Pulmonary complications of hemoglobinopathies

Rekha Vij, Roberto Machado

Research output: Contribution to journalReview article

29 Citations (Scopus)

Abstract

Hemoglobinopathies are diseases caused by genetic mutations that result in abnormal, dysfunctional hemoglobin molecules or lower levels of normal hemoglobin molecules. The most common hemoglobinopathies are sickle cell disease (SCD) and the thalassemias. In SCD, an abnormal hemoglobin alters the erythrocyte, causing a chronic hemolytic anemia, which can lead to pulmonary parenchymal damage and impaired vascular function. Pulmonary complications of SCD include the acute chest syndrome (ACS), reactive airways disease, pulmonary hypertension (PH), and pulmonary fibrosis. Episodes of ACS and the development of PH both increase the risk of death in patients with SCD. Both α and β thalassemia are characterized by impaired production of hemoglobin subunits, and severity of disease varies widely. Although screening studies suggest that PH is a common complication for patients with thalassemia, its impact on survival is unknown. Understanding the pathogenesis, diagnostic options, and prevention and treatment strategies for such complications is critical for clinicians who care for these patients. In this review, we discuss the mechanisms and clinical presentation of pulmonary complications associated with hemoglobinopathies, with a focus on recent advances in pathogenesis and treatment.

Original languageEnglish (US)
Pages (from-to)973-983
Number of pages11
JournalChest
Volume138
Issue number4
DOIs
StatePublished - Oct 1 2010
Externally publishedYes

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Hemoglobinopathies
Sickle Cell Anemia
Thalassemia
Acute Chest Syndrome
Pulmonary Hypertension
Abnormal Hemoglobins
Lung
Hemoglobin Subunits
Inborn Genetic Diseases
Pulmonary Fibrosis
Hemolytic Anemia
Blood Vessels
Patient Care
Hemoglobins
Erythrocytes
Mutation
Survival
Therapeutics

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Pulmonary complications of hemoglobinopathies. / Vij, Rekha; Machado, Roberto.

In: Chest, Vol. 138, No. 4, 01.10.2010, p. 973-983.

Research output: Contribution to journalReview article

Vij, Rekha ; Machado, Roberto. / Pulmonary complications of hemoglobinopathies. In: Chest. 2010 ; Vol. 138, No. 4. pp. 973-983.
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