Peripheral blood and bone marrow morphology of HET (1986). Peripheral blood smear shows marked thrombocytosis with slight anisocytosis and few large platelets, red cells are normocytic. Bone marrow histology showed a normocellular hematopoiesis and increase in megakaryocytes with loose clustering. There is no increase of granulopoiesis or erythropoiesis. The megakaryocytes are usually larger than normal; there are no dysmorphic megakaryocytes and there is slight focal reticulin fibrosis grade 1 (WHO: MF 0).  

Contexts in source publication

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... propositus case II3 of the Dutch HET family was referred to Dr Michiels at the Erasmus University Medical Center (Academic Hospital Dijkzigt Rotterdam) for expert evaluation. At time of diagnosis of familial ET the histopathology from bone marrow biopsy material from the propositus in 1986 and in 1991 was diagnostic for ET as described by Michiels et al. [17] (Figures 3-5). All features according to the Rotterdam Clinical and Pathological (RCP) diagnostic criteria for ET [17] were present: ...

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... Increase of platelet count in excess of 400 x10 9 /l in the absence of any cause or sign of reactive thrombocytosis ( Figure 3). ...

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... initial bone marrows histologic findings in the Dutch HET family were blindly and independently evaluated in 2014 by Drs Piche and De Raeve directly from the pictures without any knowledge of the clinical and laboratory data and not aware of the diagnosis of congenital familial thrombocythemia caused by a gain of function mutation in the TPO gene. The first bone marrow biopsy in 1986 of case II3 is consistent with ET according to the 2014 WHO clinical molecular and pathological (2014 WHO-CMP, Figures 3 and 4) [35]. A second bone marrow biopsy in 1991 showed dense clustering of large megakaryocytes megakaryocytes ( Figure 5). ...

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... marrow histology in 4 affected cases at ages between 15 and 22 years in the third generation of the Polish family showed a slightly hypercellular bone marrow due to increased megakaryopoiesis of loosely clustered small, medium and some large sized megakaryocytes and moderate reactive increase of dispersed mature eosinophils ( Figure 6) [19]. The two adult cases in the second generation of the Polish HET family had increase of megakaryopoiesis in a completely normocellular bone marrow at ages of 52 and 66 years indicating the absence of bone marrow hypercellularity similar as in the proband of the Dutch HET case II3 at age 62 years (Figures 3 and 4). All affected members in the Polish HET family had normal bone marrow myeloid/erythroid cells ratios. ...