Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil

Junior, Lacy Cardoso de Brito; Cardoso, Maria do Socorro de Oliveira; Rocha, Euzamar Gaby; Anijar, Herika; Cunha, Mariana Sequetin; Saraiva, João Carlos Pina

doi:10.5581/v33n1a12

Cited by 1 publication

(2 citation statements)

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“…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%

“…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%

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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%