How I treat hemophagocytic lymphohistiocytosis

שנת פרסום ראשונה: אוקטובר 2011
שנת עדכון:
מפרסם: BLOOD

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)

BLOOD, 13 OCTOBER 2011 , VOLUME 118, NUMBER 15