Partial Splenic Embolization in Paediatric Sickle Cell Disease Patients with Hypersplenism

Overview

Journal Cardiovasc Intervent Radiol

Specialty Cardiology & Vascular Diseases

Date 2024 Apr 5

PMID 38578371

Authors

Jacques A Bazeboso

Didier Mbuyi Mukendi

Cisse-Luc Mbongo

Wilfrid Mbombo

Michel Lelo Tshikwela

Antoine Molua

Benjamin Longo

Leon Tshilolo

Jose I Bilbao

Affiliations

Soon will be listed here.

Abstract

Purpose: To assess the safety and efficacy of partial splenic embolization (PSE) to reduce the need of transfusions and improve hematologic parameters in patients with hypersplenism and sickle cell disease (SCD).

Material And Methods: This prospective study includes 35 homozygous hemoglobin S patients with SCD and hypersplenism who underwent PSE from 2015 until 2021 in Kinshasa. Patients were evaluated, before and after PSE (1, 3 and 6 months), using clinical, laboratory and ultrasonographic methods. PSE was performed with the administration of gelatin sponge particles embolizing 60-70% of the splenic parenchyma.

Results: The mean age was 10 (± 4) years and (21/35, 60%) were male. After PSE Leucocytes decreased at 3 months (16 692.94 vs 13 582.86, p = 0.02) and at six months Erythrocytes increased 2 004 000 vs. 2 804 142 (p < 0.001), Platelets increased (168 147 vs. 308 445, p < 0.001) and Hemoglobin increased (5.05 g/dL vs. 6.31 g/dL, p < 0.001) There was a significant dicrease in the need of transfusions from 6 (2-20) before PSE to 0.06 (0-1) after PSE (p < 0.001). The most frequent complication was splenic rupture (4/35, 11.4%), seen only and in all patients with hypoechogenic nodules at baseline.

Conclusion: PSE is a safe procedure in patients with SCD and hypersplenism, that do not have hypoechogenic nodules in the spleen. PSE improves the hematological parameters and reduces the frequency of blood transfusions.

Citing Articles

Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease.

Sissoko A, Cisse A, Duverdier C, Marin M, Dumas L, Manceau S Am J Hematol. 2024; 99(12):2267-2278.

PMID: 39286963 PMC: 11560635. DOI: 10.1002/ajh.27481.

Colosplenic Fistula Following Splenic Embolization in a Sickle Cell Disease Patient.

Al Jabri A, Al Masrouri S, Al Adawi H, Al Qadhi H Cureus. 2024; 16(5):e61011.

PMID: 38800766 PMC: 11125456. DOI: 10.7759/cureus.61011.

References

Al-Salem A . Splenic complications of sickle cell anemia and the role of splenectomy. ISRN Hematol. 2011; 2011:864257. PMC: 3200071. DOI: 10.5402/2011/864257. View

Brousse V, Buffet P, Rees D . The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014; 166(2):165-76. DOI: 10.1111/bjh.12950. View

El Hoss S, Brousse V . Considering the spleen in sickle cell disease. Expert Rev Hematol. 2019; 12(7):563-573. DOI: 10.1080/17474086.2019.1627192. View

Nouri A, de Montalembert M, Revillon Y, Girot R . Partial splenectomy in sickle cell syndromes. Arch Dis Child. 1991; 66(9):1070-2. PMC: 1793058. DOI: 10.1136/adc.66.9.1070. View

Tshilolo L, Aissi L, Lukusa D, Kinsiama C, Wembonyama S, Gulbis B . Neonatal screening for sickle cell anaemia in the Democratic Republic of the Congo: experience from a pioneer project on 31 204 newborns. J Clin Pathol. 2008; 62(1):35-8. DOI: 10.1136/jcp.2008.058958. View

