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Paroxysmal nocturnal hemoglobinuria in the differential diagnosis of thrombocytopenia

Fusun Gediz, Bahriye Kadriye Payzin, Ozlem Zekiye Cakmak, Yusuf Uzum, Damla Ernur, Fahri Sahin
  • Fusun Gediz
    Department of Hematology, Izmir Katip Celebi University, Ataturk Training and Research Hospital, Izmir; PNH Education and Study Gruop, Turkey
  • Bahriye Kadriye Payzin
    Department of Hematology, Izmir Katip Celebi University, Ataturk Training and Research Hospital, Izmir, Turkey
  • Ozlem Zekiye Cakmak
    Department of Internal Medicine Izmir Katip Celebi University, Ataturk Training and Research Hospital, Izmir, Turkey
  • Yusuf Uzum
    Department of Internal Medicine Izmir Katip Celebi University, Ataturk Training and Research Hospital, Izmir, Turkey
  • Damla Ernur
    Department of Hematology, Ege University, Izmir, Turkey
  • Fahri Sahin
    Department of Hematology, Izmir Katip Celebi University, Ataturk Training and Research Hospital, Izmir; Department of Hematology, Ege University, Izmir, Turkey | drfahrisahin@gmail.com

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease which diagnosis may be delayed due to variable clinical findings. We describe herein a case of PNH in a 21 year old woman who admitted with complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent abdominal pain. On laboratory tests thrombocytopenia and iron deficiency anemia without any clinical findings were found. Flow cytometric evaluations showed a PNH clone of 15% for erythrocytes, 64% for monocytes, and 60% for granulocytes. The patient was diagnosed with PNH and an eculizumab therapy was initiated. Following initiation of eculizumab therapy, the frequency of abdominal pain attacks decreased, hemoglobin level normalized, and platelet values increased slightly. In patients submitting with a triad of symptoms such as thrombocytopenia, iron deficiency anemia, and abdominal pain attacks of unknown etiology we suggest considering PNH. We also encourage physicians to share their similar observations in order to raise the knowledge on infrequent presentations of PNH.

Keywords

thrombocytopenia, abdominal pain, paroxysmal nocturnal hemoglobinuria

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Submitted: 2016-09-04 14:24:05
Published: 2017-03-22 10:32:01
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Copyright (c) 2017 Fusun Gediz, Bahriye Kadriye Payzin, Ozlem Zekiye Cakmak, Yusuf Uzum, Damla Ernur, Fahri Sahin

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