key: cord-0871031-wv0co0vd
authors: Vagrecha, Anshul; Stanco, Joseph; Majeed, Sumreen; Kouides, Peter; Acharya, Suchitra S.
title: Primary thromboprophylaxis in a patient with type 3 von Willebrand disease and severe COVID‐19 infection
date: 2021-01-11
journal: Haemophilia
DOI: 10.1111/hae.14252
sha: 7400849027ffa51ca4190536671e34f4d9e9c74e
doc_id: 871031
cord_uid: wv0co0vd

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A 65-year-old white, obese, female with type 3 VWD (von Willebrand antigen: 3.5%, von Willebrand activity: <1%; FVIII: 18%, absent multimers), adult-onset type 1 diabetes mellitus, hypothyroidism, dyslipidaemia and angiodysplasia presented to a New York hospital in April 2020 with a 4-day history of cough, subjective fever (felt warm to touch), myalgias and shortness of breath on exertion. Before her presentation, she was followed at our Hemophilia Treatment Center and was on prophylactic replacement therapy for two months with a von Willebrand factor/coagulation factor VIII human complex (brand name Wilate) at 35 U/kg every other day along with intermittent courses of tranexamic acid for underlying angiodysplasia and history of gastrointestinal bleeding.

On presentation, she was tachypneic, tachycardiac and hypoxic (oxygen saturation was 94% at rest but 85% on walking). We summarize pertinent laboratory findings in Table 1 She was monitored closely with trough and peak VWF levels, and the factor dose was adjusted to maintain the VWF activity between 30 and 50%.

On Hospital Day 5, her clinical condition worsened. She developed respiratory failure, was intubated and moved to the ICU for mechanical ventilation. She was then started on systemic corticosteroids and H2 blockers for stomach protection. She continued LMWH thromboprophylaxis and VWF replacement therapy. By Hospital Day 13, she developed worsening anaemia. Her Hb dropped to 7.8 g/dL from 9.4 g/dL, and she received a pRBC transfusion. No source of bleeding was identified, and the anaemia was presumed to be due to her angiodysplasia and dilution from phlebotomy. Her LMWH dose was reduced to 40 mg once daily.

She continued to make gradual progress and was extubated by Hospital Day 14 and transferred to a rehabilitation facility. On Hospital Day 18, she was switched to rivaroxaban thromboprophylaxis (10 mg once daily) and was discharged after 3 weeks from her initial presentation. She did not develop significant haemorrhage and there was no evidence of clinical DVT, PE, arterial thrombosis or VTE post-discharge. No screening imaging for thrombosis was done.

She was discharged home on rivaroxaban prophylaxis, which was discontinued 4 weeks post-discharge with no evidence of bleeding.

She is now 6 months post-COVID-19 diagnosis and doing well. We started intermediate-dose LMWH in our patient and continued her prophylactic replacement therapy while monitoring peak and trough VWF activity with dosage changes. Intending to maintain that activity between 30 and 50%, we not only could safely continue thromboprophylaxis but also maintain haemostatic VWF activity. With her drop in haemoglobin and concern for bleeding, we decreased her LMWH dose. However, our patient did not develop significant bleeding or any clinical evidence of thrombosis. Therefore, monitoring factor levels on replacement therapy, and the use of standard anticoagulation practices allowed us to safely administer thromboprophylaxis both inpatient 

The authors would like to thank nurses, residents and other healthcare team members taking care of patients with COVID-19 infection.

anticoagulation, bleeding disorder, COVID-19, LMWH, thromboprophylaxis, von Willebrand disease

SA has received research support for investigator-initiated study from Bayer, LLC and Advisory Board fees from Novo Nordisk and BPL. AV, JS, SM, PK stated that they had no interests which might be perceived as posing a conflict or bias.

AV wrote the first draft of the letter. AV, SA and PK designed the manuscript. JS, SM and SA were involved in the care, inpatient management and discharge follow-up of the patient. AV, JS, SM, PK and SA revised and approved the final version.

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions. 

Thrombosis in Hospitalized Patients With COVID-19 in a New York City Health System

Scientific and Standardization Committee communication: Clinical guidance on the diagnosis, prevention, and treatment of venous thromboembolism in hospitalized patients with COVID-19

Thromboembolism and anticoagulant therapy during the COVID-19 pandemic: interim clinical guidance from the anticoagulation forum

COVID-19 and coagulation: bleeding and thrombotic manifestations of SARS-CoV-2 infection

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How I treat patients with inherited bleeding disorders who need anticoagulant therapy