Causes, clinical symptoms, and outcomes of infectious diseases associated with hemophagocytic lymphohistiocytosis in Taiwanese adults

Abstract

The infectious diseases associated with HLH were diverse and resulted in a high mortality rate. Cases in which the patients were aged more than 50 years, developed DIC, and had persistent fever even after 3 days of being diagnosed with HLH showed poor prognosis.

Prognostic Factors and Long-Term Outcome in 52 Turkish Children With Hemophagocytic Lymphohistiocytosis*

“…In addition, we found that high DIC score (≥ 5) and age below 2 years at diagnosis increased the mortality risk approximately two-fold in univariate analysis in high-risk patients with refractory or relapsed HLH. DIC has been previously reported to be associated with increased mortality in adult patients with secondary HLH (40). It has been suggested that cytokine-induced tissue factor release from activated macrophages may contribute to the development of DIC in these patients (41).…”

“…The identification of prognostic risk factors for early death in HLH is a challenging task (36)(37)(38)(39)(40). A previous comprehensive study in 232 children with HLH has suggested that hyperbilirubinemia, thrombocytopenia, hyperferritinemia (> 2,000), and persistent fever at diagnosis and at 2 weeks of HLH treatment are important risk factors predicting early death (36).…”

Hemophagocytic Lymphohistiocytosis in Adults

“…In a large study of 96 adult patients from Taiwan with HLH, 30 were associated with infection. 4 The most common types of infection were viral (41%), mycobacterial (23%), bacterial (23%), and fungal (13%). The Epstein-Barr virus (EBV) is the most common viral pathogen linked to the development of HLH.…”

Hemophagocytic lymphohistiocytosis secondary to infections: a tertiary care experience

“…37 Mortality rates were significantly better in infection associated HLH. 31,38,33 An optimistic view is that we can expect good results from infection associated HLH as infection burden is high in India. The high mortality (80%) in present study could be due to delayed presentation and multiorgan dysfunction.…”

“…Poor prognostic markers are age more than 50 years, fever of more than 3 days duration and DIC (38), immunosuppression, high ferritin, triglycerides, increased transaminases, C-Reactive Protein (CRP), LDH, low haemoglobin, leukopenia, thrombocytopenia and low sodium levels. 31 Though our patient cohort were young all of them had most of the above mentioned markers which could be the reason for high mortality.…”