Anales de la RANM

281 A N A L E S R A N M R E V I S T A F U N D A D A E N 1 8 7 9 SARS-COV-2 INFECTION IN A PATIENT WITH PAROXYSMAL NOCTURNAL HAEMOGLOBINURIA Pablo Estival An RANM · Año 2020 · número 137 (03) · páginas 281 a 285 SARS-COV-2 INFECTION IN A PATIENT WITH PAROXYSMAL NOCTURNAL HAEMOGLOBINURIA (PNH) TREATED WITH RAVULIZUMAB: A CASE REPORT INFECCIÓN POR SARS-COV-2 EN UN PACIENTE CON HEMOGLOBINURIA PAROXÍSTICA NOCTURNA (HPN) EN TRATAMIENTO CON RAVULIZUMAB: A RAÍZ DE UN CASO CLÍNICO Pablo Estival 1 ; Blanca Colás 1 ; Yang Dai 2 ; F. Ataulfo Gonzalez 1 1 Hospital Clinico Universitario San Carlos, Department of Hematology, The Clínic Institute of Hematological and Oncological Diseases, Madrid, Spain 2 Alexion Pharmaceuticals, Inc., Boston, MA Abstract The clinical course of a Paroxysmal Nocturnal Hemoglobinuria (PNH) patient receiving treatment with terminal complement by ravulizumab and SARS-CoV2 infection is described. The treatment commenced in Januar y 2016 showing adequate tolerance and symptom resolution. In April 2020 an episode of breakthrough hemolysis is observed. Chest X-Ray revealed a right infection lobar infiltrate with SARS-CoV-2 positive PCR. The patient was afebril with normal oxygen saturation. He did not require standard disease treatment and presented eventual resolution, developing only mild symptoms. Neither PNH nor ravulizumab treatment seem to influence susceptibility to Covid-19 infection. It is possible that razulizumab, a complement inhibitor, may have played a beneficial role in the favorable clinical development. Resumen Se describe el curso clínico de un paciente diagnosticado de infección por SARS-CoV-2 en tratamiento con un inhibidor terminal del complemento (Ravulizumab; ensayo clínico ALXN 1210) para Hemoglobinuria Paroxística Nocturna (HPN), que comenzó en Enero de 2016 con buena tolerancia y resolución de los síntomas. En abril del 2020, durante una consulta de seguimiento dentro del ensayo, el paciente presenta un episodio de hemólisis aguda. La radiografía de tórax mostró un infiltrado pulmonar en lóbulo inferior derecho. Se efectuó una PCR de SARS-COV2 que resultó positiva. El paciente estaba afebril, sin signos de infección o afectación respiratoria. Presentó resolución espontánea, desarrollando únicamente sintomatología leve. No requirió tratamiento habitual para la infección por coronavirus. Tanto el HPN como el ravulizumab parecen no tener influencia en la susceptibilidad individual a la infección por SARS-COV2. Es posible que el ravulizumab (un inhibidor del complemento) juegue un papel beneficioso en el desarrollo de un curso clínico favorable. Keywords: Ravulizumab; Complement C5 inhibitor; paroxysmal nocturnal hemoglobinuria; PNH; Covid-19; SARS-CoV-19. Palabras clave: Ravulizumab; Inhibidores de C5; Hemoglobinuria paroxística nocturna; HPN; COVID-19; SARS-CoV-2. Autor para la correspondencia F. Ataulfo González Hospital Clinico San Carlos, Department of Hematology Profesor Martín Lagos · 28040 Madrid, Spain. Tlf.: +34 913 303 321 | E-Mail: fernandoataulfo.gonzalez@salud.madrid.org DOI: 10.32440/ar.2020.137.03. cc01 Enviado: 16.11.20 | Revisado: 22.11.20 | Aceptado: 18.12.20 C A S O C L Í N I C O SARS-CoV2 infection has implied a major challenge for medical community, primarily for Hematology. In fact, actual evidence of the disease in hemato- logical patients, usually immunocompromise, is primarily insufficient, and they mortality approach 30% (1). PNH is a clonal stem cell hematopoietic disease characterized by chronic intravascular hemolysis and increased risk of thrombosis. This is due to acquired somatic mutation which impairs the biosynthesis of the glycosylphosphatidylinositol (GPI) anchor, and as a consequence all GPI-linked proteins are not expressed on affected hematopoi- etic stem cells and their mature progeny blood cells. Among the missing GPI-linked proteins, the lack of the two complement inhibitors, MIRL (CD59) and DAF (CD55), makes red blood cells more susceptible to lytic action by the complement system. Nowadays, monoclonal antibodies against complement (like anti C5 therapies eculizumab INTRODUCTION