PLASMA-CELL DYSCRASIA WITH MARROW FIBROSIS - A REVERSIBLE SYNDROME MIMICKING AGNOGENIC MYELOID METAPLASIA

Background. The interrelation between plasma cell dyscrasia and myelof ibrosis or agnogenic myeloid metaplasia (AMM) is unclear. The existenc e of two distinct syndromes has been proposed: (1) plasma cell dyscras ia associated with simple marrow fibrosis caused by the secretion of l ymphokines and (2) myeloma coexisting with AMM representing two distin ct clonal diseases. Methods. The authors report the case of a 68 year- old man seen initially with severe anemia, massive splenomegaly, a leu koerythroblastic blood morphology, and myelofibrosis coexisting with m assive bone marrow infiltration with IgA lambda-producing plasmacytoid cells. Results. Cyclic therapy with vincristine, carmustine, cyclopho sphamide, melphalan, and prednisone resulted in clinical remission of the myeloma lasting for 2 years and complete resolution of all the cli nical features resembling AMM. Conclusions. The authors' observations and the report of two other patients in whom remission of AMM has been observed after myeloma treatment underline the broad spectrum of seco ndary abnormalities ranging from moderate bone marrow fibrosis to the full clinical expression of a syndrome closely mimicking AMM. These se condary abnormalities are potentially reversible even in the presence of advanced bone marrow fibrosis and massive splenomegaly.