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Clinical presentation, laboratory parameters, classification and flow cytometric analysis of patients of paroxysmal nocturnal haemoglobinuria in Pakistan


Hematology & Transfusion International Journal
Rafia Mahmood, Department of Haematology, Armed Forces Institute of Pathology, Pakistan
Saleem Ahmed Khan, Department of Haematology, Armed Forces Institute of Pathology, Pakistan
Muhammad Mukarram Bashir, Department of Haematology, Armed Forces Institute of Pathology, Pakistan
Chaudhry Altaf, Department of Haematology, Armed Forces Institute of Pathology, Pakistan
Hamid Saeed Malik, Department of Haematology, Armed Forces Institute of Pathology, Pakistan
Muhammad Tahir Khadim, Department of Haematology, Armed Forces Institute of Pathology, Pakistan

Abstract

Background: Paroxysmal Nocturnal Haemoglobinuria (PNH) is a rare acquired clonal haematopoietic stem cell disorder that manifests as bone marrow failure, haemolytic anaemia and thrombosis.
Objectives: To determine the clinical presentation and laboratory parameters and to classify and perform flow cytometric analysis of PNH patients in Pakistan.
Materials and method: This cross-sectional study was conducted in the Department of Haematology and Department of Immunology, Armed Forces Institute of Pathology Rawalpindi from January 2013 to December 2017. Diagnosis was established by flow cytometry. Clinical presentation and laboratory parameters were assessed and the patients were classified according to the Working Diagnostic Classification of the International PNH Interest Group (I-PIG).
Results: A total of 43 patients of PNH were analyzed. Males were 28(65%) and 15(35%) were females. Median age was 28 years (14-48years). The most common presenting feature was pallor in 42 (98%) followed by fatigue in 40(93%) and dyspnea in 25(58%) of the patients. Twenty (47%) patients presented with anaemia alone, 8 (19%) with bicytopenia while 15 (35%) had pancytopenia at the time of presentation. Three (6.9%) patients presented with thrombosis. The median duration of disease at diagnosis was 3.8yrs. Mean haemoglobin was 7.8±2.6g/dl, mean platelet count was 94±52.8x109/l and mean ANC was 2.9±1.3x109/l. Patients were classified as classic PNH (53%), PNH in setting of another bone marrow disorder (42%) and sub-clinical PNH (4.7%). The median clone size was 60% in patients with classic PNH, 22% in patients with PNH in setting of another bone marrow disorder and 7% in sub-clinical PNH.
Conclusion: The most common presentation was pallor. Majority of patients were in the classic PNH subgroup. 

Keywords

paroxysmal nocturnal haemoglobinuria, flow cytometry, clone size,ANC, PNH, I-PIG, GPI, CD59

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