Hepatosplenic lymphoma, more specifically known as hepatosplenic T cell lymphoma or HSTCL has been in the news recently due to the US FDA's announced in the spring of 2011 that its investigation into a certain class of drug, known as TNF blockers, were believed to be causing a small but significant number of patients (mostly children) taking these drugs to develop hepatosplenic lymphoma.
In the past, and perhaps in some hidden corners of the lymphoma world, hepatosplenic lymphoma continues to be known by a few other names:
- -- Hepatosplenic Gamma delta T-cell lymphoma
- -- Diffuse small cleaved cell lymphoma, unclassified
- -- Pleomorphic small cell lymphoma, medium size cell, HTLV-1 negative
However, currently the accepted term is hepatosplenic T-cell lymphoma.
What is Hepatosplenic Lymphoma?
Hepatosplenic lymphoma is a non-Hodgkin's lymphoma (NHL) that affects the T cells or T lymphocytes in the lymphatic system. Only about 15 percent of all NHLs are T cell lymphomas, making this broad category of cancers already rather rare.
Hepatosplenic lymphoma was first presented as "a distinct clinicopathologic entity" in 1996 , meaning it was first discovered and identified as being different than any known lymphoma at the time. This means however that we don't really know very much about it. What we do know is not encouraging.
What are the Symptoms of Hepatosplenic Lymphoma?
In patients suffering from hepatosplenic T cell lymphoma, the name of the disease tells you a lot about what's going on in their bodies: namely, that both their liver (hepato-) and their spleen (splenic-) are enlarged.
Unlike many other lymphomas, it is not necessarily common for the patient to have swollen or enlarged lymph nodes.
A bone marrow biopsy taken for HSTCL will likely be positive, as bone marrow involvement is common. Blood tests meanwhile may show some evidence of cytopenia, or low blood cell counts (chiefly thrombocytopenia and anemia).
Is Hepatosplenic Lymphoma Treatable or Curable
The treatment of choice for patients diagnosed with this disease tends to be combination chemotherapy, in which one of the drugs is most likely going to be from the anthracycline class (such as doxorubicin). However, like many other T-cell lymphomas, treatment is not considered widely successful, and relapses are extremely common.
Unfortunately, this is an aggressive and refractory disease, difficult to treat, and the median survival time from diagnosis is said to be around three years.
While some patients may be candidates for an autologous or allogenic stem cell transplantation, at this time it appears that the only potential cure for HSTCL could be the allogenic transplantation.
Sources
- TNF Blockers and Cancer
- Cuneo A et al. Hepatosplenic T-cell lymphoma (HSTCL) . Atlas Genet Cytogenet Oncol Haematol. July 2008.
- Cooke CB et al. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin Blood. 1996 Dec 1;88(11):4265-74
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