Schifrin, Amsel, and Burdorf
11. 12. 13. 14. 15.
tion with other heart rate patterns during monitored labor. AM J OBSTET GYNECOL 1979;135:360-3. Kubli FW, Hon EH, Khazin AE, et ai. Observations on heart rate and pH in the human fetus during labor. AM J OBSTET GYNECOL 1969; 104: 1190-206. Zalar RW Jr, Quilligan EJ. The influence of scalp sampling on the cesarean section rate for fetal distress. AM J OBSTET GYNECOL 1979;135:239-46. Shields J, Schifrin BS. Perinatal antecedents of cerebral palsy. Obstet Gynecol 1988;71:899-905. Walker N. The case for conservatism in management of foetal distress. BMJ 1959;2:1221-6. Benson RC, Shubeck F, Deutschberger J, et ai. Fetal heart
February 1992 Am J Obstet Gyneco1
rate as a predictor of fetal distress. A report from the collaborative project. Obstet Gynecol 1968;32:259-66. 16. Techniques to evaluate fetal health. In: Pritchard JA, MacDonald PC, Gant NF, eds. Williams' obstetrics. ed 17. New York: Appleton-Century-Crofts, 1985:286-7. 17. Neutra RR, Feinberg SE, Greenland S, Friedman EA. Effect of fetal monitoring on neonatal death rates. N Engl J Med 1978;299:324. 18. Niswander KR, Gordon M. The women and their pregnancies. Washington, D.C.: United States Department of Health, Education, and Welfare, 1972; DHEW publication no. (NIH) 73-379.
Successive pregnancies complicated by idiopathic sideroblastic anemia John R. Barton, MD, David C. Shaver, MD, and Baha M. Sibai, MD Memphis, Tennessee Sideroblastic anemias are a diverse group of hypoproliferative anemias characterized by defective iron use within erythropoietic cells and a defect in heme biosynthesis. This report describes the first case of successive pregnancies in a patient with idiopathic sideroblastic anemia. Periodic transfusions with washed erythrocytes and oral pyridoxine resulted in normal maternal and perinatal outcome. (AM J CSSTET GYNECOL
1992;166:576-7.)
Key words: Sideroblastic anemia, pregnancy, pyridoxine Sideroblastic anemia is a descriptive term for a diverse group of disorders that have in common a defect in heme biosynthesis and defective iron use within erythropoietic cells. A sideroblast is a marrow erythroid precursor (specifically, a normoblast) that has Prussian blue-positive granules within the cytoplasm. The granules represent deposits of iron phosphate within mitochondria, which have not been used for heme synthesis. The abnormal accumulation may form a perinuclear ring of iron. This ringed sideroblast is the hallmark of sideroblastic anemia.' Sideroblastic anemia may be hereditary or acquired. Both sex-linked and autosomal recessive patterns of inheritance have been described. The acquired variety of sideroblastic anemia has been associated with certain diseases (especially malignant and inflammatory dis-
From the Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, The University of Tennessee, Memphis. Received for publication july 16, 1991; accepted August 15, 1991. Reprint requests: Baha M. Sibai, MD, 853 jefferson, Suite E102, Memphis, TN 38103.
6/1/33386
orders) and some drugs or toxins (especially ethyl alcohol, lead, isonicotinic acid).2 There is also an idiopathic variety of sideroblastic anemia. Sideroblastic anemia is an exceedingly unusual complication of pregnancy. Because of our inability to identify a report of successive pregnancies associated with idiopathic sideroblastic anemia, the management of the following patient is presented. Case report A 22-year-old black woman, gravida 2, para 1, was seen at 10 weeks' gestation. Two years earlier, prenatal evaluation had revealed severe anemia. At that time, the hemoglobin was 5.8 gm/ dl and hematocrit 19.1 %. There were 2,010,000 erythrocytes and 310,000 platelets per cubic millimeter. Reticulocytes were 0.7%. Leukocytes and platelets were normal. Smear of peripheral blood revealed a mixture of normocytic-normochromic cells and hypochromic-microcytic erythrocytes. Anisocytosis, poikilocytosis, target cells, schistocytes, microcytes, and rare basophilic stippling were demonstrated. There were no Pappenheimer bodies. On bone marrow biopsy, the marrow-to-fat ratio was 70: 30. Red blood cells showed erythroid hyperplasia
Sideroblastic anemia in pregnancy
Volume 166 Number 2
