title: Clinicopathological features of acute megakaryoblastic leukaemia: Relationship between fibrosis and platelet-derived growth factor.
abstract: Acute megakaryoblastic leukaemia (AMGL) is an uncommon disease with poor prognosis. Histopathologically, AMGL cases show variable degree of fibrosis and the presence of uniform blasts or mature dysplastic megakaryocytes. Here we examined 18 cases of AMGL, including idiopathic (n = 9) and secondary (n = 9) cases. Fourteen cases were males and four were females, ranging in age from 14 to 87 years (median, 58). All cases had anaemia, but leukocyte and platelet counts varied. Blast cells were detected in the peripheral blood of 14 cases. Fourteen of 16 cases showed chromosomal abnormalities. The median survival was 6 months (range, 1-48 months). Survival rates did not correlate with the severity of fibrosis, proportion of blast cells and cause of AMGL. Nine of the 11 cases examined immunohistochemically were positive for platelet-derived growth factor (PDGF)(-BB), especially megakaryoblasts and a few fibroblasts. The PDGF-positive cases showed various degrees of fibrosis, while the negative cases showed no evidence of fibrosis. Our results confirmed the poor prognosis of patients with AMGL, irrespective of the degrees of fibrosis, and demonstrated that PDGF could play an important role in the pathogenesis of marrow fibrosis.
authors.affiliation: Department of Pathology, School of Medicine, Kurume University, Kurume, Japan. niino_daisuke@med.kurumeu.ac.jp
authors.forename: Daisuke
authors.initials: D
authors.lastname: Niino
authors.forename: Takeshi
authors.initials: T
authors.lastname: Tsuchiya
authors.forename: Masao
authors.initials: M
authors.lastname: Tomonaga
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Miyazaki
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000378:metabolism*; D001752:Blast Crisis / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007564:Japan / Q000453:epidemiology; D007947:Leukemia, Megakaryoblastic, Acute / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000378:metabolism*; D010750:Phosphoproteins / Q000378:metabolism*; D055728:Primary Myelofibrosis / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D015996:Survival Rate; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins; C077773:PDGF-related protein, human; D010750:Phosphoproteins