Background

Sickle cell disease is a known risk factor for venous thromboembolism (VTE) due to multiple factors including orthopedic surgeries, such as total knee arthroplasty (THA) or total hip arthroplasty (THA), that are frequently undertaken in this population due to complications such as avascular necrosis of the hip and other procedures such as gall-bladder removal and splenectomy.3 These risks, combined with the inherit hypercoagulability in these patients due to increased platelet activation, intravascular hemolysis, and impaired fibrinolysis, leads to an increased risk of VTE in these patients particularly in the periods of increased stress such as during a TKA or THA.4

There is a paucity of published data in regards to the optimum anticoagulation for this patient population and practices remain to be heavily influenced by expert opinion and personal experience by extrapolating data from other patient populations to be used in this higher risk population.2 The incidence of VTE in hospitalized sickle cell patients is higher than that of the general population and likely underdiagnosed 2 There is some data to suggest an odds ratio as high as 7.37 for pulmonary embolism in sickle cell patients undergoing spinal fusion operations (C.I. 4.27- 12.71 , p-value 0.001).1

Given these observations and underlying pathophysiology there is a need for more information regarding the optimum anticoagulation for these patients as well as the need to describe the association of sickle cell disease and VTE which is not very well established in literature.3

Methods

Data was requested from MARCQI and compared to EPIC to identify patients with Hemoglobin SS disease, sickle cell trait and Hb C disease who underwent THA or TKA. Patients were analyzed for perioperative anticoagulants, including medication type, dosage, and length of administration. Patients were then assessed for postoperative VTE incidences, including deep vein thromboses, pulmonary emboli, or strokes.

Results

Overall, 15 patients were included in the study who met our inclusion criteria for analysis (Above 18 years or age with SS disease or sickle trait of Hb C who underwent TKA or THA between the years 2011-2021), patients who were previously receiving anticoagulation with Heparin products DOACs or Coumadin were excluded from the study, and VTE events were defined as events occurring within 3 months after the initial surgical intervention.

Of the 15 patients that were chosen for the study there were 10 females and 5 males included with a median age of 54 (Range 24-79) , there were 12 cases of Sickle Cell Trait and 3 cases of HbSS disease ,of these patients 13 had undergone a total knee replacement and two had undergone a total hip replacement, interestingly none of our 15 selected patients had a thromboembolic event within 3 months of their surgical procedure regardless of method of VTE prophylaxis or duration used.

There was significant Heterogenicity in terms of methods or VTE prophylaxis used with the majority of the patients ( 6 patients ) having been prescribed ASA at a dose of 325MG BID on discharge along with Pneumatic compression devices while being in the hospital , 4 patients were prescribed a DOAC (Xarelto was used in all 4 patients), 2 patients were prescribed low dose LMWH , while 3 patients did not receive any form of VTE prophylaxis on discharge and only had Pneumatic compression devices while inpatient , all of which were knee replacement patients.

Of all therapies chosen the average duration of therapy ranged between 21-28 days in patients who were sent home with VTE prophylaxis.

Conclusions:

While our smaller sample size was not able to identify VTE occurring in sickle cell disease/trait/HbC disease patients undergoing TKA or THA there was significant lack of standardization in the choice of the type and duration of anticoagulation prophylaxis which stems from a lack of useful guidelines with regards to this high-risk population.

Given paucity of data with regards to the subject at hand our data can perhaps be expanded to include more patients through bigger databases to better enhance our understanding on how to best manage these patients to improve clinical outcomes and serve as a basis for future prospective trials.

No relevant conflicts of interest to declare.

Author notes

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Asterisk with author names denotes non-ASH members.

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