Acute hepatitis as a prequel to very severe aplastic anemia

Abstract

Severe aplastic anemia is a rare and potentially life-threatening disease of the bone marrow often requiring allogeneic hematopoietic stem cell transplantation. Pathogenesis of the disease can vary and often remains enigmatic. Occasionally, severe aplastic anemia is associated with prior severe acute hepatitis. Differential diagnosis of acute non-viral hepatitis challenges the physician as pathogenesis remains unclear.

We here present a case of a young patient presenting with acute hepatitis followed by severe aplastic anemia successfully treated with allogeneic hematopoietic stem cell transplantation. Due to immunosuppressive treatment with azathioprine for acute hepatitis of putative autoimmune pathogenesis and coincident infection with parvovirus B19, diagnosis of the sequential disease of acute hepatitis followed by severe aplastic anemia was complicated. We discuss the caveats and present a review of the literature.

Zusammenfassung

Schwere aplastische Anämie (SAA) ist eine seltene und potentiell lebensbedrohliche Erkrankung des Knochenmarkes. Es gibt verschiedene Modelle zur Erklärung ihrer Pathogenese. Die Transplantation allogener hämatopoetischer Stammzellen ist die einzige kurative Therapiemöglichkeit. Selten ist eine schwere aplastische Anämie mit einer vorangehenden akuten Hepatitis assoziiert. Die Differentialdiagnose der akuten Hepatitis ohne Virusnachweis stellt den behandelnden Arzt häufig vor Probleme.

Hier beschreiben wir einen Fall einer jungen Patientin mit akuter nicht-viraler Hepatitis, auf die eine SAA folgte. Letztere wurde erfolgreich mit einer allogenen Stammzelltransplantation behandelt. Infolge einer Immunsuppression mit Azathioprin für die zunächst als autoimmuner Genese behandelte Hepatitis sowie einer koinzidentellen Parvovirus-B19-Infektion gestaltete sich die Diagnosestellung der SAA schwierig. Interdisziplinäre Aufarbeitung führte zur Diagnosestellung des seltenen Syndromes der akuten Hepatitis gefolgt von schwerer aplastischer Anämie.

Schlüsselwörter

Hepatitis - Schwere aplastische Anämie - Azathioprin - Parvovirus B 19 - Stammzelltransplantation

Key words

hepatitis - severe aplastic anemia - azathioprine - parvovirus B19 - stem cell transplantation

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