Exanguinotransfusión parcial en un paciente con anemia falciforme homocigota sometido a cirugía cardíaca con circulación extracorpórea: reporte de caso / Partial exchange transfusion in a patient with homozygous sickle cell disease undergoing heart surgery with cardiopulmonary bypass: a case report

En pacientes con anemia falciforme, el circuito de circulación extracorpórea promueve la polimerización de la hemoglobina y la formación de drepanocitos. La exanguinotransfusión reduce los niveles circulantes de hemoglobina S. Reportamos el manejo de un niño con anemia falciforme homocigótica que requirió cirugía de cierre de comunicación interauricular. Se realizó exanguinotransfusión parcial intraoperatoria disminuyendo los niveles de hemoglobina S de 89 por ciento a 23 por ciento. La circulación extracorpórea fue conducida en normotermia utilizando cardiología fría, realizando el procedimiento quirúrgico en paro sinusal.

In patients with sickle cell anemia, the extracorporeal circulation circuit promotes the polymerization of hemoglobin and sickle cell formation. Exchange transfusion reduces circulating levels of hemoglobin S. We report the management of a child with homozygous sickle cell anemia who required surgical closure of atrial septal defect. Partial intraoperative exchange transfusion was performed that decreased hemoglobin S levels from 89 percent to 23 percent. Cardiopulmonary bypass was conducted at normothermia with cold blood cardioplegia and the surgical procedure was performed in sinus arrest.

Jaundice in the newborns.

Hyperbilirubinemia is the commonest morbidity in the neonatal period and 5-10% of all newborns require intervention for pathological jaundice. Neonates on exclusive breast-feeding have a different pattern and degree of jaundice as compared to artificially fed babies. Latest guidelines from the American Academy of Pediatrics (AAP) for management of jaundice in a normal term newborn have been included in the protocol. Separate guidelines have been provided for the management of jaundice in sick term babies, preterm and low birth weight babies, for hemolytic jaundice and prolonged hyperbilirubinemia.

Neonatal jaundice and splenic hematoma.

In the newborn period, unconjugated hyperbilirubinemia (UHB) is common, multifactoral, and associated with a variety of physiologic and pathologic conditions. The most commonly identified pathologic cause leading to hyperbilirubinemia is hemolytic disease of the newborn. We report a five-days-old female infant with neonatal jaundice secondary to splenic hematoma.

Effect of partial exchange transfusion in asymptomatic polycythemic LBW babies.

This randomized controlled trial was conducted to determine the effect of partial exchange transfusion in polycythemic babies. Forty five asymptomatic polycythemic babies with birth weight < or = 2000 g were included and randomly assigned to undergo either partial exchange transfusion using isotonic saline within 4 hours of screening or routine medical management. Outcome measures were neonatal morbidity (especially hypoglycemia and neurological alterations) and mortality; developmental delays using DDST-II, neurological deficits, tone and DTR abnormalities over 18 months follow up period. The overall neonatal morbidity in this study was low and comparable in the two groups. Some of the polycythemic babies in the non-exchanged group found initially at 3 months age with "suspected development" grew out of their developmental delay at 18 months of age or later while those who underwent exchange transfusion and with retarded development at 3 months of age remained so even at 18 months of age.

Subgaleal hematoma and seven exchange transfusions.

A 3 kg baby was delivered by cesarean section after prolonged labor. He had massive subgaleal hematoma. He developed anemia requiring packed cell transfusions and hyperbilirubinemia requiring a total of seven exchange transfusions and highly intensive phototherapy. There were no adverse complications of the hyperbilirubinemia or the exchange transfusion.

Component therapy.

