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Hematology Reports
Volume 10
Issue 1
10.4081/hr.2018.7523
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Case Report

Paroxysmal Nocturnal Hemoglobinuria: When Delay in Diagnosis and Long Therapy Occurs

by
Salvatrice Mancuso
1,*,
Giuseppe Sucato
1,
Melania Carlisi
2,
Marco Santoro
2,
Giuseppe Tarantino
1,
Emilio Iannitto
1,
Mariasanta Napolitano
1 and
Sergio Siragusa
1
1
Department of Oncology, Haematology Unit, University of Palermo, Via del Vespro 129, 90127 Palermo, Italy
2
Department of Surgical, Oncological and Stomatological Disciplines, University of Palermo, Via del Vespro 129, 90127 Palermo, Italy
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2018, 10(1), 7523; https://doi.org/10.4081/hr.2018.7523
Submission received: 5 December 2017 / Revised: 3 March 2018 / Accepted: 5 March 2018 / Published: 29 March 2018

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disorder characterized by hemolytic anemia, bone marrow failure and thrombosis, caused by a somatic mutation in PIG-A gene that results in the absence of CD55 and CD59, two important complement regulatory proteins. In this paper, a case of PNH is retrospectively examined looking for clinical and laboratory features, and the entire course of the disease from the onset of the symptoms is described, together with an adequate follow-up over a 7-years treatment period. In this case, the not specificity and the limited clinical relevance of the symptoms led to a delay in diagnosis. After thrombosis, Eculizumab therapy has been shown to be effective, and during seven years of follow-up no events have occurred that put the patient’s life at risk. A multidisciplinary approach is crucial in cases like this, in order to allow early diagnosis and minimize the risks for the patients.
Keywords: paroxysmal nocturnal hemoglo-binuria; thrombotic events; renal failure; Eculizumab paroxysmal nocturnal hemoglo-binuria; thrombotic events; renal failure; Eculizumab

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MDPI and ACS Style

Mancuso, S.; Sucato, G.; Carlisi, M.; Santoro, M.; Tarantino, G.; Iannitto, E.; Napolitano, M.; Siragusa, S. Paroxysmal Nocturnal Hemoglobinuria: When Delay in Diagnosis and Long Therapy Occurs. Hematol. Rep. 2018, 10, 7523. https://doi.org/10.4081/hr.2018.7523

AMA Style

Mancuso S, Sucato G, Carlisi M, Santoro M, Tarantino G, Iannitto E, Napolitano M, Siragusa S. Paroxysmal Nocturnal Hemoglobinuria: When Delay in Diagnosis and Long Therapy Occurs. Hematology Reports. 2018; 10(1):7523. https://doi.org/10.4081/hr.2018.7523

Chicago/Turabian Style

Mancuso, Salvatrice, Giuseppe Sucato, Melania Carlisi, Marco Santoro, Giuseppe Tarantino, Emilio Iannitto, Mariasanta Napolitano, and Sergio Siragusa. 2018. "Paroxysmal Nocturnal Hemoglobinuria: When Delay in Diagnosis and Long Therapy Occurs" Hematology Reports 10, no. 1: 7523. https://doi.org/10.4081/hr.2018.7523

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