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Paroxysmal nocturnal haemoglobinuria. Experience over a 10 years period
Author(s) -
Canalejo K.,
Riera Cervantes N.,
Felippo M.,
Sarandría C.,
Aixalá M.
Publication year - 2014
Publication title -
international journal of laboratory hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.705
H-Index - 55
eISSN - 1751-553X
pISSN - 1751-5521
DOI - 10.1111/ijlh.12156
Subject(s) - paroxysmal nocturnal hemoglobinuria , hemoglobinuria , clone (java method) , lactate dehydrogenase , hemolytic anemia , immunology , haemolysis , population , schistocyte , medicine , haptoglobin , hemolysis , platelet , thrombotic thrombocytopenic purpura , biology , biochemistry , dna , environmental health , enzyme
Summary Introduction Paroxysmal nocturnal hemoglobinuria ( PNH ) is a hemolytic, clonal and acquired disorder of the hematopoietic stem cell with a deficiency of all glycophosphatidyl‐inositol ( GPI ) linked proteins. The aim of this retrospective study was to analyse haematological and biochemical data from 152 patients referred to our laboratory for diagnosis of PNH by flow cytometry ( FC ). Methods Patients and healthy donor (152 and 99 respectively) were studied. Ham, sucrose, lactate dehydrogenase ( LDH ), Iron, haptoglobin (Hp), blood cell morphology and Kaplow cytochemical stain for leukocyte alkaline phosphatase ( LAP ) were carried out. GPI ‐proteins anti‐ CD 55 and CD 59 in erythrocytes and the former, plus anti CD 16b and CD 66b on neutrophils were evaluated by FC . Results Anemia and/or leukopenia and/or thrombocytopenia, increased reticulocyte count and LDH were observed in patients with PNH clone. Some of them had dacriocytes, schistocytes. LAP was low. On average, we detected 50% CD 59 (−) erythrocytes and 29, 83, 78% CD 55/59 (−), CD 16b (−), CD 66b (−) neutrophils, respectively. Conclusion Paroxysmal nocturnal hemoglobinuria clone was detected in 20/152 patients. Negative population's percentages were high in patients with classic PNH , Hematimetry, LAP and adequate use of CF contribute to PNH clone detection in the laboratory.