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Hemato-Oncology- 36-Non Hodgkin’s Lymphoma-special subtypes


Question: Dr. Chiragbhai, thank you for explaining in last part about Non Hodgkin’s Lymphoma treatment options broadly, and simplifying it for our readers. There are three major categories, with low grade NHL having low grade treatment but low cure rates and long disease course, requiring intermittent treatment. On the other hand, high grade lymphomas have aggressive growth, require aggressive treatment, but have very high cure rates. Now can you tell us about the common subtypes of low grade NHL, having different clinical course and treatment options?
Answer: Yes, there are many interesting subtypes of low grade NHL. We discussed one variety last time i.e. MALT lymphoma. This lymphoma is closely linked to infection. It is thought to be due to chronic infection with persistent immune activation. For example, Helicobacter pylori infection in stomach is associated with MALT lymphoma. In early cases, eradication of this infection with only a two week course of antibiotics can cure this lymphoma in about 70% cases.
Que: This is amazing!! A cancer treated with only antibiotics…
Ans: Yes, this is true. Another interesting variety is splenic marginal zone lymphoma. These patients present with splenomegaly, and mostly no other findings. Diagnosis is frequently made after splenectomy. Some patients are diagnosed by bone marrow biopsy or circulating villous lymphocytes. This is the only lymphoma where a surgical treatment i.e. splenectomy is curative in majority of cases, and is generally the preferred first line treatment. More recently, patients have also been treated with Rituximab, without any chemotherapy or surgery, with equally good results.
Que: Another amazing variety!! First there was only antibiotics, then only surgery, to treat a lymphoma, without any chemotherapy. Very interesting.
Ans: One more such variety is Waldenstrom’s macroglobulinemia. Lymphoma cells in these patients produce high amounts of IgM, resulting in hyperviscosity of blood. This leads to impaired circulation in brain, eyes, lungs, with related symptoms. Such patients need urgent plasmapheresis to lower the level of IgM, followed by chemotherapy. Rituximab may sometime transiently increase IgM levels after first dose.
Hairy Cell Leukemia is also a rare type of lymphoma, which presents with splenomegaly and pancytopenia. Diagnosis can be missed at bone marrow biopsy if pathologist is not aware of this condition. This lymphoma interestingly is cured by only one dose of chemotherapy (most lymphomas normally need average 6 cycles). Drug is also unusual i.e. cladribine, which is not used in any other lymphoma as first option. Method of administration is also unusual i.e. over 7 days of infusion, but only one course.
Some other lymphomas, mainly of T cell origin, present with only skin involvement, at least in early stages. For example, mycosis fungoides. These may not be diagnosed till long and treated as some resistant skin disease. Once diagnosed, interestingly, disease limited to skin is treated with only topical therapy, without any systemic chemotherapy.
LGL – large granular lymphocytic leukemia – is another very low grade lymphoma, which presents with cytopenia such as low Hb, or wbc or both. They have no enlarged nodes or spleen. Diagnosis is made by calculating increased number of LGLs (large granular lymphocytes – normally a very small fraction of lymphocytes) in blood, under microscope. More recently, their number can be measured by flow cytometry as well. They are treated with low doses of methotrexate and low dose prednisone in most cases.

Dr. Chirag A. Shah; M.D. Oncology/Hematology (USA), 079 26754001. Diplomate American Board of Oncology and Hematology. Ahmedabad.
Shyam Hem-Onc Clinic. 402 Galaxy, Near Shivranjani, Opp Jhansi ki Rani BRTS, Ahmedabad.