Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Abstract

FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease.
 Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients.
 This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The diagnosis of alpha-thal was made by the multiplex ligation dependent probe amplification (MLPA) technique. Sickle cell pregnancies were followed according to the protocol of care in force in our service. The variables of interest were: hematological variables, sickle cell crises during pregnancy, maternal and fetal complications. Statistical analyzes were performed with SPSS 20.0 software. Means and standard deviations were compared with the Student's t and Annova tests. The value of p <0.05 was considered the significance level.
 The Hb-SS / alpha-thal and HbSS / HPFH genotypes were observed in 101 and 121 women respectively. Otherwise 758 women had the HbSS genotype. The morbidity related to sickle cell complications in the mother and fetus, were less frequent in the Hb-SS / alpha-thal and HbSS / HPFH groups. The statistical differences were statistically significant.
 This study showed a significant protective effect of alpa-thal and HPFH during pregnancy in sickle-cell pregnant women.
 FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease.
 Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients.
 This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The diagnosis of alpha-thal was made by the multiplex ligation dependent probe amplification (MLPA) technique. Sickle cell pregnancies were followed according to the protocol of care in force in our service. The variables of interest were: hematological variables, sickle cell crises during pregnancy, maternal and fetal complications. Statistical analyzes were performed with SPSS 20.0 software. Means and standard deviations were compared with the Student's t and Annova tests. The value of p <0.05 was considered the significance level.
 The Hb-SS / alpha-thal and HbSS / HPFH genotypes were observed in 101 and 121 women respectively. Otherwise 758 women had the HbSS genotype. The morbidity related to sickle cell complications in the mother and fetus, were less frequent in the Hb-SS / alpha-thal and HbSS / HPFH groups. The statistical differences were statistically significant.
 This study showed a significant protective effect of alpa-thal and HPFH during pregnancy in sickle-cell pregnant women.

Fetal hemoglobin in β hemoglobinopathies: Is enough too much?

“…Four women, all with severe forms of β thalassemia and HbF of 70%–100%, had growth‐restricted or small for gestational age fetuses; fetal growth was normal in 17 heterozygotes with different genotypes of β thalassemia who had HbF 11%–29% 19 . Women with sickle cell disease and HbF >15% had superior pregnancy outcomes and larger fetuses than patients with lower HbF 20 . This is consistent with the observation that HbF benefits many of the complications of sickle cell disease 21 …”

“…19 Women with sickle cell disease and HbF >15% had superior pregnancy outcomes and larger fetuses than patients with lower HbF. 20 This is consistent with the observation that HbF benefits many of the complications of sickle cell disease. 21 Is HbF with its reduced P 50 detrimental at altitude?…”

THE EFFECT OF ALPHA THALASSEMIA, HBF and HBC ON HAEMATOLOGICAL PARAMETERS OF SICKLE CELL DISEASE PATIENTS IN IBADAN, NIGERIA.

“…In the Democratic Republic of Congo, among pregnant women, morbidity related to sickle cell complications in the mother and foetus were less frequent in the HbSS/alphathal than in HbSS. 24 The protective role of α-thalassemia against the risk of abnormal TCD in Nigerian children with SCA has been described. 25 The coinheritance of alpha thalassemia and SCA has also been associated with lower consultation rates and increased survival.…”

Current Issues and Options for Hormonal Contraception in Adolescents and Young Adult Women With Sickle Cell Disease: An Update for Health Care Professionals

“…There has been very little research on reproductive attitudes, beliefs and health knowledge of patients with SCD. [83][84][85][86][87] The decision to have a child is influenced by the risks of the genetic transmission, the perceptions Table 3. General risk conditions that may have an impact on eligibility criteria for contraceptive use (From ref.…”

“…However, the need for repeated venous access and the associated risks and complications, such as alloimmunization and iron overload, limits its use. 85,86,91,92 Chelation therapy can be used to remove excess iron in patients with evidence of iron overload. 91 During pregnancy, chelation should be restricted for cases where the potential benefit outweighs the potential fetal risk.…”