The effects of exchange transfusion for prevention of complications during pregnancy of sickle hemoglobin C disease patients.

BACKGROUND: Pregnancy represents a challenge for women with sickle cell disease (SCD), with higher rates of both maternal and fetal complications. The aim of this study was to evaluate the impact of prophylactic transfusion support administered specifically to pregnant women with sickle hemoglobin C disease. MATERIALS AND METHODS: Patients were divided into two groups according to the type of transfusion support received: 10 women received prophylactic erythrocytapheresis or manual exchange transfusion at 28 weeks of gestation, and 14 received transfusions only on demand, due to acute complications, or received no transfusions at all. RESULTS: Our results indicated higher frequencies of SCD-related complications in the group that did not receive prophylactic transfusion support (35.7% vs. only 10% in the erythrocytapheresis group). Furthermore, these complications were more severe in this group and included all cases of acute chest syndrome. A significant difference was observed concerning gestational age at birth (38.7 weeks in the transfusion group vs. 34.4 weeks, p = 0.037), with a higher frequency of preterm births in the nontransfused group (69.23% vs. 30% in the transfusion group). CONCLUSION: We demonstrated a clear reduction of unfavorable outcomes in patients receiving prophylactic transfusions, probably reflecting better maternal and fetal conditions, which corroborated to the more satisfactory indices of vitality, observed in newborns. Considering that erythrocytapheresis or manual exchange transfusions both represent feasible and safe procedures, they could represent important tools for the optimal management of these patients.

[Assessment of the use of transfusion therapy and complications in orthopedic surgery in patients with sickle-cell anemia: retrospective study]. / Analyse des pratiques transfusionnelles et complications chez le patient drépanocytaire en situation de chirurgie orthopédique: étude rétrospective.

Red blood cells (RBCs) transfusion is a common practice in the treatment or for the prevention of complications of patients with sickle-cell disease. In surgery, pre-operative transfusions are frequently given to prevent peri-operative complications. There is no consensus however on the best regimen of transfusion for this purpose. The transfusion techniques are muliple. In addition, pre-operative transfusion therapy is reported to be largely responsible for an increased morbidity and mortality in patients with sickle cell anemia undergoing surgery. During the period 1990-2000, 16 patients (4 men and 12 women) with a mean age of 37 years and various major sickle cell hemoglobinopathies underwent 32 total hip arthroplasty for femoral head necrosis. Nine patients with sickle-cell trait were included as control group. Twelve of them had haemoglobin SS (HbSS), 2/16 had HbSC, 2/16 had HbS/betathalassemia. Operative transfusion were given in only 12/32 procedures, 4 were performed pre-operatively and 8 intra-operatively. Simple transfusion (mean: 2.5 packed red cells) were administered in all the procedures. The main complications observed in our patients were anemia by hemolysis and haemorrhagic shock, vaso-occlusive crisis and chest syndrome. Anemia requiring transfusions was significatively related to the procedures with pre-operative transfusion. In the light of our result, we would like to propose transfusional protocol--if needed--only intra-operatively.

The obstetric performance of sickle cell disease patients and homozygous hemoglobin C disease patients in Ile-Ife, Nigeria.

In a 9-year period (1980-1988), 46 delivered hemoglobinopathy patients were studied. There was a high incidence of complications during pregnancy. The perinatal and maternal mortalities were 13.4% and 9.2% respectively. The complications could be minimized by adequate supervision in pregnancy, labor and puerperium.

The hemoglobinopathies and pregnancy in Lagos.

Thirty-four patients with abnormal hemoglobin were studied through 42 pregnancies under one obstetrician. There were 30 patients with sickle cell anemia (HbSS), two with sickle cell hemoglobin C disease (HbSC) and two with homozygous hemoglobin C disease (HbCC). There were 39 live births (including one pair of twins), and four perinatal deaths. The patients with HbSC and HbCC had five uncomplicated pregnancies and deliveries. Of the 36 pregnancies in patients with HbSS one aborted at 12 weeks. Intra-uterine growth retardation (14.3%) and pregnancy-induced hypertension (14.3%) were the most serious pregnancy complications. No patient had more than one crisis. Only one out of the 10 patients transfused needed more than two units of blood throughout pregnancy. The mean gestation at delivery was 37.5 +/- 3.2 (S.D.) weeks. The mean birth weight was 2.7 +/- 0.6 (S.D.) kg. The perinatal mortality was 114.3 per thousand live births and there was one maternal death.

Pregnancy and sickle cell hemoglobinopathies: results with and without prophylactic transfusions.

