The Role of Infection in the Pathogenesis of Vaso-Occlusive Crisis in Patients With Sickle Cell Disease.

Abstract

Sickle cell disease (SCD) is characterized by recurrent vaso-occlusive crisis (VOC). Patients with SCD have impaired immunity and are thus predispose to infections. The vast majority of SCD patients live in underdeveloped nations with high prevalence and transmission rates of infections. This makes the SCD patients prone to infections, which frequently precipitate VOC. We reviewed the role of infection in the pathogenesis of VOC, taking into consideration all potential mechanisms from previous studies and hypothetical perspectives. The potential mechanisms through which infections may lead to VOC involve several pathological changes including pneumonitis, pyrexia, acute phase reaction, hypercoagulability, neutrophilia, eosinophilia, thrombocytosis, bronchospasm, red cell cytopathic and membrane changes, auto-antibodies mediated red cell agglutination and opsonization, diarrhoea and vomiting, which may act singly or in concert to cause red cell sickling. These changes can induce sickling directly or indirectly through their adverse effects on Hb oxygenation and polymerization, hydration, blood viscosity, red cell metabolism, procoagulant activation, intercellular adherence and aggregation, culminating in VOC. There is therefore the need to ameliorate the burden of infection on SCD through immunization, prophylactic and therapeutic use of antimicrobials, barrier protection and vector control in communities with high prevalence of SCD.

Sickle cell anemia in Brazil: personal, medical and endodontic patterns

“…Additionally, neutrophilia in SCA individuals can be accompanied by eosinopoenia. 7,8 Our findings showed that only one SCA patient presented with neutrophilia. Despite the significant difference (p = 0.045) observed in the eosinophil counts when the groups with and without the need for endodontic treatment were compared, eosinopoenia was not observed among the eligible patients.…”

“…Neutrophilia is a useful marker of infection in many clinical settings, as well as in SCA, 7,8 because the prevalence and intensity of neutrophilia are higher in SCA patients with bacterial infections than in those without infections. Additionally, neutrophilia in SCA individuals can be accompanied by eosinopoenia.…”

“…5,6,7,8 Studies have shown an association between hematological factors and pathologic alterations in SCA. 9,10 The elevated white blood cell counts in sickle cell disease have been reported as being able to predict morbid events in patients with the disease.…”

Homozygous Sickle Cell Disease Presenting As Acute Pulmonary Infection: A Case Report

“…[13] It is therefore imperative that sickle cell disease patients with acute or chronic infections of the lungs must be closely monitored with blood gas analyzers so as to detect and correct hypoxia and its deleterious effect on red cell sickling. [14] …”

Multiple bone and joint disease in a sickle cell anaemia patient: a case report

“…While (b) may explain the femoral and humeral head necrosis in this patient, haematogenous bacteria infection (c) appears to take prominence in this index patient in that severe abdominal crisis which the patient presented with among others could allow seepage of normal gut flora into the blood stream. Haematogenous spread is possible because SCD patients have sluggish circulation of blood in bones and also have compromised immune status attributable to many inherent factors such as defective microbial opsonization, tissue infarction and splenic hypofunction 11,12…”