Lymphoplasmacytic Lymphoma with IgA Gammopathy, Case Report and Review of Literature.

The Second International Workshop on Waldenstrom’s macroglobulinemia Consensus Panel has recommended diagnostic criteria that have been modified by the Mayo Clinic and large cohort studies ^1–5. Essential to the criteria: IgM monoclonal gammopathy regardless of the size of the M protein, 10% or greater bone marrow infiltration by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation with an intertrabecular pattern, and typical immunophenotype. We present a 72 year old WM who is free of any past medical history, presented with worsening ataxic gait for four months, mild headache, and frequent urination at night. No history of fever, weight loss, bone pain, numbness, muscle weakness, or back pain. On examination, patient had ataxic gait, positive Romberg test, otherwise normal exam, patient had no palpable lymph nodes, spleen or liver. Ophthalmic exam was normal. His Haemoglobin was 14.7 g/dl, white cell count was 9.9 × 109/l, no shift in neutrophils, no abnormal cells were seen, MCV was 92.4 fL, and no rouleaux formation. 24 Hour total urine protein is 66 mg/24 hours, no free monoclonal light chains (BJP). BUN, creatinine and electrolytes were normal, serum calcium 9.8 mg/dl, albumin 3.8 g/dl, and globulin 3.6 g/dl. After an extensive neurological work up including LP, CSF viral, bacterial, and fungal cultures and radiological studies that were all negative a thinking started about paraneoplastic syndrome. Serum protein electrophoresis was obtained and showed monoclonal paraproteinemia of 0.24 g/dL, immunofixation sowed IgA of 1110 mg/dl, 953 mg/dl, and 1190 mg/dl on three different occasions. IgM, and IgG were within normal limits. Anti Hu,”ANNA-1” and anti Ri “ANNA-2” were negative. Viscosity was 1.6 centipoises. Absence of anemia, bone lytic lesions, renal failure and normal serum calcium levels made Multiple Myeloma “MM” diagnosis less likely. Bone Marrow biopsy “BMB” was done and showed typical diffuse proliferation of small lymphocytes, plasmacytoid lymphocytes, and plasma cells which is the morphologic features of lymphoplasmacytic lymphoma “LPL”. The B cells expressed CD19, CD20, and CD138 without expression of CD5, CD10, CD11c, CD23, or CD25. Immunostains on the clot sections for IGA, kappa, lambda, CD20 and CD138 revealed up to 20 percent B lymphocytes with cytoplasmic and membrane kappa and IgA expression consistent with LPL. The challenge became how to explain such a combination between IgA gamopathy and LPL which classically has IgM macroglobulinemia. The patient was given a regimen of six cycles of cyclophosphamide, prednisone and rituxan, a follow up of six months showed dramatic improvement of his neurological symptoms, a second BMB was done after the chemotherapy course and showed no more evidence of LPL. Few case reports of LPL has been described and showed that monoclonal gammopathy isn’t restricted to the IgM class, it could be IgA ^6–13, IgG ^{8, 10, 12}, or non at all ¹², all these cases were not given the diagnosis of Waldenstrom’s macroglobulinemia for the abscnce of IgM gammopathy.