Clinical Characteristics of Adult Hemophagocytic Lymphohistiocytosis in the Emergency Department

Abstract

Purpose To determine the clinical manifestations and results of adult hemophagocytic lymphohistiocytosis (HLH) patients in our emergency department. Methods We retrospectively evaluated patients with HLH from 1 April 2018 to 31 December 2020. The clinical data of these patients (basic information, symptoms, vital signs, laboratory results, HLH diagnostic criteria, H Score, main treatments, outcomes) were collected. Results Thirty-three patients (23 males and 10 females; 40.55±18.78 years) with 34 clinical episodes (one male had two clinical episodes and died during the second episode) were enrolled. Twenty-five patients were placed in a “survivor” group, and nine patients were categorized into a “deceased” group. Fever, splenomegaly, hemoglobin <90 g/L and platelet count <100×10 9 /L most commonly met the diagnostic standard for HLH. The H Score results in the survival group and deceased group was 212.4±37.18 and 252.1±40.95, respectively. Viral infection was the most common reason for HLH, followed by immune-system disease and cancer. Laboratory tests showed that deceased-group patients had multiple-organ dysfunction. Multivariate logistic regression showed that the lactate dehydrogenase (lactate dehydrogenase) level (P = 0.039; odds ratio, 0.999) was significantly related to death. Conclusion In the emergency department, HLH should be considered for critically ill patients with fever, splenomegaly, low hemoglobin and low platelet count. The H Score might be useful to diagnose HLH quickly. In our study, 26.47% of HLH patients died in the emergency department, and patients with a significantly increased lactate dehydrogenase level had a markedly increased risk of death.

Complete Response to Donor Lymphocyte Infusion for Primary Hemophagocytic Lymphohistiocytosis Relapse after Allogeneic Hematopoietic Cell Transplantation

“…As this case demonstrates, primary HLH is a severe, life-threatening disorder that can be refractory to multiple lines of treatment. Although HLH-94 is applicable to both the treatment of adults and pediatric HLH, the response rate is significantly lower in adults, likely owing to the more complex etiology [15]. A number of secondline treatments have been investigated for disease refractory to the HLH-94 protocol [4] including DEP (doxorubicin, etoposide, and methylprednisolone) [16], emapalumab (anti-IFNy) [17], ruxolitinib (both alone and in combination with DEP) [18,19], and alemtuzumab [20].…”