Maternal morbidity and perinatal outcome in 108 pregnancies complicated by sickle cell anemia (hemoglobin SS disease), sickle cell-hemoglobin C disease (hemoglobin SC disease), or sickle cell-beta-thalassemia disease were analyzed. Women given prophylactic red cell transfusions (1973-1982) during pregnancy were matched with historic controls whose care was almost identical except that blood was not given unless indicated (1955 to 1972). In women with hemoglobin SS disease who received prophylactic red cell transfusions, there was a sevenfold reduction in perinatal mortality and negligible maternal morbidity. In pregnancies complicated by hemoglobin SC disease during which transfusions were given, there were no perinatal losses, whereas there were in 18% of women not given transfusions. Maternal morbidity in women given transfusions was negligible; however, half of those not transfused experienced morbidity and, importantly, pulmonary complications were common. Transfusion-related complications included hepatitis and alloimmunization. From these experiences the authors conclude that prophylactic red cell transfusions reduce maternal morbidity and perinatal mortality appreciably, although perinatal morbidity is not eliminated. Transfusion therapy is justifiably started early in pregnancy for women with hemoglobin SS disease; however, transfusions may be withheld until the end of the second trimester for women with hemoglobin SC or sickle cell-beta-thalassemia disease.

Outcome of pregnancy in sickle cell anemia and sickle cell-hemoglobin C disease. An analysis of 181 pregnancies in 98 patients, and a review of the literature.

The outcome of pregnancy has been analyzed in 72 women with sickle cell anemia (SS) and 26 women with sickle cell-hemoglobin C disease (SC), part of an unselected population of 148 women over 18 years of age with these hemoglobinopathies, who have been followed at a sickle cell disease clinic for 8 years. In SS women, 22% of first pregnancies were aborted spontaneously, and the overall early fetal loss was 19.2%. A similar figure was calculated from the literature since 1956. In SC women, 12% of first pregnancies were lost, but the overall early fetal loss was only 8.9%. The perinatal mortality, under quite variable conditions of prenatal care and delivery, was 10.2% in SS women and 2% in SC women. There were no stillbirths or midterm deaths in utero among SC women, but these accounted for most of the perinatal mortality in SS women, particularly in first and second pregnancies. There was one neonatal death in each group, but eight third pregnancies in SS women were completed without perinatal mortality. A remarkable finding among the SC women was the number of successful pregnancies, ten in one woman, and many of the pregnancies were completed without the supervision of a physician. Our findings were compared with those in the literature, and the conclusion that we drew is that termination of pregnancy and sterilization of young women are not generally indicated solely on the basis of these hemoglobinopathies.

Red cell exchange in the pregnancy complicated by a major hemoglobinopathy.

A new method of partial erythrocyte exchange for pregnancies complicated by a major hemoglobinopathy is described. The Haemonetics 30 Cell Separator allows efficient withdrawal and discard of the patient's erythrocytes. The patient's leukocytes, platelets, and plasma are conserved and returned with washed, compatible donor red cells which contain Hb AA. Three pregnant black women with significant hemoglobinopathies were treated by this modality during their pregnancies. Vaginal term deliveries were accomplished in each case. The method offers advantages in the efficiency of blood quantity displacement, patient comfort, adaptability to the patient with severe anemia, and the use of outpatient facilities. The method is suggested for use whenever a patient with an obstetrically significant hemoglobin variant needs transfusions of erythocytes.

Prophylactic transfusions of normal red blood cells during pregnancies complicated by sickle cell hemoglobinopathies.

Prophylactic transfusions of normal donor red cells were administered during 37 pregnancies to women with sickle cell anemia, sickle cell-hemoglobin C disease, or sickle cell-beta thalassemia disease. Once the diagnosis was confirmed, the transfusions were administered intermittently throughout the rest of the pregnancy in such amounts and at such frequencies that no more than 60% of the circulating red cells contained hemoglobin S and the hematocrit was above 25. The maternal mortality rate was zero and maternal morbidity as the consequence of the sickle cell hemoglobinopathy was minimal. The perinatal mortality rate was appreciably reduced when compared to that previously observed without prophylactic transfusions but perinatal morbidity was still excessive. Evidence that the intrauterine environment was compromised, in spite of the transfusions consisted of an increased frequency of growth-retarded fetuses, of meconium staining of amnionic fluid, and of ominous decelerations of fetal heart rate. Morbidity from the transfusions was troublesome. Nonetheless, it is concluded tentatively that both the mother with a sickle cell hemoglobinopathy and her fetus are likely to benefit from prophylactic transfusions of normal donor red cells administered during one pregnancy according to the protocol employed in this study.

Hemoglobin S/C disease in a pregnant woman with crisis and fat embolization syndrome.

The complications of painful crisis and megaloblastic anemia are hallmarks of the pregnant patient with hemoglobin S/C disease. We describe here the clinical course in a patient with hemoglobin S/C disease in whom painful crisis and the fat embolization syndrom developed postpartum with severe neurologic abnormalities. Response to exchange blood transfusion was dramatic, and the patient recovered without neurologic impairment.

Sickle cell crisis treated by exchange transfusion. Treatment of two patients with heterozygous sickle cell syndrome.

In two patients with severe painful sickle cell crisis and a deteriorating clinical course, recovery coincided with large-scale exchange transfusions (6 liters). The one patient, with sickle cell beta thalassemia, achieved a 90% exchange, calculated on the sickle cell hemoglobin present before and after transfusion. The other patient, with sickle cell hemoglobin Hb A, S, and (Hb) C disease, also achieved an 80% exchange calculated on the level of HbA.S. AND C in samples before and after transfusion.