with increased numbers of prorubricytes and rubricytes. Progressive hemoglobinization was seen. Special stains for iron showed a marked increase in sideroblasts. The majority of sideroblasts demonstrated perinuclear ring distribution of coarse granules. There was no other abnormality. The diagnosis was sideroblastic anemia. There was no hereditary predisposition, drug-toxin exposure, and no associated disease. The anemia did not respond to pyridoxine therapy (50 mg daily). Intermittently, the patient required transfusions of washed erythrocytes to maintain the hemoglobin in the 9 to 10 gm/dl range. She experienced spontaneous onset of labor at term but required cesarean section for cephalopelvic disproportion. Transfusion was necessary at the time of surgery. The postoperative course was uneventful. Three weeks after surgery, the hemoglobin was 7.9 gm/dl and hematocrit 19%. She was lost to follow-up until 3 months post partum. At that time, the hematocrit was 11.2%, and again she required transfusions. The patient used no form of contraception and failed to keep appointments until she suspected pregnancy. When seen at 10 weeks' gestation in the subsequent pregnancy, hemoglobin was 4.7 gm/dl and hematocrit 13.4%. She was transfused with two units of washed erythrocytes and begun on 200 mg pyridoxine daily and 2 mg folate daily. The pregnancy proceeded in an uneventful fashion except for the requirement of intermittent transfusions. Antepartum evaluation of fetal well-being with serial ultrasonography and nonstress testing were normal. There was never any evidence of antibody production as a result of the repeated blood transfusions. After the spontaneous onset of labor at 37 weeks' gestation, a repeat cesarean section and tubal sterilization were performed. The female infant weighed 2296 gm with Apgar scores of 9 and 9. There
577
were no complications. Postoperative hematocrit was 29.5%. Comment
Anemia is frequently encountered in pregnancy. On occasion, a chronic anemia will require bone marrow analysis. The finding of ringed side rob lasts is diagnostic of sideroblastic anemia. This report demonstrates one patient with idiopathic sideroblastic anemia who was managed successfully throughout two pregnancies by periodic transfusions of washed erythrocytes and oral pyridoxine therapy. Management is directed toward maintenance of the hematocrit by periodic transfusions of washed erythrocytes. Obvious potential complications include isoimmunization and viral infections. Although only a minority of patients with idiopathic sideroblastic anemia respond to pyridoxine, patients should be treated with 100 to 200 mg of pyridoxine per day (about 100 times the physiologic daily requirement). Further, oral folate supplementation (1 to 2 mg per day) is recommended because concomitant folate deficiency is frequent with idiopathic sideroblastic anemia. Because sideroblastic anemia is allied with myelodysplastic syndromes, patients should also be monitored for the development of acute leukemia. REFERENCES 1. Desnick ID, Anderson KE. Heme biosynthesis and its disorders: the porphyrias and sideroblastic anemias. In: Hoffman R, Bent EJ Jr, Shattil SJ, et ai, eds. Hematology: basic principles and practice. New York: Churchill Livingstone, 1991 :350-67. 2. Hines JD, Grasso JA. The sideroblastic anemias. Semin Hematol 1970;7(1):86-206.
11. 12. 13. 14. 15.
tion with other heart rate patterns during monitored labor. AM J OBSTET GYNECOL 1979;135:360-3. Kubli FW, Hon EH, Khazin AE, et ai. Observations on heart rate and pH in the human fetus during labor. AM J OBSTET GYNECOL 1969; 104: 1190-206. Zalar RW Jr, Quilligan EJ. The influence of scalp sampling on the cesarean section rate for fetal distress. AM J OBSTET GYNECOL 1979;135:239-46. Shields J, Schifrin BS. Perinatal antecedents of cerebral palsy. Obstet Gynecol 1988;71:899-905. Walker N. The case for conservatism in management of foetal distress. BMJ 1959;2:1221-6. Benson RC, Shubeck F, Deutschberger J, et ai. Fetal heart
February 1992 Am J Obstet Gyneco1
rate as a predictor of fetal distress. A report from the collaborative project. Obstet Gynecol 1968;32:259-66. 16. Techniques to evaluate fetal health. In: Pritchard JA, MacDonald PC, Gant NF, eds. Williams' obstetrics. ed 17. New York: Appleton-Century-Crofts, 1985:286-7. 17. Neutra RR, Feinberg SE, Greenland S, Friedman EA. Effect of fetal monitoring on neonatal death rates. N Engl J Med 1978;299:324. 18. Niswander KR, Gordon M. The women and their pregnancies. Washington, D.C.: United States Department of Health, Education, and Welfare, 1972; DHEW publication no. (NIH) 73-379.
Successive pregnancies complicated by idiopathic sideroblastic anemia John R. Barton, MD, David C. Shaver, MD, and Baha M. Sibai, MD Memphis, Tennessee Sideroblastic anemias are a diverse group of hypoproliferative anemias characterized by defective iron use within erythropoietic cells and a defect in heme biosynthesis. This report describes the first case of successive pregnancies in a patient with idiopathic sideroblastic anemia. Periodic transfusions with washed erythrocytes and oral pyridoxine resulted in normal maternal and perinatal outcome. (AM J CSSTET GYNECOL
1992;166:576-7.)
