A case report on the first confirmed case of PNH in Ethiopia
PNH
Keywords:
PNH paroxysmal nocturnal hemoglobinuriaAbstract
- PNH is an acquired hemolytic disorder caused by somatic mutation characterized by intravascular hemolysis with pancytopenia and a tendency to thrombosis. It is a rare disease with incidence of 1 to 10 cases per million populations. We are reporting the first confirmed case of PNH in Ethiopia. He is a 38 years old man who presented with anemia and jaundice of 3 years duration; he has mild pallor; lab examinations showed moderate anemia with features of coomb’s negative intravascular hemolysis; and finally very large clone of PNH cells on immunophenotypic analysis
References
References
Luzzatto L, Bessler M, Rotoli B: Somatic mutations in paroxysmal nocturnal hemoglobinuria: a blessing in disguise? Cell. 1997; 88(1): 1–4
Screzenmeir H, Muus P, Socie G, et al. Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry. Hematologica 2014; 99:922
Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005; 106: 3699-3709.
Fletcher M, Sutherland DR, Whitby L, et al. Standardizing Leucocyte PNH clone detection: An international study. Cytometry B Clin Cytom 2014.
Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med 1995; 333: 1253-58.
S. Hussain, A. Qureshi, J. Kazi: Renal involvement in paroxysmal nocturnal hemoglobinuria. Nephron Clin Pract 2013; 123:28–35
Garrick Laudin, Lizemarie Wium: A case of paroxysmal nocturnal hemoglobinuria (PNH) in an obstetric patient: A South African perspective. CMI 2019; 13(1)
Issaragrisil S, Piankijagum A, Tang-naitrisorana Y: Corticosteroids therapy in paroxysmal nocturnal hemoglobinuria. Am J Hematol 1987; 25: 77-83
McKeage K: Eculizumab: a review of its use in paroxysmal nocturnal haemoglobinuria. Drugs. 2011; 71(17): 2327–45.
Dinerstein C. Why Is Soliris the Most Expensive Drug In The US? American Council in Science and Health. May 27, 2017.
Luzzatto L, Bessler M, Rotoli B: Somatic mutations in paroxysmal nocturnal hemoglobinuria: a blessing in disguise? Cell. 1997; 88(1): 1–4
Screzenmeir H, Muus P, Socie G, et al. Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry. Hematologica 2014; 99:922
Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005; 106: 3699-3709.
Fletcher M, Sutherland DR, Whitby L, et al. Standardizing Leucocyte PNH clone detection: An international study. Cytometry B Clin Cytom 2014.
Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med 1995; 333: 1253-58.
S. Hussain, A. Qureshi, J. Kazi: Renal involvement in paroxysmal nocturnal hemoglobinuria. Nephron Clin Pract 2013; 123:28–35
Garrick Laudin, Lizemarie Wium: A case of paroxysmal nocturnal hemoglobinuria (PNH) in an obstetric patient: A South African perspective. CMI 2019; 13(1)
Issaragrisil S, Piankijagum A, Tang-naitrisorana Y: Corticosteroids therapy in paroxysmal nocturnal hemoglobinuria. Am J Hematol 1987; 25: 77-83
McKeage K: Eculizumab: a review of its use in paroxysmal nocturnal haemoglobinuria. Drugs. 2011; 71(17): 2327–45.
Dinerstein C. Why Is Soliris the Most Expensive Drug In The US? American Council in Science and Health. May 27, 2017.
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Published
2020-12-31
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Case Report