathology	The proliferation consists of medium-sized lymphocytes with a rim of pale cytoplasm. The nuclei show condensed chromatin with inconspicuous nucleoli (Feldman et al., 2006). Typically these cells show an intrasinusoidal pattern of growth sparing the portal triads and the white pulp. Intrasinusoidal bone marrow involvement may occur. The neoplastic lymphocytes are CD3+, CD4- and may express CD8 and CD56. These features, along with negativity for granzyme B and for perforin, indicate a proliferation of Gamma delta resting T lymphocyte.
Treatment	Multiagent chemotherapy, including anthracyclines is the treatment of choice. Autologous or allogeneic transplantation may have a role in selected patients (Cooke et al., 1996).
Prognosis	The patients usually respond to chemotherapy, but relapses occur frequently and median survival is around three years. Allogeneic BMT may cure some patients.

Cytogenetics

Cytogenetics Morphological

Extra-copies of the long arm of chromosome 7q deriving from one or more isochromosome 7q are frequently found in this lymphoma. Trisomy 8 may also occur. Multiple copies of 7q were also documented by FISH. An increased number of 7q signals was found in cases with cytologic features of progression, indicating a tendency of HSTCL to multiply the i(7)(q10) chromosome during evolution. (Wlodarska et al 2002).

Bibliography

Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin.

Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, Abruzzo L, Frantz C, Kaviani M, Jaffe ES.

Blood 1996; 88(11): 4265-4274.

PMID 8943863

Classification and histopathology of the lymphomas.

Feldman A, Pittaluga S, Jaffe ES.

In: Canellos GP, Lister TA, Young BD: The Lymphomas 2nd edition. Saunders Elsevier, Philadelphia, 2006, pp 2-38

Fluorescence in situ hybridization study of chromosome 7 aberrations in hepatosplenic T-cell lymphoma: isochromosome 7q as a common abnormality accumulating in forms with features of cytologic progression.

Wlodarska I, Martin-Garcia N, Achten R, De Wolf-Peeters C, Pauwels P, Tulliez M, de Mascarel A, Briere J, Patey M, Hagemeijer A, Gaulard P.

Genes Chromosomes Cancer. 2002; 33: 243-251.

PMID 11807981

Citation

This paper should be referenced as such :

Cuneo, A ; Cavazzini, F ; Rigolin, GM

Hepatosplenic T-cell lymphoma (HSTCL)

Atlas Genet Cytogenet Oncol Haematol. 2009;13(7):510-511.

Free journal version : [ pdf ]   [ DOI ]

On line version : http://AtlasGeneticsOncology.org/Anomalies/HepatoTlymphoID2099.html

Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes

IDH2

External links

COSMIC	Histo = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMap (UZH-SIB Zurich)	Topo ( C42) Morph ( 9716/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]

REVIEW articles	automatic search in PubMed
Last year articles	automatic search in PubMed
All articles	automatic search in PubMed

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