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126 Part III: Epochal Hematology Chapter 8: Hematology during Pregnancy 127

labor, as well as rates of maternal mortality, were higher in mothers 8. Acker DB, Johnson MP, Sachs BP, et al: The leukocyte count in labor. Am J Obstet Gyne-
with sickle cell anemia. The issue of prophylactic versus need-based col 153:737, 1985.
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transfusion in sickle cell patients is controversial. A Cochrane Review 9. Watts DH, Krohn MA, Wener MH, et al: C-reactive protein in normal pregnancy.
Obstet Gynecol 77:176, 1991.
identified only two small randomized studies conducted in the United 10. van den Broe NR, Letsky EA: Pregnancy and the erythrocyte sedimentation rate. BJOG
States in the 1980s addressing this issue. These studies demonstrated no 108:1164, 2001.
difference in perinatal outcome between the offspring of mothers with 11. Greer IA: Thrombosis in pregnancy: Maternal and fetal issues. Lancet 353:1258, 1999.
12. Clark P, Brennand J, Conkie JA, et al: Activated protein C sensitivity, protein C, protein
sickle cell disease who were assigned to treatment with prophylactic S and coagulation in normal pregnancy. Thromb Haemost 79:1166, 1998.
transfusions and those who were not. 1,150 13. Halligan A BJ, Sheppard B, et al: Haemostatic, fibrinolytic and endothelial variables in
Although the incidence of cesarean section in sickle cell patients is normal pregnancies and pre-eclampsia. Br J Obstet Gynaecol 101:448, 1992.
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reported to be as high as 36 percent, delivery can generally be accom- 14. Brabin BJ, Hakimi M, Pelletier D: An analysis of anemia and pregnancy-related mater-
nal mortality. J Nutr 131:604S, 2001.
plished vaginally. Most experts recommend avoiding induction of labor 15. Centers for Disease Control (CDC): CDC criteria for anemia in children and childbearing-
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as this can lead to sickle crisis. Epidural anesthesia is reported to be aged women. MMWR Morb Mortal Wkly Rep 38:400, 1989.
safe and to decrease the risk of peripartum painful crises. 153 16. Sifakis S, Pharmakides G: Anemia in pregnancy. Ann N Y Acad Sci 900:125, 2000.
17. FAO/WHO: Joint Expert Consultation Report: Requirements of Vitamin A, Iron, Folate,
and Vitamin B12. FAO Food and Nutrition Series 23. FAO, Rome, 1988.
18. Harthoorn-Lasthuizen EJ, Lindemans J, Langenhuijsen MM: Does iron-deficient ery-
THALASSEMIA SYNDROMES thropoiesis in pregnancy influence fetal iron supply? Acta Obstet Gynecol Scand 80:392,
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β-Thalassemia Syndromes 19. Scholl TO, Hediger ML, Fischer RL, et al: Anemia vs iron deficiency: Increased risk of
Preconception evaluation of patients with β-thalassemia syndromes preterm delivery in a prospective study. Am J Clin Nutr 55:985, 1992.
is recommended and should include assessment of transfusion needs, 20. Hemminki E, Rimpela U: A randomized comparison of routine versus selective iron
supplementation during pregnancy. J Am Coll Nutr 10:3, 1991.
chelation therapy, body iron status and organ function, and the pres- 21. Horner RD, Lackey CJ, Kolasa K, et al: Pica practices of pregnant women. J Am Diet
ence of antibodies to red cell antigens. Patients with β-thalassemia Assoc 91:34, 1991.
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minor generally tolerate pregnancy well; however, doses of at least 4 mg 22. Shojania AM: Folic acid and vitamin B12 deficiency in pregnancy and in the neonatal
period. Clin Perinatol 11:433, 1984.
of folate per day are recommended in the preconception period and 23. Van de Velde A, Van Droogenbroeck J, Tjalma W, et al: Folate and Vitamin B(12) defi-
the first trimester as there is some data to suggest an increased risk of ciency presenting as pancytopenia in pregnancy: A case report and review of the litera-
neural tube defects in their offspring. Transfusion and iron chelation ture. Eur J Obstet Gynecol Reprod Biol 100:251, 2002.
