Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders

Clin Chest Med. 2013 Dec;34(4):739-52. doi: 10.1016/j.ccm.2013.08.006. Epub 2013 Oct 17.


Pulmonary hypertension (PH) has emerged as a major complication of several hematologic disorders, including hemoglobinopathies, red cell membrane disorders, chronic myeloproliferative disorders, and splenectomy. With the exception of sickle cell disease, there are a limited number of studies systematically evaluating the prevalence of PH using the gold standard right heart catheterization in these disorders. The cause of the PH in patients with hematologic disorders is multifactorial, and a thorough diagnostic evaluation is essential. More importantly, there are virtually no high-quality data on the safety and efficacy of PH-targeted therapy in this patient population.

Keywords: Myeloproliferative disorders; Pulmonary hypertension; Sickle cell disease; Splenectomy; Thalassemia.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Anemia, Hemolytic / complications*
  • Anemia, Sickle Cell / complications
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Risk Factors
  • Splenectomy / adverse effects