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Die Osteomyelofibrose

Myelofibrosis

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Summary

A survey is presented on the theories concerning the nosology as well as the clinical and laboratory findings in myelofibrosis. In some cases the disease is a proliferative process, while in others necrosis of myeloid tissue is provoked by unknown stimuli and terminates in fibrosis of the marrow. This concept is underscored by experimental studies which show generalized marrow fibrosis in rabbits during repeated antigen-antibody reactions.

Clinical and laboratory findings as well as the course vary widely from one case to the other. Histological examination of a marrow biopsy is essential for the diagnosis; the histological pattern sometimes allow to distinguish between proliferative and reactive-necrotizing forms. The immunoglobulins (mostly γ-G-globulins) are elevated in more than 50% of the cases, which together with sometimes positive findings of antibodies against collagen point in the direction of an inflammatory process.

In therapy the indications of splenectomy are stressed, whereas cytostatic drugs or corticoids are used only in selected cases.

Zusammenfassung

Bei der Osteomyelofibrose handelt es sich z.T. um autonom-proliferative Erkrankungen, z.T. um zu Nekrosen und terminaler Fibrosierung führende, durch unbekannte Stimuli ausgelöste Knochenmarks-Prozesse. Diese zweite Entstehungsmöglichkeit wird u.a. durch tierexperimentelle Untersuchungen gestützt, die die Entwicklung von generalisierten Markfibrosen bei rezidivierenden Antigen-Antikörper-Reaktionen zeigen.

Díese durch die Trias „Markfibrose (evtl. mit Knochenumbau), extramedulläre Blutbildung und erythroleukämisches Blutbild“ charakterisierte Krankheitsgruppe zeigt im Einzelfall weite Unterschiede hinsichtlich der klinischen Befunde, der Labor-Untersuchungen und des Verlaufs. So ist eine sichere Diagnose nur durch die histologische Knochenmarks-Untersuchung möglich, die gelegentlich die Abgrenzung proliferativer Krankheitsformen gegenüber reaktiv-nekrotisierenden Markprozessen gestattet. Bemerkenswerterweise sind in über der Hälfte der Fälle die Immunglobuline (in der Regel γ-G-Globuline) vermehrt. Bei der Therapie werden die Indikationen zur Splenektomie hervorgehoben, während Cytostatika oder Corticoide nur ausnahmsweise Verwendung firden.

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Authors and Affiliations

  1. Medizinische Univ.-Poliklinik Heidelberg, Germany

    W. Hunstein & Ch. Hauswaldt

  2. Medizinische Univ.-Klinik und Poliklinik Göttingen, Germany

    W. Hunstein & Ch. Hauswaldt

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  1. W. Hunstein
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  2. Ch. Hauswaldt
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Hunstein, W., Hauswaldt, C. Die Osteomyelofibrose. Klin Wochenschr 52, 305–317 (1974). https://doi.org/10.1007/BF01468828

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  • DOI: https://doi.org/10.1007/BF01468828

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