Nicole Ryman and Michael McNeal, M.D.
Sickle cell trait individuals are known to suffer from certain complications, such as renal papillary necrosis. A complication that may not be as well established in the literature is superior mesenteric venous thromboembolism.
A 34 year old African American female with a medical history significant for sickle cell trait, bleeding disorder in an aunt, and two miscarriages, presents with severe, diffuse abdominal pain. She reports nausea, emesis, and several episodes of diarrhea. In addition, her history included oral contraceptive use, episodic tobacco use, obesity, and cholecystitis. CT abdomen showed inflammatory changes of the central mesentery with scattered mesenteric lymph nodes with occlusion, dilation of the superior mesenteric vein, and thickening of the small bowel wall suggesting vascular congestion.
Uncomplicated cystitis without hematuria was suggested by urinalysis, and ceftriaxone was started. There was no evidence of peritonitis or gastrointestinal bleeding. Heparin was started. Her pain was managed with morphine, and she remained on bowel rest with clear liquid diet. Labs were insignificant; no rise in lactate and lipase was 29. Then, 24 hours later, repeat CT abdomen revealed edema throughout the central mesentery with the superior mesenteric vein dilated. Upon discharge, she was prescribed xarelto for 3 months and tramadol for pain. A repeat CT abdomen/pelvis in 4 weeks was also ordered. She was assigned to follow up with hematology in order for her hypercoagulable state to be further investigated.
This case demonstrates an uncommon finding that likely occurred due to a perfect storm of risks. This patient was recently post-partum, has sickle cell trait, and a history of smoking, oral contraceptives use, and obesity.1 It is possible that a combination of these risks led to the formation of superior mesenteric thromboembolism, a rare entity.2 Recognizing the risk of venous thromboembolism in sickle cell trait patients has not been widely reported. Folson et al demonstrated in a prospective study comparing individuals with sickle cell trait and those without any HbS allele that a 1.5 fold increase risk of venous thromboembolism, particularly with pulmonary embolism, for those with sickle cell trait.3 A recent study conducted in the UK suggests an association with sickle cell trait and venous thromboembolism, specifically pulmonary embolism.4 The pathophysiology has not been completely illustrated, but some have reported increase in coagulation activity in sickle cell trait individuals like significantly higher levels of d-dimer, thrombin-antithrombin complexes, and prothrombin fragment 1.2 Blood monocytes were increased compared to match controls, and monocytes play a role in endothelial damage, atherogenesis, and plaque rupture.5 More studies can further elucidate a potential association between venous thromboembolism and sickle cell trait and thus allow medical professionals to counsel these individuals on risk factors and improve early identification and management.
1.Naik, RP, Haywood C. Sickle cell trait diagnosis: clinical and social implications. Hematology/the Education Program of the American Society. 2015;(1): 160-167. doi:10.1182/asheducation-2015.1.160.
2.Clair, DG, Beach J. Mesenteric Ischemia. New England Journal of Medicine. 2016;(374): 959-968. doi:10.1056/NEJMra1503884.
3.Folsom AR, Tang W, Roetker NS, et al. Prospective study of sickle cell trait and venous thromboembolism incidence. Journal of thrombosis and haemostasis:JTH. 2015; 13(1): 2–9. doi:10:1111/jth.12787.
4.Little I, Vinogradova Y, Orton E, et al. Venous thromboembolism in adults screened for sickle cell trait: a population-based cohort study with nested case–control analysis.BMJ Open. 2017;7(3): doi: 10.1136/bmjopen.2016.012665.
5.Tsaras G, Owusu-Ansah Amma, Boateng Freda, et al. Complications associated with sickle cell trait: a brief narrative review. The American Journal of Medici