Hepatosplenic gamma-delta T-cell lymphoma in a female patient after delivery.

ABSTRACT: Hepatosplenic ?? T-cell lymphoma (HSTCL) is a very rare peripheral T-cell lymphoma characterized by extranodal infiltration of mature malignant post-thymic T-lymphocytes into sinusoids of the liver and spleen without lymphadenopathy and significant cytopenias. The aetiology of the disease is unknown. We describe the case of a female patient in whom HSTCL developed after delivery and who was previously without disease. Flow cytometry and liver puncture are essential for diagnosing HSTCL, especially in patients with unexplained pancytopenia and hepatosplenomegaly. Since phenotypic results can easily be misinterpreted as non-malignant, the examiner should have enough experience to recognize clonal changes of T-lymphocytes. Namely, in contrast to B-lymphocytes, T-lymphocytes do not have an efficient indicator of clonality and are recognized by flow cytometry based only on aberrant expression of commonly present antigens of T-cell and NK-cell subsets. At present, there is no known cure for HSTCL with a maximum survival up to 2 years.

SUBMITTER: Glaser M 

PROVIDER: S-EPMC3343453 | biostudies-other | 2012 Jan

REPOSITORIES: biostudies-other