Preoperative Exchange Transfusion for Sickle Cell Disease Patients Undergoing Open‐Heart Surgery: An Exception to the Rule

Abstract Preoperative exchange transfusion is a routine practice in patients with sickle cell disease having elevated sickle cell hemoglobin levels (>40%) undergoing open‐heart surgery on cardiopulmonary bypass. A new approach toward acceptance and management of sickle cell disease patients with high sickle cell hemoglobin levels for open‐heart surgery without preoperative exchange transfusion of blood is presented. (J Card Surg 2010;25:691‐693)     

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

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 transfusions (STX) and 54.1% had no transfusion (NTX). 14.1% of all patients had postoperative complications, and 50% of those, had complications 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 does not prevent perioperative complications of sickle cell disease patients. HBSS > 40% carries a higher risk of postoperative complications.     

Treatment and prevention of stroke in children with sickle cell disease

Opinion statement Stroke is one of the major complications in children with sickle cell disease (SCD). Ischemic stroke is associated with small asymptomatic subcortical infarcts to large territorial lesions causing major disability. Intracranial hemorrhages may be caused by aneurysm rupture or by leakage from moyamoya vessels or venous sources. There have been no acute stroke treatment studies in SCD, but hydration and exchange transfusion are often recommended. However, there is an evidence base for primary and to some extent secondary stroke prevention. Primary prevention of stroke was demonstrated in the Stroke Prevention Trial in Sickle Cell Anemia (STOP), in which children with transcranial Doppler (TCD) mean blood flow velocities of 200 cm/second (previously shown to indicate high stroke risk) or higher were randomized to either regular blood transfusions or no transfusion. The study showed a very significant 90% reduction in first stroke with transfusion. In STOP2, discontinuing transfusions after 30 months or more (even with normal TCD) resulted in a high rate of reversion to abnormal TCD values and stroke. TCD screening of all children with SCD, and initiation and maintenance of chronic transfusion to maintain hemoglobin S below 30% in the high-risk group, is the only proven prevention strategy for stroke in SCD. Hydroxyurea is being studied as secondary stroke prevention at this time. No recommendation specific to SCD regarding the use of antiplatelet agents or anticoagulants in ischemic stroke can be made. Bone marrow transplantation can be curative for SCD, and limited data support its use to prevent stroke in SCD.     

Delayed haemolytic transfusion reaction and hyperhaemolysis complicating peri-operative blood transfusion in sickle cell disease

We present a case of delayed haemolytic transfusion reaction and hyperhaemolysis syndrome in a patient with sickle cell disease. A 32-year-old woman with a history of sickle cell disease was scheduled for total hip replacement. She was transfused pre-operatively and suffered a delayed haemolytic transfusion reaction. Postoperatively the patient continued to haemolyse, despite the use of antigen compatible blood, suggesting that she had developed hyperhaemolysis syndrome following her delayed haemolytic transfusion reaction. Although rare, both conditions must be borne in mind when dealing with patients who have undergone multiple transfusions.     

Transfusion-related acute lung injury or acute chest syndrome of sickle cell disease? — A case report

PURPOSE: To describe how to differentiate transfusion-related acute lung injury from acute chest syndrome of sickle cell disease. Clinical features: A neurosurgical patient with sickle cell disease received two units of packed red blood cells postoperatively. Four hours later she developed progressive respiratory distress, diffuse geographical airspace disease and bilateral pulmonary edema. The patient recovered sufficiently to be transferred from the intensive care unit within four days. The temporal relationship to transfusion, features on computerized tomographic scan, and the rapid resolution of severe edema point to a diagnosis of transfusion related acute lung injury. Granulocyte or human leukocyte antigen antibodies in donor plasma may confirm a diagnosis of transfusion injury. CONCLUSION: The clinician should appreciate that erythrocyte transfusion to prevent or treat acute chest syndrome may cause transfusion related acute lung injury, a condition that mimics, exacerbates or possibly triggers the syndrome it was intended to treat.     

Drépanocytose et thrombose des veines cérébrales internes

Cerebrovascular disorders are frequently associated with sickle cell disease, mainly in homozygous children. We report the case a 25-old-patient with known sickle cell disease who presented with coma inaugurated by manifestations of intracranial hypertension. CT revealed bilateral thalamic infarcts and angiography confirmed the thrombosis of internal cerebral veins. Treatment included heparin and blood transfusion. Severe cerebral oedema resulted in the lethal outcome three days later.     

