Reversal of pre-capillary pulmonary hypertension in a patient with sickle cell anemia who underwent haploidentical peripheral blood stem cell transplantation

In patients with sickle cell disease (SCD), deoxygenated sickle hemoglobin (HbS) polymerizes, resulting in rigid, adherent RBC that obstruct microcirculation and cause hemolysis. Patients

experience many acute and chronic complications, including pain crises, acute chest syndrome and pulmonary hypertension (PH), among others.

PH remains a serious complication in SCD, and has been reported as a major cause of morbidity and mortality.1, 2, 3 It is defined as a resting mean pulmonary arterial pressure (mPAP) ⩾25 mm 

Hg measured by right heart catheterization (RHC),4, 5 and is estimated to impact 6–11% of adults with SCD.5, 6 Transthoracic echocardiography (ECHO) estimates pulmonary arterial systolic

pressure via the surrogate tricuspid regurgitant velocity (TRV) to screen for PH.6, 7, 8 Similarly, elevated N-terminal-pro brain natriuretic peptide (NT-proBNP) levels have been associated

with increased TRV in adults with SCD;9 furthermore, NT-proBNP level of 160 ng/L or higher was independently associated with mortality.9 While TRV and NT-proBNP are indirect estimates of

pulmonary pressure, RHC is required for a definitive diagnosis of PH. For the purposes of this report, RHC, 6-min walk tests (6MWT), NT-proBNP and ECHO values were retrieved to monitor the

change in estimated pulmonary arterial pressure in the patient presented.

This research was supported by the intramural programs of the National Heart, Lung and Blood Institute, and the National Institute of Diabetes, and Digestive and Kidney Diseases.

Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD, USA

Molecular and Clinical Hematology Branch, National Institute of Diabetes and Digestive and Kidney Diseases/National Heart, Lung, and Blood Institute, NIH, Bethesda, MD, USA

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