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Critical Issues About the Diagnosis of MPNs: Bone Marrow Histopathology

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Myeloproliferative Neoplasms

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

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Abstract

Controversy persists regarding the role of histopathology for the diagnosis of polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) diagnosed according to the World Health Organization (WHO) criteria. While morphological features play a minor role in PV, these are of major importance in ET and PMF, particularly regarding the early/prefibrotic stages of PMF. Discrimination between ET and early PMF by characteristic histological bone marrow (BM) patterns is of clinical significance. Recognition of these differences requires a systematic assessment of standardized BM features concerning age-matched cellularity, erythro- as well as neutrophil granulopoiesis, and especially megakaryocyte abnormalities. In PV, a trilineage proliferation (panmyelosis) of all hematopoietic cells is a characteristic feature, whereas in early PMF, a granulocytic and megakaryocytic myeloproliferation is prevalent. Contrasting ET with a predominant increase in large to giant mature megakaryocytes, in PMF clustering of grossly abnormal cells of this lineage, is detectable. Only in more advanced stages of PMF, moderate to overt reticulin and collagen fibrosis develops and is associated with the clinical evolution of the disease process. Concerning the complex BM alterations, standardization of prominent morphological parameters is emphasized as a first step for reproducibility of the WHO classification.

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Authors and Affiliations

  1. Institute of Pathology, University of Cologne, Cologne, Germany

    Jürgen Thiele

  2. Senckenberg Institute of Pathology, University of Frankfurt, Frankfurt, Germany

    Hans Michael Kvasnicka

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  1. Jürgen Thiele
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Correspondence to Jürgen Thiele .

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  1. Div. Ematologia, Ospedali Riuniti di Bergamo, Largo Barozzi 1, Bergamo, 24128, Italy

    Tiziano Barbui

  2. Dept. Internal Medicine, Div. Hematology, Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Ayalew Tefferi

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Thiele, J., Kvasnicka, H.M. (2012). Critical Issues About the Diagnosis of MPNs: Bone Marrow Histopathology. In: Barbui, T., Tefferi, A. (eds) Myeloproliferative Neoplasms. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-24989-1_3

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