Hepatosplenic T-cell lymphoma: clinicopathologic, immunophenotypic, and molecular characterization of17 Chinese cases

Pathology of the liver sinusoids

“…The first is a diffuse linear infiltrate of small‐ to medium‐sized lymphoid cells with little or no sinusoidal dilatation and no evidence of hepatocellular injury. This is seen most typically in NK cell leukaemia, hepatosplenic T cell lymphoma, usually of the gamma–delta subtype, but may also occur in hairy cell leukaemia. The bland morphology of cells infiltrating sinusoids and lack of significant liver cell injury may cause difficulty in recognizing the sinusoidal infiltrates as being neoplastic in nature, as a similar pattern of sinusoidal infiltration can also occur in reactive conditions such as tropical splenomegaly syndrome, Felty syndrome and some types of viral hepatitis (e.g.…”

“…In the case of hairy cell leukaemia, immunohistochemistry may help to demonstrate a pure B cell population – contrasting with reactive sinusoidal infiltrates, which are composed predominantly of T lymphocytes. Immunohistochemistry is less helpful in the diagnosis of hepatosplenic T cell lymphoma, and in cases where there is diagnostic uncertainty DNA extraction and PCR studies can be used to demonstrate T cell receptor gene arrangement as a marker of clonal T cell expansion . NK cell leukaemia, another form of malignancy that can involve the liver by diffuse infiltration of the sinusoids, is thought to arise from the large granular lymphocytes of the liver.…”

“…Immunohistochemistry is less helpful in the diagnosis of hepatosplenic T cell lymphoma, and in cases where there is diagnostic uncertainty DNA extraction and PCR studies can be used to demonstrate T cell receptor gene arrangement as a marker of clonal T cell expansion. 56,61 NK cell leukaemia, another form of malignancy that can involve the liver by diffuse infiltration of the sinusoids, is thought to arise from the large granular lymphocytes of the liver. This is an unusual, highly aggressive tumour that has a predilection for young adults, but no gender preference.…”

Hepatosplenic T‐Cell Lymphoma in an Immunocompetent Male with Central Nervous System Invasion: A Rare Clinical Entity

“…Massive splenomegaly is usually the most consistent physical finding (2,3,(9)(10)(11)(12), hepatomegaly was seen in 40%-88% of all the patients, lymphadenopathy is less common, perhaps less than 25% (2,(9)(10)(11)(12). Massive splenomegaly is usually the most consistent physical finding (2,3,(9)(10)(11)(12), hepatomegaly was seen in 40%-88% of all the patients, lymphadenopathy is less common, perhaps less than 25% (2,(9)(10)(11)(12).…”

“…As a rare and aggressive extranodal T-cell lymphoma, patients with HSTCL usually present with B-symptoms: fever, night sweats and weight loss. Massive splenomegaly is usually the most consistent physical finding (2,3,(9)(10)(11)(12), hepatomegaly was seen in 40%-88% of all the patients, lymphadenopathy is less common, perhaps less than 25% (2,(9)(10)(11)(12). The most frequent sites of involvement include spleen, liver and BM, PB involvement is usually late in the course of the disease, involvement of the CNS is extremely rare, but has been reported (13,14).…”