Abstract
Bone marrow fibrosis is a hallmark of primary and post ET/PV myelofibrosis. To investigated the impact of replacement of the hematopoietic system in myelofibrosis patients by allogeneic stem cell transplantation on bone marrow fibrosis, we studied bone marrow fibrosis on bone marrow samples from 24 patients with myelofibrosis before and after dose-reduced conditioning followed by allogeneic stem cell transplantation from related or unrelated donor. Using the European Consensus on Grading Bone Marrow Fibrosis, before allografting all patients had advanced fibrosis MF-2 (n = 13) or MF-3 (n = 11). After transplantation, a complete (MF-0) or nearly complete (MF-1) regression of bone marrow fibrosis was seen in 59 % at day +100, in 90 % at day +180, and in 100 % at day +360. No correlation between occurrence of acute graft-versus-host disease, and fibrosis regression on day +180 was seen. We conclude that dose-reduced conditioning, followed by allogeneic stem cell transplantation, resulted in a rapid resolution of bone-marrow fibrosis suggesting the bone marrow fibrogenesis is a highly dynamic rather than static process in patients with myelofibrosis.
Highlights
Myelofibrosis with myeloid metaplasia (MMM) is a rare malignant hematological disease of the hematopoietic stem cells with a median age at diagnosis of 60 years
Clinical and preclinical observation suggested that cellular and extracellular levels of fibrogenic and angiogenic cytokines are altered in patients with myelofibrosis Conventional therapy is mainly used to control symptoms whereas no such therapy has so far shown to change the natural course of the disease
From all patients, bone marrow histology was obtained before allogeneic stem cell transplantation (n = 24)
Summary
Myelofibrosis with myeloid metaplasia (MMM) is a rare malignant hematological disease of the hematopoietic stem cells with a median age at diagnosis of 60 years. Myelofibrosis is characterized by splenomegaly, extramedullary hematopoiesis, bone marrow collagen fibrosis, and osteosclerosis [1]. Bone marrow fibroblasts in patients with myelofibrosis are polyclonal and exhibit normal function [2]. Clinical and preclinical observation suggested that cellular and extracellular levels of fibrogenic and angiogenic cytokines are altered in patients with myelofibrosis Conventional therapy is mainly used to control symptoms whereas no such therapy has so far shown to change the natural course of the disease. The only curative treatment option is hematopoietic stem cell
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