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Casano et al describe a case of refractory pulmonary hypertension with severe Bordetella pertussis infection.1 Their description of the literature is incomplete. We described four cases of fatal pulmonary hypertension (PHT) in a series of 13 critically ill infants with B pertussis.2 The cases that developed PHT all presented with severe hyperleukocytosis (WCC>100 × 109/l) which was unresponsive to all currently available modalities including extra-corporeal membrane oxygenation. Hyperleukocytosis was an independent predictor of death when corrected for presentation severity of illness. We suggested the existing histological evidence3 was such that extreme leukocytosis prediposes to the formation of lymphocyte aggregates in the pulmonary vasculature and increased pulmonary vascular resistance via obstruction rather than hypoxic vasoconstriction. Therefore Dr Casano’s recommendation for the early use of pulmonary vasodilators is unlikely to be sufficient in this context. We are assessing the impact of strategies aimed at reducing lymphocyte numbers and adhesion in addition to standard treatments for pulmonary hypertension.
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Authors’ reply
As Peters comments in his letter, we know that hyperleukocytosis has been postulated as a factor for pulmonary hypertension in Pertussis infection, but necessary brevity did not make it possible to report. Nevertheless, our patient never reached these values of leucocytosis; it’s possible, as in many other diseases, that several pathogenic mechanisms contribute to pulmonary hypertension, making a concomitant treatment approach necessary.