A Retrospective Analysis of the Significance of Haemoglobin SS and SC in Disease Outcome in Patients With Sickle Cell Disease and Dengue Fever

Abstract

BackgroundLittle is known about the significance of haemoglobin genotype in dengue fever severity. This study was undertaken to determine the case fatality ratio and the impact of genotype in patients with sickle cell disease and confirmed dengue fever.MethodsThis retrospective analysis included 40 patients with confirmed dengue and sickle cell disease, during the study period (2010–2012).FindingsThere was a significantly higher case fatality ratio, 12.5% among patients with either haemoglobin SC disease or homozygous SS disease when compared to that of the general population 0.41% (p < 0.0001). The unadjusted odds of dying among those with haemoglobin SC disease compared with the group with homozygous SS disease was OR = 4.4 (95% CI 0.6 to 31.7). The predictors of mortality independent of sickle cell disease genotype were haemoglobin concentration at presentation OR = 0.57 (95% CI, 0.35 to 0.94) and the change in haemoglobin concentration from steady state OR = 0.59 (95% CI, 0.37 to 0.94). Adjusting for haemoglobin concentration at presentation increased the risk of death for the SC genotype relative to SS genotype OR = 13.4 (95% CI 1.1 to 160.3).InterpretationThe risk of fatal dengue may be higher among patients with a relatively mild genotype (haemoglobin SC).

Fatal Sickle Cell Disease and Zika Virus Infection in Girl from Colombia

“…Reports of patients co-infected with DENV and CHIKV are rare, few details are available, and mostly restricted to few fatal cases of dengue ( 9 ). In patients with dengue, deaths might be higher among those who have a hemoglobin SC genotype, as recently reported ( 10 ). Onset of vaso-occlusion in persons with sickle cell disorders is often triggered by inflammation, as has been reported in DENV infections and which probably occurred in our patient ( 8 ).…”

Vaso‐occlusive crisis in a sickle cell patient after transfusion‐transmitted dengue infection

“…A retrospective analysis in Jamaica found a higher case‐fatality rate in SCD patients when compared to that in the general population (12.5% vs 0.41%, P < .0001) and also described a higher risk of death in the SC genotype comparing to the SS genotype. 8 Similarly, a study performed in Guadeloupe, French Guiana, and Martinique in a pediatric population found a higher risk of severe dengue in SCD patients (57.1% for SC vs 37.1% for SS and 0% for controls, P < .001) and a higher case‐fatality rate (14.2% for SC, 2.8% for SS, and 0% for controls, P = .02) 10 …”

“…A systematic review and meta‐regression analysis of 77 studies on DF found a 1.14% case‐fatality rate in the general population 13 . In contrast, case reports and retrospective studies performed during dengue outbreaks in the Caribbean region indicate a higher risk of severe dengue and a high case‐fatality rate in SCD patients 8,14‐16 . A retrospective analysis in Jamaica found a higher case‐fatality rate in SCD patients when compared to that in the general population (12.5% vs 0.41%, P < .0001) and also described a higher risk of death in the SC genotype comparing to the SS genotype.…”

“…Red blood cell (RBC) transfusion is still the main therapeutic alternative for both acute and chronic complications of sickle cell disease (SCD). Despite the lack of large studies on DF in SCD patients, the few published articles on the topic suggest a higher risk of severe clinical outcome and a higher case‐fatality rate in such patients 8‐10 . Herein, we report the first case, as far as we know, of a patient with sickle cell anemia who developed DF transmitted by blood transfusion.…”

Risk of severe dengue is higher in patients with sickle cell disease: a scoping review

“…This is in contrast to dengue haemorrhagic fever in patients without SCD where an increasing haematocrit heralds severe dengue..Adjusting for haemoglobin concentration at presentation increased the risk of death for the SC genotype relative to SS genotype with an OR = 13.4 (95% CI 1.1 to 160.3). 6 From the limited data available to date, it appears that the risk of fatal dengue may be higher among patients with a relatively mild genotype (haemoglobin SC), although no firm conclusions can be drawn on the incidence and extent of risk. The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, tribal regions of India and the Middle East.…”