[Hepatitis-associated aplastic anemia preceded by a hemophagocytic syndrome-like state].
A 21-year-old man was admitted to our hospital for acute hepatitis of unknown cause. His liver function improved with rest, but worsened 2 months later. He developed a high fever and pancytopenia. The serum level of cytokines including TNF-alpha, IFN-gamma, IL-6, and M-CSF was elevated, and hemophagocytes were seen in bone marrow. These findings suggested a hemophagocytic syndrome-like state. With prednisolone, gamma-globulin, and G-CSF, the high fever disappeared and the patient's liver function gradually recovered. However, the severe pancytopenia persisted. The bone marrow became acellular with a small number of hemophagocytes, and hepatitis-associated aplastic anemia was diagnosed. After immunosuppressive therapy with ATG, CyA and G-CSF was started, and the patient showed hematopoietic reconstitution. The bone marrow CD4+/CD8+ lymphocyte ratio recovered to within the normal range, and the serum cytokines including TNF-alpha and IFN-gamma decreased. The increase in serum cytokines, particularly TNF-alpha and INF-gamma, as well as the presence of activated T cells associated with the preceding hemophagocytic syndrome-like state may have predisposed this patient to aplastic anemia.
Matsunawa
Manabu
M
Department of Hematology, Showa University School of Medicine.
Kawakami
Keiichiro
K
Hisatake
Jun-ichi
J
Suzuki
Junko
J
Nakamaki
Tsuyoshi
T
Hino
Ken-ichiro
K
Tomoyasu
Shigeru
S
D000328:Adult; D000741:Anemia, Aplastic / Q000150:complications*; D006505:Hepatitis / Q000150:complications*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology*; D006801:Humans; D008297:Male; D010198:Pancytopenia / Q000209:etiology