INFECTION- AND MALIGNANCY-ASSOCIATED HEMOPHAGOCYTIC SYNDROMES: Secondary Hemophagocytic Lymphohistiocytosis
Section snippets
INFECTION-ASSOCIATED HEMOPHAGOCYTIC
SYNDROME (IAHS)
In 1979, Risdall and coworkers36 described a clinical syndrome characterized by fever, hepatosplenomegaly, and pancytopenia. Lymphadenopathy, pulmonary infiltrates, and skin rash were often present. Many patients had elevated transaminases, a high bilirubin, or increased alkaline phosphatase. Clotting abnormalities included prolonged partial thromboplastin time and hypofibrinogenemia. Bone marrow and lymph nodes showed an infiltration with morphologically benign histiocytes
MALIGNANCY-ASSOCIATED HEMOPHAGOCYTIC SYNDROME (MAHS)
Secondary forms of hemophagocytic lymphohistiocytosis have also been reported in children in association with malignant diseases. This is of great importance with regard to differential diagnosis, in particular in older children, adolescents, and adults. As shown in Table 3, the associated malignant diseases can be divided into two major groups.
In the first, hemophagocytic symptoms develop in patients with acute lymphoblastic leukemia (ALL)37, 42 and various kind of tumors before or during
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Address reprint requests to Jan-Inge Henter, MD, PhD, Pediatric Hematology and Oncology, Karolinska Hospital, S-171 76 Stockholm, Sweden
This research is supported by grants from the Children's Cancer Foundation of Sweden and the Medical Research Council of Sweden.