Atypical Overt Hemorrhagic Stroke in an Adult With Sickle Cell-Hemoglobin C Disease: A Report of a Rare Case

TL;DR

This paper reports a rare case of a hemorrhagic stroke in an adult with sickle cell-hemoglobin C disease, challenging the belief that this condition is mild.

Contribution

The paper presents a rare clinical case of overt hemorrhagic stroke in an adult with HbSC disease.

Findings

An adult with HbSC disease experienced a hemorrhagic stroke during a sickle cell crisis.

The case demonstrates that HbSC disease can lead to severe neurological complications.

The patient developed chronic neurological deficits requiring skilled nursing care.

Abstract

Sickle cell disease (SCD) is an inherited hemoglobinopathy in which deformed erythrocytes impair microvascular blood flow, leading to complications across multiple organ systems. Variants of SCD exist, and a less common form involves the inheritance of one hemoglobin S allele and one hemoglobin C allele, manifesting as sickle cell-hemoglobin C (HbSC) disease. Although HbSC disease can cause significant sequelae, a rare but serious complication is hemorrhagic stroke. In such cases, chronic endothelial injury, vascular fragility, and abnormal hemodynamics associated with this hemoglobinopathy may predispose patients to vessel rupture, resulting in hemorrhage. We report the case of a 54-year-old man with HbSC disease who was admitted for an acute sickle cell crisis and subsequently experienced an overt hemorrhagic stroke. The patient developed chronic neurological deficits and remains under skilled nursing care. This case highlights the rare occurrence of hemorrhagic stroke in HbSC disease and challenges the perception that HbSC is a benign condition.

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