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Priapism, hemoglobin desaturation, and red blood cell adhesion in men with sickle cell anemia.

ABSTRACT: Priapism is a serious, but episodic, complication of sickle cell disease (SCD). We had previously reported that subjects with SCD had variable red blood cell (RBC) adhesion to the immobilized sub-endothelial protein laminin (LN). We examined adhesion to LN in a microfluidic device, of RBCs from men with homozygous sickle cell anemia. Adhesion under hypoxic, but not ambient, conditions was greater in men with a history of priapism, with median adhesion of 529 RBCs per 32?mm2/unit area (range 5-5248) rising to 3268 RBCs per 32?mm2/unit area (range 49-18,368, P?=?0.004), under ambient and hypoxic conditions, respectively (n?=?14). This was not seen in RBCs from men without a history of priapism (median 402 (range 14-785) and 122 (range 31-4112) RBCs per 32?mm2/unit area, ambient and hypoxic conditions, respectively (P?=?N.S., N?=?12)). We also observed an association between hypoxia-enhanced RBC adhesion in vitro and a history of hemoglobin desaturation in vivo independent of priapism. Prolonged Hb desaturation may increase sickle polymer formation and RBC damage, resulting in enhanced RBC adhesion, hemolysis, and endothelial dysfunction. The identification of distinct RBC phenotypes could prompt clinical evaluation for suitability for novel or under-used therapies, like oxygen.

SUBMITTER: Yuan C

PROVIDER: S-EPMC6744332 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature

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Priapism, hemoglobin desaturation, and red blood cell adhesion in men with sickle cell anemia.

Yuan Charlotte C Quinn Erina E Kucukal Erdem E Kapoor Sargam S Gurkan Umut A UA Little Jane A JA

Blood cells, molecules & diseases 20190805

Priapism is a serious, but episodic, complication of sickle cell disease (SCD). We had previously reported that subjects with SCD had variable red blood cell (RBC) adhesion to the immobilized sub-endothelial protein laminin (LN). We examined adhesion to LN in a microfluidic device, of RBCs from men with homozygous sickle cell anemia. Adhesion under hypoxic, but not ambient, conditions was greater in men with a history of priapism, with median adhesion of 529 RBCs per 32 mm<sup>2</sup>/unit area ...[more]

PMID: 31404907

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Project description:BackgroundSickle cell disease comprises a group of genetic haemoglobin disorders. The predominant symptom associated with sickle cell disease is pain resulting from the occlusion of small blood vessels by abnormally 'sickle-shaped' red blood cells. There are other complications, including chronic organ damage and prolonged painful erection of the penis, known as priapism. Severity of sickle cell disease is variable, and treatment is usually symptomatic. Priapism affects up to half of all men with sickle cell disease, however, there is no consistency in treatment. We therefore need to know the best way of treating this complication in order to offer an effective interventional approach to all affected individuals. This is an update of a previously published review.ObjectivesTo assess the benefits and risks of different treatments for stuttering (repeated short episodes) and fulminant (lasting for six hours or more) priapism in sickle cell disease.Search methodsWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also searched trial registries. Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 09 September 2019. Date of most recent search of trial registries and of Embase: 01 October 2019.Selection criteriaAll randomised or quasi-randomised controlled trials comparing non-surgical or surgical treatment with placebo or no treatment, or with another intervention for stuttering or fulminant priapism.Data collection and analysisThe authors independently extracted data and assessed the risk of bias of the trials.Main resultsThree trials with 102 participants were identified and met the criteria for inclusion in this review. These trials compared stilboestrol to placebo, sildenafil to placebo and a four-arm trial which compared ephedrine or etilefrine to placebo and ranged in duration from two weeks to six months. All of the trials were conducted in an outpatient setting in Jamaica, Nigeria and the UK. None of the trials measured our first primary outcome, detumescence. However, all three trials reported on the reduction in frequency of stuttering priapism, our second primary outcome; and from the evidence included in this review, we are uncertain whether stilboestrol, etilefrine or ephedrine reduce the frequency of stuttering priapism as the certainty of the evidence has been assessed as very low. Additionally, we conclude that sildenafil may make little or no difference (low-certainty evidence). Two trials reported on immediate side effects and we are uncertain whether etilefrine or ephedrine reduce the occurrence of these (very low-certainty of evidence) and also conclude that sildenafil may make little or no difference in side effects (low-quality evidence). Given that all of the trials were at risk of bias and all had low participant numbers, we considered the certainty of the evidence to be low to very low.Authors' conclusionsThere is a lack of evidence for the benefits or risks of the different treatments for both stuttering and fulminant priapism in sickle cell disease. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions for priapism in sickle cell disease.

Dataset Information

Priapism, hemoglobin desaturation, and red blood cell adhesion in men with sickle cell anemia.

Publications

Priapism, hemoglobin desaturation, and red blood cell adhesion in men with sickle cell anemia.

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