Sickle cell disease-associated pulmonary hypertension: an integrated framework linking pathologies, mechanisms, and clinical phenotypes.

Abstract excerpt

Sickle cell disease-associated pulmonary hypertension (SCD-PH) affects approximately 10% of adults with SCD and markedly increases mortality, yet mechanistic and haemodynamic heterogeneity complicates classification, trial design, and treatment selection. We propose an integrated framework linking five interacting axes-anaemia/high-output, haemolysis/haem/iron toxicity, hypoxia, inflammation, and thrombosis-to...

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