Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex
            evolution and liver transplant

Alencar, Railene Célia B.; Guimarães, Andréa M.; Junior, Lacy Cardoso de Brito

doi:10.5935/1676-2444.20160051

Cited by 2 publications

(3 citation statements)

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“…PNH can occur at any age, but it is generally diagnosed between third and fifth decades. [2][3][4][5] 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. [6] The diagnosis of PNH is made by means of clinical findings and laboratory tests to confirm the degree of hemolysis (haptoglobin, lactate dehydrogenase, direct Coombs test, reticulocyte count and total bilirubin and bilirubin fraction) and deficiency of anchored proteins of the complement system (CD55, CD59, and FLAER) in granulocytes (CD15, CD33, and CD24) and monocytes (CD14 and CD64) by flow cytometry, the gold standard method.…”

Section: Introductionmentioning

confidence: 99%

“…As regards sex, in Europe, PNH is more common in women, whereas in Asia it is more common in men. PNH can occur at any age, but it is generally diagnosed between third and fifth decades [2–5] . 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 [6] …”

Section: Introductionmentioning

confidence: 99%

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COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria

Sokol¹,

Nehaj

Mokáň

et al. 2021

Medicine

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Introduction: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, life-threatening hemopoietic stem cell disorder characterized by the triad of hemolytic anemia, thrombosis, and impaired bone marrow function. Evidence suggests that severe outcomes in COVID19 infection are attributed to the excessive activation of the complement cascade leading to acute lung injury and associated is with an increased prothrombotic state. Patient concerns: A 27-year-old Caucasian man with PNH presented to the Emergency Department of our hospital with acute onset shortness of breath, cough and blood in urine. Diagnosis: The patient was diagnosed with acute hemolytic exacerbation of PNH complicated with moderate COVID19 pneumonia. Outcomes: The patient was initiated with an anticoagulant unfractionated heparin, dexamethasone, and cefuroxime injection. His symptoms quickly resolved, and he was discharged after 5 days. Conclusion: The complement system activation is a critical component in the sequalae of COVID19 infection. Evidence suggests that severe outcomes in COVID19 infection are attributed to the excessive activation of the complement cascade leading to acute lung injury and associated is with an increased prothrombotic state. Notably, C5a concentration was noted to be higher in patients with COVID19 infection. The use of complement inhibitors to attenuate immune mediated damage in COVID19 nevertheless represents a very interesting theoretical approach. However, careful consideration as to which patients may benefit will be required and the outcome of clinical trials needed.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria

Sokol¹,

Nehaj

Mokáň

et al. 2021

Medicine

View full text Add to dashboard Cite

show abstract

“…O gene PIG-A é responsável pela codificação de uma enzima crítica na formação da proteína âncora glicosil-fosfatidil-inositol (GPI), a qual realiza a fixação de múltiplas proteínas na membrana citoplasmática. A deficiência de GPI conduz a uma carência de proteínas reguladoras do SC ligada à superfície dos eritrócitos, tornando-os susceptíveis a hemólise intravascular mediada pelo sistema complemento (ARAUJO CJ, et al, 2002;PARKER C, et al, 2005;ALENCAR RCB et al, 2016).…”

Section: Introductionunclassified

Diagnóstico e monitoramento por imunofenotipagem da hemoglobinúria paroxística noturna (HPN) e correlação com a eficácia do tratamento com eculizumab

Silveira

Silva²,

Pereira

et al. 2019

Acervo Saúde

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Objetivo: Ressaltar o monitoramento da HPN por imunofenotipagem por citometria de fluxo (CMF) e avaliar a eficácia do tratamento com o anticorpo monoclonal eculizumab. Metodologia: O trabalho é uma análise retrospectiva de dados de prontuários dos pacientes encaminhados a um Instituto de referência em doenças hematológicas no Estado de Goiás para diagnóstico e monitoramento da HPN no período de janeiro de 2010 a dezembro de 2017. Resultados: Dos 31 pacientes diagnosticados com HPN, 5 foram selecionados com dados completos nos prontuários. No paciente 1, houve uma melhora significativa com o tratamento, com um aumento do número relativo para os marcadores dos eritrócitos (CD55 e CD59) de 22,7% para 71,7%. Para o paciente 3, a variação média de marcadores foi de 0,5% para 5,2%. No paciente 5 a variação média ficou entre 12,6% e 36,9%. O eculizumab reduziu o quadro hemolítico, melhorando os sintomas e estabilizando os resultados dos testes de hemólise. Conclusão: A imunofenotipagem por CMF é padrão ouro na investigação de HPN por avaliar a expressão de proteínas ancoradas pela GPI com alta sensibilidade e especificidade. Este estudo é o primeiro a demonstrar a eficácia do eculizumab, sem a progressão da doença.

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Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant

Cited by 2 publications

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COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria

COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria

Diagnóstico e monitoramento por imunofenotipagem da hemoglobinúria paroxística noturna (HPN) e correlação com a eficácia do tratamento com eculizumab

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