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Anaesthetic management of caesarean section in a parturient with acute myelodysplastic syndrome
Internafiod Journal 01 Obstetric Anesthesia (1997) 6, 27&273 0 1997 Pearson Professional Ltd
CASE REPORT
Anaesthetic managementof caesareansection in a parturient with acute myelodysplastic syndrome F. Christiaens, D. Burrini,” C. Verborgh, N. Fontaine, L. de Catte,* F. Camu Department of Anaesthesiologyand *Department of Foeto-maternalM e d icine, University Hospital A.Z.- KU B., F lemish Free University of Brussels,Brussels,Belgium SUMMARY: A 34-year-old pregnant woman developed a myelodysplastic syndrome during pregnancy which resulted in a refractory anaemia and an extreme thrombocytopenia. The report describes the anaesthetic management of elective caesarean section and successful childbirth in this patient. Following replacement therapy with packed red cells and platelets, general anaesthesiawas used for the procedure.
INTRODUCTION Obstetric anaesthetistsare on occasion confronted by the need to anaesthetizeparturients with haematological problems for delivery or surgery. W e report the managementof elective caesareansection in a parturient with refractory anaemia and extreme thrombocytopenia due to a myelodysplastic syndrome (MDS). CASE REPORT A 34-year-old m u ltigravid (G,P,A,) woman (weight 65 kg, height 162 cm) was scheduled for elective caesarean section becauseof transverselie of the fetus, at a gestational age of 35 weeks and 4 days. M e d ical and surgical history included an appendectomy,asthma in childhood and a true allergy to penicillin. At 21 weeks of pregnancy, she had developed pharyngitis which was treated orally by erythromycin and acetaminophen. She had been referred to the department of fetomaternal m e d icine at a gestational age of 24 weeks and 5 days for severe anaemia (haemoglobin [Hb] 7.9 g/dL and haematocrit [Hct] 23.9%; normal values
F. Christhens MD, C. Verborgh MD, N. Fontaine MD, F. Camu MD, Department of Anaesthesiology, University Hospital A.Z.V.U.B., Flemish Free University of Brussels, Laarbeeklaan 101, B- 1090 Brussels, Belgium and D. Burrini MD, L. de Catte MD, Department of Foeto-maternal Medicine, University Hospital A.Z.-V.U.B., Flemish Free University of Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium. Correspondence to: Dr. Frank Christiaens, Tel: 00 32 2 477 53 60; Fax: 00 32 2 477 5 148. 270
pregnant female at term: 11.6 g/dL and 35.5% respectively) and thrombocytopenia (5 1xl 09/L; normal range pregnant female: 150-400x109/L).She presently complained of fatigue at rest and dyspnoea at night and during exercise. Physical examination revealed anaemic conjunctivae, pallor and a systolic murmur l/6; she was apyretic and had no purpura nor hepatosplenomegaly.The results of an extensive laboratory screeningat this tim e are listed in Table 1. Since no aetiology for this increasing thrombocytopenia and anaemia was found at this moment, a conservative therapeutic attitude was adopted: no transfusion of packed red cells (PRC) nor platelets would be given unless the haemoglobin level decreasedbelow 6 g/dL, the platelet count decreased below 20x109/L or the patient became symptomatic. As the patient became asthenic 4 days later and haemoglobin level turned out to be only 6.9 g/dL, 2 units of PRC were transfused. At a gestational age of 26 l/7 weeks, a diagnostic bone marrow puncture was performed and demonstrated MDS. Karyotype analysis of the bone marrow aspirate showed m u ltiple complex abnormalities, indicating a high risk to leukaemia transformation. Spontaneous epistaxis occurring some tim e later was treated by electrocauterization. Progressive anaemia developed and required m u ltiple leukocyte-free PRC transfusions. A moderate transfusion reaction occurred during a PRC transfusion and several HLAantibodies were demonstrated. At a gestational age of 33 weeks, there were no obstetrical contraindications left for the delivery of the fetus. Fetal pulmonary maturation was induced weekly with corticosteroids. The fetus had developed well. An ultrasound at 34 weeks showed head and
