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Case Reports
. 2016 Dec;6(4):614-615.
doi: 10.1086/688490.

A case of pulmonary arterial hypertension associated with adult hemophagocytic lymphohistiocytosis

Julius Koifman  1 , John Granton  2 , John Thenganatt  2
Affiliations
Case Reports

A case of pulmonary arterial hypertension associated with adult hemophagocytic lymphohistiocytosis

Julius Koifman et al. Pulm Circ. 2016 Dec.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH. To our knowledge, PAH associated with adult HLH has only been described in the literature once before. PAH may now be a potential differential diagnosis for patients with HLH who present with respiratory symptoms.

Keywords: hemophagocytic lymphohistiocytosis; hemophagocytosis; pulmonary hypertension; stem cell transplantation.

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References

    1. Galie N, Humbert M, Vachiery J, Gibbs S, Lang I, Torbicki A, Simonneau G, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2015;46:1855–1856. - PubMed
    1. Gerard LM, Xing K, Sherifi I, Granton J, Barth D, Abdelhaleem M, Bergeron C, Weitzman S, Cserti-Gazdewich C. Adult hemophagocytic lymphohistiocytosis with severe pulmonary hypertension and a novel perforin gene mutation. Int J Hematol 2012;95(4):445–450. - PubMed
    1. Zeilhofer U, Ashworth M, Amrolia P, Rao A, Chiesa R, Veys P, Rao K. Pulmonary hypertension following haematopoietic stem cell transplantation for primary haemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2013;60(3):521–523. - PubMed
    1. Scott RB, Robb-Smith AH. Histiocytic medullary reticulocytosis. Lancet 1939;2:194–198.
    1. Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant 2010;16:S82–S89. - PubMed

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