Key words: Sideroblastic anemia, pregnancy, pyridoxine Sideroblastic anemia is a descriptive term for a diverse group of disorders that have in common a defect in heme biosynthesis and defective iron use within erythropoietic cells. A sideroblast is a marrow erythroid precursor (specifically, a normoblast) that has Prussian blue-positive granules within the cytoplasm. The granules represent deposits of iron phosphate within mitochondria, which have not been used for heme synthesis. The abnormal accumulation may form a perinuclear ring of iron. This ringed sideroblast is the hallmark of sideroblastic anemia.' Sideroblastic anemia may be hereditary or acquired. Both sex-linked and autosomal recessive patterns of inheritance have been described. The acquired variety of sideroblastic anemia has been associated with certain diseases (especially malignant and inflammatory dis-
From the Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, The University of Tennessee, Memphis. Received for publication july 16, 1991; accepted August 15, 1991. Reprint requests: Baha M. Sibai, MD, 853 jefferson, Suite E102, Memphis, TN 38103.
6/1/33386
orders) and some drugs or toxins (especially ethyl alcohol, lead, isonicotinic acid).2 There is also an idiopathic variety of sideroblastic anemia. Sideroblastic anemia is an exceedingly unusual complication of pregnancy. Because of our inability to identify a report of successive pregnancies associated with idiopathic sideroblastic anemia, the management of the following patient is presented. Case report A 22-year-old black woman, gravida 2, para 1, was seen at 10 weeks' gestation. Two years earlier, prenatal evaluation had revealed severe anemia. At that time, the hemoglobin was 5.8 gm/ dl and hematocrit 19.1 %. There were 2,010,000 erythrocytes and 310,000 platelets per cubic millimeter. Reticulocytes were 0.7%. Leukocytes and platelets were normal. Smear of peripheral blood revealed a mixture of normocytic-normochromic cells and hypochromic-microcytic erythrocytes. Anisocytosis, poikilocytosis, target cells, schistocytes, microcytes, and rare basophilic stippling were demonstrated. There were no Pappenheimer bodies. On bone marrow biopsy, the marrow-to-fat ratio was 70: 30. Red blood cells showed erythroid hyperplasia
Sideroblastic anemia in pregnancy
Volume 166 Number 2
with increased numbers of prorubricytes and rubricytes. Progressive hemoglobinization was seen. Special stains for iron showed a marked increase in sideroblasts. The majority of sideroblasts demonstrated perinuclear ring distribution of coarse granules. There was no other abnormality. The diagnosis was sideroblastic anemia. There was no hereditary predisposition, drug-toxin exposure, and no associated disease. The anemia did not respond to pyridoxine therapy (50 mg daily). Intermittently, the patient required transfusions of washed erythrocytes to maintain the hemoglobin in the 9 to 10 gm/dl range. She experienced spontaneous onset of labor at term but required cesarean section for cephalopelvic disproportion. Transfusion was necessary at the time of surgery. The postoperative course was uneventful. Three weeks after surgery, the hemoglobin was 7.9 gm/dl and hematocrit 19%. She was lost to follow-up until 3 months post partum. At that time, the hematocrit was 11.2%, and again she required transfusions. The patient used no form of contraception and failed to keep appointments until she suspected pregnancy. When seen at 10 weeks' gestation in the subsequent pregnancy, hemoglobin was 4.7 gm/dl and hematocrit 13.4%. She was transfused with two units of washed erythrocytes and begun on 200 mg pyridoxine daily and 2 mg folate daily. The pregnancy proceeded in an uneventful fashion except for the requirement of intermittent transfusions. Antepartum evaluation of fetal well-being with serial ultrasonography and nonstress testing were normal. There was never any evidence of antibody production as a result of the repeated blood transfusions. After the spontaneous onset of labor at 37 weeks' gestation, a repeat cesarean section and tubal sterilization were performed. The female infant weighed 2296 gm with Apgar scores of 9 and 9. There
577
were no complications. Postoperative hematocrit was 29.5%. Comment
Anemia is frequently encountered in pregnancy. On occasion, a chronic anemia will require bone marrow analysis. The finding of ringed side rob lasts is diagnostic of sideroblastic anemia. This report demonstrates one patient with idiopathic sideroblastic anemia who was managed successfully throughout two pregnancies by periodic transfusions of washed erythrocytes and oral pyridoxine therapy. Management is directed toward maintenance of the hematocrit by periodic transfusions of washed erythrocytes. Obvious potential complications include isoimmunization and viral infections. Although only a minority of patients with idiopathic sideroblastic anemia respond to pyridoxine, patients should be treated with 100 to 200 mg of pyridoxine per day (about 100 times the physiologic daily requirement). Further, oral folate supplementation (1 to 2 mg per day) is recommended because concomitant folate deficiency is frequent with idiopathic sideroblastic anemia. Because sideroblastic anemia is allied with myelodysplastic syndromes, patients should also be monitored for the development of acute leukemia. REFERENCES 1. Desnick ID, Anderson KE. Heme biosynthesis and its disorders: the porphyrias and sideroblastic anemias. In: Hoffman R, Bent EJ Jr, Shattil SJ, et ai, eds. Hematology: basic principles and practice. New York: Churchill Livingstone, 1991 :350-67. 2. Hines JD, Grasso JA. The sideroblastic anemias. Semin Hematol 1970;7(1):86-206.
