Grade of bone marrow fibrosis is associated with relevant hematological findings - a clinicopathological study on 865 patients with chronic idiopathic myelofibrosis

Controversy continues to exist regarding not only the exact definition and grading of myelofibrosis (MF), but also whether, and to what extent, this feature may be correlated with clinical findings. A retrospective study was performed involving 865 bone marrow (BM) biopsies together with the clinical records from patients with chronic idiopathic myelofibrosis (CIMF). Diagnosis was established according to the World Health Organization criteria, and assessment of MF followed a consensus scoring system that included four grades (MF-0 to MF-3). Histopathological and clinical evaluations were carried out in an independent fashion. Prefibrotic and early CIMF (MF-0/-1) were presented by 565 patients showing borderline to mild anemia and no or slight splenomegaly, but frequently, thrombocytosis exceeding 500x10(9)/l was shown. In 300 patients, manifest reticulin and collagen fibrosis (MF-2/-3) were characterized by marked anemia, gross splenomegaly, peripheral blasts, and normal to decreased platelet and leukocyte counts. The latter cohort was consistent with findings generally in keeping with MF with myeloid metaplasia. Regarding the stepwise evolution of disease, sequential BM examinations showed that in 103 patients, prefibrotic and early CIMF transformed into advanced stages accompanied by correspondingly developing clinical and histomorphological features. Survival analysis (univariate calculation) revealed a significantly more favorable prognosis in prefibrotic vs advanced stages of CIMF. On the other hand, higher classes of MF also exerted a higher clinical risk profile (Lille score). In conclusion, the dynamics of the disease process in CIMF are characterized by evolving MF in the BM and closely associated changes of relevant hematological findings.