Abstract
BackgroundHemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation. We aimed to describe the clinical and laboratory findings of HLH patients secondary to Visceral leishmaniasis (VL) and their treatment outcome during a 4-year follow-up period compared to primary HLH.MethodForty children with primary HLH confirmed by genetic study and 20 children with HLH secondary to VL confirmed by a blood or bone marrow polymerase chain reaction from 2014 to 2018 in Shiraz, Fars province, Southern Iran, were enrolled.ResultsThe median age at diagnosis was 11.5 months (range 1–170), and 56.7% were male. Fever and splenomegaly were the most frequent clinical presentations. 93.3% of the subjects had an HScore > 169, which had a good correlation with HLH-2004 criteria (r = 0.371, P = 0.004). Patients with primary HLH experienced more thrombocytopenia (P = 0.012) and higher alanine transaminase (P = 0.016), while patients with VL-associated HLH had higher ferritin (P = 0.034) and erythrocyte sedimentation rate (P = 0.011). Central nervous system (CNS) involvement occurred in 38.3% of patients. The mortality rate was higher in patients with CNS disease (61% vs. 35%, P = 0.051). The 3-yr overall survival rate was 35.9%. (24% in primary HLH and 100% in VL-associated HLH, P < 0.001). In Cox regression analysis, platelet count < 100,000/ μ l (hazard ratio 4.472, 95% confidence interval 1.324–15.107, P = 0.016) correlated with increased mortality in patients with primary HLH.ConclusionVL is a potential source of secondary HLH in regions with high endemicity. Treatment of the underlying disease in VL-associated HLH is sufficient in most cases, with no need to start etoposide-based chemotherapy.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation and tissue destruction due to abnormal immune activation
Despite a high prevalence of Visceral leishmaniasis (VL) in Southern Iran [11], there are few reports of VLassociated HLH in our region [12, 13]. In this prospective study, we aimed to describe the clinical and laboratory findings and the outcome of children diagnosed with VL-associated HLH during a 4-year followup period compared to patients with primary HLH
Binary logistic regression revealed that only age < 2 years (Odds ratio (OR) 6.446, 95% confidence interval (CI) 1.538–27.012, P = 0.011) increased the odds of Central nervous system (CNS) involvement
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation and tissue destruction due to abnormal immune activation. The absence of normal downregulation by activated macrophages and lymphocytes is possibly the cause of hyper inflammation and dysregulated immune state [1]. HLH presents as a febrile illness associated with multiple organ involvement. The diagnosis of HLH is primarily based on fulfilling at least 5 out of 8 criteria based on the published diagnostic criteria used in the HLH-2004 trial [4]. Homozygosity or compound heterozygosity for verified HLH-associated mutations will confirm the diagnosis [5]. A scoring system has been developed to generate a diagnostic score referred to as “HScore,” which estimates the probability of HLH (Table 1). Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation. We aimed to describe the clinical and laboratory findings of HLH patients secondary to Visceral leishmaniasis (VL) and their treatment outcome during a 4-year follow-up period compared to primary HLH
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