DOI: http://dx.doi.org/10.18203/2349-2902.isj20191280

Splenectomy in sickle cell haemoglobinopathies

Brajamohan Mishra, Malaya Krishna Nayak, Sandeep Mishra, Itishree Das

Abstract


Background: Sickle cell disease is one of the common haemoglobinopathies in the world. Among its various clinical presentations, splenic complications are associated with high morbidity and substantial mortality, the only effective preventive strategy for which is prophylactic splenectomy. The aim of the present study was to observe the safety and effectiveness of splenectomy to prevent frequent requirement of hospitalizations, blood transfusions and splenic complications in patients of sickle cell disease.

Methods: The study was carried out in 72 patients of SCD with splenomegaly who underwent splenectomy for some indication in the surgery department of VSSIMSAR, Burla during the period from October 2016 to September 2018. Their preoperative baseline hematological parameters and frequency of transfusion requirement was compared with postoperative status.

Results: Of these 72 SCD patients, 49 (68.7%) patients had repeated requirement of blood transfusion and hypersplenism, 22 (30.5%) patients had history of one or more episodes of splenic sequestration crises, one patient had splenic abscess. After splenectomy the mean increase in haemoglobin level, TLC and TPC was respectively 2.83±0.9 gm%, 1.7±0.8 lac/cmm and 2726±1618/cmm. Operative mortality was 0%. None of the patients required any blood transfusion and no major postoperative complications during 6 months follow up period.

Conclusions: The morbidity of the patients of SCD in terms of repeated hospitalizations, blood transfusion, living with a huge spleen, accompanying symptoms and its complications can be effectively minimized by the elective splenectomy. With good preoperative preparation and post-operative management, splenectomy in SCD patients is a safe procedure with minimal risk of post-operative complications.


Keywords


Sickle cell disease, Hypersplenism, Splenectomy

Full Text:

PDF

References


Rao VR. Genetics and epidemiology of sickle cell anemia in India. ICMR Bull. 1988;18:87-90.

Parmar D, Likhar KS. Prevalence of Splenomegaly in Sickle cell Anemia patients in relation to Haemoglobin F. IJRRMS. 2014;4(1):12-5.

Serjeant GR, Chin N, Asnani MR, Serjeant BE, Mason KP, Hambleton IR, et al. Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth. PLoS ONE. 2018;13(3):e0192710.

Serjeant GR. Evolving locally appropriate models of care for Indian sickle cell disease. Indian J Med Res. 2016;143(4):405–13.

Kar BC, Satapathy RK, Kulozik AE, Kulozik M, Sirr S, Serjeant BE, et al. Sickle cell disease in Orissa State, India. Lancet. 1986;2:1198-201.

Powars DR, Pagelow CH. The spleen in sickle cell disease and thalassemia. Am J Pediatr Hematol Oncol. 1979;1(4):343–51.

Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010;85:6-13.

Rezende PV, Viana MB, Murao M, Chaves AC, Ribeiro AC. Acute splenic sequestration in a cohort of children with sickle cell anemia. J Pediatr (Rio J). 2009;85:163-9.

Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364:1343.

al-Salem AH, Qaisaruddin S, Nasserallah Z, al Dabbous I, al Jam'a A. Splenectomy in patients with sickle-cell disease. Am J Surg. 1996;172:254-8.

Sorrells DL, Morrissey TB, Brown MF:Septic complications after splenectomy for sickle cell sequestration crisis. Pediatr Surg Int. 1998;13:100.

Serjeant GR. Sickle-cell disease. Lancet. 1997;350:725–30.

Pearson HA, Spencer RP, Cornelius EA. Functional asplenia in sickle cell anemia. N Engl J Med. 1969;281:923-6.

Pearson HA, McIntosh S, Ritchey AK, Lobel JS, Rooks Y, Johnston D. Developmental aspects of splenic function in sickle cell diseases. Blood. 1979;53:358-65.

Casper JT, Koethe S, Rodney GE, Thatcher LG. A new method for studying splenic reticuloendothelial dysfunction in sickle cell disease patients and its clinical application: a brief report. Blood. 1976;47:183-8.

Wright JG, Hambleton IR, Thomas PW, Duncan ND, Venugopal S, Serjeant GR. Postsplenectomy course in homozygous sickle cell disease. J Pediatr. 1999;134:304.

Emond AM, Morais P, Venugopal S, Carpenter RG, Serjeant GR. Role of splenectomy in homozygous sickle cell disease in childhood. Lancet. 1984;1:88.

Lesher AP, Kalpatthi R, Glenn JB. Outcome of splenectomy in children younger than 4 years with sickle cell disease. J Pediatr Surg. 2009;44:1134.

Maliyil J, Caire W, Nair R, Bridges D. Splenic abscess and multiple brain abscesses caused by Streptococcus intermedius in a young healthy man. Proc (Bayl Univ Med Cent). 2011;24:195-9.

Alvi AR, Kulsoom S, Shamsi G. Splenic abscess:outcome and prognostic factors. J Coll Physicians Surg Pak. 2008;18:740-3.

Sprague CC, Paterson JCS. Role of the Spleen and Effect of Splenectomy in Sickle Cell Disease. Blood. 1958;13:569-81.

Singhal A, Thomas PW, Keatney T, Venugopal S, Serjeant GR. Acceleration in linear growth after splenectomy for hypersplenism in homozygous sickle cell disease. Arch Dis Child. 1995;72:227–9.

Topley JM, Rogers DW, Stevens MCG, Serjeant GR. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Childhood. 1981;56:765-9.