Hematological Features and Diagnosis

Barosi and colleagues25 have published diagnostic criteria for CIMF (Table 10-4). There is a progressive change in the marrow histology over time. Initially, there is a proliferative phase, in which the marrow is hypercellular, all hemopoietic elements are retained, and the increased fibrous tissue consists of increased coarse reticulin in parallel bundles (Table 10-3). Finally, an osteomyelosclerotic stage is reached, in which there is replacement with highly vascular fibrous tissue containing new bone and markedly dysplastic megakaryocytes. As a result of these marrow changes, the peripheral blood findings are variable, although leukoerythroblastic change is typical. Anemia develops over time. Thrombocytosis may be present initially but can develop later in some patients. The white count is variable. Often pancytopenia with the presence of a small percentage of blast cells eventually occurs. There is progressive splenic and often hepatic enlargement, and myeloid metaplasia in other organs may occasionally be found late in the disease process. Transformation to acute myeloblastic leukemia occurs in approximately 20 percent of patients.

The differential diagnosis encompasses the many causes of secondary bone marrow fibrosis (Table 10-5). Whereas

Table 10-4. The Italian Criteria for the Diagnosis of Chronic Idiopathic Myelofibrosis

Necessary criteria Necessary criteria

Absence of Philadelphia chromosome or bcr-abl rearrangement in peripheral blood cells Optional criteria Splenomegaly

Anisopoikilocytosis with tear-drop erythrocytes Presence of circulating immature myeloid cells Presence of circulating erythroblasts Presence of clusters of megakaryocytes and dysmegakaryopoiesis in bone marrow sections Myeloid metaplasia

Adapted from those given by the Italian Cooperative Study Group on Myelofibrosis with Myeloid Metaplasia.

Diagnosis of idiopathic myelofibrosis is acceptable if the following combinations are present: the two necessary criteria plus any other two optional criteria when splenomegaly is present; the two necessary criteria plus any other four criteria when splenomegaly is absent. Based on the publication of Barosi et al.25

Table 10-5. Conditions Associated with Bone Marrow Fibrosis



Chronic idiopathic myelofibrosis

Infections (e.g., tuberculosis,

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