Clinical complications in pregnant women with sickle cell disease: prospective study of factors predicting maternal death or near miss

Patrícia Santos Resende Cardoso, Regina Amélia Lopes Pessoa de Aguiar, Marcos Borato Viana, Patrícia Santos Resende Cardoso, Regina Amélia Lopes Pessoa de Aguiar, Marcos Borato Viana

Abstract

Objective: To evaluate complications in pregnant women with sickle cell disease, especially those leading to maternal death or near miss (severe obstetric complications).

Methods: A prospective cohort of 104 pregnant women registered in the Blood Center of Belo Horizonte (Hemominas Foundation) was followed up at high-risk prenatal units. They belonged to Group I (51 hemoglobin SS and three hemoglobin S/β(0)-thalassemia) or Group II (49 hemoglobin SC and one hemoglobin S/β(+)-thalassemia). Both groups had similar median ages. Predictive factors for 'near miss' or maternal death with p-value≤0.25 in the univariate analysis were included in a multivariate logistic model (significance set for p-value≤0.05).

Results: Group I had more frequent episodes of vaso-occlusive crises, more transfusions in the antepartum and postpartum, and higher percentage of preterm deliveries than Group II. Infections and painful crises during the postpartum period were similar in both the groups. The mortality rate was 4.8%: three deaths in Group I and two in Group II. One-third of the women in both the groups experienced near miss. The most frequent event was pneumonia/acute chest syndrome. Alpha-thalassemia co-inheritance and β-gene haplotypes were not associated with near miss or maternal death. In multivariate analysis predictors of near miss or death were parity above one and baseline red blood cell macrocytosis. In Group I, baseline hypoxemia (saturation<94%) was also predictive of near miss or death.

Conclusion: One-third of pregnant women had near miss and 4.8% died. Both hemoglobin SS and SC pregnant women shared the same risk of death or of severe complications, especially pulmonary events.

Keywords: Anemia, Sickle cell; Hemoglobin SC disease; Maternal death; Pregnancy; Pregnancy complications.

Copyright © 2014 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier Editora Ltda. All rights reserved.

Figures

Figure 1
Figure 1
Period of pulmonary complications/acute chest syndrome.
Figure 2
Figure 2
Near-miss and maternal death causes according to genotype.

