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Background and Objectives Erythrocytapheresis and leukapheresis ( LPE) of small children are logistically complex and many centres are reluctant to perform these procedures. In children, both sickle cell and leukaemic emergencies demand prompt action to prevent additional morbidity but detailed protocols for small children are lacking, and often are performed using guidelines shown to work in larger patients. We report a 3-year experience with children weighing 11-25 kg at a large academic medical centre. Materials and Methods All patients were treated with the COBE® Spectra apheresis system; circuit was primed with blood not adjusted for haematocrit and anticoagulant citrate dextrose A was used as anticoagulation. Procedures were performed in the paediatric intensive care unit by apheresis nursing staff. Results Twenty-five apheresis procedures in 19 patients were performed; 17 of 19 patients presented with sickle cell-related acute complications and two (2/19) with newly diagnosed acute leukaemia and hyperleucocytosis. None of the patients required medications during the procedures. Vital signs and clinical condition remained stable and did not worsen during or postapheresis. One patient had a delayed haemolytic transfusion reaction 1 week posterythrocytapheresis as he developed alloantibodies as a result of the procedure. All sickle cell patients achieved a target haematocrit of 21-30% and Haemoglobin A of ≥68%. Both leukaemia patients who underwent LPE had no further signs of leukostasis and achieved marked reductions in leucocyte counts. Conclusions Apheresis of children weighing 11-25 kg can be safely performed without increased morbidity. We outline a protocol that can be used to perform apheresis with minimal complications. [ABSTRACT FROM AUTHOR]

Background: D-RBCs are produced by a rare gene that fails to encode for CcEe. Such persons may produce an antibody (Ab) of complex specificity such as anti-Rh17 (Hro). The anti-Rh 17 Ab may cross the placenta and cause severe hydrops fetalis. Case Report: A 34-year old pregnant woman G2P1001 was found to have a high-titer IgG Ab against high-frequency antigen with negative DAT. Further investigation showed her RBCs are D-. Her mother's and father's RBC phenotypes are O, D+ c+ e+ and A, D+, C+ e+ respectively. Her parents are unrelated. Her husband's RBC phenotype is O, D+C+c+E+e+. Sensitization presumably resulted from transfusion of two units of blood after her first delivery. Prenatal ultrasonography showed polyhydramnios and double bubble consistent with duodenal atresia. Amniocentesis at 31 weeks disclosed zone III OD at 450 on Liley graph. Hydrops fetalis developed two days prior to cesarean section. Two courses of betamethasone were given prior to delivery. The baby, delivered at 33 weeks with birth weight of 2121 g, had severe hydrops fetalis and was intubated immediately. Apgar scores were 4 and 7 at 1 and 5 min, respectively. He received surfactant and mechanical ventilation until day 19, and oxygen until day 25. Initial Hb was 6.9g/dl, platelets 100,000/ul, bilirubin 7.1/1.0 mg/ dl, and RBC type is O, C+D+e+. Two exchange transfusions (EXT) were done: a partial EXT and double volume EXT with D—blood reconstituted with FFP. Ab titer decreased from 1:256 after EXT to 1:16 at 5 weeks of life, with an estimated t1/2 of 6.7 days. Mixed jaundice was treated first with phototherapy, IVIG, and phenobarbital. Because of limited availability of D— blood, erythropoietin was initiated immediately. Cardiovascular complications included systemic hypotension, pulmonary hypertension, and patent ductus arteriosus which required surgical ligation. Laparotomy disclosed a preampullary duodenal atresia with malrotation; a duodenoduodenostomy and appendectomy were done. Persistent cholestatic jaundice was treated with phenobarbital, ursodiol, elementary diet (with MCT oil), and liposoluble vitamins. Conclusions: This is a case of severe hydrops fetalis due to hemolytic disease associated with D— possible de novo mutation, accompanied with duodenal atresia, and treated successfully with a combination of D— RBC and erythropoietin. [ABSTRACT FROM AUTHOR]

Focuses on the successful perinatal management of hydrops fetalis due to hemolytic disease associated with D-- maternal phenotype. Efficacy of intravenous immunoglobulins in treating hemolytic disease of the newborn infant; Serial assessments of fetal ultrasonogram.