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Year : 2007  |  Volume : 1  |  Issue : 1  |  Page : 5

Assessment of perioperative transfusion therapy and complications in sickle cell disease patients undergoing surgery

1 College of Medicine and Medical Science, Arabian Gulf University and Anesthesia Department, Salmaniya Medical Complex, Bahrain
2 Faculty of Medicine, Zagazig University, Egypt and Consultant anesthetist, Anesthesia Department, Salmaniya Medical Complex, Bahrain
3 Anesthesia Department, Salmaniya Medical Complex, Bahrain

Correspondence Address:
Zahra M Al-Sammak
College of Medicine and Medical Science, Arabian Gulf University and Anesthesia Department, Salmaniya Medical Complex
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Source of Support: None, Conflict of Interest: None

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Date of Web Publication5-Oct-2009


Background : Perioperative blood transfusion is usually given to sickle cell disease patients to reduce or prevent perioperative morbidity. Assessment of such a practice was the subject of our study. Methods : A retrospective one year survey of sickle cell disease patients undergoing surgery at Salmaniya Medical Complex, Bahrain, was conducted. The medical records were reviewed to characterize the surgical procedure, transfusion management and perioperative complications. Results : 85 sickle cell disease patients who underwent surgery were studied. Preoperatively, 21.2% had exchange transfusion (ETX), 24.7% had simple transfusion (STX) and 54.1% had no transfusion (NTX). 14.1% of all patients had postoperative complications and 50% of those who had complications were from the laparoscopic cholecystectomy group. The incidence of sickle cell crisis postoperatively was 22.2% in ETX group, 9.5% in STX group and 4.34% in the NTX group. The incidence of acute chest syndrome postoperatively was found to be 5.55 % in the ETX group, 4.76% in the STX group and 4.34% in the NTX group. No intraoperative complications were recorded in all groups. All patients who had postoperative complications had a preoperative HBSS > 40%. Conclusion : Exchange transfusion doesn't prevent perioperative complications of sickle cell disease. HBSS > 40% carries a higher risk of postoperative complications.

Keywords: Sickle cell disease patients, perioperative transfusion therapy, complications.

How to cite this article:
Al-Sammak ZM, Falaki M, Pasha AA. Assessment of perioperative transfusion therapy and complications in sickle cell disease patients undergoing surgery. Saudi J Anaesth 2007;1:5

How to cite this URL:
Al-Sammak ZM, Falaki M, Pasha AA. Assessment of perioperative transfusion therapy and complications in sickle cell disease patients undergoing surgery. Saudi J Anaesth [serial online] 2007 [cited 2022 Nov 29];1:5. Available from:

   Introduction Top

Sickle cell disease (SCD) is one of the most common inherited diseases in the world. It is most commonly seen in individuals whose genetic origins are in sub-Saharan Africa, southern India, and the Mediterranean. Approximately 1 in 350 African Americans are born with HBSS, whereas 1 in 835 have HBSC and 1 in 1700 have HBSβ- thalassemia [1] .

Pathophysiologicaly, SCD is a hemoglobin structure disorder in which glutamic acid at the sixth residue of theβ chain of hemoglobin is substituted by valine , resulting in the formation of a poorly soluble hemoglobin tetramer (α2/β2 S) [2] . Homozygote (SS) will show clinical symptoms and heterozygote (AS) or traits are usually asymptomatic. . Sickle cell hemoglobin C disease and Sickle cell beta thalassemia are other varieties.

The hallmarks of SCD are anemia and vasculopathy. The major sources of morbidity and mortality in SCD are acute painful syndromes, severe anemia, infections, acute chest syndrome and organ failure.

Surgical procedures are often required. Preoperative blood transfusions are frequently given to prevent perioperative complications. There is no consensus however on the best regimen for transfusion for this purpose. Blood transfusion can be life saving and can ameliorate some of the complications of SCD. Nevertheless, blood transfusions, even when correctly used , are not without complications [3] .

The aim of this study is to assess the relative risks and benefits of preoperative blood transfusion regimens in patients with sickle cell disease undergoing surgery unrelated or directly attributed to consequences of their illness.

