A 74-year-old febrile male with myelodysplastic syndrome (MDS) associated with Ig (λ) monoclonal gammopathy was initially treated with melphalan and prednisone for 27 days without apparent benefit. The patient was then admitted to our hospital on June 26, 1984. Hematological study revealed severe pancytopenia with hypogranular and Pelger-Huët-like abnormalities of neutrophils. Bone marrow aspirate was normocellular and showed marked dysplasia of trilineage blood cells. Plasma cells accounted for 12% of the myelogram but neither bone destruction nor abnormal renal function could be found. Bone marrow chromosome study revealed that the karyotype was 43, X, -Y, -2, -5, -7, -12, -16, -17, -20, -22, 15q+, +6 mar in all 20 banded cells analyzed.
He was then treated with metenolon enantate and prednisone. After 29 days, peripheral blood film showed a sudden increase in number of mature neutrophils with Pelger-Hüet-like abnormality and hypogranular appearance. He died of respiratory failure on July 28, 1984 with evidence of extensive diffuse shadows on his chest X-ray examination.
At autopsy the bone marrow was hypercellular with a number of neutrophils and sparse plasma cells. A surprising evidence was that diffuse infiltrates of plasma cells were seen in multiple organs such as lungs, liver, spleen, kidneys, pancreas and gastrointestinal canals. Furthermore, proliferation of these plasma cells seemed polyclonal rather than monoclonal because, using the PAP method for cytoplasmic (c-) Ig staining, c-κ positive cells and c-λ positive ones were almost equal in number on the liver specimen.
It is of interest that such polyclonal prolyferation of plasma cells as observed in this case may be ascribed to the idea of genetically unstable myeloid-lymphoid stem cells in MDS.
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