Acute myelodysplastic syndrome
27 1
Table 1. Laboratory results at the time of referral to the department of feto-maternal medicine
Blood group Haemoglobin Haematocrit Red blood cell count Mean cell volume Platelet count White blood cell count Activated partial thromboplastin time Prothrombin time Blood glucose Blood urea Creatinine Creatine kinase Total lactate dehydrogenase Aspartate aminotransferase Alanine aminotransferase Total bilirubin Total protein Albumin Amylase Lipase C-Reactive protein Serum folic acid Intra-erythrocyte folate Ferritin Transferrin Iron binding capacity True vitamin B,, Haptoglobin Fibrinogen Thyroid stimulating hormone Free T, Anticardiolipin antibodies Lupus anticoagulant
Result
Normal non-pregnant range
A Rhesus + (CCDce) 7.0 g/dL 21.4% 2.2xlO’VL 98 fL 33X109/L 9.6xlOVL 25.1 s 85% 105 mg/dL 14 mg/dL 0.52 mg/dL < 20 W/L 972 IUlL 25 IUlL 25 IU/L 0.3 mg/dL 5.7 gldL 3.3 gldL 51 IUlL 25 IU/L < 7 mg/dL 7.1 ug/L 1664 pg/L 154 ug/L 302 mg/dL 352 ugldL 0.76 ug/L < 10 mg/dL 233 mg/dL 1.01 mu/L 8.8 rig/L negative
11.5 -16.5 g/dL 3747% 3.8-5.8~1O’~/L 7696 fL 150-400x109/L 410x10~1L 23-28 s 70-100% 7GlOO mg/dL 15540 mg/dL 0.4-1.2 mg/dL S-130 IU/L 181-502 IUlL 941 IU/L 4-39 IUlL 0.2-1.0 mg/dL 6.3-8.2 gldL 3.9-5.0 g/dL cl13 IUlL 50-200 IU/L < 0.2 mg/dL 2.0-14.0 ug/L 200-800 ug/L lo-260 ug/L 208-382 mg/dL 225420 ug/dL 0.22-0.94 ug/L 27-l 39 mg/dL 200400 mg/dL 0.15-3.0 mu/L 9.0-21.8 rig/L
The viral screening (including: parvo B-19, pica RNA, rubella, cytomegalovirus, Epstein Barr virus, Herpes simplex virus and human immunodeficiency virus) and parasite screening (toxoplasmosis) were not contributive.
abdominal circumference and femur length were in concordance with 33 weeks. Weight was estimated at 2300 g which accorded with the fiftieth centile. Umbilical blood flow was normal. Since the fetus was in transverse lie, the patient was scheduled for caesarean section at 35 3/7 weeks. Fetal heart rate was monitored daily and showed good variability, many accelerations and no decelerations. The evening before surgery, a 16-gauge intravenous catheter was placed and 24 g of immunoglobulins were given. The platelet count was then 9x109/L and the patient was assessed ASA physical class III. The platelet count on the morning of surgery was 12x109/L and increased to 34x109/L after administration of the first unit of platelets. After intravenous administration of 20 mg of methylprednisolone, a second transfusion of platelets was performed resulting in a platelet count of 61x109/L. When these transfusions were completed, the patient was transferred to the operating room. A second 16-gauge catheter was placed intravenously and a 19-gauge catheter was inserted under local
anaesthesia in the left radial artery. Monitoring included maternal heart rate, electrocardiogram, noninvasive and invasive blood pressure, end tidal carbon dioxide, urinary output, pulse oximetry and arterial blood gas analysis. Isoflurane concentration was measured with a Datex Capnomac. Following preoxygenation, general anaesthesia was induced intravenously with vecuronium 0.5 mg, ketamine 100 mg and succinylcholine 75 mg given in a rapid sequence to facilitate endotracheal intubation. Before the baby was born, anaesthesia was maintained with an additional bolus of ketamine (25 mg) and succinylcholine (25 mg), and 100% oxygen was administered. Isoflurane concentration was kept at 0.6%. The incision to delivery time (I-D interval) and the uterine incision to delivery time (U-D interval) were 7 min and 58 s respectively. After delivery, an intravenous bolus of 4 U of oxytocin was given and 6 U were added to 1000 mL Hartmann’s solution. Anaesthesia was further maintained with isoflurane concentrations of 0.4-0.6% in a 40% oxygen/air mixture, fentanyl
272
International
Table 2.
