Hepatosplenic T Cell Lymphoma
Definition
- Lymphoma of cytotoxic T cell phenotype with a marked predilection for sinusoidal involvement of liver, spleen and bone marrow
Alternate/Historical Names
- Hepatosplenic gamma delta T cell lymphoma
Diagnostic Criteria
- Infiltrate confined to sinusoids of liver, spleen and bone marrow
- May be rather inconspicuous
- Portal spaces and white pulp generally spared
- Peripheral blood may be involved late in disease
- Lymph nodes only rarely involved
- Uniform medium sized cells in most cases
- Clumped chromatin and inconspicuous nucleoli
- Occasional cases show more pleomorphism and mixture with large cells
- Progression to larger cells with blastic features has been described in some cases
- All cases T lineage with cytotoxic protein expression
- Most cases have gamma delta T cell receptors
- A subset of cases may have alpha beta T cell receptors
- Other types of lymphoma may express gamma delta T cell receptors
Dita Gratzinger MD PhD
Yasodha Natkunam MD PhD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Initial posting : September 1, 2007
