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Case Report

Cerebral Stroke in a Teenage Girl with Paroxysmal Nocturnal Hemoglobinuria

1
Specialistic Oncology Laboratory Unit, ARNAS Hospitals Civico, Di Cristina e Benfratelli, 90127 Palermo, Italy
2
Pediatric Hematology and Oncology Unit, Oncology Department, ARNAS Hospitals Civico, Di Cristina e Benfratelli, 90127 Palermo, Italy
3
Pediatric Neurology and Psychiatric Unit, ARNAS Hospitals Civico, Di Cristina e Benfratelli, 90127 Palermo, Italy
4
Department of Pediatrics, ARNAS Hospitals Civico, Di Cristina e Benfratelli, 90127 Palermo, Italy
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2017, 9(2), 7012; https://doi.org/10.4081/hr.2017.7012
Submission received: 22 December 2016 / Revised: 22 February 2017 / Accepted: 28 February 2017 / Published: 1 June 2017

Abstract

We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children’s hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL) with negative Coombs test, mild leucopenia (white blood cells 4.9 × 109/L) and thrombocytopenia (platelets 97 × 109/L) and high values of lactate dehydrogenase (2855 U/L) were identified; a packed red cells transfusion was administered. Her condition worsened and she subsequently presented complete right hemiplegia, aphasia and coma; magnetic resonance imaging revealed a massive ischemic lesion. A diagnosis of PNH was eventually made following high sensitivity flow cytometry, which identified a PNH clone (CD66b negative equal to 93.7% of granulocytes). Fast recovery from neurologic and hematological problems occurred in response to anticoagulant therapy and intravenous therapy with eculizumab. We are convinced that PNH should be included in the differential diagnosis of children presenting with cytopenia.
Keywords: case report; hemoglobinuria; thrombosis; fatigue; eculizumab case report; hemoglobinuria; thrombosis; fatigue; eculizumab

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MDPI and ACS Style

Gervasi, F.; D’Amelio, L.; Trizzino, A.; Ferraro, F.; Russo, D.; Santangelo, G.; Cardella, F.; Trizzino, A.; Di Marco, F.; Farruggia, P. Cerebral Stroke in a Teenage Girl with Paroxysmal Nocturnal Hemoglobinuria. Hematol. Rep. 2017, 9, 7012. https://doi.org/10.4081/hr.2017.7012

AMA Style

Gervasi F, D’Amelio L, Trizzino A, Ferraro F, Russo D, Santangelo G, Cardella F, Trizzino A, Di Marco F, Farruggia P. Cerebral Stroke in a Teenage Girl with Paroxysmal Nocturnal Hemoglobinuria. Hematology Reports. 2017; 9(2):7012. https://doi.org/10.4081/hr.2017.7012

Chicago/Turabian Style

Gervasi, Francesco, Lucia D’Amelio, Antonino Trizzino, Fabrizia Ferraro, Delia Russo, Giuseppe Santangelo, Francesca Cardella, Angela Trizzino, Floriana Di Marco, and Piero Farruggia. 2017. "Cerebral Stroke in a Teenage Girl with Paroxysmal Nocturnal Hemoglobinuria" Hematology Reports 9, no. 2: 7012. https://doi.org/10.4081/hr.2017.7012

APA Style

Gervasi, F., D’Amelio, L., Trizzino, A., Ferraro, F., Russo, D., Santangelo, G., Cardella, F., Trizzino, A., Di Marco, F., & Farruggia, P. (2017). Cerebral Stroke in a Teenage Girl with Paroxysmal Nocturnal Hemoglobinuria. Hematology Reports, 9(2), 7012. https://doi.org/10.4081/hr.2017.7012

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