Splenic Reirradiation In Waldenstr(o|··)m’s Macroglobulinemia(Case Report And Review Of The Literature)

ObjectiveTo discuss the effect of three-dimensional conformal radiotherapy (3D-CRT) for persistent severe pancytopenia and splenomegaly in Waldenstrom’s macroglobulinemia(WM), and to explore its etiology, pathogenesis, diagnosis, treatment and prognosis by literature review.MethodWe analyzed a case of fatigue, high IgM concentration, lots of lymphoplasmacytoid cells in the bone marrow, which was finally diagnosed as WM. We assessed the effect of splenic irradiation for persistent profound pancytopenia and splenomegaly, and reviewed the related literature to discuss its etiology, pathogenesis, diagnosis, treatment and prognosis.ResultsA69-year-old Asian female was proved to be WM by fatigue, anemia, massive splenomegaly, high IgM concentration, paratrabecular deposits of lymphoplasmacytoid cells on the trephine biopsy. She was treated with chlorambucil, thalidomide, and then fludarabine plus cyclophosphamide therapy. But the patient developed persistent grade3anemia plus grade3leukopenia. Although she received blood transfusion, Erythropoietin (EPO) and human granulocyte colony stimulating factors (hG-CSF) repeatedly, anemia and leukopenia relapsed soon. Then the patient was referred for splenic irradiation, with24Gy in12treatment fractions. She tolerated the treatment well, with a37%reduction of IgM, an impalpable spleen, and improved hematological laboratory tests for4months. But our patient refused to have a splenectomy, she was then treated with splenic reirradiation for recurrent hypersplenism. She tolerated reirradiation up to24Gy well and with a55%reduction of IgM after the treatment. She showed no evidence of progression almost two years after reirradiation. The etiology and pathogenesis of WM are unknown clearly, clinical manifestations are multiple, corresponding treatments can be chosen and prognosis is unfavorable.ConclusionOur experience demonstrates that splenic irradiation is an effective treatment to control the progression of WM. In cases of patients who refuse to have a splenectomy for recurrent splenomegaly, splenic reirradiation may be another choice.