References

    1. Serjeant G.R. The emerging understanding of sickle cell disease. Br J Haematol. 2001;112(1):3–18.
    1. Steinberg M.H. Pathopysiologically based drug treatment of sickle cell disease. Trends Pharmacol Sci. 2006;27:204–210.
    1. Rogers D.T., Molokie R. Sickle cell disease in pregnancy. Obstet Gynecol Clin North Am. 2010;37:223–237.
    1. Hassel K. Pregnancy and sickle cell disease. Hematol Oncol Clin North Am. 2005;19:903–916.
    1. Charache S., Scott J., Niebyl J., Bonds D. Management of sickle cell disease in pregnant patients. Obstet Gynecol. 1980;55:407–410.
    1. Dickinson F.T. Sickle-cell hemoglobin C disease in pregnancy: report of a case with review of the literature. J Am Osteopath Assoc. 1980;79:591–594.
    1. Rahimy M.C., Gangbo A., Adjou R., Deguenon C., Goussanou S., Alihonou E. Effect of active prenatal management on pregnancy outcome in sickle cell disease in an African setting. Blood. 2000;96:1685–1689.
    1. Sun P.M., Wilburn W., Raynor B.D., Jamieson D. Sickle cell disease in pregnancy: twenty years of experience at Grady Memorial Hospital, Atlanta, Georgia. Am J Obstet Gynecol. 2001;184:1127–1130.
    1. Serjeant G.R., Loy L.L., Crowther M., Hambleton I.R., Thame M. Outcome of pregnancy in homozygous sickle cell disease. Obstet Gynecol. 2004;103:1278–1285.
    1. Yu C.K., Stasiowska E., Stephens A., Awogbade M., Davies A. Outcome of pregnancy in sickle cell disease patients attending a combined obstetric and haematology clinic. J Obstet Gynaecol. 2009;29:512–516.
    1. Afolabi B.B., Iwuala N.C., Iwuala I.C., Ogedengbe O.K. Morbidity and mortality in sickle cell pregnancies in Lagos, Nigeria: a case control study. J Obstet Gynaecol. 2009;29:104–106.
    1. Al Jama F.E., Gasem T., Burshaid S., Rahman J., Al Suleiman S.A., Rahman M.S. Pregnancy outcome in patients with homozygous sickle cell disease in a university hospital. Eastern Saudi Arabia. Arch Gynecol Obstet. 2009;280:793–797.
    1. Nomura R.M., Igai A.M., Tosta K., Fonseca G.H., Gualandro S.F., Zugaib M. Resultados maternos e perinatais em gestações complicadas por doenças falciformes. Rev Bras Ginecol Obstet. 2010;32:405–411.
    1. Reichenheim M.E., Zylbersztajn F., Moraes C.L., Lobato G. Severe acute obstetric morbidity (near miss): a review of the relative use of its diagnostic indicators. Arch Gynecol Obstet. 2009;280:337–343.
    1. Souza J.P., Cecatti J.G., Fagundes A., Morais S.S., Villar J., Carroli G.A. Maternal near miss and maternal death in the World Health Organization's 2005 global survey on maternal and perinatal health. Bull World Health Org. 2010;88:113–119.
    1. Koshy M., Burd L., Wallace D., Moawad A., Baron J. Prophylactic red-cell transfusions in pregnant patients with sickle cell disease: a randomized cooperative study. N Engl J Med. 1988;319:1447–1452.
    1. Al-Farsi S.H., Al-Riyami N.M., Al-Khabori M.K., Al-Hunaini M.N. Maternal complications and the association with baseline variables in pregnant women with sickle cell disease. Hemoglobin. 2013;37:219–226.
    1. Pritchard J.A., Scott D.E., Whalley P.H., Cunningham F.G., Mason R.A. The effects of maternal sickle cell hemoglobinopathies and sickle cell trait on reproductive performance. Am J Obstet Gynecol. 1973;117:662–670.
    1. Villers M.S., Jamison M.G., De Castro L.M., James A.H. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol. 2008;199:125e1–1255e.
    1. Nomura R.M., Igai A.M., Tosta K., Fonseca G.H., Gualandro S.F., Zugaib M. Acute chest syndrome in pregnant women with hemoglobin SC disease. Clinics. 2009;64:927–928.
    1. Elsayegh D., Shapiro J.M. Sickle cell vasooclusive crisis and acute chest syndrome at term pregnancy. South Med J. 2007;100:77–79.
    1. Martin J.N., Jr., Martin R.W., Morrison J.C. Acute management of sickle cell crisis in pregnancy. Clin Perinatol. 1986;13:853–868.
    1. Belisário A.R., Martins M.L., Brito A.M., Rodrigues C.V., Silva C.M., Viana M.B. β-globin gene cluster haplotypes in a cohort of 221 children with sickle cell anemia or Sβ0-thalassemia and their association with clinical and hematological features. Acta Haematol. 2010;124:162–170.
    1. Castro O., Brambilla D.J., Thorington B., Reindorf C.A., Scott R.B., Gillette P. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994;84:643–649.
    1. Smith J.A., Espeland M., Bellevue R., Bonds D., Brown A.K., Koshy M. Pregnancy in Sickle Cell Disease: experience of the Cooperative Study of Sickle Cell Disease. Obstet Gynecol. 1996;87:199–204.
    1. Quinn C.T., Ahmad N. Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease. Br J Haematol. 2005;131:129–134.
    1. Parrish M.R., Morrison J.C. Sickle cell crisis and pregnancy. Semin Perinatol. 2013;37:274–279.
    1. Royal College of Obstetricians and Gynaecologists Green-top, Guideline Management of sickle cell disease in pregnancy. RCOG. 2011;61:1–20.
    1. Bellina J.H., Bickers J.N. Modern management of sickle cell disease in pregnancy. South Med J. 1974;67:426–430.
    1. Danielson C.F. The role of red blood cell exchange transfusion in the treatment and prevention of complications of sickle cell disease. Ther Apher. 2002;6:24–31.
    1. Zanette A.M. Gravidez e contracepção na doença falciforme. Rev Bras Hematol Hemoter. 2007;29:309–312.

Source: PubMed

Sponsor e collaboratori

Condizioni mediche

Interventi farmacologici

3
Sottoscrivi