Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China

Abstract

BackgroundHemophagocytic lymphohistiocytosis (HLH) is a relatively rare but life-threatening disease with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality.MethodsTo improve the recognition as well as understanding of this disorder, we analyzed clinical characteristics and prognostic factors from 85 adult patients diagnosed with HLH in our hospital from April 2005 to June 2014.ResultsPatients with HLH displayed variable clinical markers across a wide spectrum. These included fever and hyperferritinemia (100%), elevated lactate dehydrogenase (LDH) (98.8%), two or three cytopenia (92.2%), splenomegaly (72.9%), hypofibrinogenemia (69.4%), hypertriglyceridemia (64.7%), hemophagocytosis (51.7%), and hepatomegaly (24.7%). Patients with active Epstien-Barr Virus (EBV) infection had a median overall survival (OS) of 65 days. Those displaying malignancy had very poor survival (median OS: 40 days). However, patients in rheumatic and non-EBV infection groups had relatively superior prognosis (not reached). Univariate analysis showed that Fibrinogen (Fbg) <1.5 g/L, platelet number (PLT) <40 × 109/L and LDH ≥1000 U/L were factors that negatively affected survival (P = 0.004, 0.000, 0.002). Multivariate analysis showed that PLT <40 × 109/L was the independent adverse factor (HR = 0.350, 95% CI: 0.145-0.844, P = 0.019).ConclusionsHLH had very complex clinical manifestations and high death rate. Patients with active EBV infection, malignancy, Fbg <1.5 g/L, PLT <40 × 109/L and LDH ≥1000 U/L had high risk of death as well as inferior survival, and these patients require systemic targeted treatments as early as possible.

Hemophagocytic lymphohistiocytosis secondary to infections: a tertiary care experience

“…31 Age, clinical features, lab abnormalities like ferritin level and cytopenia were in concordance with other studies. 32,33 Lactate dehydrogenase (LDH) is one finding not included in diagnostic criteria but elevated in all our patients similar to studies done by Fei Li et al and Riviere. 31,33 Low glycosylated Ferritin was found to be useful in assisting the diagnosis in patients with ferritin values between 500 and 10,000 µg/L.…”

“…32,33 Lactate dehydrogenase (LDH) is one finding not included in diagnostic criteria but elevated in all our patients similar to studies done by Fei Li et al and Riviere. 31,33 Low glycosylated Ferritin was found to be useful in assisting the diagnosis in patients with ferritin values between 500 and 10,000 µg/L. 34,35 Bone Marrow (BM) score is based upon number of hemophagocytic cells per high power field, and classified as mild (1-2), moderate (3-5), severe (6)(7)(8), very severe (˃8).…”

“…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.…”

Between a rock and a hard place

“…The mutations identified to date lead to abnormalities in the perforin cytotoxic pathway causing decreased function of NK-and/or cTC, indicating a pivotal role of this event in the pathogenesis of HLH [2]. Recently, there have been increasing reports of adult onset HLH in the context of conditions associated with immune dysregulation: hematologic malignancies, infections, autoimmune diseases, organ/ stem cell transplants and chemo-or immunosuppressive therapies [3][4][5][6][7]. Some of the cases affecting young adults represent attenuated familial forms of the disease due to partial loss of function of the HLH-causing genes [8], while in older adults (>60 yr.) malignancy associated HLH is most common.…”

Limited Role of Random Skin Biopsy in the Diagnosis of Intravascular Lymphoma in Adult Patients with Hemophagocytic Lymphohistiocytosis

“…Malignancy-associated HLH has been identified as an independent risk factor for a worse prognosis, with survival rates as low as 12% at the 5-year follow-up [1,2]. While natural killer (NK)/T-cell lymphoma has been reported as the most common type of malignancy associated with HLH [3], other malignancies may also be associated with HLH [4].…”