splenic marginal zone lymphoma | Lymphoma of the splenic marginal zone

Lymphoma of the splenic marginal zone






The lymphoma of the splenic marginal Zone (LZMS) is a slow (indolent) B-lymphocyte non-Hodgkin's lymphoma (NHL). It usually appears in the spleen, bone marrow and blood. It sometimes affects the lymph nodes of the abdomen, but in most cases the LZMS does not reach the lymph nodes.

LZMS is associated with viral infections, in particular hepatitis C virus (HCV) and herpes virus related to Kaposi sarcoma (HVSK). It usually affects seniors, mostly older men. LZMS rarely appears before 50 years. The average age of diagnosis varies between 65 and 70 years. In most people, the disease is advanced stage (stage IV) when diagnosed.

The LZMS can turn into a rapidly evolving (aggressive) NHL type, but it is not very common.

Symptoms
People with a LZMS often have:

A spleen bigger than normal
A small number of red blood cells (anemia);
A small number of platelets (thrombocytopenia);
A liver larger than normal (in some cases).
Treatments
The treatment options for the LZMS are as follows.

Vigilant Observation
Vigilant observation, also known as active surveillance, may be an option since LZMS develops slowly and may not need to be treated immediately. The care team carefully monitors the person with a LZMS and begins treatment when symptoms appear or if there are signs that the disease is evolving more quickly.

Surgery
Surgery to remove the spleen (splenectomy) can be proposed as a treatment for LZMS. It increases the number of red blood cells or platelets. It is also used to relieve discomfort when a spleen larger than normal exerts pressure on other organs.

Radiotherapy
When splenectomy is not an option, an external radiation therapy can be administered to the spleen to treat the LZMS.

Chemotherapy
LZMS does not respond as well to chemotherapy as other types of slow-moving NHL (indolent), but it can still be offered to some people.

The following combinations of chemotherapeutic agents may be used to treat LZMS:

CHOP – Cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin), and prednisone
R-Chop – Chop with rituximab (Rituxan)
CVP – cyclophosphamide, vincristine and prednisone
R-CVP – CVP with rituximab
FND – Fludarabine (Fludara), mitoxantrone (Novantrone) and dexamethasone (Decadron, Dexasone)
BR – Bendamustine (Treanda) and rituximab
Targeted treatment
Targeted treatment uses drugs to target specific molecules (such as proteins) located on the surface of cancer cells. These molecules contribute to the sending of signals that tell cells to grow or divide. By targeting these molecules, medications interrupt the growth and spread of cancer cells while limiting damage to normal cells.

Rituximab is a targeted drug that is used to treat LZMS. It can be administered alone or associated with chemotherapy.

Biological therapy
Biological therapy uses natural or artificial substances to modify the behaviour of the cells. It is sometimes used to treat the NHL.

Some people with a LZMS will also have HCV infection. It is then possible that they are given alpha interferon (intron A, Wellferon), which is a type of biological medicine. It can be administered alone or associated with a drug called ribavirin, which is used to treat viral infections such as HCV.