Treatment and Outcomes of Hepatocellular Carcinoma in Patients with Sickle Cell Disease: a Population-based Study in the U.S

Overview

Journal HPB (Oxford)

Publisher Elsevier

Specialty Gastroenterology

Date 2021 Jul 23

PMID 34294525

Authors

Arianna Barbetta

Cameron Goldbeck

Angelina Lim

Sean P Martin

Jeffrey A Kahn

M Raashid Sheikh

Juliet Emamaullee

Affiliations

Soon will be listed here.

Abstract

Background: Sickle cell disease (SCD) is a rare hemoglobinopathy which can result in chronic liver disease and cirrhosis. Patients with SCD have an increased risk of hematologic malignancy, but the prevalence of hepatocellular carcinoma (HCC) in this population is unknown. Herein, the association of SCD with HCC was examined using registry data.

Methods: The SEER-Medicare database was queried to identify patients diagnosed with HCC between 2000 and 2015, and further stratified by SCD status. Propensity matching was performed to examine cancer-related survival and treatment outcomes.

Results: Overall 56,934 patients with HCC were identified, including 81 patients with SCD. Patients with SCD more frequently had cirrhosis [48.1% (39/81) vs 23.5% (13,377/56,853), p < 0.01] yet presented with smaller tumors [<5 cm: 51.9% (42/81) vs 38.5% (21,898/56,853), p = 0.01]. After propensity matching, SCD was not associated with attenuated survival (aHR 0.73 95%CI 0.52-1.01). When stratified by treatment, patients with SCD had equivalent outcomes to chemotherapy (p = 0.65), TACE/TARE (p = 0.35), resection (p = 0.15) and transplantation (p = 0.67) when compared to non-SCD patients.

Conclusion: This study confirms that a subset of patients with SCD will develop HCC. Importantly, therapeutic options for HCC should not be limited by pre-existing SCD, and similar survival should be expected when compared to non-SCD patients.

References

Paydas S . Sickle cell anemia and hematological neoplasias. Leuk Lymphoma. 2002; 43(7):1431-4. DOI: 10.1080/1042819022386833. View

Brunson A, Keegan T, Mahajan A, Paulukonis S, Wun T . Cancer specific survival in patients with sickle cell disease. Br J Haematol. 2018; 185(1):128-132. PMC: 8966490. DOI: 10.1111/bjh.15687. View

Traina F, Jorge S, Yamanaka A, de Meirelles L, Costa F, Saad S . Chronic liver abnormalities in sickle cell disease: a clinicopathological study in 70 living patients. Acta Haematol. 2007; 118(3):129-35. DOI: 10.1159/000107744. View

Piel F, Steinberg M, Rees D . Sickle Cell Disease. N Engl J Med. 2017; 376(16):1561-1573. DOI: 10.1056/NEJMra1510865. View

Seminog O, Ogunlaja O, Yeates D, Goldacre M . Risk of individual malignant neoplasms in patients with sickle cell disease: English national record linkage study. J R Soc Med. 2016; 109(8):303-9. PMC: 4976723. DOI: 10.1177/0141076816651037. View

Bauer T, Moore G, Hutchins G . The liver in sickle cell disease. A clinicopathologic study of 70 patients. Am J Med. 1980; 69(6):833-7. DOI: 10.1016/s0002-9343(80)80008-8. View

Ramdath A, Zeineddin A, Nizam W, Kearse L, Olufajo O, Williams M . Outcomes after Cholecystectomy in Patients with Sickle Cell Disease: Does Acuity of Presentation Play a Role?. J Am Coll Surg. 2020; 230(6):1020-1024. DOI: 10.1016/j.jamcollsurg.2020.02.046. View

Fako V, Martin S, Pomyen Y, Budhu A, Chaisaingmongkol J, Franck S . Gene signature predictive of hepatocellular carcinoma patient response to transarterial chemoembolization. Int J Biol Sci. 2019; 15(12):2654-2663. PMC: 6854367. DOI: 10.7150/ijbs.39534. View

Levesque E, Lim C, Feray C, Salloum C, Quere A, Robin B . Liver transplantation in patients with sickle cell disease: possible but challenging-a cohort study. Transpl Int. 2020; 33(10):1220-1229. DOI: 10.1111/tri.13669. View

10.

Feld J, Kato G, Koh C, Shields T, Hildesheim M, Kleiner D . Liver injury is associated with mortality in sickle cell disease. Aliment Pharmacol Ther. 2015; 42(7):912-21. PMC: 6478018. DOI: 10.1111/apt.13347. View

11.

Stricker R, Linker C, Crowley T, Embury S . Hematologic malignancy in sickle cell disease: report of four cases and review of the literature. Am J Hematol. 1986; 21(2):223-30. DOI: 10.1002/ajh.2830210212. View

12.

Ahn H, Li C, Wang W . Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients. Pediatr Blood Cancer. 2005; 45(2):184-90. DOI: 10.1002/pbc.20317. View

13.

Cusano J, Curry E, Kingston K, Klings E, Li X . Epidemiology and Perioperative Complications in Patients With Sickle Cell Disease After Orthopaedic Surgery: 26 Years' Experience at a Major Academic Center. J Am Acad Orthop Surg. 2019; 27(23):e1043-e1051. DOI: 10.5435/JAAOS-D-18-00288. View

14.

Brunson A, Keegan T, Bang H, Mahajan A, Paulukonis S, Wun T . Increased risk of leukemia among sickle cell disease patients in California. Blood. 2017; 130(13):1597-1599. PMC: 5620417. DOI: 10.1182/blood-2017-05-783233. View

15.

Theocharidou E, Suddle A . The Liver in Sickle Cell Disease. Clin Liver Dis. 2019; 23(2):177-189. DOI: 10.1016/j.cld.2018.12.002. View

16.

Khurmi N, Gorlin A, Misra L . Perioperative considerations for patients with sickle cell disease: a narrative review. Can J Anaesth. 2017; 64(8):860-869. DOI: 10.1007/s12630-017-0883-3. View

17.

Emre S, Kitibayashi K, Schwartz M, Ahn J, Birnbaum A, Thung S . Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis. Transplantation. 2000; 69(4):675-6. DOI: 10.1097/00007890-200002270-00036. View

18.

Hogen R, Kim M, Lee Y, Lo M, Kaur N, Kahn J . Liver Transplantation in Patients with Sickle Cell Disease in the United States. J Surg Res. 2020; 255:23-32. PMC: 7541438. DOI: 10.1016/j.jss.2020.05.015. View

19.

Mehta K, Farnaud S, Sharp P . Iron and liver fibrosis: Mechanistic and clinical aspects. World J Gastroenterol. 2019; 25(5):521-538. PMC: 6371002. DOI: 10.3748/wjg.v25.i5.521. View

20.

Hamideh D, Alvarez O . Sickle cell disease related mortality in the United States (1999-2009). Pediatr Blood Cancer. 2013; 60(9):1482-6. DOI: 10.1002/pbc.24557. View