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Polycythemia vera: Electron microscopy of the bone marrow in 10 non-treated patients

A thin section and freeze-fracture study

Polycythämia vera: Elektronenmikroskopische Befunde am Knochenmark von zehn unbehandelten Patienten

Eine Untersuchung mit Hilfe der Dünnschnitt- und Gefrierbrechungsmethode

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Zusammenfassung

Elektronenmikroskopische Untersuchungen (Dünnschnitte und Gefrierbrechungen) wurden am Knochenmark von zehn Patienten mit Polycythämia vera vor der Behandlung durchgeführt. Au\er der Hyperplasie aller drei Zellreihen und der Sinus wurden vor allem Atypien der reifenden Erythroblasten gesehen. Diese Abweichungen der normalen Entwicklung bestanden hauptsächlich aus tiefen Einstülpungen der Kernhülle im Proerythroblasten und in auffallenden Kernspalten in Erythro- und Normoblasten. Im Vergleich zu ähnlichen Veränderungen bei dyserythropoetischen und aplastischen Anämien ebenso wie bei Leukämien werden diese Anomalien im Zusammenhang mit Störungen der DNA-Synthese diskutiert. Weitere Atypien betrafen die granulocytäre Reihe mit abnormen Kernschleifen und die Megakaryopoese, die Mikroformen aufwies, was wohl als ein Ausdruck der Reifungshemmung zu werten ist. Diese ultrastrukturellen Anomalien mit ihren morphologischen Kriterien der neoplastischen Proliferation aller drei Zellreihen bei der Polycythämia vera passen gut in das neue Konzept der Transformation einer pluripotenten Stammzelle mit klonalem Charakter.

Summary

Electron microscopy (thin sections and freeze-fracture replicas) was performed on the bone marrow of ten patients with Polycythemia vera prior to any treatment. In addition to a hyperplasia of all three cell lineages and the sinuses, atypias were observed in the maturing erythroblasts. These aberrations of normal development consisted mainly of deep invaginations of the nuclear envelope in proerythroblasts and conspicuous nuclear clefts in erythro- and normoblasts. In comparison with similar changes in dyserythropoietic and aplastic anemia as well as leukemia these alterations are discussed in connection with disturbances of DNA synthesis. Further atypias involved megakaryopoiesis which displayed microforms probably as an evidence for maturation arrest. These ultrastructural abnormalities with their morphological features of a neoplastic proliferation of all three cell lineages in Polycythemia vera are in good agreement with the new concept of a transformation of a pluripotent stem cell with clonal character.

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Authors and Affiliations

  1. Institut für Pathologie der Medizinischen Hochschule Hannover, Karl-Wiechert-Allee 9, D-3000, Hannover 61, Germany

    J. Thiele, K. F. Vykoupil & A. Georgii

  2. Klinische Immunologie und Bluttransfusion der Medizinischen Hochschule Hannover, Karl-Wiechert-Allee 9, D-3000, Hannover 61, Germany

    W. Stangel

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  1. J. Thiele
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  4. A. Georgii
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Supported by the Deutsche Forschungsgemeinschaft (DFG Ge 121/19)

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Thiele, J., Stangel, W., Vykoupil, K.F. et al. Polycythemia vera: Electron microscopy of the bone marrow in 10 non-treated patients. Blut 38, 407–420 (1979). https://doi.org/10.1007/BF01007902

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