Tshilolo L, Summa V, Gregorj C, Kinsiama C, Bazeboso J, Avvisati G . Foetal haemoglobin, erythrocytes containing foetal haemoglobin, and hematological features in congolese patients with sickle cell anaemia. Anemia. 2012; 2012:105349. PMC: 3398577. DOI: 10.1155/2012/105349. View

Tshilolo L, Mukendi R, Wembonyama S . Blood transfusion rate in Congolese patients with sickle cell anemia. Indian J Pediatr. 2007; 74(8):735-8. DOI: 10.1007/s12098-007-0129-4. View

Owusu-Ofori S, Remmington T . Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev. 2017; 11:CD003425. PMC: 6486322. DOI: 10.1002/14651858.CD003425.pub4. View

Vick L, Gosche J, Islam S . Partial splenectomy prevents splenic sequestration crises in sickle cell disease. J Pediatr Surg. 2009; 44(11):2088-91. DOI: 10.1016/j.jpedsurg.2009.06.007. View

10.

Spigos D, JONASSON O, Mozes M, capek V . Partial splenic embolization in the treatment of hypersplenism. AJR Am J Roentgenol. 1979; 132(5):777-82. DOI: 10.2214/ajr.132.5.777. View

11.

Stanley P, Shen T . Partial embolization of the spleen in patients with thalassemia. J Vasc Interv Radiol. 1995; 6(1):137-42. DOI: 10.1016/s1051-0443(95)71079-x. View

12.

Pringle K, Spigos D, Tan W, Politis C, Pang E, Reyez H . Partial splenic embolization in the management of thalassemia major. J Pediatr Surg. 1982; 17(6):884-91. DOI: 10.1016/s0022-3468(82)80461-2. View

13.

Jimenez M, Azcona C, Castro L, Bilbao J, Leon P, Sierrasesumaga L . Partial splenic embolization in a child with hereditary spherocytosis. Eur J Pediatr. 1995; 154(6):501-2. DOI: 10.1007/BF02029370. View

14.

Pratl B, Benesch M, Lackner H, Portugaller H, Pusswald B, Sovinz P . Partial splenic embolization in children with hereditary spherocytosis. Eur J Haematol. 2007; 80(1):76-80. DOI: 10.1111/j.1600-0609.2007.00979.x. View

15.

Molica M, Massaro F, Annechini G, Baldacci E, DElia G, Rosati R . Life-Threatening Autoimmune Hemolytic Anemia and Idhiopatic Thrombocytopenic Purpura. Successful Selective Splenic Artery Embolization. Mediterr J Hematol Infect Dis. 2016; 8(1):e2016020. PMC: 4848020. DOI: 10.4084/MJHID.2016.020. View

16.

Araten D, Iori A, Brown K, Torelli G, Barberi W, Natalino F . Selective splenic artery embolization for the treatment of thrombocytopenia and hypersplenism in paroxysmal nocturnal hemoglobinuria. J Hematol Oncol. 2014; 7:27. PMC: 3984395. DOI: 10.1186/1756-8722-7-27. View

17.

Bazeboso J, Tshilolo L, Mbongo C, Bilbao J . Partial Splenic Embolization in a Child with Sickle Cell Disease and Hypersplenism. J Vasc Interv Radiol. 2016; 27(11):1738-1739. DOI: 10.1016/j.jvir.2016.06.026. View

18.

Ballas S, Lieff S, Benjamin L, Dampier C, Heeney M, Hoppe C . Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2009; 85(1):6-13. PMC: 5046828. DOI: 10.1002/ajh.21550. View

19.

Cober M, Phelps S . Penicillin prophylaxis in children with sickle cell disease. J Pediatr Pharmacol Ther. 2012; 15(3):152-9. PMC: 3018247. View

20.

Sangro B, Bilbao I, Herrero I, Corella C, Longo J, Beloqui O . Partial splenic embolization for the treatment of hypersplenism in cirrhosis. Hepatology. 1993; 18(2):309-14. View