Journal of Obstetric Anesthesia
Results of peroperative arterial blood gas (ABG) analysis
PH PaCO, (mmHg) PaO, (mmHg) HCO,- (mmol/L) Base deficit (mmol/L) Haemoglobin (g/dL) Haematocrit (%)
ABG 1
ABG 2
ABC 3
1.46 29.5 266.5 20.9 -1.1 11.8 34.7
7.41 33.2 198.8 21.2 -2.2 7.1 22.6
7.27 46.2 505.8 21.0 -5.5 10.2 30.0
200 pg and vecuronium 2.5 mg. Results of the first arterial blood gas analysis (performed 15 min after induction), second arterial blood gas analysis (performed before transfusion of one unit of leukocytefree PRC because of estimated operative blood loss of 960 mL) and third arterial blood gas analysis (3 min post extubation) are shown in Table 2. After the second arterial blood gas analysis, 20 mg of methylprednisolone was given intravenously and a third unit of platelets was transfused. Forty-one minutes after induction, a second transfusion of leukocyte-free PRC was given. Peroperative diuresis amounted to 100 mL. The induction to extubation time and the skin to skin operation time were 70 and 55 min respectively. The patient did not move at any time and did not report any awareness.A male infant was born, weighing 2300 g. The Apgar scores were 3, 7 and 9 at 1, 5 and IO min respectively. The placental weight was 650 g and the venous cord blood showed a pH of 7.40, PaCO, 36.2 mmHg, P,O, 33.1 mmHg, HCO,- 22.0 mmol/L and a base deficit of -1.7 mmol/L. On arrival in the Post Anaesthesia Care Unit, laboratory tests showed a Hb of 10.9 g/dL, a Hct of 32.1% and a platelet count of 63x10g/L. Twenty milligrams of methylprednisolone were repeated intravenously and a fourth unit of platelets was transfused and resulted in a platelet count of 66x10g/L. Another 10 U of oxytocin were given over a 12 h period. Postoperative analgesia consisted of 2 mg boluses of piritramide (10 mg in total) and 2 g of proparacetamol intravenously. On the morning of the first postoperative day, laboratory tests showed a Hb of 9.5 g/dL, a Hct of 28.3% and a platelet count of 58x10g/L and the patient was transferred to the maternity ward. The platelet count on the third and fourth postoperative day decreased to 34x10g/L and 24x10g/L respectively. A fifth transfusion of platelets was administered and resulted in a platelet count of 77x10g/L. The patient was discharged from the hospital on the seventh postoperative day with a Hb of 8.2 g/dL, a Hct of 24.3% and a platelet count of 57x10g/L. A bone marrow puncture performed 20 days postpartum demonstrated an evolution to acute myeloid leukaemia (AML) type M6. Despite intensive
treatment, including several courses of chemotherapy, the patient died 8 months postpartum. DISCUSSION
Myelodysplastic syndromes are a group of haematological disorders that are characterized by dysplastic haematopoiesis.’The proportion of blast cells in the peripheral blood and/or bone marrow is one of the main distinguishing features of these conditions.2 The MDS are subdivided in five different types according to the French-American-British classification: 1. 2. 3. 4. 5.
refractory anaemia (RA) RA with ring sideroblasts RA with excessof blasts (RAEB) chronic myelomonocytic leukaemia (CMML) RAEB in transformation.2
The diagnostic bone marrow puncture performed at a gestational age of 26 l/7 weeks was compatible with a MDS type RA with ring sideroblasts. To our knowledge, 12 cases of pregnancy-induced MDS were reported in oncologic or haematologic journals. However, specific anaesthetic management was not discussed in these reports and, furthermore, anaesthetic literature on this subject is not available.Pagliuca et al3 reported four cases of pregnancy-associated MDS and reviewed seven cases from literature.‘,4J Vorst et aI6 reported a twelfth case.From these 12 cases of pregnancy-associated MDS, only three parturients were delivered by caesarean section while the others had spontaneous vaginal delivery or termination of pregnancy.3,4In the present case, the mode of delivery was enforced by the transverse lie of the fetus. The decision to perform a caesareansection was also influenced by the fact that maternal antibodies might have crossed the placenta with unknown influence on the coagulation status of the fetus. The potential trauma provoked by vaginal delivery in such a fetus might carry a risk for intracranial haemorrhage. Since regional anaesthesiais absolutely contraindicated in patients with severecoagulopathy due to the risk of an epidural haematoma, surgery was performed under general anaesthesia. To our knowledge, this case represents the most severethrombocytopenia due to MDS that underwent caesarean section. After an extensive search, only two units of HLA-compatible platelets (from one donor) and two units of partial HLA-compatible platelets were available preoperatively. The patient responded well to the first platelet transfusion from the most compatible donor and showed no transfusion reaction. To suppress an immune response, methylprednisolone was administered before each subsequent platelet transfusion.
