The value of bone marrow histology in differentiating between early stage Polycythemia vera and secondary (reactive) Polycythemias.

作者: Volker Diehl , Rudolph Zankovich , Juergen Thiele , Hans Michael Kvasnicka

DOI: 10.3324/%X

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摘要: BACKGROUND AND OBJECTIVES: The diagnostic criteria of the Polycythemia Vera Study Group (PVSG), although generally acknowledged as gold standard for establishing a diagnosis polycythemia vera (PV), do not consider bone marrow features. It may, therefore, be speculated that initial-early stages PV are overlooked. In this retrospective study we tried to investigate whether morphology patients with an only borderline slight increase in hemoglobin/hematocrit conforming postulates PVSG enabled clear-cut differentiation between and secondary (reactive) polycythemias (SP). DESIGN METHODS: From series 348 pronounced erythrocytosis representative pre-treatment trephine biopsies cohort 86 cases was selected showing hemoglobin (males < 18.5 g/dL, females 16.5 g/dL). Biopsies clinical records were evaluated independently following histologic work-up straightforward consensus reached. impact findings tested by means discriminate analysis 20 standardized morphologic features based on histochemical immunohistochemical staining techniques. RESULTS: Bone histopathology 47 diagnosed having SP characterized minimal cellularity predominance erythroid lineage. Neutrophil granulocytopoiesis prominent left-shifted small medium-sized megakaryocytes without maturation defects scattered throughout marrow. There increased number eosinophils, marked perivascular plasmacytosis, histiocytic reticular cells accumulated cell debris many iron-laden macrophages. Contrasting appearance our 39 stage revealed hypercellular trilineage proliferation (pan-myelosis) confluent sheets erythropoiesis loose clusters megakaryocytes. Megakaryocytopoiesis pleomorphous appearance, i.e. giant lying adjacent ones, but lacked obvious cytologic abnormality. usually no inflammatory reaction interstitial compartment. ten lymphoid nodules found, conspicuous iron deposits six reticulin fibers present. Following stepwise set parameters emerged including megakaryocyte size, plasma cells, overall cellular debris. This pattern exerted significant separation (Wilks' lambda statistics = 0.110, p 0.0001) early from SP. Most had underlying bronchopulmonary condition, frequently associated heavy smoking or rarely renal pathology. addition histopathologic features, splenomegaly, thrombocyte count, LDH, LAP erythropoietin levels proved different two groups patients. INTERPRETATION CONCLUSIONS: Initial-early is specific histopathology. Clinical distinctive include particular level. Taking these into consideration, reactive-secondary causes (SP) clearly distinguishable autonomous ones (PV).

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