Anesthetic management of a patient with sickle [beta+] thalassemia

Sickle cell disease is a congenital condition and its most common clinical manifestation is anemia due to chronic hemolysis. Persistent and accelerated hemolysis associated with multiple transfusions is a recognized risk factor for the development of cholelithiasis. The occurrence of gallstones is one of the most important manifestations of sickle cell disease in the digestive tract. Most gallstones are pigmented and characteristically occur at younger ages and the prevalence of cholelithiasis increases progressively with age, affecting 50% of young adults. Cholecystectomy is the most common surgical procedure performed in sickle cell disease patients. Anesthesia in this population of patients for major surgeries deserves special attention due to various complications particularly silent infarctions of end organs are common. We are reporting a 14-year-old girl diagnosed with sickle cell anemia and [beta+] thalassemia with cholelithiasis went for cholecystectomy under general anesthesia. Although the patient has both beta[+] thalassemia and sickle cell disease component, the latter is of more concern for anesthesia

Splenectomy in thalassemia

The aims of our study were to illustrate the most common indications for splenectomy in patients with beta thalassemia and to clarify the effect of splenectomy on blood transfusion requirements in our patients. This is a retrospective study of 36 patients with B thalassemia who underwent splenectomy in Prince Rashed Ben Al-Hassan Hospital in Irbid in the northern area of Jordan over a period of 10 years, from January 1993 to January 2003. Case histories were reviewed with regard to age, sex, indication for splenectomy, blood transfusion requirements [both amount and frequency], hematocrit level [pre and post splenectomy], hepatitis C virus [HCV] antibodies, preparations made for surgery, and post-operative complications. Increased transfusion requirements, hypersplenism, and massive splenomegaly were the most common indications for splenectomy in beta thalassemia patients. For 6-12 preoperative months, the frequency of blood transfusion had increased from nearly monthly intervals [10-12 times per year] up to more than 20 to 24 times per year. The amount of blood required had increased from a mean of 150 cc /kg/year to more than a mean of 280cc/kg/year during the same period of preoperative time. Post splenectomy, patients with beta thalassemia major returned to a monthly transfusion regimen [15-28 months, mean 21.5 months]. In patients with beta thalassemia intermedia, the effect of splenectomy was longer lasting [36-48 months, mean 42 months]. Hepatitis C infection was seen in 20 beta thalassemia patients [56%] before splenectomy. Splenectomy is beneficial for children with beta thalassemia and hypersplenism because it reduces blood transfusion requirements even if only for a temporary period. Polyvalent vaccines should be given to all patients before splenectomy to reduce complications

[Early hepatic complication in frst year after bone marrow transplantation in major beta thalassemic patients]

Bone marrow transplantation is a good therapeutic modality for beta thalassemia. Liver complications is one of the major causes of morbidity and mortality following BMT. Determination of the factors of liver injury leads to earlier diagnosis after BMT and improves prognosis. We studied 113 major Beta thalassemic patients who have been transplanted from 1990- 2000 in bone marrow transplantation center of Shariati Hospital. 62 were male and 51 were female. 27 patients were class one, 56 were class two and 30 were class three. The median age of each classes were 6.5, 6.3 and 8.7 year. Conditioning regime consisted of busulfan [3.5-4mg/Kg] and cyclophophamide [40-50 mg/kg]. For GVHD prophylaxis we gave cyclosporine +/- metothrexate. Grade of liver fibrosis defined by biopsy in all patients before BMT. All patients and their donors tested for HBsAg, HBsAb, HCVAb, CMVAb with RIA method. We assessed causes of liver dysfunction before and after transplantation and effect of high ferritin level on liver function. Hepatic dysfunction in first year after transplantation were seen in 86 [76%] patients. Causes of liver dysfunction were consisted of 53.1% GVHD, 15.93% cyclosporine hepatotoxicity, 5.3% conditioning regime hepatotoxicity and 1.77% VOD. In all three classes hepatic GVHD, cyclosporine toxicity, death and normal liver function post BMT had significant relation with hepatic dysfunction before BMT [p=0.001]. In patients with ferritin level more than 1000, there were significant hepatotoxicity with conditioning regime [p=0.001]. 17 [15.04%] of patients have been died. In this study we determined incidence and causes of hepatic dysfunction before and after BMT in major beta thalassemic patients. According to our study the incidence of hepatic dysfunction was 76.1% and hepatic GVHD and drug hepotoxicity were the most common causes of hepatic dysfunction in all three classes. Serum ferritin level had not significant relation to GVHD, cyclosporine hepatotoxicity and VOD

Umbilical cord blood transplantation in children with beta-thalassemia diseases.

To evaluate factors affecting the outcome of sibling and unrelated donor umbilical cord blood transplantation (CBT) in Thai children with beta-thalassemia diseases. The case-series study of all children undergoing such transplants in our institute was conducted Six children with thalassemia major were diagnosed at a median age of 1.5 years and CBT was performed at a median age of 5.5 years (range 2-15). Six donors consisted of three HLA-identical siblings, one two-allele, one three-antigen mismatched sibling, and one one-allele mismatched unrelated cord blood. The median number of nucleated cells infused was 2.83 x 10(7)/kg (range 1.49-5.3); the median number of CD34+ cells infused was 1.94 x 10(5)/kg (range 0.2-5.3). In all, two patients had complete donor engraftment; three had mixed chimerism (MC); one patient died of cerebral thrombosis and neutropenic septicemia. Of the two complete donor-engrafted patients, two developed grade 2 acute graft-versus-host disease (GVHD) which responded well to immunosuppressive therapy. Of the three mixed-chimeric patients, two were clinically cured. With a median follow-up of 7 months (range 2-30), five children survived and have done well with transfusion-independent. Umbilical cord blood provides a reasonable option for hematopoietic stem cell source to transplant for beta-thalassemia diseases and the outcome in the present study was good.

Portal vein thrombosis after splenectomy for beta-thalassemia major

Portal vein thrombosis is a recognized complication after splenectomy in beta-thalassemia major due to the chronic hypercoagulable state which has been recognized to exist in childhood thalassemia and contribute to thromboembolic events. We are reporting one patient with beta-thalassemia major developed portal vein thrombosis following splenectomy

Hemodynamic responses to captopril during splenectomy in thalassemic children.

Splenectomy in beta-thalassemic children is frequently accompanied by perioperative hypertension which occasionally is followed by convulsion. The efficacy of captopril in attenuating the hypertensive response to splenectomy was investigated in 82 thalassemic children. The control group, consisting of 40 patients, received intravenous furosemide (1 mg/kg) preoperatively; whereas, 42 children were randomly allocated into 2 groups to receive oral captopril (0.7 mg/kg) or a combination of captopril (0.7 mg/kg) and furosemide (1 mg/kg) before the operation. Before anesthetic induction, both systolic and diastolic arterial pressures in the captopril and the combined groups were significantly lower than the furosemide group (P < 0.001), whereas, the heart rates in all groups were comparable. Changes in arterial pressure in response to the operation were significantly smaller in the combined group when compared with the other two groups (P < 0.001). Immediate postoperative hypertension requiring additional management occurred in 20 per cent of the furosemide group, and 14.3 per cent in the other two groups. One patient in the combined group had a convulsion in association with hypertension. The authors conclude that captopril combined with furosemide effectively controls intraoperative hypertension in thalassemic children undergoing splenectomy; however, postoperative hypertension remains common, and needs appropriate treatment immediately.