Acute myelodysplastic syndrome In a highly susceptible patient, the administration of nitrous oxide (N,O) during general anaesthesia could induce haematological abnormalities and even bone marrow depression. 7~8Nitrous oxide probably inactivates the vitamin B,,-dependent enzyme methionine synthase, an enzyme that participates in folic acid metabolism.7 Although this phenomenon only occurs after several hours of N,O administration, we avoided N,O entirely in view of this patient’s haematological dysfunction. REFERENCES 1. Siddiqui T, Elfebein G J, Noyes W D, Moreb J S, Oblon D, Weiner R S. Myelodysplastic syndromes presenting in pregnancy. Cancer 1990; 66: 377-381. 2. Bennet J M, Catovsky D, Daniel M T et al. The FrenchAmerican-British Cooperative Group. Proposals for the
273
classification of the myelodysplastic syndromes. Br J Haematol 1982; 51: 189-199. Pagliuca A, Mufti G J, Fenaux P, De Silva C, Samaratunga I. Myelodysplastic syndromes during pregnancy (letter). Europ J Haematol 1991; 47: 310-312. Boultwood J, Rack K, Buckle V J, Madden J, Oscier D G, Wainscoat J S. Homozygous deletion of FMS in a patient with the Sq-syndrome. Br J Haematoll990; 76: 310-3 11. Furukawa Y, Enomoto M, Sato Y, Yoshida M, Sakamoto S, Miura Y. Myeloplastic syndrome in pregnancy with haematological improvement following delivery. Acta Haematol 1988; 51: 7680. Vorst E J, Levene N A, Nisani R, Berrebi A. Fragile X syndrome and myelodysplasia discovered during pregnancy. Br J Haematol Jpn 1993; 85: 415416. I. Koblin D D, Tomerson B W, Waldman F M, Lampe G H, Wauk L Z, Eger E I. Effect of nitrous oxide on folate and vitamin B,, metabolism in patients. Anesth Analg 1990; 71: 610-617. 8. Waldman F M, Koblin D D, Lampe G H, Wauk L Z, Eger E I. Hematologic effects of nitrous oxide in surgical patients. Anesth Analg 1990; 71: 618-624
CASE REPORT
Anaesthetic managementof caesareansection in a parturient with acute myelodysplastic syndrome F. Christiaens, D. Burrini,” C. Verborgh, N. Fontaine, L. de Catte,* F. Camu Department of Anaesthesiologyand *Department of Foeto-maternalM e d icine, University Hospital A.Z.- KU B., F lemish Free University of Brussels,Brussels,Belgium SUMMARY: A 34-year-old pregnant woman developed a myelodysplastic syndrome during pregnancy which resulted in a refractory anaemia and an extreme thrombocytopenia. The report describes the anaesthetic management of elective caesarean section and successful childbirth in this patient. Following replacement therapy with packed red cells and platelets, general anaesthesiawas used for the procedure.
INTRODUCTION Obstetric anaesthetistsare on occasion confronted by the need to anaesthetizeparturients with haematological problems for delivery or surgery. W e report the managementof elective caesareansection in a parturient with refractory anaemia and extreme thrombocytopenia due to a myelodysplastic syndrome (MDS). CASE REPORT A 34-year-old m u ltigravid (G,P,A,) woman (weight 65 kg, height 162 cm) was scheduled for elective caesarean section becauseof transverselie of the fetus, at a gestational age of 35 weeks and 4 days. M e d ical and surgical history included an appendectomy,asthma in childhood and a true allergy to penicillin. At 21 weeks of pregnancy, she had developed pharyngitis which was treated orally by erythromycin and acetaminophen. She had been referred to the department of fetomaternal m e d icine at a gestational age of 24 weeks and 5 days for severe anaemia (haemoglobin [Hb] 7.9 g/dL and haematocrit [Hct] 23.9%; normal values