   Patients and Methods Top

A retrospective one year survey of sickle cell disease patients undergoing surgery at Salmaniya Medical Complex, Bahrain, was conducted. Patients were eligible for enrollment in the study if they had a diagnosis of sickle cell disease documented by the presence of hemoglobin SS on electrophoresis.

The patients were grouped into three categories, exchange transfusion (EXT), simple transfusion (STX), and no transfusion (NTX).Patients' data were collected from the medical records and reviewed to characterize anaesthetic risk ( ASA physical status) ,the surgical procedure, transfusion management and perioperative complications. The medical history, anesthetic management and recovery period were also recorded.

Data on patients' characteristics, transfusion, perioperative management and complications are expressed as number and means΁ SD

   Results Top

The data from 85 sickle cell disease patients who underwent surgery were analysed. All the patients were hydrated and premedicated with tranquilizers and prophylactic antibiotics. Both sexes were included with the mean age being 21 (1-50) years. [Table 1], [Figure 1] .45% of patients underwent majour surgeries while 55% underwent minour surgeries [Figure 2]. The surgeries that were done were laparoscopic cholecystectomy 24%, splenectomy 5%, ENT procedures 20%, orthopedic surgeries 18% , dilatation and curettage and minor surgical procedures33%. [Table 2], [Figure 3] The patients were categorized into NTX, ETX and STX groups [Table 3] according to their preoperative transfusion status. Criteria for accepting the patients for surgery were either a pre operative Hb% of 10-12.4 G% and/or HbS of 45- 60 % [Table 4]. Incidence of sickle cell crisis was found to be 25% in laparoscopic cholecystectomy, 50% in ENT procedures and 7.1% in orthopedic surgeries. Incidence of ACS was found to be 5% in laparoscopic cholecystectomy patients, 11.7% in ENT patients and 7.1% in orthopedic patients [Table 5], [Figure 4]. The NTX group had sickle cell crisis in 2.35% and ACS in 2.35%. STX category showed sickle cell crisis in 2.35% and 1.17% had ACS. Patients in ETX group had crisis in 4.47% and 1.17% of the patients had ACS [Figure 5].

   Discussion Top

Persons with sickle cell disease (SCD) are more likely to undergo surgery than are the general population during their lifetime [4] . Orthopedic procedures, cholecystectomies, splenectomies are the most common operations performed in (SCD) patients. In our study 45% of patients had undergone major surgery like laparoscopic cholecystectomies, splenectomies, and total hip replacement.

Cholecystectomy is often necessary as a result of cholelithiasis, a condition more frequent in patients with SCD than in the general population owing to chronic hemolytic anemia [12] . During the early 1990s, laparoscopic cholecystectomy superseded open cholecystectomy, and the procedure is now widely used in patients with SCD [13] .

In our study, the sickle cell disease patients who underwent laparoscopic cholecystectomy were more liable to develop sickle cell crisis than those who underwent other operations since 25% of them developed crisis and 5%developed acute chest syndrome. 50% of laparoscopic cholecystectomy patients received exchange transfusion (ETX), and 50% received simple transfusion (STX). Because surgery exposes patients to many of the factors that are known to precipitate red blood cell sickling, persons with SCD undergoing surgery require meticulous clinical care to prevent perioperative sickle cell-related complications. Even with meticulous care, approximately 25%-30% of patients will have postoperative complications [5] .In our study 14% of all the patients had sickle cell disease complications. Routine perioperative transfusions have been standard practice for (SCD) patients undergoing major surgery. However, data supporting this practice is mixed [7] . This procedure may be an exchange transfusion, which is given to lower the level of HBS, usually to around 30%, or a simple transfusion of 1 or 2 units of blood to raise the overall hemoglobin level to approximately 10g/dl [6] , improving the overall oxygen- carrying capacity.The case for preoperative transfusion is based on improving oxygen delivery, because most patients with SCD are chronically anemic, although they develop compensatory mechanisms to ensure reasonable functioning at low hemoglobin levels. In addition, by lowering the level of HbS and the number of sickled red blood cells, whole blood viscosity should fall, reducing the risk of vaso-occlusion.In our study the two practices were used with 24.7% had their surgery after getting simple transfusion while 21.2% had their surgery after getting exchange transfusion.

