How I treat How I treat hemophagocytic lymphohistiocytosis
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چکیده
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is essential for survival, but timely diagnosis may be challenging because of the rarity of HLH, its variable presentation, and the time required to perform diagnostic testing. Therapy is complicated by dynamic clinical course, high risk of treatment-related morbidity, and disease recurrence. Here, we review the clinical manifestations and patterns of HLH and describe our approach to the diagnosis and therapy for this elusive and potentially lethal condition. (Blood. 2011;118(15):4041-4052)
منابع مشابه
How I treat hemophagocytic lymphohistiocytosis in the adult patient.
Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation. This syndrome can be caused by genetic mutations affecting cytotoxic function (familial HLH) or be secondary to infectious, rheumatologic, malignant, or metabolic conditions (acquired HLH). Prompt recognition is paramount...
متن کاملHow I Treat Hemophagocytic Lymphohistiocytosis in the Adult Patient Alison
Hemophagocytic Lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation. This syndrome can be caused by genetic mutations affecting cytotoxic function (familial HLH) or secondary to infectious, rheumatologic, malignant, or metabolic conditions (acquired HLH). Prompt recognition is paramount an...
متن کاملHow I treat hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is ...
متن کاملHow I Treat How I treat hemophagocytic lymphohistiocytosis in the adult patient
Hemophagocytic lymphohistiocytosis (HLH) is a rare and devastating disorder of uncontrolled immune activation that has gained increasing recognition over the past decade. Patients with HLH present with clinical and laboratory evidence of extreme inflammation. This syndrome was first described in 1939 by Scott and Robb-Smith and again in 1952 when Farquhar and Claireaux reported a case of 2 infa...
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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation, which is characterized by fever, hepatosplenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, and/or hypofibrinogenemia, and evidence of hemophagocytosis. Secondary HLH is often seen in adults and categorized based on autoimmune, infections-related, and malignancy-associated etiol...
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تاریخ انتشار 2011