F. Christhens MD, C. Verborgh MD, N. Fontaine MD, F. Camu MD, Department of Anaesthesiology, University Hospital A.Z.V.U.B., Flemish Free University of Brussels, Laarbeeklaan 101, B- 1090 Brussels, Belgium and D. Burrini MD, L. de Catte MD, Department of Foeto-maternal Medicine, University Hospital A.Z.-V.U.B., Flemish Free University of Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium. Correspondence to: Dr. Frank Christiaens, Tel: 00 32 2 477 53 60; Fax: 00 32 2 477 5 148. 270
pregnant female at term: 11.6 g/dL and 35.5% respectively) and thrombocytopenia (5 1xl 09/L; normal range pregnant female: 150-400x109/L).She presently complained of fatigue at rest and dyspnoea at night and during exercise. Physical examination revealed anaemic conjunctivae, pallor and a systolic murmur l/6; she was apyretic and had no purpura nor hepatosplenomegaly.The results of an extensive laboratory screeningat this tim e are listed in Table 1. Since no aetiology for this increasing thrombocytopenia and anaemia was found at this moment, a conservative therapeutic attitude was adopted: no transfusion of packed red cells (PRC) nor platelets would be given unless the haemoglobin level decreasedbelow 6 g/dL, the platelet count decreased below 20x109/L or the patient became symptomatic. As the patient became asthenic 4 days later and haemoglobin level turned out to be only 6.9 g/dL, 2 units of PRC were transfused. At a gestational age of 26 l/7 weeks, a diagnostic bone marrow puncture was performed and demonstrated MDS. Karyotype analysis of the bone marrow aspirate showed m u ltiple complex abnormalities, indicating a high risk to leukaemia transformation. Spontaneous epistaxis occurring some tim e later was treated by electrocauterization. Progressive anaemia developed and required m u ltiple leukocyte-free PRC transfusions. A moderate transfusion reaction occurred during a PRC transfusion and several HLAantibodies were demonstrated. At a gestational age of 33 weeks, there were no obstetrical contraindications left for the delivery of the fetus. Fetal pulmonary maturation was induced weekly with corticosteroids. The fetus had developed well. An ultrasound at 34 weeks showed head and
Acute myelodysplastic syndrome
27 1
Table 1. Laboratory results at the time of referral to the department of feto-maternal medicine
Blood group Haemoglobin Haematocrit Red blood cell count Mean cell volume Platelet count White blood cell count Activated partial thromboplastin time Prothrombin time Blood glucose Blood urea Creatinine Creatine kinase Total lactate dehydrogenase Aspartate aminotransferase Alanine aminotransferase Total bilirubin Total protein Albumin Amylase Lipase C-Reactive protein Serum folic acid Intra-erythrocyte folate Ferritin Transferrin Iron binding capacity True vitamin B,, Haptoglobin Fibrinogen Thyroid stimulating hormone Free T, Anticardiolipin antibodies Lupus anticoagulant
Result
Normal non-pregnant range
A Rhesus + (CCDce) 7.0 g/dL 21.4% 2.2xlO’VL 98 fL 33X109/L 9.6xlOVL 25.1 s 85% 105 mg/dL 14 mg/dL 0.52 mg/dL < 20 W/L 972 IUlL 25 IUlL 25 IU/L 0.3 mg/dL 5.7 gldL 3.3 gldL 51 IUlL 25 IU/L < 7 mg/dL 7.1 ug/L 1664 pg/L 154 ug/L 302 mg/dL 352 ugldL 0.76 ug/L < 10 mg/dL 233 mg/dL 1.01 mu/L 8.8 rig/L negative
11.5 -16.5 g/dL 3747% 3.8-5.8~1O’~/L 7696 fL 150-400x109/L 410x10~1L 23-28 s 70-100% 7GlOO mg/dL 15540 mg/dL 0.4-1.2 mg/dL S-130 IU/L 181-502 IUlL 941 IU/L 4-39 IUlL 0.2-1.0 mg/dL 6.3-8.2 gldL 3.9-5.0 g/dL cl13 IUlL 50-200 IU/L < 0.2 mg/dL 2.0-14.0 ug/L 200-800 ug/L lo-260 ug/L 208-382 mg/dL 225420 ug/dL 0.22-0.94 ug/L 27-l 39 mg/dL 200400 mg/dL 0.15-3.0 mu/L 9.0-21.8 rig/L
The viral screening (including: parvo B-19, pica RNA, rubella, cytomegalovirus, Epstein Barr virus, Herpes simplex virus and human immunodeficiency virus) and parasite screening (toxoplasmosis) were not contributive.