In reality, many hospitals and sickle cell centers have policies that err on the side of caution and advocate giving a transfusion to patients before surgery. Many centers have adopted a more conservative policy following the publication of the study by the preoperative transfusion in sickle cell disease group [9] . This showed that this policy was as safe as exchange transfusion. Some centers do not routinely transfuse patients before surgery but rely heavily on the delivery of excellent care from a wide multidisciplinary team to prevent the development of postoperative sickle cell events [10] . In a recent national survey of practice in the United Kingdom, it was shown that most patients undergoing cholecystectomy and adenoidectomy do so without preoperative blood transfusion, whereas almost all patients undergoing hip arthroplasty are prepared by an exchange transfusion. There was no difference in the rate of postoperative complications in patients who received a transfusion and those who did not, suggesting a growing trend in the United Kingdom to avoid transfusion when possible [11] .

In 1997 Haberkern et al. published the results of a study of 364 patients undergoing cholecystectomy [14] . This study compared 110 patients randomized to aggressive transfusion, 120 patients randomized to conservative transfusion, 37 patients nonrandomized to nontransfusion, and 97 patients nonrandomized to transfusion. This confirmed the previous findings of Vichinsky et al. [9] of no improved prophylactic benefit from an aggressive transfusion compared with conservative transfusion.

A retrospective observational study reported by Griffin and Buchanan in 1993 examined outcomes in children undergoing 66 surgical procedures without preoperative transfusion. [10] There was only one episode of ACS and no pain crises after 46 minor procedures. The authors concluded that any potential benefit from transfusion would therefore be low and the risks of transfusion were not justified for minor procedures. The retrospective study of 1,079 procedures by Koshy et al. also examined the effects of transfusion. [12] This found that perioperative transfusion was associated with a lower rate of SCD-related complications in hemoglobin SS patients undergoing low-risk procedures, with crude rates of 4.8% with transfusion and 12.9% without transfusion. The discrepancy between this outcome and that reported by Griffins et al. [10] may be attributable to differing populations. In 1995 Vichinsky et al. published the results of a prospective randomized trial that examined target goals for transfusion for 604 surgical procedures. [9] This study compared an aggressive transfusion regimen, designed to decrease the hemoglobin S concentration to less than 30%, with a conservative regimen.

By reviewing the files of sickle cell disease patients, we noticed that the exchange blood transfusion did not prevent occurrence of postoperative complications if HBS was still more than 40% regardless of the level of HBA. 22.2% of those patients who were exchanged developed sickle cell crisis and 5.5% developed acute chest syndrome.

The acute chest syndrome, one of the most frequent clinical complications, developed in 4.9% of the patients in our study. It is an acute pneumonia-like complication of SCD, is defined on the basis of the finding of a new pulmonary infiltrate involving at least one complete lung segment on chest radiograph, consistent with alveolar consolidation, but excluding atelectasis. Additional diagnostic features include chest pain, pyrexia >38.5, tachypnoea, wheezing, or cough. Precipitants include a variety of infectious pathogens, fat embolism after bone marrow infarction, pulmonary infarction and surgical procedures. In our study, there were four cases of ACS. These patients had had pre existing pulmonary pathology in the previous year and underwent adenotonsillectomy, laparoscopic cholecystectomy, and arthroscopy. Two of these patients did not receive any transfusion and two had EXT and SXT. The Hb concentration was >10 gms and HbS was 40% - 60%. Past history of pulmonary disease with decrease in oxygenation and ventilation-perfusion mismatch may increase the risk of ACS(12). Our patients improved with the standard treatment of incentive spirometry, bronchodilators, oxygen, adequate analgesia, broad spectrum antibiotics and simple transfusion. They were hospitalized for about 11 days and were discharged home.