Perioperative management of sickle cell disease in paediatric cardiac surgery

In sickle cell disease, cardiopulmonary bypass may induce red cell sickling. Partial exchange transfusion reduces the circulating haemoglobin S level. We report the management of a child with sickle cell disease who required surgical closure of a ventricular septal defect. Preoperative exchange transfusion of 50% of the total blood volume was performed with fresh packed red cells over three days. Further exchange transfusion was performed as cardiopulmonary bypass commenced. The haemoglobin S level was reduced from 76% to 37%. The blood removed from the patient during the exchanges was processed allowing storage and re-infusion of the patient's plasma and platelets. Combined preoperative and intraoperative exchange transfusions, instead of a single stage 50% volume exchange, was effective and potentially avoids larger haemodynamic effects. Cardiopulmonary bypass was conducted at normothermia and cold cardioplegia was avoided (fibrillatory arrest was used during the surgical repair).     

Stroke in children with sickle cell disease

Opinion statement Children with sickle disease are at high risk for ischemic stroke and transient ischemic attacks, usually secondary to intracranial arteriopathy involving the terminal internal carotid and proximal middle cerebral and anterior cerebral arteries, which may be diagnosed using transcranial Doppler ultrasound or magnetic resonance angiography (MRA). Other central nervous system (CNS) complications include seizures and coma, which may be secondary to ischemic stroke, sinovenous thrombosis, reversible posterior leukoencephalopathy, or acute demyelination. The immediate priority after an acute CNS event is to improve cerebral oxygenation with oxygen supplementation to maintain peripheral saturation measured using pulse oximetry between 96% and 99%, and a simple transfusion of packed cells within an hour of presentation if the patient’s hemoglobin is less than 10 g/dL. The patient then should have erythrocytapheresis or manual exchange to reduce the hemoglobin S percentage to below 30%. Computed tomography to exclude hemorrhage is mandatory and MR T2-weighted imaging with MRA, fat-saturated imaging of the neck (dissection), MR venography (sinovenous thrombosis), and diffusion-weighted imaging usually distinguishes between arterial ischemic stroke and the differential diagnoses. Comatose patients with widespread focal or global cerebral edema may have good functional outcome after surgical decompression. Anticoagulation may be indicated for dissection or sinovenous thrombosis and steroids for demyelination. Blood pressure should be reduced slowly if raised in patients with reversible posterior leukoencephalopathy. Seizures should be treated aggressively and electroencephalogram monitoring should be done to exclude subclinical seizures if the patient is unconscious. Hemorrhagic stroke may require craniectomy and drainage and/or management of vasospasm. Interventional neuroradiology with coils is an alternative to surgical clipping for aneurysms. For secondary prevention, regular blood transfusion to hemoglobin S of less than 30% reduces the risk of recurrent stroke from approximately 67% to approximately 10%. Hydroxyurea and phlebotomy may be used in patients who are alloimmunized. Moyamoya syndrome is a risk factor for recurrence despite prophylactic blood transfusion. Revascularization may prevent additional stroke. Bone marrow transplantation may be offered to patients with human leukocyte antigen-compatible siblings. Blood transfusion prevents stroke in patients with velocities greater than 200 cm per second on TCD; a phase III trial studying the prevention of the progression of silent infarction is being done. Emerging primary prophylaxis regimens being tested include citrulline and arginine, aspirin, and overnight oxygen supplementation. Physicians caring for children with sickle cell disease also should ensure adequate nutrition, including five servings of fruit and vegetables a day. The role of vitamin supplementation is controversial, particularly when patients must take daily penicillin prophylaxis.     

Guideline on the peri-operative management of patients with sickle cell disease

Sickle cell disease is a multisystem disease characterised by chronic haemolytic anaemia, painful vaso-occlusive crises and acute and chronic end-organ damage. It is one of the most common serious inherited single gene conditions worldwide and has a major impact on the health of affected individuals. Peri-operative complications are higher in patients with sickle cell disease compared with the general population and may be sickle or non-sickle-related. Complications may be reduced by meticulous peri-operative care and transfusion, but unnecessary transfusion should be avoided, particularly to reduce the risk of allo-immunisation. Planned surgery and anaesthesia for patients with sickle cell disease should ideally be undertaken in centres with experience in caring for these patients. In an emergency, advice should be sought from specialists with experience in sickle cell disease through the haemoglobinopathy network arrangements. Emerging data suggest that patients with sickle cell disease are at increased risk of COVID-19 infection but may have a relatively mild clinical course. Outcomes are determined by pre-existing comorbidities, as for the general population.     