abdominal circumference and femur length were in concordance with 33 weeks. Weight was estimated at 2300 g which accorded with the fiftieth centile. Umbilical blood flow was normal. Since the fetus was in transverse lie, the patient was scheduled for caesarean section at 35 3/7 weeks. Fetal heart rate was monitored daily and showed good variability, many accelerations and no decelerations. The evening before surgery, a 16-gauge intravenous catheter was placed and 24 g of immunoglobulins were given. The platelet count was then 9x109/L and the patient was assessed ASA physical class III. The platelet count on the morning of surgery was 12x109/L and increased to 34x109/L after administration of the first unit of platelets. After intravenous administration of 20 mg of methylprednisolone, a second transfusion of platelets was performed resulting in a platelet count of 61x109/L. When these transfusions were completed, the patient was transferred to the operating room. A second 16-gauge catheter was placed intravenously and a 19-gauge catheter was inserted under local
anaesthesia in the left radial artery. Monitoring included maternal heart rate, electrocardiogram, noninvasive and invasive blood pressure, end tidal carbon dioxide, urinary output, pulse oximetry and arterial blood gas analysis. Isoflurane concentration was measured with a Datex Capnomac. Following preoxygenation, general anaesthesia was induced intravenously with vecuronium 0.5 mg, ketamine 100 mg and succinylcholine 75 mg given in a rapid sequence to facilitate endotracheal intubation. Before the baby was born, anaesthesia was maintained with an additional bolus of ketamine (25 mg) and succinylcholine (25 mg), and 100% oxygen was administered. Isoflurane concentration was kept at 0.6%. The incision to delivery time (I-D interval) and the uterine incision to delivery time (U-D interval) were 7 min and 58 s respectively. After delivery, an intravenous bolus of 4 U of oxytocin was given and 6 U were added to 1000 mL Hartmann’s solution. Anaesthesia was further maintained with isoflurane concentrations of 0.4-0.6% in a 40% oxygen/air mixture, fentanyl
272
International
Table 2.
Journal of Obstetric Anesthesia
Results of peroperative arterial blood gas (ABG) analysis
PH PaCO, (mmHg) PaO, (mmHg) HCO,- (mmol/L) Base deficit (mmol/L) Haemoglobin (g/dL) Haematocrit (%)
ABG 1
ABG 2
ABC 3
1.46 29.5 266.5 20.9 -1.1 11.8 34.7
7.41 33.2 198.8 21.2 -2.2 7.1 22.6
7.27 46.2 505.8 21.0 -5.5 10.2 30.0
200 pg and vecuronium 2.5 mg. Results of the first arterial blood gas analysis (performed 15 min after induction), second arterial blood gas analysis (performed before transfusion of one unit of leukocytefree PRC because of estimated operative blood loss of 960 mL) and third arterial blood gas analysis (3 min post extubation) are shown in Table 2. After the second arterial blood gas analysis, 20 mg of methylprednisolone was given intravenously and a third unit of platelets was transfused. Forty-one minutes after induction, a second transfusion of leukocyte-free PRC was given. Peroperative diuresis amounted to 100 mL. The induction to extubation time and the skin to skin operation time were 70 and 55 min respectively. The patient did not move at any time and did not report any awareness.A male infant was born, weighing 2300 g. The Apgar scores were 3, 7 and 9 at 1, 5 and IO min respectively. The placental weight was 650 g and the venous cord blood showed a pH of 7.40, PaCO, 36.2 mmHg, P,O, 33.1 mmHg, HCO,- 22.0 mmol/L and a base deficit of -1.7 mmol/L. On arrival in the Post Anaesthesia Care Unit, laboratory tests showed a Hb of 10.9 g/dL, a Hct of 32.1% and a platelet count of 63x10g/L. Twenty milligrams of methylprednisolone were repeated intravenously and a fourth unit of platelets was transfused and resulted in a platelet count of 66x10g/L. Another 10 U of oxytocin were given over a 12 h period. Postoperative analgesia consisted of 2 mg boluses of piritramide (10 mg in total) and 2 g of proparacetamol intravenously. On the morning of the first postoperative day, laboratory tests showed a Hb of 9.5 g/dL, a Hct of 28.3% and a platelet count of 58x10g/L and the patient was transferred to the maternity ward. The platelet count on the third and fourth postoperative day decreased to 34x10g/L and 24x10g/L respectively. A fifth transfusion of platelets was administered and resulted in a platelet count of 77x10g/L. The patient was discharged from the hospital on the seventh postoperative day with a Hb of 8.2 g/dL, a Hct of 24.3% and a platelet count of 57x10g/L. A bone marrow puncture performed 20 days postpartum demonstrated an evolution to acute myeloid leukaemia (AML) type M6. Despite intensive
treatment, including several courses of chemotherapy, the patient died 8 months postpartum. DISCUSSION
Myelodysplastic syndromes are a group of haematological disorders that are characterized by dysplastic haematopoiesis.’The proportion of blast cells in the peripheral blood and/or bone marrow is one of the main distinguishing features of these conditions.2 The MDS are subdivided in five different types according to the French-American-British classification: 1. 2. 3. 4. 5.
