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1/2. Anesthetic management of Cesarean delivery in a patient with hypoplastic anemia and severe pre-eclampsia.

    PURPOSE: To describe the anesthetic management of Cesarean delivery in a patient with hypoplastic anemia and severe pre-eclampsia. CLINICAL FEATURES: A 28-yr-old parturient with a history of thrombocytopenia was admitted with signs of pre-eclampsia (blood pressure of 140/90 mmHg, heavy proteinuria and moderate bilateral ankle edema) at 25 weeks of gestation. Laboratory studies revealed pancy-topenia (hemoglobin 6.4 g.dL(-1), white cell count 3.43 x 10(9).L(-1), platelet count 20 x 10(9).L(-1)) and bone marrow biopsy showed hypoplastic anemia. As pre-eclampsia worsened, a Cesarean delivery was performed at 27 weeks with prophylactic platelet transfusion and meticulous blood pressure control. The procedure was uneventful, conducted under general anesthesia with an estimated blood loss of around 600 mL and a live female baby was delivered. Postoperatively her blood pressure and neurological symptoms improved but thrombocytopenia remained at discharge. CONCLUSIONS: Hypoplastic anemia is rare in pregnancy but it poses an increased risk for both mother and fetus. The mother is at risk of life-threatening episodes of bleeding and infection and a multidisciplinary team approach (obstetrician, anesthesiologist, hematologist and pediatrician) is essential. An accurate assessment of the hematological condition should be made and abnormalities corrected before surgery. Regional anesthesia may not be possible in this circumstance. ( info)

2/2. Reduced intensity hematopoietic stem-cell transplantation across human leukocyte antigen barriers in a patient with congenital amegakaryocytic thrombocytopenia and monosomy 7.

    Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare inherited bone marrow failure syndrome that has the potential to progress to pancytopenia and acute myeloid leukemia. Hematopoietic stem-cell transplantation (HSCT) is presently the only curative treatment approach. We used a reduced intensity transplantation regimen in a CAMT patient with aplastic anemia and monosomy 7 who had no matched related donor. The patient had rapid and durable engraftment with minimal complications and is well 24 months post-transplantation. Thus, reduced intensity conditioning might be a feasible approach to stem-cell transplantation in patients with CAMT who do not have a related donor and who are at increased risk of toxicity from standard conditioning regimens. ( info)


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