Surgery in patients with hemoglobin SC disease

Neumayr, Lynne; Koshy, Mabel; Haberkern, Charles M.; Earles, Ann N.; Bellevue, Rita; Hassell, Kathryn L.; Miller, Scott T.; Black, Dennis M.; Vichinsky, Elliott

doi:10.1002/(sici)1096-8652(199802)57:2<101::aid-ajh2>3.0.co;2-#

Cited by 66 publications

(14 citation statements)

References 41 publications

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“…Based on these findings, simple transfusions are at present preferable in the preoperative management of SS patients. Limited exchange transfusions might still be required for those few SS patients with higher haematocrits or for patients with Hb SC disease ( Neumayr et al , 1998 ).…”

Section: Randomized Trials Of Red Cell Transfusions: Efficacy and Thementioning

confidence: 99%

Management of Sickle Cell Disease: Recent Advances and Controversies

Castro

1999

Br J Haematol

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Section: Randomized Trials Of Red Cell Transfusions: Efficacy and Thementioning

confidence: 99%

Management of Sickle Cell Disease: Recent Advances and Controversies

Castro

1999

Br J Haematol

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“…Abdominal procedures may also be associated with increased risk, particularly in patients with HbSC disease. 26 Anecdotal experience suggests that prophylactic transfusions can reduce the frequency of painful episodes in patients refractory to hydroxyurea, but only as part of a comprehensive pain program.…”

Section: Resultsmentioning

confidence: 99%

Best practices for transfusion for patients with sickle cell disease

Wun

Hassell

2010

Hematol Rep

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The β-globin gene mutation in sickle cell anemia results in anemia and repeated bouts of vascular occlusion. The cumulative effect of these vasocclusive events is progressive damage to many organs including the kidneys, lungs, and brain. The transfusion of red blood cells (RBC) can ameliorate many of these complications, but can be associated with both acute and chronic complications, including iron overload. The objective of the Best Practices in Transfusion Medicine for Patients with Sickle Cell Disease (SCD) Conference was to review the available published evidence and clinical experience surrounding the use of RBC transfusions for sickle cell disease by a panel of experts. The expert panel developed explicit clinical guidelines for the use of RBC in SCD patients. The panel also made recommendations for further research. A set of guidelines were produced for dissemination to pertinent stakeholders. If implemented, these clinical pathways have the potential to optimize the use of red blood cell transfusions in SCD.

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“…described a statistically significant reduction of SCD-dependent complications from 12.9% to 4.8% using blood transfusions [4], and Neumayr et al . reported that transfusions decreased complications from 18% to 9% [11]. In contrast, Vichinsky et al .…”

Section: Discussionmentioning

confidence: 99%

The use of partial exchange blood transfusion and anaesthesia in the management of sickle cell disease in a perioperative setting: two case reports

et al. 2010

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IntroductionHomozygous sickle cell carriers have an increased perioperative mortality. Some indications may justify an exchange blood transfusion to reduce the proportion of haemoglobin S. The advantages of general blood transfusion in a perioperative setting have not been proven and thus remain controversial. It is not clear whether reducing the proportion of haemoglobin S minimizes perioperative complications or whether patients with sickle cell disease in a stable clinical condition benefit from an exchange blood transfusion in a perioperative setting.Case presentationWe report the case of two Angolan children aged 10 and 11 respectively, of African origin with sickle cell anaemia who underwent surgery to treat chronic necrosis, fistula of the bones and bone destruction. This presentation describes the perioperative course, including general anaesthesia. A partial exchange blood transfusion decreased S-haemoglobin levels from 81% to 21% and simultaneously treated the anaemia.ConclusionThere is a consensus that imbalances in homoeostasis, including operative procedures, can cause a critical exacerbation of sickle cell disease. The case presented here illustrates a strategy for successfully managing sickle cell disease in the perioperative period to minimize its complications. It is important for the anaesthesiologist to carefully manage pulmonary gas exchange and to ensure sufficient tissue perfusion, balanced fluid resuscitation and normothermia, while keeping in mind the level of organ impairment in order to prevent an acute exacerbation of sickle cell disease.We performed a partial exchange blood transfusion due to the following factors: high haemoglobin S-fraction, anaemia, operating procedure at several sites, and difficult management of body temperature. Esmarch ischemia is an established tool for preventing uncontrolled blood loss. There is no known contraindication for this, but attention must be paid to prevent uncontrolled tissue ischemia and acidosis. The use of regional anaesthesia should be considered for postoperative pain management.

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Surgery in patients with hemoglobin SC disease

Cited by 66 publications

References 41 publications

Management of Sickle Cell Disease: Recent Advances and Controversies

Management of Sickle Cell Disease: Recent Advances and Controversies

Best practices for transfusion for patients with sickle cell disease

The use of partial exchange blood transfusion and anaesthesia in the management of sickle cell disease in a perioperative setting: two case reports

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