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Splenic marginal zone lymphoma



 

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.

Diagnosis and Prognosis

SMZL usually presents with massive splenomegaly, marrow and blood involvement. Patients often have anaemia and lymphocytosis. Overall the prognosis is very good for long term survival, with the median being approximately 13 years. The two most important risk factors are anaemia and lymphocytosis.

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. (1)

Although SMZL is usually CD5 negative some patients are CD5 positive, and in fact it can be quite variable in its presentation. (2) (3)

Prognostic index

There is no currently accepted International Prognostic Index for SMZL like there is for some other types of non-Hodgkin's lymphoma.  Nevertheless there are recognized risk factors which may form the basis for an IPI in the future. There are three risk factors:

  • Haemoglobin level < 12 g/dL
  • LDH greater than normal
  • Albumin level < 3.5 g/dL

0 risk factors = Low risk, 88% 5 year cause specific survival (CSS)

1 risk factor = intermediate risk, 73% 5 year CSS

2 or more risk factors = high risk, 50% 5 year CSS

Splenic marginal zone lymphoma: a prognostic model for clinical use

Another contender for a valid international prognostic index uses Haemoglobin, platelet count, LDH and extrahilar lymphadenopathy to risk stratify patients. That study was published in August 2012. (4)

Treatments

Historically splenectomy has been the preferred treatment of choice over chemotherapy. (5)Rituximab has changed this thinking.  Due to its high efficacy and low toxicity it would seem to make the removal of the spleen unnecessary as an upfront treatment. Most people would probably rather keep their spleen if possible.  Some research even suggests that Rituximab alone is superior to splenectomy alone and equal to Rituximab and chemotherapy. Rituximab and splenectomy obtains the highest complete remission rate but may not improve overall survival. (6)

Since losing the spleen is not very appealing to most people Rituxan monotherapy is likely to be the most beneficial option as a first line therapy. (7) (8) Splenectomy can always be used later if needed.

When chemotherapy stronger than just Rituxan monotherapy is required the purine analogues such as Fludarabine have been used with some success. Although Fludarabine is effective, it also has significant haematologic toxicity and is therefore often not recommended. (9) (10) (11) (12)

Other purine analogues such as Pentostatin (2-deoxycoformycin), 2-CDA (2-chlorodeoxyadenosine)  appear to be showing promise. Though they have not taken the place of other treatments they may have a place in future treatments.

Splenic marginal zone lymphoma: from genetics to management


Other information

Splenic marginal zone lymphoma a review - from the journal Blood

A focused search about Splenic Marginal Zone lymphoma from PubMed

Splenic Marginal Zone Lymphoma: Current Knowledge and Future Directions

Marginal Zone Lymphomas: Management of Nodal, Splenic, and MALT NHL

How I diagnose and treat splenic lymphomas: Emilio Iannitto

References

  1. Intrasinusoidal bone marrow involvement by splenic lymphoma with villous lymphocytes: a helpful immunohistologic feature

  2. Splenic marginal zone lymphoma: hydra with many heads?

  3. CD5 expression identifies a subset of splenic marginal zone lymphomas with higher lymphocytosis: a clinico-pathological, cytogenetic and molecular study of 24 cases

  4. Risk stratification for Splenic Marginal Zone Lymphoma based on haemoglobin concentration, platelet count, high lactate dehydrogenase level and extrahilar lymphadenopathy: development and validation on 593 cases

  5. Prognostic features of splenic lymphoma with villous lymphocytes:

  6. Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma

  7. Treatment of Splenic Marginal Zone Lymphoma with Rituximab Monotherapy in 59 Patients

  8. Splenic Lymphomas: Is There Still a Role for Splenectomy?

  9. A Phase 2 Study of Concurrent Fludarabine and Rituximab for the Treatment of Marginal Zone Lymphomas

  10. High incidence of non-neutropenic infections induced by rituximab plus fludarabine and associated with hypogammaglobulinemia: a frequently unrecognized and easily treatable complication

  11. Cyclophosphamide and fludarabine (CF) in advanced indolent lymphoma: Results from the ECOG/CALGB intergroup E1496 trial

  12. Fludarabine as a risk factor for poor stem cell harvest, treatment-related MDS and AML in follicular lymphoma patients after autologous hematopoietic cell transplantation


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