AA-01. COVID-19 in patients with bone marrow failure and paroxysmal nocturnal hemoglobinuria
Darya A. Chebykina, Irina K. Golubovskaya, Yuriy N. Kuznetsov, Maria V. Bogomolova
RM Gorbacheva Research Institute, Pavlov University, St. Petersburg, Russia
Darya A. Chebykina, phone: +7(931) 965-39-45, e-mail: firstname.lastname@example.org
Patients with acquired and inherited bone marrow failure syndromes (BMFS), including aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH), are exposed to a significant risk of COVID-19 infection and mostly to its possible complications. Treatment programs for patients with a BMFS include two main therapeutic options: allogeneic hematopoietic stem cell transplantation (allo-HSCT) and combined immunosuppressive therapy (IST). IST may increase the risk of developing serious infection in patients with AA, but at the same time can reduce hyperinflammatory tissue destruction. A serious viral infection such as COVID-19 could serve as an initiating event for AA or drop blood counts in patients in remission. One of the most common complications in PNH patients associated with COVID-19 infection is vascular thrombosis. Many of the thrombotic complications arising from COVID-19 infections may be triggered and/or exacerbated by excessive complement activation. This is of major potential clinical relevance, as currently available anti-complement therapies could be efficacious in COVID-19. The aim of our study was to analyze the course of COVID-19 infection in patients with BMF syndromes.
Patients and methods
A total of 55 patients with the BMF and COVID-19 infection between 04.2020 and 05.2021 were included in the study. The median age was 30 (18-72) years. The median follow-up was 8 months (1-15). The majority were patients with AA (n=23, 42%). Nine patients (16%) received allo-HSCT. Forty six (84%) patients received the conservative therapy (n=19, 41%). Ten patients (22%) were dependent from blood transfusions. The anti-complement therapy was used in 13 patients (23%) with active hemolytic PNH and AA/PNH.
Thirty patients (55%) had a mild form of COVID-19. Severe forms were registered in 4 (7%) cases. The specific pneumonia was diagnosed in 36 % of the patients (CT-1 – 29%, CT-2 – 7%). A total of 2 patients died (vSAA 1, SAA/MDS 1). With a median follow-up of 8 months overall survival was 95%. The main causes of death were the secondary bacterial and fungal infections. Two patients with hemolytic PNH developed a breakthrough hemolysis on eculizumab with subsequent small-vessels pulmonary thrombosis. Hepatic vein thrombosis occurred in 1 patient before eculizumab therapy starting. The majority of the patients irrespective of baseline remission status showed a decline of Hb level and platelets after COVID-19 infection. The median HB level was 119 g/L before and 105 g/L after 2 months, (p <0.001), platelet level was 132×109/l and 85×109/l (p <0.001), respectively. The median neutrophils level decreased from 1.82×109/l to 1.42×109/l (p <0.001).
Mostly the COVID-19 disease occurred in the mild and moderate forms in the patients with BFMs, including those receiving IST. However, the patients with severe cytopenia had a risk of death due to secondary infectious complications. According to our data COVID-19 was associated with significant Hb and PLT decreasing. In the patients with the hemolytic PNH, the breakthrough hemolysis and the thrombotic complications can develop during and after COVID-19 infection.
COVID-19, aplastic anemia, paroxysmal nocturnal hemoglobinuria, bone marrow failure.