[Lymphoma-associated hemophagocytic syndrome(LAHS)].
Hemophagocytic syndrome(HPS) is characterized by a systemic proliferation of histiocytes showing hemophagocytosis in bone marrow, liver, or spleen. Clinical features of HPS are idiopathic fever, cytopenia, liver dysfunction, and coagulopathy. Adult HPS includes lymphoma-associated hemophagocytic syndrome(LAHS) and the incidence of LAHS was about 40% of the patients with HPS according to our study. About 70% of patients with LAHS had hepatosplenomegly without significant peripheral lymphoadenopathy or nodal lesions and showed HPS at the time of presentation. These clinical features were previously diagnosed as malignant histiocytosis (MH). The prognosis of LAHS depends mostly on the immunophenotype of lymphomas, T/NK-LAHS or B-LAHS. The median survival is 69 days in T/NK-LAHS and 242 days in B-LAHS.
Takahashi
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Third Department of Internal Medicine, Akita University School of Medicine.
D001402:B-Lymphocytes / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000145:classification / Q000473:pathology; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology; D011379:Prognosis; D013577:Syndrome; D013601:T-Lymphocytes / Q000473:pathology