Bone marrow transplantation for acute myelosclerosis

✍ Scribed by J. Walter Smith; Howard M. Shulman; E. Donnall Thomas; Alexander Fefer; C. Dean Buckner

• Publisher: John Wiley and Sons
• Year: 1981
• Tongue: English
• Weight: 671 KB
• Volume: 48
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/1097-0142(19811115)48:10<2198::aid-cncr2820481014>3.0.co;2-m

✦ Synopsis

Four patients with acute myelosclerosis were treated with chemoradiotherapy and bone marrow transplantation. Three of the four had prompt engraftment with greater than 0.10 X lo9 granulocytes/liter three weeks after transplantation. One patient continues in complete remission one year after transplantation. One patient experienced a partial remission and two patients died early in the post-transplantation period. Marrow fibrosis disappeared in three of the four patients. The patient with residual marrow fibrosis died 22 days after transplantation. It is apparent that marrow fibrosis is reversible. In view of the absence of other effective therapy, intensive therapy with marrow transplantation should be considered as primary therapy for patients with acute myelosclerosis who have a suitable marrow donor.

Cancer 48:2198-2203, 1981. CUTE (MALIGNANT) MYELOSCLEROSIS is a myelo-A proliferative disorder with some features of agnogenic myeloid metaplasia and some of acute leukemia with fibrosis. The original description and diagnostic criteria proposed by Lewis and Szur16 have recently been confirmed by Bearman et al. Acute myelosclerosis is characterized by pancytopenia, a peripheral blood smear with mild or absent anisocytosis, poikilocytosis, and minimal or no splenomegaly. Usually a small number of blast cells are present in the peripheral blood. The bone marrow is sufficiently fibrotic to prevent aspiration, and the biopsy specimen shows hypercellularity. There is proliferation of immature cells of all three marrow cell lines with a prominence of megakaryocytes that are sometimes atypical. Acute myelosclerosis is rapidly progressive and fatal within a year of diagnosis. Treatment with corticosteroids, androgens, single-agent