Paroxysmal nocturnal hemoglobinuria

Čermák, Jaroslav

doi:10.36290/vnl.2018.071

Cited by 1 publication

(2 citation statements)

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“…"Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis" [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…GPI deficiency in virtually all PNH cases is the result of a somatic mutation in class A phosphatidylinositol glycan (PIGA), resulting in a deficiency of complement inhibitor proteins CD55 and CD59, which leads to complement-mediated chronic hemolysis. of GPI-deficient erythrocytes, as well as the activation of platelets, monocytes and granulocytes [1,[3][4][5][6][7].…”

Section: Introductionmentioning

confidence: 99%

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Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria

Miguel

Filho²,

Miguel³

et al. 2022

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Aims: To describe a Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria. Presentation of Case: A 25 years old, male, student, in regular follow-up in the Hematology sector due to Paroxysmal Nocturnal Hemoglobinuria in regular use of Eculizumab. He reports a month ago that he suddenly noticed, upon waking up in the morning, blurring and decreased visual acuity in her left eye. Discussion: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by hemolysis, thrombosis, and bone marrow failure caused by defective expression of glycosylphosphatidylinositol-anchored (GPI-anchored) complement inhibitors. Most commonly, PNH is caused by the loss of PIGA function, which is necessary for GPI biosynthesis. Conclusions: Patients with hemolytic anemia, unexplained thrombosis, especially in uncommon sites, cytopenias and bone marrow failure syndromes, dysphagia, and unexplained abdominal pain should be screened for PNH. PNH patients can benefit greatly from the treatments available, with a reduction in the risk of serious sequelae and a considerable improvement in their quality of life. So that the patient continues to be followed up in the ophthalmology department, with decreased visual acuity ipsilateral to the lesion, with a relative afferent pupillary defect on that side.

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“…"Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis" [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%