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Research and Practice in Thrombosis and Haemostasis ; 5(SUPPL 2), 2021.
Article in English | EMBASE | ID: covidwho-1508988


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.

Research and Practice in Thrombosis and Haemostasis ; 5(SUPPL 2), 2021.
Article in English | EMBASE | ID: covidwho-1508987


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.