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עמוד בית
Wed, 12.06.24

Original Articles

IMAJ | volume

Journal 1, January 2000
pages: 22-24

Paroxysmal Nocturnal Hemoglobinuria Associated with in vitro Inhibition of Erythropoiesis by Bone Marrow T Lymphocytes


    Background: The etiology of bone marrow failure, a prominent feature of paroxysmal nocturnal hemoglobulinuria, is presently unknown.

    Objectives: To evaluate the possible influence of cellular immune mechanisms in the bone marrow failure of PNH.

    Methods: We studied marrow erythroid colony formation in a patient with paroxysmal nocturnal hemoglobinuria without hypoplastic/aplastic marrow complications.

    Results: In vitro assays revealed a pronounced inhibition of primitive erythroid (BFU-E) progenitor cell growth by marrow T lymphocytes. Removal of T cells prior to culture resulted in a 4.5-fold enhancement of BFU-E numbers. Reevaluation of in vitro erythropoiesis during steroid administration indicated a persistent, albeit less prominent, T cell inhibitory effect.

    Conclusion: Our findings provide the first direct evidence for a cellular immune inhibitory phenomenon accompanying PNH.

    PNH= paroxysmal nocturnal hemoglobinuria

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