• Toshihiko Kakiuchi, Katsuhide Eguchi, Daisuke Koga, Hiroi Eguchi, Masanori Nishi, Motoshi Sonoda, Masataka Ishimura, and Muneaki Matsuo.
• Department of Pediatrics, Faculty of Medicine, Saga University, Saga, Japan.
• Medicine (Baltimore). 2022 Feb 25; 101 (8): e28953e28953.

RationaleHepatitis-associated aplastic anemia (HAAA) is a rare illness that results in bone marrow failure following hepatitis development. The etiological agent remains unknown in most HAAA cases. However, clinical features of the disease and immunotherapy response indicate that immune-mediated factors play a central role in the pathogenesis of HAAA. Activation of cytotoxic T cells and increase in CD8 cells could exert cytotoxic effects on the myelopoietic cells in the bone marrow.Patient ConcernsA 15-month-old boy was brought to our hospital with complaints of generalized petechiae and purpura observed a week prior to hospitalization. His liver was palpated 3 cm below the costal margin, platelet count was 0 × 104/μL, and alanine aminotransferase level was 1346 IU/L. A blood test indicated cytomegalovirus infection, and 3 bone marrow examinations revealed progressive HAAA. As the disease progressed to the 3rd, 6th, and 9th week after onset, CD4+ T cells were markedly decreased, CD8+ T cells were markedly increased, and the CD4/CD8 ratio was significantly decreased. The number of B cells and natural killer cells decreased with time, eventually reaching 0.0%.DiagnosisHAAA.InterventionsRabbit antithymocyte globulin and eltrombopag olamine (a thrombopoietin receptor agonist) were administered.OutcomesThe patient's platelet count returned to normal, and bone marrow transplantation was avoided. The peripheral blood lymphocytes (PBLs) improved as the patient's general condition recovered.LessonsThis case demonstrates that HAAA induced by cytomegalovirus infection features decreasing CD4+ and increasing CD8+ PBLs as the bone marrow hypoplasia progresses. The PBLs return to their normal levels with the recovery from the disease. Our case findings thus support the involvement of immunological abnormality in HAAA.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

31 keywords...

hide…