Management of painful sickle cell crisis in pregnancy

A case of sickle cell disease crisis is described in a Saudi patient at 28 weeks of gestation. She was successfully managed with intravenous fluids, oxygen, opiates and blood transfusion.     

Autologous transfusion and hemoglobin SC disease

Three autologous blood units were transfused during elective orthopaedic surgery in a patient with undiagnosed haemoglobin SC disease. The packed red blood cells had been stored at 4 degrees C on SAG-M under standard conditions for 10 to 31 days. There was no evidence of adverse clinical reactions during the perioperative period. Six months later, a blood unit was collected at the initial step of an exchange transfusion in the same patient. Haemolysis was moderate after a 12-day-storage period and more significant after 32 days. This observation, as some other case reports, suggest that autologous blood transfusion may be considered for haemorrhagic surgery in selected patients with sickle cell disease.     

Stroke in children

Opinion statement  

Liver transplantation in a patient with S(beta)o-thalassemia

BACKGROUND: Patients presenting sickle cell disease may develop different types of hepatic complications. Intrahepatic cholestasis is a potentially fatal complication of the disease, and sometimes the only possible solution is transplantation. Postoperative transfusion management has not yet been well established. In this report, we describe the transfusional program of a patient presenting sickle cell disease and intrahepatic cholestasis who underwent liver transplantation 2 years ago. METHODS: Data were obtained from the chart and the blood bank records. RESULTS: The liver transplantation was performed successfully. Despite mild allograft dysfunction 3 months after surgery, secondary to intrahepatic sickling, the patient has been doing well with the transfusional management adopted (sickle-cell hemoglobin <20%). CONCLUSION: Sickle cell disease should not be a criterion for exclusion from liver transplantation. Regular transfusion with monitoring of sickle-cell hemoglobin is a very important measure to minimize the risk of intrahepatic sickling and possible rejection.     

Bilateral total knee replacement with tourniquets in a homozygous sickle cell patient

A 27-yr-old male patient, with homozygous sickle cell disease was scheduled for bilateral total knee replacement under tourniquet. The use of tourniquet in sickle cell patients is not without hazard. After preoperative exchange transfusion, total knee replacement was performed. The patient tolerated the procedure well. Patients with sickle cell disease should not be denied the benefit of a tourniquet if hematological correction has been undertaken. IMPLICATIONS: The use of a tourniquet in patients with sickle cell is controversial. The author describes a case of bilateral total knee replacement performed using a tourniquet in a patient with sickle cell disease.     

Laparoscopic cholecystectomy in sickle cell disease

STUDY AIM: Sickle cell affection is a public health problem in Africa. The aims of this prospective study were to evaluate the early results of laparoscopic cholecystectomy in sickle cell patients in Senegal. METHOD: From January 1998 to June 2002 all the sickle cell patients undergoing a laparoscopic cholecystectomy were included. Intra- and post-operative protocol (blood transfusion if Hb < 9 g/dl, rehydration, oxygenotherapy) was standardized. RESULTS: Forty-two patients with sickle cell of types SS-33 and AS9 were operated upon by same surgeon. One case of conversion due to an effraction of biliary junction was reported. One homozygote patient died post-operatively because of peritonitis. Two acute thoracic syndromes, three vaso-occlusive crisis, and two cases of wound infection constituted the post-operative morbidity. No case of complication was noted in those who underwent pre-operative transfusion. CONCLUSION: Laparoscopic cholecystectomy can be carried out in sickle cell patients with biliary lithiasis provided that general anaesthetic rules are respected.     

Elective cholecystectomy for the patient with sickle cell disease and asymptomatic cholelithiasis

Experience with elective cholecystectomy in ten patients with sickle cell disease and one patient with sickle-thalassemia from 1977-1984 was reviewed. In contrast to an earlier review from our institution, the current series had a low morbidity. This improvement is attributed to careful perioperative care, especially preoperative transfusion, hydration, and oxygenation. Because of the increasing longevity of sickle cell patients, because of the incidence of eventual significant complications of even asymptomatic cholelithiasis, and in order to simplify medical management by eliminating the diagnostic confusion between acute cholecystitis and sickle cell hepatobiliary crisis, the authors believe that elective cholecystectomy is to be recommended for the sickle cell patient with asymptomatic gall stones.     

