Non Hodgkin's Lymphoma
Cyberfamily
Splenic Marginal Zone Lymphoma
Splenic marginal zone lymphoma is generally an indolent lymphoma that involves the spleen, bone marrow and other organs. Splenomegaly is usually observed and when localized to the spleen then removal of the spleen often results in complete remission.
SMZL is also known as Splenic Lymphoma with Villous Lymphocytes. (villous means having the appearance of being covered with fine long hairs) It resembles hairy cell leukaemia and is considered to be the a leukaemic form of lymphoma. It is extremely rare and only accounts for about 1% of all lymphomas.
Previously a definitive diagnosis
could only be made after removal of the spleen, but it has now been
established that a conclusive diagnosis can be made from a bone
marrow biopsy due to the peculiar intrasinusoidal BM involvement
Intrasinusoidal bone marrow involvement by splenic lymphoma with
villous lymphocytes: a helpful immunohistologic feature
Like other indolent lymphomas SMZL has
a good prognosis for long term survival. Median survival varies
according to risk factors but is approximately 13 years overall. The
two primary risk factors are anaemia (low haemoglobin count), and
lymphcytosis (high lymphocyte count).
Prognostic features of splenic lymphoma with villous lymphocytes: a
report on 129 patients
Optimal treatment has not yet been
established but studies indicate that splenectomy may prove more
beneficial than chemotherapy in terms of survival.
Prognostic features of splenic lymphoma with villous lymphocytes:
When chemotherapy is necessary Rituxan monotherapy is effective in many cases. When something stronger is required the purine analogues such as Pentostatin (2-deoxycoformycin), 2-CDA (2-chlorodeoxyadenosine) and Fludarabine appear to be more effective than to the alkylating agents such as Cyclophosphamide, although combination chemotherapy is also common.
For an excellent overview of SMZL lymphoma the following review from Blood Journal is an excellent resource.
Splenic Marginal Zone Lymphoma from Blood Journal
Further information about SMZL can be found at Pubmed. Just click the link below.
A focused search about Splenic Marginal Zone lymphoma from PubMed
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