Paroxysmal nocturnal haemoglobinuria: a case presenting with isolated thrombocytopenia
Biotechnology and Biotechnological Equipment, 2016 Paroxysmal nocturnal haemoglobinuria (PNH) is a rare disease characterized by intravascular haemolysis, haemoglobinuria and thrombosis. The present study reported PNH patients presenting with isolated thrombocytopenia.
Mustafa Yilmaz
exaly +3 more sources
Paroxysmal Nocturnal Haemoglobinuria‐Associated Acute Cutaneous Thrombosis and Haemolysis in the Setting of Parvovirus B19 and Varicella Zoster Virus Infection [PDF]
eJHaemA 28‐year‐old male with paroxysmal nocturnal haemoglobinuria (PNH) presented with headache, nasal bridge discomfort and haemoglobinuria. He developed a macular‐papular rash which rapidly progressed into purpura, necrosis and peri‐orbital oedema ...
Louise J. Potter +9 more
doaj +3 more sources
Medicina (Lithuania), 2022 During an acute SARS-CoV-2 infection, a diagnosis of Aplastic Anaemia associated with Paroxysmal Nocturnal Haemoglobinuria (AA/PNH) was made in a 78-year-old woman who had presented to the emergency department with severe pancytopenia.
Giovanni D'Angelo, Iannuzzo Gabriella
exaly +4 more sources
Therapeutic Advances in Rare Disease, 2020 Paroxysmal nocturnal haemoglobinuria (PNH) is an ultra-orphan disease, which until 15 years ago had limited treatment options. Eculizumab, a monoclonal antibody that inhibits C5 in the terminal complement cascade, has revolutionised treatment for this ...
Morag Griffin
exaly +2 more sources
MUC4 mutations promote a thrombotic phenotype in patients with paroxysmal nocturnal haemoglobinuria by increasing the deposition of terminal complement [PDF]
Clinical and Translational MedicineBackground Thrombosis is a common complication in paroxysmal nocturnal haemoglobinuria (PNH) patients, but primary prevention remains controversial. Identifying high‐risk individuals could enable risk‐stratified prophylactic anticoagulation strategies ...
Yingying Chen +8 more
doaj +2 more sources
Eculizumab for paroxysmal nocturnal haemoglobinuria: catastrophic health expenditure in Nepalese patients [PDF]
Orphanet Journal of Rare Diseases, 2023 Paroxysmal nocturnal hemoglobinuria (PNH) results from a mutation in the phosphatidylinositol glycan class-A gene which causes uncontrolled complement activation with resultant intravascular hemolysis and its sequelae. Eculizumab is a terminal complement
Sugat Adhikari +4 more
doaj +2 more sources
Role of NK cells in immune escape in patients with classical paroxysmal nocturnal haemoglobinuria [PDF]
Clinical and Translational MedicineBackground Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal haematopoietic stem cell disorder. Immune escape is crucial in PNH, and our previous studies revealed that natural killer (NK) cells potential participate in the immune escape of
Chaomeng Wang +10 more
doaj +2 more sources
Severe renal haemosiderosis in a patient with untreated paroxysmal nocturnal haemoglobinuria: a case report [PDF]
BMC NephrologyBackground Paroxysmal nocturnal haemoglobinuria (PNH) is a life-threatening disease in which intravascular haemolysis of the red blood cells frequently manifests with chronic haemolysis, anaemia and thrombosis.
Zhong Zhen Goh +3 more
doaj +2 more sources
Higher doses of eculizumab may be beneficial in paroxysmal nocturnal haemoglobinuria in pregnancy [PDF]
European Journal of Case Reports in Internal MedicineIntroduction: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare bone marrow failure disorder that results in complement-mediated haemolysis (mostly intravascular), thrombosis and bone marrow suppression.
Harshank Vishnubhai Patel +6 more
doaj +2 more sources
Haploidentical allogeneic haematopoietic stem cell transplantation for paroxysmal nocturnal haemoglobinuria: a retrospective analysis [PDF]
Annals of HematologyObjective: To evaluate the efficacy and prognostic factors of haploidentical haematopoietic stem cell transplantation (haplo-HSCT) for paroxysmal nocturnal haemoglobinuria (PNH).
Jing-Yi Bi +17 more
doaj +2 more sources