A physician in the United Kingdom wonders how others regard intraoperative cell salvage for patients with sickle cell disease (HbSS or HbSC). He has been asked to consult on the suitability of red cell salvage in a Jehovah's Witness who has SS disease and who needs to undergo myomectomy. He wonders if there a significant risk of sickling in the extracorporeal flow? For non-Jehovah's Witness patients with a similar medical condition, but who do not refuse transfusions, would elective red cell exchange be advisable prior to surgery?

The following comments have been received.

ADDENDA Mar 23, 2005

1. According to Dr. Ch. P. Henny (an Anesthesiologist/Intensivist at the University of Amsterdam in The Netherlands - attribution used with permission) intraoperative cell salvage is contraindicated in patients with sickle cell anemia. In addition, he is of the opinion that other (relative) contraindications for intraoperative cell salvage include: salvage of blood from a site of oncological surgery (unless the blood is irradiated with 50Gy, although some say that an adequate reduction of tumor cells may be accomplished by using a leukocyte filter), infection, use of local hemostatics, and other substances which may activate coagulation. As to published experience about red cell salvaging in sickle cell patients, as far as he knows, there are only a few case reports (Cleve Clin J Med 1994;61(2):137-40, Anesthesiology 1990;73(1):177-9, Anesthesiology 1989;70(5):878-9). Concerning Jehovah's witnesses who are not sickle cell disease patients, he uses the following strategy in case of elective surgery: preoperative optimization of the hemoglobin concentration up to 9.5 mmol/l using rH-erythropoietin in combination with either oral or i.v. iron, intraoperative acute nomovolemic hemodilution (in such a way that continuation with the patient's circulation is maintained, which is generally accepted by Jehovah's witnesses), intraoperative cell salvage (in case of a relative contraindication only used if it will save the patien's life), administration of tranexamic acid and/or DDAVP when necessary, postoperative cell salvaging and if necessary treatment with i.v. iron sucrose with or without rH-EPO. In his experience, in some cases it is even necessary to keep the patient under anesthesia using muscle relaxants for several days or longer, due to extremely low Hb concentrations. If blood samples have to be taken it is important to use micro samples (pediatric collection tubes) since frequent blood sampling may cause iatrogenic anemia. Finally when it concerns a female patient the operation should be planned when she is not menstruating.

2. According to Dr. Keith Quirolo of Children's Hospital in Oakland, California (attribution used with permission) his experience is that exchange transfusion is NOT superior to simple transfusion with regards to clinical outcome of surgery for patients who have sickle cell anemia (hemoglobin SS). He will employ exchange transfusion for sickle cell disease patients in advance of surgery if the patient has a relatively high hemoglobin level of 9.0 g/dl or higher, in order to cause a significant change in the patient's hemoglobin S level. He acknowledges having no experience with using a cell saver during surgery, but his guess would be that hemoglobin polymerization and hemolysis might occur. His group has performed surgery after treating patients with erythropoietin to increase low hemoglobin levels when the patients could not be transfused. The group has even collected autologous units of blood from some Sickle Cell disease patients, but the units were never used during surgery. He suggests colleagues might find the following references of interest:

• Vichinsky EP, Haberkern CM, Neumayr L, Earles AN, Black D, Koshy M, Pegelow C, Abboud M, Ohene-Frempong K, Iyer RV. 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. N Engl J Med. 1995 Jul 27; 333(4):206-13.
• Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK, Khako Y, Kinney TR. Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood. 1995 Nov 15; 86(10):3676-84.
• Haberkern CM, Neumayr LD, Orringer EP, Earles AN, Robertson SM, Black D, Abboud MR, Koshy M, Idowu O, Vichinsky EP. 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 Mar 1; 89(5):1533-42.
• Neumayr L, Koshy M, Haberkern C, Earles AN, Bellevue R, Hassell K, Miller S, Black D, Vichinsky E. Surgery in patients with hemoglobin SC disease. Preoperative Transfusion in Sickle Cell Disease Study Group. AM J Hematol. 1998 Feb; 57(2):101-8.
• Waldron P, Pegelow C, Neumayr L, Haberkern C, Earles A, Wesman R, Vichinsky E. Tonsillectomy, adenoidectomy, and myringotomy in sickle cell disease: perioperative morbidity. Preoperative Transfusion in Sickle Cell Disease Study Group. J Pediatr Hematol Oncol. 1999 Mar-Apr; 21(2):129-35.
• Vichinsky EP, Neumayr LD, Haberkern C, Earles AN, Eckman J, Koshy M, Black DM. The perioperative complication rate of orthopedic surgery in sickle cell disease: report of the National Sickle Cell Surgery Study Group. Am J Hematol. 1999 Nov; 62(3):129-38.

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