Ultrastructure of blastic crisis in osteomyelofibrosis. A report of 2 cases with some unusual features.
The clinical and morphological findings are presented in two patients suffering from myelofibrosis and osteomyelosclerosis which terminated in an acute blastic crisis. Clinical follow-up data and light microscopy of the bone marrow however, revealed a chronic megakaryocytic-granulocytic myelosis (CMGM) with progression into myelofibrosis during the course of disease. In one patient the blastic transformation involved predominantly basophils, and in the other, neutrophils, with an accompanying abnormal proliferation of megakaryocytes in both cases. Electron microscopy of this cell population demonstrated remarkable atypicalities of the neutrophilic, basophilic and megakaryocytic cell lines. These abnormalities consisted of a nuclear-cytoplasmic asynchrony and a partial arrest of maturation, sometimes resulting in bizarre cell forms. Our investigations support the hypothesis of a mixed cellularity type of myelosis with a gradual and insiduous progression into osteomyelofibrosis/-sclerosis and a potential blastic crisis. In the evolution of blastic crisis all cell lines may be transformed, but with predominance of one population - basophils and neutrophils in our two cases - in addition to atypicalities of megakaryocytes.
D000208:Acute Disease; D000328:Adult; D001853:Bone Marrow / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007938:Leukemia / Q000648:ultrastructure*; D007951:Leukemia, Myeloid / Q000648:ultrastructure; D008297:Male; D008533:Megakaryocytes / Q000648:ultrastructure; D008854:Microscopy, Electron; D008875:Middle Aged; D009504:Neutrophils / Q000648:ultrastructure; D055728:Primary Myelofibrosis / Q000473:pathology*