A clinicopathological study was performed on 114 patients (46 male/68 female, median age 67 years) with the diagnosis of agnogenic myeloid metaplasia (AMM) respectively primary osteo-myelofibrosis which was not preceded by any other or allied subtype of chronic myeloproliferative disorders. On admission patients revealed a striking variability of laboratory data as well as different histopathological features of initial bone marrow biopsies. For this reason discrimination was done into two groups based on bone marrow findings: group I patients (n = 46, 19 male/27 female) showed a hypercellular marrow without or only borderline (n = 24) to slight (n = 22) reticulin fibrosis and group II cases (n = 68, 27 male/41 female) displayed coarse bundles of collagen fibres (n = 18) frequently accompanied by osteosclerosis (n = 50). Statistical analysis of the corresponding initial hematological findings resulted in significant differences. These differences concerned also the complications occurring during the lengthy course of disease, which included a higher incidence of pancytopenia and severe marrow failure with hemorrhage and blast crisis in group II patients. However, overall survival time was not different in both groups. This may be related to the similarity of age distribution (64 resp. 65 years) and its significant association with arteriosclerotic vascular lesions. Consequently acute myocardial infarction and congestive heart failure were frequent causes of death in addition to infections due to marrow failure and blast crisis. Repeated bone marrow biopsies in 24 patients revealed an insidious transition from hypercellular lesions (group I) into advanced fibro-osteosclerotic changes (group II) concurring with laboratory data. Therefore our discrimination into two groups of patients represents variable stages or static histological and corresponding hematologic features in the evolution of a dynamic disease process in AMM.

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Primary myelofibrosis-osteomyelosclerosis (agnogenic myeloid metaplasia): correlation of clinical findings with bone marrow histopathology and prognosis.

A clinicopathological study was performed on 90 patients (39 males - 51 females, age 68 years) with primary (idiopathic) myelofibrosis - osteomyelosclerosis (OMF) in order to correlate laboratory and histomorphological parameters with each other and to calculate factors of prognostic impact on survival. In addition to multiple interactions between various laboratory features, there was a significant correlation between degree of medullary fibrosis and osteosclerotic changes with sizes of spleen and liver, level of LDH and duration of relevant prediagnostic symptoms. In trephine biopsies of the bone marrow, reduction of hematopoietic tissue was assessed by evaluating the amount of fat cells plus the degree of osteosclerotic lesions. This histological parameter did not reveal significant relationships with hepatosplenomegaly, duration of relevant symptoms or length of disease, but was correlated with the clinical findings of bone marrow failure. On univariate analysis, several clinical (age greater than 45 years, presence of relevant prediagnostic symptoms, hemoglobin level less than 9 g/dl, counts of myelo- and normoblasts, thrombocyte count less than 100 and greater than 700 x 10(9)/l, spleen size and LDH level) and histological features (reduction of hematopoiesis, counts for megakaryocytes and lymphoid nodules) were found to exert a predictive value on prognosis. However, on multivariate regression analysis only age remained significant. This result apparently reflects the numerous interactions between the various clinical as well as histological variables tested.

Bone Marrow↗