Bone marrow histopathology following cytoreductive therapy in chronic idiopathic myelofibrosis

• Published in: Histopathology 2003-11, Vol.43 (5), p.470-479
• Main Authors: Thiele, J, Kvasnicka, H M, Schmitt-Graeff, A, Diehl, V
• Format: Article
• Language: English
• Subjects: Aged; Antineoplastic Agents - therapeutic use; Biological and medical sciences; Bone Marrow - drug effects; Bone Marrow - pathology; bone marrow biopsies; Busulfan - therapeutic use; Female; Hematologic and hematopoietic diseases; Hematopoiesis - drug effects; Humans; Hydroxyurea - therapeutic use; hypoplasia; idiopathic myelofibrosis; Interferons - therapeutic use; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Medical sciences; Middle Aged; myelodysplasia; myelofibrosis; Primary Myelofibrosis - drug therapy; Retrospective Studies; scleroedema; therapeutic regimens
• ISSN: 0309-0167; 1365-2559
• DOI: 10.1046/j.1365-2559.2003.01732.x

Aims:  To analyse systematically therapy‐induced lesions of haematopoiesis in chronic idiopathic myelofibrosis (IMF). Methods and results:  A total of 759 sequential bone marrow (BM) biopsies (median interval 32 months) were performed in 261 patients with IMF. Besides a control group (symptomatic treatment), monotherapies included busulfan, hydroxyurea and interferon. In all therapy groups hypoplasia of varying degree was a frequent finding and often accompanied by a patchy distribution of haematopoiesis. Most conspicuous was gelatinous oedema showing a tendency to develop discrete reticulin fibrosis (scleroedema). Minimal to moderate maturation defects of megakaryopoiesis and erythroid precursors occurred, but overt myelodysplastic features were most prominent following hydroxyurea and busulfan therapy. Acceleration and blastic crisis were characterized by the appearance of immature and CD34+ progenitor cells. Concerning the dynamics of fibrosis, no differences were observed between controls and the various therapy groups. In 143 patients (55%) without or with little reticulin at onset, an increase in myelofibrosis was detectable that progressed to overt collagen fibrosis. Conclusions:  Therapy‐related bone marrow lesions in IMF comprise a strikingly variable spectrum that may include aplasia with scleroedema and a patchy distribution of myelodysplastic haematopoiesis associated with progressive myelofibrosis.