Which organ complication is most commonly responsible for mortality in adult patients with sickle cell disease?

Question

Accepted Answer

B. Acute chest syndrome progressing to pulmonary hypertension and cor pulmonale. ## Organ Complications and Mortality in Sickle Cell Disease

### Leading Cause of Death in Adults with SCD

**Key Point:** **Acute chest syndrome (ACS)** and its progression to pulmonary hypertension and cor pulmonale is the leading cause of mortality in adult patients with sickle cell disease.

### Acute Chest Syndrome: Pathophysiology

1. **Pulmonary infarction** from vaso-occlusion
2. **Fat embolism** from bone marrow necrosis
3. **Infection** (bacterial pneumonia, atypical organisms)
4. **In situ thrombosis** in pulmonary vessels
5. **Acute lung injury** from inflammatory cascade

### Pulmonary Complications Timeline

```mermaid
flowchart TD
  A[Sickle Cell Disease]:::outcome --> B[Recurrent ACS episodes]:::action
  B --> C[Chronic pulmonary damage]:::action
  C --> D[Pulmonary hypertension]:::outcome
  D --> E[Right ventricular dysfunction]:::action
  E --> F[Cor pulmonale]:::urgent
  F --> G[Cardiogenic shock & death]:::urgent
```

### Comparison of Major SCD Complications

| Complication | Age of Onset | Mortality Impact | Reversibility |
|--------------|--------------|------------------|---------------|
| Splenic sequestration | Childhood | High in children | Potentially reversible |
| Acute chest syndrome | Adolescence/Adult | **Highest in adults** | Reversible if early |
| Pulmonary hypertension | Adult | Very high | Irreversible |
| Stroke | Childhood/Adult | High | Irreversible |
| Osteonecrosis | Adult | Low | Irreversible but not fatal |

**High-Yield:** Pulmonary hypertension develops in 30–40% of adults with SCD and is associated with a 4-fold increase in mortality risk. It is the single most important predictor of poor prognosis in adult SCD.

**Clinical Pearl:** Elevated tricuspid regurgitant jet velocity (TRV) on echocardiography is used to screen for pulmonary hypertension in SCD patients; those with TRV >2.8 m/s have significantly reduced survival.

Answer

A. Acute splenic sequestration crisis

Answer

C. Osteonecrosis of the femoral head

Answer

D. Proliferative retinopathy with retinal detachment

Which organ complication is most commonly responsible for mortality in adult patients with sickle cell disease?

Explanation

Organ Complications and Mortality in Sickle Cell Disease

Leading Cause of Death in Adults with SCD

Acute Chest Syndrome: Pathophysiology

Pulmonary Complications Timeline

Comparison of Major SCD Complications

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Complication	Age of Onset	Mortality Impact	Reversibility
Splenic sequestration	Childhood	High in children	Potentially reversible
Acute chest syndrome	Adolescence/Adult	Highest in adults	Reversible if early
Pulmonary hypertension	Adult	Very high	Irreversible
Stroke	Childhood/Adult	High	Irreversible
Osteonecrosis	Adult	Low	Irreversible but not fatal