How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders

Evans M. Machogu, Roberto Machado

Research output: Contribution to journalArticle

Abstract

Hemoglobinopathies are caused by genetic mutations that result in abnormal hemoglobin molecules, resulting in hemolytic anemia. Chronic complications involving the lung parenchyma, vasculature, and cardiac function in hemoglobinopathies result in impaired gas exchange, resulting in tissue hypoxia. Hypoxia is defined as the deficiency in the amount of oxygen reaching the tissues of the body and is prevalent in patients with hemoglobinopathies, and its cause is often multifactorial. Chronic hypoxia in hemoglobinopathies is often a sign of disease severity and is associated with increased morbidity and mortality. Therefore, a thorough understanding of the pathophysiology of hypoxia in these disease processes is important in order to appropriately treat the underlying cause and prevent complications. In this article, we discuss management of hypoxia based on three different cases: sickle cell disease, b-thalassemia, and hereditary spherocytosis. These cases are used to review the current understanding of the disease pathophysiology, demonstrate the importance of a thorough clinical history and physical examination, explore diagnostic pathways, and review the current management.

Original languageEnglish (US)
Pages (from-to)1770-1780
Number of pages11
JournalBlood
Volume132
Issue number17
DOIs
StatePublished - Oct 25 2018

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Hemoglobinopathies
Tissue
Abnormal Hemoglobins
Hereditary Spherocytosis
Thalassemia
Hemolytic Anemia
Sickle Cell Anemia
Gases
Physical Examination
Oxygen
Molecules
Hypoxia
Morbidity
Lung
Mutation
Mortality

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders. / Machogu, Evans M.; Machado, Roberto.

In: Blood, Vol. 132, No. 17, 25.10.2018, p. 1770-1780.

Research output: Contribution to journalArticle

Machogu, Evans M. ; Machado, Roberto. / How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders. In: Blood. 2018 ; Vol. 132, No. 17. pp. 1770-1780.
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