We concluded from this retrospective study that Type of procedure is a significant predictor of SCD specific complications. Blood transfusion is a critical and, in many circumstances, life-saving part of evolving strategies for the treatment of patients with SCD. Because of the complications associated with transfusions, and to a lesser extent the cost, many areas of controversy remain regarding some indications for transfusions. Simple conservative transfusions may be required for major surgeries. Exchange transfusions carry more complications and are not proved to be more effective than conservative approach. Optimal management of patients with SCD undergoing major surgery is undefined. A likely predictor of the need for transfusion maybe the patients prior history of complications associated with anesthesia.

Preoperative evaluation of patients by a hematologist is recommended. Our results have to be substantiated by a prospective study conducted on a larger group of patients.[16]

   References Top

1.National Heart, Lung, and Blood Institute. Clinical alert from the National Heart, Lung, and Blood Institute (December 5, 2004). Available at prof/blood/sickle/clinical-alert-scd.htm. Accessed March 15, 2005.  Back to cited text no. 1      
2.WF, Telen MJ, Ware RE. Transfusion support for patients with sickle cell disease. Bethesda (MD): AABB Press; 1998.  Back to cited text no. 2      
3.Germain S, Brahimi L, Rohrlich P, et al. [Transfusion in sickle cell anemia]. Pathol Biol(Paris) 1999;47(1):65-72 [in French].  Back to cited text no. 3      
4.B, Sally C. Surgery in sickle cell disease. Hematology/oncology clinics of North America, W.B. Saunders Company, 2005; Volume 19(5)  Back to cited text no. 4      
5.Wali Y.A., al Okbi H., al Abri R., A comparison of two transfusion regimens in the perioperative management of children with sickle cell disease undergoing adenotonsillectomy. Pediatr Hematol Oncol 2003; 20: 7-13  Back to cited text no. 5      
6.Ohene-Frempong K., Indications for red cell transfusion in sickle cell disease. Semin Hematol 2001; 38: 5-13  Back to cited text no. 6      
7.Riaddington C, Williamson L. Preoperative blood transfusion for sickle cell disease. Cochrane Database Syst Rev.2001; 3:CD003149  Back to cited text no. 7      
8.Adams D.M., Ware R.E., Schultz W.H., et al. Successful surgical outcome in children with sickle hemoglobinopathies: the Duke University experience. J Pediatr Surg (1998) 33: 428-432.   Back to cited text no. 8      
9.Vichinsky E.P., Haberkern C.M., Neumayr L., et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease: the Preoperative Transfusion in Sickle Cell Disease Study Group [see comment]. N Engl J Med 1995; 333 : 206-213.   Back to cited text no. 9      
10.Griffin T.C., Buchanan G.R., Elective surgery in children with sickle cell disease without preoperative blood transfusion. J Pediatr Surg 1993; 28: 681-685.   Back to cited text no. 10      
11.Buck J., Casbard A., Llewelyn C., et al. Preoperative transfusion in sickle cell disease: a survey of practice in England. Eur J Haematol 2005; 75 : 14-21.  Back to cited text no. 11      
12.Koshy M., Weiner S.J., Miller S.T., et al. Surgery and anesthesia in sickle cell disease: Cooperative Study of Sickle Cell Diseases. Blood 1995; 86 : 3676-3684.   Back to cited text no. 12      
13.Wales P.W., Carver E., Crawford M.W., et al. Acute chest syndrome after abdominal surgery in children with sickle cell disease: is a laparoscopic approach better?. J Pediatr Surg 2001; 36 : 718-721.   Back to cited text no. 13      
14.Haberkern CM , Neumayr LD , Orringer EP , et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Preoperative Transfusion in Sickle Cell Disease Study Group . Blood 1997 ; 89 : 1533-42   Back to cited text no. 14      
15.Eckman JR. Techniques for blood administration in sickle cell patients. Semin Hematol2001;38(Suppl 1):23-29.  Back to cited text no. 15      
16.Kemp J.S., Obstructive sleep apnea and sickle cell disease [comment]. J Pediatr Hematol Oncol 1996;18 : 104-105.  Back to cited text no. 16      


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


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