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Primary myelofibrosis (PMF).
Bone Marrow, Iliac Crest
Haematoxylin & Eosin (H&E)
64 years
64-year old male patient with myeloproliferative disease, splenomegaly. Genetics: mutation for Calreticulin is positive.
Bone marrow trephine biopsy with few intertrabecular spaces with age-correlated hypercellularity (70-80%). Megacaryocytes are increased in number and form dense clusters. They show hyperchromatic nuclei with plump bulbous or cloud-like lobulation. Erythropoiesis is reduced in quantity. Myelopoiesis is increased with normal maturation. Fibre stain reveals diffuse myelofibrosis grade 2.
Immunohistochemistry for CD 34+ did not show an increase in blasts.
Bone marrow fibrosis is graded as follows: MF-0 correspondes to normal bone marrow with scattered linear reticulin fibres especially around vessels. MF-1 shows a loose network of reticulin fibres. MF-2 reveals a dense increase of reticulin fibres with some coarse bundles of collagen. In MF-3 fibrous bundles show extensive intersections, it is often accompanied by osteosclerosis. Besides JAK2 mutation, which is the most frequent genetic aberration in myeloproliferative neoplasms, calreticulin mutation represents another common genetic abnormality.
Andrea Bodmer, University of Basel
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