A prospective comparison of four techniques for diagnosis of paroxysmal nocturnal hemoglobinuria.

نویسندگان

  • R Gupta
  • P Pandey
  • R Choudhry
  • R Kashyap
  • M Mehrotra
  • S Naseem
  • S Nityanand
چکیده

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder with altered expression of glycosylphosphatidylinositol (GPI)-anchored proteins, resulting in the increased susceptibility of erythrocytes to complement-mediated lysis. This study compared the available laboratory methods for detection of PNH cells and evaluated their utility in routine clinical practice. Fifty patients were evaluated by flow cytometric immunophenotyping (FCMI) using CD55 and CD59 monoclonal antibodies, PNH gel card test (GCT), Ham test and sucrose lysis test (SLT). A PNH clone was detectable in erythrocytes in 14 (28%) patients by FCMI, 13 (26%) by GCT and 10 (20%) by Ham test and SLT. The GCT and lytic tests showed 100% specificity and sensitivity was 92.8% and 71.1%, respectively. The GCT results correlated with type III cells (positive for > or =3.21% type III cells) and lytic test results correlated with CD59(-) type III cells (positive for > or =5% CD59(-) type III cells). The GCT and lytic tests were comparable in their sensitivity to detect type II cells (positive for > or =18.5% type II cells). Among the available methods, FCMI is most sensitive, can quantify and delineate PNH cells with differential expression of GPI-anchored proteins. The GCT is a useful screening tool as it is fairly sensitive, easy to perform and interpret. Well-standardized lytic tests are fairly reliable as screening tests.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...

متن کامل

Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry.

Paroxysmal nocturnal hemoglobinuria is a rare, acquired disease associated with hemolytic anemia, bone marrow failure, thrombosis, and, frequently, poor quality of life. The International PNH Registry is a worldwide, observational, non-interventional study collecting safety, effectiveness, and quality-of-life data from patients with a confirmed paroxysmal nocturnal hemoglobinuria diagnosis or d...

متن کامل

Paroxysmal nocturnal hemoglobinuria clone in 103 Brazilian patients: diagnosis and classification

BACKGROUND Paroxysmal nocturnal hemoglobinuria is an acquired chronic hemolytic anemia, which often manifests as peripheral blood cytopenias and thrombosis. OBJECTIVE The aim of this study is to describe a Brazilian population of paroxysmal nocturnal hemoglobinuria patients. METHODS One hundred and three paroxysmal nocturnal hemoglobinuria cases were retrospectively reviewed and the clinica...

متن کامل

گزارش یک مورد بیمار مبتلا به Paroxysmal Nocturnal Hemoglobinuria طی حاملگی

سابقه و هدف: PNH فرم نادری از آنمی همولیتیک غیر ارثی است که با همولیز حاد و مزمن داخل عروقی و هموگلوبینوری مکرر مشخص می شود و اغلب منجر به سقط و عوارض عروقی از قبیل ترمبوزهای وریدی می گردد اما برخی موارد حاملگی موفق و بدون عارضه نزد بیماران PNH مشاهده می گردد. در این راستا اقدام به معرفی یک مورد بیمار حامله مبتلا به PNH مراجعه کننده به بیمارستان رسول اکرم تهران در زمستان سال گذشته می شود. گزارش...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • International journal of laboratory hematology

دوره 29 2  شماره 

صفحات  -

تاریخ انتشار 2007