refractory anaemia (RA) RA with ring sideroblasts RA with excessof blasts (RAEB) chronic myelomonocytic leukaemia (CMML) RAEB in transformation.2
The diagnostic bone marrow puncture performed at a gestational age of 26 l/7 weeks was compatible with a MDS type RA with ring sideroblasts. To our knowledge, 12 cases of pregnancy-induced MDS were reported in oncologic or haematologic journals. However, specific anaesthetic management was not discussed in these reports and, furthermore, anaesthetic literature on this subject is not available.Pagliuca et al3 reported four cases of pregnancy-associated MDS and reviewed seven cases from literature.‘,4J Vorst et aI6 reported a twelfth case.From these 12 cases of pregnancy-associated MDS, only three parturients were delivered by caesarean section while the others had spontaneous vaginal delivery or termination of pregnancy.3,4In the present case, the mode of delivery was enforced by the transverse lie of the fetus. The decision to perform a caesareansection was also influenced by the fact that maternal antibodies might have crossed the placenta with unknown influence on the coagulation status of the fetus. The potential trauma provoked by vaginal delivery in such a fetus might carry a risk for intracranial haemorrhage. Since regional anaesthesiais absolutely contraindicated in patients with severecoagulopathy due to the risk of an epidural haematoma, surgery was performed under general anaesthesia. To our knowledge, this case represents the most severethrombocytopenia due to MDS that underwent caesarean section. After an extensive search, only two units of HLA-compatible platelets (from one donor) and two units of partial HLA-compatible platelets were available preoperatively. The patient responded well to the first platelet transfusion from the most compatible donor and showed no transfusion reaction. To suppress an immune response, methylprednisolone was administered before each subsequent platelet transfusion.
Acute myelodysplastic syndrome In a highly susceptible patient, the administration of nitrous oxide (N,O) during general anaesthesia could induce haematological abnormalities and even bone marrow depression. 7~8Nitrous oxide probably inactivates the vitamin B,,-dependent enzyme methionine synthase, an enzyme that participates in folic acid metabolism.7 Although this phenomenon only occurs after several hours of N,O administration, we avoided N,O entirely in view of this patient’s haematological dysfunction. REFERENCES 1. Siddiqui T, Elfebein G J, Noyes W D, Moreb J S, Oblon D, Weiner R S. Myelodysplastic syndromes presenting in pregnancy. Cancer 1990; 66: 377-381. 2. Bennet J M, Catovsky D, Daniel M T et al. The FrenchAmerican-British Cooperative Group. Proposals for the
273
classification of the myelodysplastic syndromes. Br J Haematol 1982; 51: 189-199. Pagliuca A, Mufti G J, Fenaux P, De Silva C, Samaratunga I. Myelodysplastic syndromes during pregnancy (letter). Europ J Haematol 1991; 47: 310-312. Boultwood J, Rack K, Buckle V J, Madden J, Oscier D G, Wainscoat J S. Homozygous deletion of FMS in a patient with the Sq-syndrome. Br J Haematoll990; 76: 310-3 11. Furukawa Y, Enomoto M, Sato Y, Yoshida M, Sakamoto S, Miura Y. Myeloplastic syndrome in pregnancy with haematological improvement following delivery. Acta Haematol 1988; 51: 7680. Vorst E J, Levene N A, Nisani R, Berrebi A. Fragile X syndrome and myelodysplasia discovered during pregnancy. Br J Haematol Jpn 1993; 85: 415416. I. Koblin D D, Tomerson B W, Waldman F M, Lampe G H, Wauk L Z, Eger E I. Effect of nitrous oxide on folate and vitamin B,, metabolism in patients. Anesth Analg 1990; 71: 610-617. 8. Waldman F M, Koblin D D, Lampe G H, Wauk L Z, Eger E I. Hematologic effects of nitrous oxide in surgical patients. Anesth Analg 1990; 71: 618-624