Overcoming challenges of venous thromboembolism in sickle cell disease treatment

ABSTRACT

Introduction: Venous thromboembolism (VTE) is a common comorbid condition found in sickle cell disease (SCD) and is associated with increased mortality for adults with SCD. The pathophysiology that leads to the thrombophilic state in SCD has been previously reviewed; however, evidence-based guidelines to aid in diagnosis, prevention, and management of VTE are lacking.

Areas covered: This review article will cover the pathophysiology underlying the hypercoagulable state, the epidemiology of VTE, and management strategies of VTE in SCD.

Expert opinion: Providers should have a high suspicion for diagnosing VTE to help reduce morbidity and mortality in the SCD population. Unlike other thrombophilias, the risk of life-threatening anemia while being treated with anticoagulation is compounded with the potential complications surrounding red blood cell transfusions in this population (i.e. alloimmunization, hyperhemolysis) and this provides another complexity to managing VTE in this population. Clinical trials evaluating the risk and benefit of treatment and treatment duration are needed.

KEYWORDS:

• Sickle cell disease
• venous thromboembolism
• epidemiology of venous thromboembolism in sickle cell disease
• management of venous thromboembolism in sickle cell disease
• deep venous thrombosis
• pulmonary embolism

Article highlights

• VTE occurs relatively frequently in people living with SCD, and clinical providers should have a high level of suspicion for diagnosing VTE.

• The hypercoagulable state in SCD result from multifactorial causes related to endothelial injury, venous stasis, and activated pro-coagulant activity at both steady and non-steady state in SCD.

• CTPA and V/Q scan are the two most common modalities used to diagnose PE in SCD and have comparable diagnostic capability for diagnosing PE.

• Patients with SCD often encounter traditional VTE risk factors of frequent hospitalizations, surgeries, and central venous catheter line placements.

• Inpatient pharmacologic thromboprophylaxis should be considered for adults with SCD who are hospitalized or undergoing surgery.

• Providers should consider extending the duration of therapeutic anticoagulation therapy for patients with SCD. However, it is imperative that providers who care for patients with SCD risk stratify patients for both recurrent VTE requiring extended treatment duration and the risk of bleeding after an initial VTE event.

This box summarizes key points contained in the article.

Declaration of interest

SM Lanzkron has received research funding from Pfizer, AstraZeneca, Prolong, Selexys, and Global Blood Therapeutics. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or conflict with the subject matter or materials discussed in this manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Notes

Additional information

Funding

This paper was not funded.