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therapy has improved both life expectancy and fertility in patients with 24. Walker SP, Wein P, Ihle BU: Severe folate deficiency masquerading as the syndrome of
hemolysis, elevated liver enzymes, and low platelets. Obstet Gynecol 90:655, 1997.
β-thalassemia intermedia and major, and successful pregnancies have 25. Mahomed K: Folate supplementation in pregnancy. Cochrane Database Syst Rev
been reported in both disorders. A high rate of maternal mortal- (2):CD000183, 2000.
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ity is reported in women with thalassemia and cardiac iron overload 26. Bruinse HW, van den Berg H: Changes of some vitamin levels during and after normal
pregnancy. Eur J Obstet Gynecol Reprod Biol 61:31, 1995.
emphasizing the need for aggressive management of iron status prior to 27. Frenkel EP, Yardley DA: Clinical and laboratory features and sequelae of deficiency of
undertaking a pregnancy. During pregnancy, regular transfusions are folic acid (folate) and vitamin B12 (cobalamin) in pregnancy and gynecology. Hematol
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recommended to keep the hemoglobin level at 10 mg/dL and transfu- Oncol Clin North Am 14:1079, 2000.
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Iron-chelation therapy with deferoxamine in pregnancy is controversial 29. Baker RI, Manoharan A, de Luca E, et al: Pure red cell aplasia of pregnancy: A distinct
and most authorities recommend a hiatus during pregnancy; however, clinical entity. Br J Haematol 85:619, 1993.
no fetal abnormalities have been reported in pregnancies in which it 30. Makino Y, Nagano M, Tamura K, et al: Pregnancy complicated with pure red cell apla-
sia: A case report. J Perinat Med 31:530, 2003.
was continued (Chap. 48). 159 31. Mant MJ: Chronic idiopathic pure red cell aplasia: Successful treatment during preg-
nancy and durable response to intravenous immunoglobulin. J Intern Med 236:593,
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α-Thalassemia Syndromes 32. Thachil J, Toh C-H: Disseminated intravascular coagulation in obstetric disorders and
Patients with the silent carrier state or α-thalassemia trait have no its acute haematological management. Blood Rev 23: 167, 2009.
increase in pregnancy complications; however, identification of patients 33. Erez O, Novack L, Beer-Weisel R, et al: DIC score in pregnant women—a population
with heterozygous α-thalassemia trait is important in assessing the risk based modification of the International Society on Thrombosis and Hemostasis score.
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of having a fetus that has hemoglobin H or hemoglobin Bart. Although 34. Tuffnell DJ: Amniotic fluid embolism. Curr Opin Obstet Gynecol 15:119, 2003.
women with hemoglobin H are generally able to have successful preg- 35. Awad IT, Shorten GD: Amniotic fluid embolism and isolated coagulopathy: Atypical
presentation of amniotic fluid embolism. Eur J Anaesthesiol 18:410, 2001.
nancies, the chronic anemia often worsens, requiring blood transfusion. 36. Clark SL: Amniotic Fluid Embolism. Obstet Gynecol 123:337, 2014.
Patients with hemoglobin H are sensitive to oxidizing compounds and 37. Goldszmidt E, Davies S: Two cases of hemorrhage secondary to amniotic fluid embolus
medications, which should be borne in mind, particularly during preg- managed with uterine artery embolization. Can J Anaesth 50:917, 2003.
nancy (Chap. 48). 38. Letsky EA: Disseminated intravascular coagulation. Best Pract Res Clin Obstet Gynaecol
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39. Rodeghiero F: Von Willebrand disease: Pathogenesis and management. Thromb Res 131
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