Blood transfusion in patients with sickle cell disease requiring laparoscopic cholecystectomy

Background:

Surgery in patients with sickle cell disease is associated with high morbidity. To reduce this high morbidity, different preoperative transfusion regimens were introduced. However, blood transfusion is associated with problems. This prospective study aims to establish the safety of conducting laparoscopic cholecystectomy without transfusion in sickle cell disease patients.

Methods:

Forty patients (16 males and 24 females; mean age 26.6 years) undergoing laparoscopic cholecystectomy for cholelithiasis were divided into 2 matched groups: Group I “no transfusion” (n=24 patients; 60%) and Group II “transfusion” (n=16; 40%). In Group II, 9 patients (22.5%) received a simple transfusion and 7 (17.5%) a partial exchange transfusion.

Results:

Group II patients had significantly higher levels of Hb-S prior to transfusion. They developed a significantly higher complication rate (25% vs. 0%) and subsequently longer hospital stay (3.9±2 vs. 2.1±1.4). Moreover, there was no significant difference in the complications between the simple transfusion and partial exchange transfusion subgroups.

Conclusion:

Surgery in SCD patients is safe without a preoperative blood transfusion. Moreover, preoperative blood transfusion is associated with significantly higher postoperative complications and longer hospital stay. Hence, a “no transfusion” policy is recommended.     

Pediatric and newborn stroke

Opinion statement  In children, arterial ischemic stroke is more common than hemorrhage. The clinical presentation differs according to age, stroke type, and location. Seizures are more common with ischemia in children, especially in newborns. The presentation of pediatric ischemic stroke is more complex than in adults, so the clinical phenotype of ischemic stroke is modified. Risk factors for ischemic and hemorrhagic stroke include congenital heart disease, blood disorders, vasculopathies, infections (both current and preceding the stroke), and vascular malformations, but often no discernible etiology is determined. Current treatment is based on consensus rather than large, case-controlled studies. There is no strategy for primary prevention of pediatric or newborn stroke except in sickle cell disease. Most clinicians use aspirin for secondary prevention. Transfusion therapy is proven effective for secondary prevention of stroke associated with sickle cell disease. Prospective cohort studies are needed to understand the natural history of pediatric stroke and to determine which individuals are at greatest risk for incident and recurrent stroke. Effective treatment and prevention strategies can only be developed once the causes of stroke in children are understood and populations at greatest risk are identified.     

Sickle cell nephropathy: challenging the conventional wisdom

This review explores the current model of sickle cell nephropathy and the limitations of the model. Renal abnormalities are common complications of sickle cell disease (SCD). Beginning in childhood, patients with SCD develop a urinary concentrating defect resulting in polyuria and a predisposition to nocturnal enuresis and dehydration. The current model of sickle cell nephropathy suggests that destruction of the renal medulla induces production of renal vasodilating substances that feedback to the glomerulus causing hyperfiltration. Hyperfiltration leads to glomerulosclerosis and proteinuria, with eventual reduction in kidney function. The crucial steps of vasodilating substance production and hyperfiltration in children with SCD have not been proven. Treatment of sickle cell nephropathy is aimed at the reduction of proteinuria with angiotensin converting enzyme inhibitors or angiotensin receptor blockers. Hydroxyurea and chronic transfusion therapy may also alter the progression of sickle cell nephropathy in children. Further studies are needed to identify an accurate model and effective treatments for sickle cell nephropathy.     

Bone and joint infection in patients with sickle cell disease

Because of recent literature reports of the rare occurrence of osteomyelitis in patients with sickle cell disease, we reviewed 5 years of experience at Dhahran Health Center (Dhahran, Saudi Arabia). Twelve cases of bone and/or joint infection were identified in patients with sickle cell disease; 83% caused by Salmonella species. This relatively high incidence might be related to the common occurrence of infection with Salmonella in this region. Long bone and multiple site involvements were noticed. Differentiation from acute bone infarcts is difficult, and a systemic and aggressive approach to early diagnosis, management, and follow-up is suggested. Before therapy is started, full history, physical examination, blood cultures, local cultures, stool and urine cultures, and measurement of febrile agglutinin levels should be done. Once diagnosis is confirmed or highly suspected, adequate surgical drainage, prolonged parenteral antibiotic therapy, and transfusion of packed red blood cells should be used. A prolonged follow-up is recommended.