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How to treat Waldenstrom's macroglobulinemia?

Q: My father is diagnosed with Waldenstrom's macroglobulinemia and was suffering from low haemoglobin levels due to it. He was treated with Rituximab (Mabthera) in 2004 followed by injections of Erythropoetin (Neorecormon) on regular basis. As a result his haemoglobin level stabilized at 11. Since last 2 months his haemoglobin level has again started decreasing, around 7, for which he received blood transfusion of one unit recently. Now the doctor has advised treatment with Rituximab in combination with flubardin. Could you please comment on the line of treatment.

A:Waldenstrom's macroglobulinemia (WM) is a rare, indolent, malignant disorder of a type of cells called B-lymphocytes that are responsible for producing antibodies. It is quite similar to multiple myeloma except that involvement of bone and kidneys is unusual in this disease. Patients are treated when clinically symptomatic. As yet, it is still not known which is the best modality of treatment and the drugs used include steroids, alkylating agents, biological agents, purine analogues, monoclonal antibody and combination therapy. The decision regarding when to treat and how to treat is governed by factors like patient’s age and clinical status. The disease at present cannot be cured but can be controlled over a prolonged period of time. As the malignant cells usually express an antigen called CD20, anti-CD20 antibody (Rituximab) is used to treat patients. The drug singly produces a response rate of around 50% but does not produce a complete response and needs to be combined with other drugs. The patients most likely to benefit from it have not been clearly defined. Patient response is usually slow and it takes around 12 weeks for the drug to be effective. Its use may result in a sudden increase in IgM levels (called 'flare phenomenon') that may require plasmapheresis. The effectiveness of therapy is judged by repeat protein electrophoresis to estimate serum IgM levels and the clinical state of the patient. The treating physician is best placed to discuss all these issues with you and guide you.

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