Improvement of Myelodysplasia and Partial Correction of panhypogammaglobulinemia after IV IgG Infusions and Splenectomy

A man born in 1951 underwent a bone marrow examination, on May 10, 1991, for investigation of his pancytopenia. The bone marrow slides were sent to me for interpretation. I made the diagnosis of myelodysplasia (refractory anemia without excess blast). The patient was subsequently referred to a hematologist in another hospital for further investigation and management. A repeat bone marrow aspiration and biopsy on May 30, 1991 again demonstrated myelodysplasia. Bone marrow biopsy, flow cytometry and cytogenetic studies were normal. His Hgb was 124 G/L, WBC 2.2 and platelet (Plt) 151 × 10e9/L. CT scan of the chest and abdomen showed mediastinal and retroperitoneal lymphadenopathy and marked splenomegaly suggestive of lymphoma. There was no symptoms suggestive of lymphoma. The patient was followed without any therapy. In March 2002 he was referred to an immunologist because of frequent episodes of pneumonias. He was found to have panhypogammaglobulinemia. The immunologist recommended monthly I V immunoglobulin(IgG). Initially the patient refused this treatment; but subsequently he agreed and he was sent to me on November 3, 2003 for consideration of monthly IV IgG infusion. Hgb 125 g/L, WBC 2.5 and Plt 112 ×10e9/L.IgG 2.31, IgA <0.07 and IgM 0.1 g/L, IgD<0.01 G/L and IgE <2 KU/L. Repeat bone marrow aspriration and biopsy in February 2002 was unchanged compared to those 1991 and cytogenetics and immunophenotyping were again normal. He was started on IV IgG 40 G Q 4-weeks. The dose was reduced to 30 G Q 4-weeks on April 30, 2004 and then reduced to 25 G Q 4-weeks on April 8, 2005. On March 12,2004 because the pancytopenia was getting worse and CT scan had shown increasing size of the spleen and nodes, repeat bone marrow examination was performed. The marrow aspirate showed normal morphology and no evidence of myelodysplasia, Bone marrow biopsy showed normocellular marrow with occasional granuloma but no evidence of lymphoma. On June 4,2004, he underwent splenctomy. The spleen, and hilar splenic nodes showed non-caseating granulomas consistent with sarcoidosis, but no evidence of lymphoma. Post splenctomy hematological parameters became normal. After September 30, 2005 no more IV IgG was infused. His IgG and IgM remained persistently above pretreatment level. The result of Immunoglobulin levels and CBC before and after therapy are shown in the Table below. Unfortunately in February 2007 he died suddenly from overwhelming pneumococcal infection, despite the fact that he was given pneumovax prior to splenectomy. Conclusion: Repeated IV IgG infusion in this case, caused partial improvement of IgG and IgM, and was possibly responsible for recovery from myelodysplasia.