The Laboratory Diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH): Update 2010

Krauss, J S

doi:10.1309/lmr59zn0mfzmgqrb

Cited by 4 publications

(8 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This allows a more accurate assessment of the GPI anchor defect in PNH. Indeed, PNH clones lower that 1% can be detected . Sutherland et al .…”

Section: Diagnosis Approachmentioning

confidence: 99%

“…In addition to FCM, the diagnosis approach of PNH may require other tests such as complete blood count, reticulocyte count, lactate dehydrogenase (LDH), bilirubin, haptoglobin and hemosiderinuria levels, iron stores evaluation, bone marrow aspirate, and biopsy, cytogenetics. Leukocyte alkaline phosphatase is frequently decreased in PNH but is not routinely used for PNH diagnosis .…”

Section: Diagnosis Approachmentioning

confidence: 99%

See 1 more Smart Citation

Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review

Devalet

Mullier

Châtelain

et al. 2015

European J of Haematology

View full text Add to dashboard Cite

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder of the hematopoietic stem cell that makes blood cells more sensitive to the action of complement. Patients experience intravascular hemolysis, smooth muscle dystonia, renal failure, arterial and pulmonary hypertension, recurrent infectious diseases and an increased risk of notably dreadful thrombotic complications. The diagnosis is made by flow cytometry. Efforts have been recently performed to improve the sensitivity and the standardization of this technique. PNH is frequently associated with aplastic anemia or low-risk myelodysplasia and may be asymptomatic. Management of the classical form of PNH has been dramatically revolutionized by the development of eculizumab, which brings benefits in terms of hemolysis, quality of life, renal function, thrombotic risk, and life expectancy. Prophylaxis and treatment of arterial and venous thrombosis currently remain a challenge in PNH.

show abstract

“…This allows a more accurate assessment of the GPI anchor defect in PNH. Indeed, PNH clones lower that 1% can be detected . Sutherland et al .…”

Section: Diagnosis Approachmentioning

confidence: 99%

Section: Diagnosis Approachmentioning

confidence: 99%

Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review

Devalet

Mullier

Châtelain

et al. 2015

European J of Haematology

View full text Add to dashboard Cite

show abstract

“…Pemeriksaan ini dapat mengukur kadar CD55 dan CD59, dan defisiensi keduanya merupakan diagnosa yang definitif terhadap PNH. 8,9 Alat bantu diagnosis lain yang sebelumnya luas digunakan adalah tes Ham yang pertama kali diperkenalkan oleh TH Ham pada tahun 1937. Prinsip tes ini adalah mencetuskan hemolisis eritrosit menggunakan zat asam, yaitu amonium klorida.…”

Section: Diskusiunclassified

Seorang Pria 21 Tahun dengan Urin Berwarna Gelap

Sibarani

2019

NJM

View full text Add to dashboard Cite

First published by Strubing in 1882, Paroxysmal Nocturnal Hemoglobinuria (PNH) is a chronic acquired disorder characterized by the occurrence of intravascular hemolysis and hemoglobinuria which commonly occurs when patients sleep at night, caused by cellular abnormalities due to somatic mutations that cause intrinsic damage on the red blood cell membrane, making it more susceptible to complement lysis. The incidence of PNH varies greatly in various populations and is more common in Southeast Asia. In general, the incidence is estimated to be 1 -1.5 cases / million population. This case is more common in young adults, but can also be found in children and parents. In general the clinical picture of PNH includes symptoms of anemia, hemoglobinia, signs of bleeding, and gastrointestinal complaints. Diagnosis can be determined through blood, urine, bone marrow and cytogenetic examination. We reported the case of a 21-year-old man with complaints of pale face, easy fatigue and tea colored urine in the morning. After several laboratory tests and aspiration of the bone marrow, PNH diagnosis is made. Glucocorticoids used as therapy, and patients are discharged with clinical improvement.

show abstract

“…So far, the current diagnosis for PNH had been flow cytometric analysis of surface CD59, a GPI-AP implicated in the protection against complement lysis. Aerolysin is, however, a far more sensitive tool to detect PNH populations, in addition to the fact that it is a far simpler and less expensive test [114,115].…”

Section: Paroxysmal Nocturnal Hemoglobinuria Diagnosticsmentioning

confidence: 99%