Abdominal Pain in Combination with an Unexplained Hemolytic Anemia Are Crucial Signs to Test for Paroxysmal Nocturnal Hemoglobinuria: A Case Report

Overview

Journal Clin Case Rep

Date 2019 Jan 19

PMID 30656036

Citations 1

Authors

N S Elias

Jurgen Riedl

Karlijn Stouten

Mark-David Levin

Frank Wolfhagen

Mathijs Eefting

Henricus Jan Vermeer

Affiliations

Soon will be listed here.

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH), a rare benign hematological disorder, presents with a wide variety of clinical symptoms. A direct Coombs-negative hemolytic anemia combined with an increased LDH = Lactate dehydrogenase level are signs to test for PNH. Follow-up does not need any microscopic review's only flow cytometric PNH clone size.

Citing Articles

Abdominal pain in combination with an unexplained hemolytic anemia are crucial signs to test for paroxysmal nocturnal hemoglobinuria: A case report.

Elias N, Riedl J, Stouten K, Levin M, Wolfhagen F, Eefting M Clin Case Rep. 2019; 7(1):175-179.

PMID: 30656036 PMC: 6333074. DOI: 10.1002/ccr3.1771.

References

Pu J, Brodsky R . Paroxysmal nocturnal hemoglobinuria from bench to bedside. Clin Transl Sci. 2011; 4(3):219-24. PMC: 3128433. DOI: 10.1111/j.1752-8062.2011.00262.x. View

Sutherland D, Illingworth A, Keeney M, Richards S . High-Sensitivity Detection of PNH Red Blood Cells, Red Cell Precursors, and White Blood Cells. Curr Protoc Cytom. 2015; 72:6.37.1-6.37.29. DOI: 10.1002/0471142956.cy0637s72. View

Hillmen P, Muus P, Roth A, Elebute M, Risitano A, Schrezenmeier H . Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2013; 162(1):62-73. PMC: 3744747. DOI: 10.1111/bjh.12347. View

Hill A, Kelly R, Hillmen P . Thrombosis in paroxysmal nocturnal hemoglobinuria. Blood. 2013; 121(25):4985-96. DOI: 10.1182/blood-2012-09-311381. View

Maciejewski J, Rivera C, Kook H, Dunn D, Young N . Relationship between bone marrow failure syndromes and the presence of glycophosphatidyl inositol-anchored protein-deficient clones. Br J Haematol. 2002; 115(4):1015-22. DOI: 10.1046/j.1365-2141.2001.03191.x. View

Peffault de Latour R, Mary J, Salanoubat C, Terriou L, Etienne G, Mohty M . Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories. Blood. 2008; 112(8):3099-106. DOI: 10.1182/blood-2008-01-133918. View

Parker C . Management of paroxysmal nocturnal hemoglobinuria in the era of complement inhibitory therapy. Hematology Am Soc Hematol Educ Program. 2011; 2011:21-9. DOI: 10.1182/asheducation-2011.1.21. View

Lee J, Jang J, Kim J, Yoon S, Lee J, Kim Y . Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. Int J Hematol. 2013; 97(6):749-57. DOI: 10.1007/s12185-013-1346-4. View

Parker C . Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria. Hematology Am Soc Hematol Educ Program. 2016; 2016(1):208-216. PMC: 6142517. DOI: 10.1182/asheducation-2016.1.208. View

10.

Schrezenmeier H, Muus P, Socie G, Szer J, Urbano-Ispizua A, Maciejewski J . Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry. Haematologica. 2014; 99(5):922-9. PMC: 4008114. DOI: 10.3324/haematol.2013.093161. View

11.

Borowitz M, Craig F, DiGiuseppe J, Illingworth A, Rosse W, Sutherland D . Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry. Cytometry B Clin Cytom. 2010; 78(4):211-30. DOI: 10.1002/cyto.b.20525. View

12.

Elias N, Riedl J, Stouten K, Levin M, Wolfhagen F, Eefting M . Abdominal pain in combination with an unexplained hemolytic anemia are crucial signs to test for paroxysmal nocturnal hemoglobinuria: A case report. Clin Case Rep. 2019; 7(1):175-179. PMC: 6333074. DOI: 10.1002/ccr3.1771. View

13.

Risitano A . Paroxysmal nocturnal hemoglobinuria and the complement system: recent insights and novel anticomplement strategies. Adv Exp Med Biol. 2013; 735:155-72. DOI: 10.1007/978-1-4614-4118-2_10. View