Pulmonary hypertension (PH) is severe complication of hematopoietic stem cell transplantation (HSCT) in children that is often underestimated; however, aggressive and timely treatment can improve survival, according to the results of a retrospective study published in the European Respiratory Journal.1

The incidence of PH after HSCT has been estimated to be between 15% and 28%. PH after HSCT is frequent in malignant osteoporosis, suggesting that the underlying disease could be a risk factor for developing PH after HSCT.2,3 Prognosis is poor in these patients, and the risk factors for developing PH after HSCT and the predictors of outcomes are unknown.1

Therefore, researchers retrospectively reviewed 31 cases of children referred for suspected PH after HSCT at a children’s hospital in Paris, France. They found that 22 patients had confirmed PH. Among the 13 responders at reactivity test, only 1 patient responded to calcium channel blockers. Overall, 7 (32%) patients died and 15 patients with PH were alive after a mean follow-up of 6.5±2.3 years (range, 2-10 years). All survivors were weaned off PH treatment after a median follow-up of 5 months (range, 3-16 months). In addition, the researchers observed a longer delay between clinical symptoms and initiation of PH therapy in patients who subsequently died (33.5±23 days) compared with survivors (7±3 days; P <.001).

“Pulmonary hypertension is a rare and severe complication of hematopoietic stem cell transplantation with a high mortality if misdiagnosed,” the researchers concluded.1

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“Early diagnosis and aggressive treatment allow a rapid clinical improvement with progressive normalization of pulmonary arterial pressure in most cases.”

References

1. Levy M, Moshous D, Szezepanski I, et al. Pulmonary hypertension after bone marrow transplantation in children [published online October 24, 2019]. Eur Respir J. doi:10.1183/13993003.00612-2019

2. Steward CG, Pellier I, Mahajan A, et al. Severe pulmonary hypertension: a frequent complication of stem cell transplantation for malignant infantile osteoporosis. Br J Haematol. 2004;124:63-71.

3. Schechter T, Leucht S, Bouffet E, et al. Pulmonary hypertensive vasculopathy following tandem autologous transplantation in pediatric patients with central nervous system tumors. Biol Blood Marrow Transplant. 2013;19:235-239.