ABSTRACT
Introduction: Traditionally, the administration of intramuscular vaccination has been avoided in patients with congenital coagulopathies due to its possible deleterious effects, including large hematomas, contractures or fibrosis. However, vaccination against SARS-CoV- 2 received approval from regulatory agencies only for intramuscular administration, since its efficacy by other routes has not been studied, so its administration was generalized by this route in this group of patients. The objective is to evaluate the possible adverse effects secondary to vaccination against COVID-19 in patients diagnosed with congenital coagulopathies in a single center. Method(s): Retrospective, analytical and single-center study documenting the adverse effects of vaccination against COVID-19 in a population diagnosed with congenital coagulopathies in a rural center in Spain. Clinical and epidemiological results were collected. Result(s): Twenty-six patients (61.5% male population) with a median age of 24 years (range 3-79 years) were evaluated. The most frequent congenital coagulopathies were deficiency of factor VII (34.6%), VIII (19.2%) and XI (19.2%). 11.5% received prophylaxis. 20% of the sample was infected during the first wave of the pandemic (pre-vaccination) without the need for hospital admission. The entire sample received vaccination in the complete scheme (3 doses, 57.7% were administered from the Pfizer-Bi brand, the difference from the Moderna-Rec brand) without evidence of significant hemorrhagic or thrombotic complications, presenting the entire sample of humoral immune response. No patient in the sample has required hospital admission secondary to severe COVID-19. Discussion/Conclusion: After completing the vaccination schedule against SARS-CoV-2, it can be inferred that the intramuscular administration of this vaccine does not produce significant hemorrhagic adverse events, being the route of preference at present.
ABSTRACT
Background : Although immune thrombocytopenia (IT) is uncommon, it has been described as one of the complications secondary to infection by SARS-CoV-2, probably secondary, amongst other factors, to the underlying immune dysregulation. The very few cases reported in the literature show excellent responses to corticoid therapy. Aims : The objective of this study is to show our experience with 2 patients who had severe symptomatic corticoid-refractory IT as a complication of COVID-19. Methods : A descriptive, single-centre study that shows our experience with 2 patients diagnosed with severe COVID-19 and a long-term stay at the Intensive Care Unit (ICU), who had severe symptomatic corticoid-refractory IT, an uncommon manifestation according to the very few series reported to date. Results : Two patients (women aged 65-67 years) with no relevant medical history, diagnosed in 03-2020 and 04-2020, respectively, with bibasal pneumonia secondary to severe COVID-19, needing stay at the ICU and assisted ventilation and long-term stay. On discharge they had severe IT associated with skin bleeding events, initially treated with oral prednisone mg/kg/day plus unspecific IVIg g/kg/day, initially obtaining a good response, but followed by early loss of response in both cases. It was decided to switch protocol to IV dexamethasone 40 mg for 4 days plus unspecific IVIg g/kg/day every 15 days, of which they received 3 cycles, without response and with increased skin stigmata of bleeding. Second-line treatment was started with TPO analogues (oral eltrombopag 50 mg/day, with dose escalation to 75 mg/day), obtaining haematological response on day +7 of start and complete remission on day +21 of treatment. Currently complete response persists, with no documented adverse events. Conclusions : Although uncommon, corticoid-refractory IT is a documented bleeding diathesis in severe COVID-19. Our experience shows that the early start of TPO analogues (in the absence of response to corticoid therapy) is an effective, well-tolerated option in patients with this complication.
ABSTRACT
Background : rIX-FP, a recombinant factor IX (rFIX) linked with recombinant human albumin, has demonstrated efficacy in prophylactic regimens up to every 21 days in patients with hemophilia B. Aims : To describe real world experience for a rural pediatric patient during the COVID-19 pandemic. Methods : Demographic, clinical (HEAD-US and Hemophilia Joint Health Score) and pharmacokinetic (PK) data were collected for up to one year following a switch from rFIX to rIX-FP. Results : In May 2018, a 3-year-old patient (moderate hemophilia B, FIX: 2.7 IU/dL) experienced hemarthrosis in the left knee and initiated prophylaxis with rFIX (40 IU/kg 2×/week). In March 2020, the patient switched to weekly rIX-FP prophylaxis (40 IU/kg), due to difficulties with venous access and accessing the clinic during the pandemic. Trough levels of 7.7% two weeks post-infusion and 5.1% 20 days post-infusion were maintained. Functional and joint assessment on day 90 of rIX-FP treatment showed no change, and no FIX inhibitors developed. On day 95 of treatment, a post-traumatic otorrhagia subsided after a single 40 IU/kg dose of rIX-FP. On day 120, the treatment interval was extended to 10 days without complications. After 160 days, the patient was diagnosed with an asymptomatic SARS-CoV-2 infection and self-isolated at home for 14 days with no changes to prophylactic treatment. On day 200, after a new PK study (trough levels of 6.9% at 14 days post-infusion), the prophylaxis interval was prolonged to 70 IU/kg every 14 days without incident. With 14-day dose intervals, monthly consumption reduced by 56.2% and the number of infusions by 75% compared with the initial rFIX treatment, without affecting efficacy or adherence. Conclusions : Switching to rIX-FP in this pediatric patient reduced the number of administrations and clinic visits without affecting efficacy or adherence, a fact that is relevant in the current context of the pandemic.