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Dig Dis Sci (2007) 52:24382443 DOI 10.1007/s10620-006-9280-4
Hepatitis Associated with Aplastic Anemia: Do CD8(+) Kupffer Cells Have a Role in the Pathogenesis?
Cem Cengiz Nesrin Turhan O. Faruk Yolcu Sevinc Ylmaz
Received: 4 December 2005 / Accepted: 1 March 2006 / Published online: 19 April 2007
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Springer Science+Business Media, Inc. 2007
Keywords Hepatitis . Aplastic anemia . Cytotoxic T cells . Kupffer cells . Immunohistochemistry . Immunosuppressive therapy
Introduction
Hepatitis-associated aplastic anemia (HAAA), rst described by Lorenz and Quaiser in 1955 [1], is a rare phenomenon which usually affects adolescents and young men [2] and is more common in the East than in the West [3 5]. It represents a minor proportion of all aplastic anemia cases [6] and may occur in 28% of young adults after liver transplantation for non-A, non-B hepatitis [7]. Although a limited number of aplastic anemia cases have been reported in association with hepatitis A, B, and G, parvovirus B19, Epstein-Barr virus (EBV), transfusion transmitted virus (TTV), and echovirus [820], the causative agent in most of the cases still remains unknown [21, 23]. Patients typically develop severe aplastic anemia 2 to 3 months after an episode of acute hepatitis. Aplastic anemia is always fatal if untreated [2, 11] and the hepatitis associated with this
C. Cengiz ([envelopeback]) O. F. Yolcu Department of Gastroenterology, Turkiye Yuksek Ihtisas Hastanesi, 8 Cadde, 81 Sokak, No. 7/18, Emek, 06510 Ankara, Turkeye-mail: [email protected]
N. TurhanDepartment of Pathology, Turkiye Yuksek Ihtisas Hastanesi, Ankara, Turkey
S. YlmazDepartment of Hematology, Turkiye Yuksek Ihtisas Hastanesi, Ankara, Turkey
may be mild and transient [2, 22], fulminant [7, 23], or chronic [2]. There is no known relation to blood transfusion, drugs, or toxins. Though not clearly dened thus far, immunologic mechanisms seem to be responsible for HAAA, particularly because immunosuppressive therapy has shown some promising results. Nonetheless, most successful outcomes have been achieved by HLA-matched bone marrow transplantation (BMT) [21].
We report an adolescent male admitted with acute hepatitis of unknown etiology whose course was subsequently complicated by severe aplastic anemia. As an original observation, many CD8-expressing Kupffer cells were detected in the liver biopsy specimen. Despite continuing immunosuppressive therapy, the patient died after an intracranial hemorrhage. Etiopathogenesis of and treatment options for this intriguing case are also discussed.
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