Sickle cell disease and pregnancy: analysis of 34 patients followed at the Regional Blood Center of Ribeirão Preto, Brazil.

OBJECTIVE: The objective of this study was to verify the evolution of pregnancies in sickle cell patients followed at one institution over a period of 12 years (January 2000 to June 2012). METHODS: The study evaluated 34 pregnant women with sickle cell disease with a mean age of 23.9±5.3 years. The incidence of obstetric complications, non-obstetric complications linked to sickle cell disease and complications in the newborn were analyzed. RESULTS: A total of 26% of the cases reported previous miscarriages, 20% had preterm labor, 10% had pre-eclampsia, and 5% had gestational diabetes. Forty-one percent of the deliveries were cesarean sections and 29% of patients required blood transfusions. In respect to sickle cell disease, 62% of patients had vaso-occlusive crises, 29% had acute chest syndrome, 23% had urinary tract infection, 15% had impaired cardiac function and 6% developed pulmonary hypertension. Only one patient died in the postnatal period due to acute chest syndrome. The mean gestational age was 37.8±2.63 weeks, and mean newborn weight was 2.809±643.8g. There were seven fetal losses, including three stillbirths and four miscarriages. The impact of transfusion therapy on the incidence of maternal-fetal complications during pregnancy was evaluated. CONCLUSIONS: Pregnancy in sickle cell patients is still associated with complications. Although no statistical difference was observed between transfused and non-transfused women, there were no deaths (fetal or maternal) in transfused patients whereas one maternal death and three stillbirths occurred in non-transfused women. A larger study of sickle cell pregnant women will be necessary to elucidate the actual role of transfusion during pregnancy in sickle cell disease.

Intrahepatic cholestasis in sickle cell disease: a case report.

Intrahepatic cholestasis (SCIC) is an uncommon but potentially fatal complication of sickle cell disease (SCD), with a high death rate, observed mainly in patients with homozygous sickle cell anemia. Herein, we describe a case of severe SCIC treated successfully with aggressive manual exchange transfusion (ET). The patient was admitted with enlarged liver and signs of hepatic failure, such as hyperbilirubinemia and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. We performed several sessions of ET in order to reduce the percentage of HbS to levels inferior to 30%, which was successfully accomplished. The patient had a complete recovery of hepatic function. This case has shown that ET is an effective treatment of SCIC and should be introduced early on the onset of this severe complication.

Effects of hydroxyurea on the membrane of erythrocytes and platelets in sickle cell anemia.

BACKGROUND AND OBJECTIVES: Adhesion molecules on the surface of erythrocytes, leukocytes and platelets are involved in vascular occlusion in sickle cell anemia. Hydroxyurea treatment of sickle cell anemia patients leads to clinical improvement and reduces the incidence of vaso-occlusive episodes. It has been previously demonstrated that hydroxyurea treatment also reduces the expression of adhesion molecules on the surface of erythrocytes. Phosphatidylserine (PS) exposure on the surface of erythrocytes has been considered to be the main determinant of altered erythrocyte adhesion in sickle cell anemia. In this study we examine the expression of PS on the surface of erythrocytes and platelets of sickle cell anemia patients before and during treatment with hydroxyurea. DESIGN AND METHODS: Blood samples from 15 sickle cell anemia patients were analyzed before and during treatment with hydroxyurea. The profile of PS expression was examined by flow cytometry. RESULTS: Hydroxyurea was effective, as determined by the patients clinical improvement and increased hemoglobin (8.3 vs 9.1 g/dL, p< 0.005), F cells (15.9% vs 37.1%, p< 0.005) and mean corpuscular volume ( 82 fL vs 101 fL, p< 0.005). PS expression on the surface of erythrocytes and platelets decreased from 6.27% to 2.96% (p< 0.005) and from 13.5% to 4.7% (p< 0.005), respectively. INTERPRETATION AND CONCLUSIONS: Hydroxyurea treatment reduces PS expression on the surface of erythrocytes and platelets, thus contributing to the favorable effects of this therapy.

Molecular investigation of GB virus C RNA in hemodialysis and thalassemics patients from Brazil.

The GB virus C (GBV-C)/hepatitis G virus (HGV) is a member of the Flaviviridae family. Based on the clinical and epidemiological profiles, this virus could be acquired mainly by parenteral transmission through contaminated blood. We therefore investigated the presence of GBV-C/HGV and its relation with the other blood borne viruses as hepatitis B and C viruses (HBV, HCV) in hemodialysis and thalassemic individuals and blood donors from Ribeirão Preto-Brazil. Detection of blood borne virus markers including HBV surface antigen (HbsAg), HBV core antibody (anti-Hbc) and HCV antibody was carried out. HIV-1, HIV-2, HTLV-1 and HTLV-2 were also investigated. GBV-C/HGV RNA was detected by reverse transcriptase and polymerase chain reaction (RT-PCR). Ninety-four serum samples from patients with chronic renal failure were analyzed. GBV-C/HGV RNA was identified in 12 (12.8%) patients, anti-HCV antibodies in 28 (29.8%), anti-Hbc in 9 (9.6%), anti-HIV in 1 (1%), HBsAg in 33 (35.1%), and HBsAg/ anti-HBc was observed in 2 (2.1%) patients. Thirty-six (38.3%) samples were non-reactive. Seven of the 12 GBV-C/HGV RNA infected samples were co-infected with other viruses: 3 (25%) with HBsAg, 2 (16.7%) with anti-HCV and 2 (16.7%) with anti-HBc/anti-HCV/HBsAg. Among the 42 thalassemic patients GBV-C/HGV RNA was detected in 6/42 patients (14.2 %). Three patients presented GBV-C/HGV, with other blood borne markers. We also detected GBV-C/ HGV in 6/50 (12%) blood donors. In these GBV-C/HGV positive thalassemics patients, 50% (3/6) were young individuals (lesser 15 years old) and 67% (4/6) were female patients. The presence of GBV-C RNA in the absence of hepatitis B and C infection in the young patients and healthy donors could be indicate that this virus is capable of independent transmission and does not contribute to liver disease.