Original articlePrognostic Factors and Outcomes of Adults With Hemophagocytic Lymphohistiocytosis
Section snippets
Methods
The Mayo Clinic electronic medical record database was queried for the terms hemophagocytic syndrome, hemophagocytosis, hemophagocytic lymphohistiocytosis, and macrophage activation syndrome from January 1, 1996, through December 31, 2011. All adult patients (age ≥18 years) whose medical records contained these terms were manually reviewed. The Mayo Clinic hematopathology database was also cross-referenced for the terms hemophagocytic syndrome and hemophagocytosis during the same period. All
Baseline Clinical and Laboratory Characteristics
Of 250 medical records reviewed in which the diagnosis was listed, 62 patients met the stringent eligibility criteria for inclusion in the study. Table 1 outlines the baseline characteristics of our study population. The median age of patients was 49 years (range, 18-87 years), and 42 (68%) were male. Thirty-two patients (52%) were diagnosed with tumor–associated HLH. Of the remaining patients (n=30) with non-tumor–associated HLH, 21 (34%) had an infection, 5 (8%) had an autoimmune condition,
Discussion
Adult HLH is a rare disease, and most of our understanding of this complex disorder comes from small retrospective case series or individual patient case reports.29, 30, 31, 32, 33, 34, 35, 36, 37, 38 To the best of our knowledge, this study is the largest single-institution retrospective analysis of adult HLH patients. In our series of adult HLH patients, 52% of patients had an underlying malignant cause, and their outcomes were markedly inferior to HLH patients with a nonmalignant cause. In
Conclusion
Our study is one of the largest retrospective studies comparing the clinical characteristics and outcomes of adult HLH patients with a variety of secondary causes. A high index of suspicion for HLH should be maintained for adults who present with unexplained fever, cytopenias, and hyperferritinemia. The underlying cause of HLH is the most important determinant of outcome in this heterogeneous population of patients. Unfortunately, the median OS of adult patients with HLH remains poor, in
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