# Unexpected Death of a Sickle Cell Disease Patient: A Case Report and Literature Review

**Authors:** Gang Zhou, Amin Mohammad, Yuan Shan

PMC · DOI: 10.7759/cureus.62645 · 2024-06-18

## TL;DR

This paper presents a case of unexpected death in a sickle cell disease patient to highlight the ongoing challenges in preventing mortality despite medical advancements.

## Contribution

The novelty lies in presenting a specific case of unexpected death to emphasize the need for improved intervention strategies in sickle cell disease.

## Key findings

- SCD remains associated with high mortality due to complications like sepsis and acute chest syndrome.
- Despite medical progress, preventing death in young SCD patients remains a significant challenge.
- The case underscores the urgent need for continued research and better intervention strategies.

## Abstract

Sickle cell disease (SCD) is an autosomal recessive genetic disorder characterized by the abnormal formation of sickle hemoglobin (HbS). Under conditions of deoxygenation, HbS undergoes polymerization, resulting in microvascular occlusion, tissue hypoxia, and infarction. The elevated mortality rate associated with SCD is primarily attributed to complications such as sepsis, acute chest syndrome, stroke, acute multiorgan failure, and pulmonary hypertension. Despite advancements in awareness and treatments, preventing mortality in young individuals with SCD remains a formidable challenge. In an effort to shed light on these challenges, we present a case of unexpected death associated with SCD to emphasize the pressing need for continued research and intervention strategies to improve patient outcomes.

## Linked entities

- **Diseases:** sickle cell disease (MONDO:0011382), acute chest syndrome (MONDO:0005632), stroke (MONDO:0005098), pulmonary hypertension (MONDO:0005149)

## Full-text entities

- **Diseases:** sepsis (MESH:D018805), autosomal recessive genetic disorder (MESH:D030342), stroke (MESH:D020521), infarction (MESH:D007238), acute chest syndrome (MESH:D056586), pulmonary hypertension (MESH:D006976), SCD (MESH:D000755), multiorgan failure (MESH:D051437), Unexpected Death (MESH:D000080485), tissue hypoxia (MESH:D000860), microvascular occlusion (MESH:D017566)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11258939/full.md

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Source: https://tomesphere.com/paper/PMC11258939