Familial hemophagocytic lymphohistiocytosis. Primary hemophagocytic lymphohistiocytosis.
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Familial hemophagocytic lymphohistiocytosis. Primary hemophagocytic lymphohistiocytosis. / Henter, J I; Aricò, M; Janka-Schaub, Gritta; Imashuku, S; Janka, G.
In: HEMATOL ONCOL CLIN N, Vol. 12, No. 2, 2, 1998, p. 417-433.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Familial hemophagocytic lymphohistiocytosis. Primary hemophagocytic lymphohistiocytosis.
AU - Henter, J I
AU - Aricò, M
AU - Janka-Schaub, Gritta
AU - Imashuku, S
AU - Janka, G
PY - 1998
Y1 - 1998
N2 - Hemophagocytic lymphohistiocytosis represents a spectrum of pathogenetically different diseases including the rapidly fatal autosomal recessive disease of familial hemophagocytic lymphohistiocytosis (FHL). The onset is usually during the first years of life with fever, cytopenia, and hepatosplenomegaly. Neurologic symptoms may supervene. Similar symptoms may occur in the infection-(virus-)associated or malignancy-associated hemophagocytic syndromes (IAHS/MAHS). Triggering infections can be found in all these diseases and do not allow for reliable differentiation. An international treatment protocol (HLH-94) has been developed for FHL, but immunomodulatory treatment may be justified in IAHS and MAHS as well, since they also have a high fatality rate.
AB - Hemophagocytic lymphohistiocytosis represents a spectrum of pathogenetically different diseases including the rapidly fatal autosomal recessive disease of familial hemophagocytic lymphohistiocytosis (FHL). The onset is usually during the first years of life with fever, cytopenia, and hepatosplenomegaly. Neurologic symptoms may supervene. Similar symptoms may occur in the infection-(virus-)associated or malignancy-associated hemophagocytic syndromes (IAHS/MAHS). Triggering infections can be found in all these diseases and do not allow for reliable differentiation. An international treatment protocol (HLH-94) has been developed for FHL, but immunomodulatory treatment may be justified in IAHS and MAHS as well, since they also have a high fatality rate.
M3 - SCORING: Zeitschriftenaufsatz
VL - 12
SP - 417
EP - 433
IS - 2
M1 - 2
ER -