The dramatic advances that have taken place in recent years in the care of sick and premature infants also have been matched by a similar increase in the use of blood transfusion therapy. Haematological features indicate that a newborn has a blood volume of 85-125 ml/kg the foetal haemoglobin is 60-85% and average Hb in full term infant is 18 gm/dl. By 2-3 months it falls to 11-12 g/dl the main cause of anemia are iron poor diet, weaning diets recurrent or chronic infections and hemolytic episodes in malarious areas. The red cells transfusions are usually top up transfusions, exchange transfusions, partial exchange transfusions. Top up- are for investigational losses and correction of mild degrees of anemias, upto to 5-15 ml/kg. They comprise 90% of all neonatal transfusions and are used in low birth babies in special care units for a maximum of 9-10 episodes. The walk in donor programs once popular are not much in vogue. The threshold for transfusion is 8-10 g/dl Hb for upto 5 weeks. Exchange transfusions are done for correction of anemia, removal of bilirubin, removal of antibodies and replacement of red cells. Ideally plasma reduced red cells that are not older than 5 days are used. It is prepared by removal of 120 ml of standard whole blood donation. The advantage of fresh cells is that hyperkalemia is avoided and good post transfusion survival acceptable red cell oxygen affinity. However it has to be screened for sickle cell disease and G6PD deficiency. Indications for exchange transfusion are kernicterus, neonatal hemolysis, G6PD deficiency, ARDS, neonatal sepsis, DIC and neonatal isoimmune thrombocytopaenia. Complications include over transfusion, perforation of major vessels, hypocalcaemia, citrate toxicity, hypothermia, hypoglycaemia, thrombocytopenia, necrotizing enterocolitis, GVHD, bacterial, viral infections. Partial exchange transfusions are done for symptomatic anemia, where Hb<10 g/dl, it is indicated in polycythemia and hyperviscosity syndromes. Exchange volume = Blood volume x (observed Hct-Desired HCt) divided observed Hct. Points to consider-there is weak expression of ABO antigens so particular care while grouping. Transfusing volumes should be 2-5 ml/kg/hour in paediatric bags of 50-100 ml with infusion devices. Platelet transfusion are indicated in neonatal throbocytopaenia, thrombocytopaenia due to sepsis, DIC, bacterial pathogens, CMV, TORCHS, Obstetric conditions such as pre eclampsia, intrauterine death abruption placenta birth injury hypoxia schock neonatal iso immune thrombocytopaenia and maternal ITP. Administration 1 RDE/pack per 2.5 kg single dose of fresh platelets less than 24hrs which contains 55 x 10(9) cells. This also contributes fresh plasma so is useful for coagulation defects also, though there is a risk of CMV and GVHD due to leucocyte contamination. Granulocyte concentrate; Gravity leucopheresis-1:8 ratio of 60 ml of 6% HES made to stand for 1hr.

Jaundice in the newborn.

Hyperbilirubinemia is the commonest morbidity in the neonatal period and 5-10% of all newborns require intervention for pathological jaundice. Neonates on exclusive breast-feeding have a different pattern of physiological jaundice as compared to artificially fed babies. Guidelines from American Academy of Pediatrics (AAP) for management of jaundice in a normal term newborn have been included in the protocol. Separate guidelines have been provided for the management of jaundice in sick term babies, preterm and low birth weight babies, for jaundice secondary to hemolysis and for prolonged hyperbilirubinemia. Although hour specific bilirubin charts are available, these have to be validated in Indian infants before they are accepted for widespread use.

Exchange transfusion in severe falciparum malaria: a simple method modified from hemodialysis circuit.

We set up a simple extracorporeal circuit, modified from the extracorporeal method generally used in conventional hemodialysis, for exchange transfusion. Temporary vascular access was used in exchange transfusion for both draining the infected blood and infusion of the freshly non-infected blood. This method of exchange transfusion was performed in 3 severe complicated falciparum malaria patients who had a percentage of parasitemia of 80, 40, and 35. The magnitude of parasitemia decreased immediately to less than one per cent and this value persisted twenty-four hours after the procedure. No complications of exchange transfusion were detected in all patients. Erythrocyte morphology determined by scanning electron microscopy was unaltered by exchange transfusion. Because of the simplicity, the effectiveness, and the safety of the procedure, this extracorporeal circuit modified from hemodialysis circuit would be a more beneficial exchange transfusion method in the treatment of severe complicated falciparum malaria than the manually-performed one.

High dose intravenous immunoglobulin therapy in neonatal immune hemolytic jaundice

Isoimmune hemolytic jaundice due to blood group [ABO] and Rhesus [Rh] incompatibility is an important problem in the neonatal period. A controlled study was conducted to assess the role of high dose intravenous immunoglobulin [HDIVIG] therapy in neonatal immune hemolytic jaundice. Newborn patients with ABO and/or Rh incompatibilities proved by significant hyperbilirubinemia [>15mg/dl], positive direct Coombs' test and high reticulocytic count [>6%] were randomly assigned to receive either conventional treatment measures alone, including phototherapy and exchange transfusion if needed [control group, n=10 newborns] or phototherapy with high dose i.v. immunoglobulin [1gm/kg] over 4 hours [study group, n=30 newborns] as soon as the diagnosis was established. One of the 30 patients in the HDIVIG group required exchange transfusion, while this became necessary in 3 of 10 patients in the control group [p<0.001]. The duration of phototherapy and hospitalization, in terms of hours were significantly shorter in the HDIVIG group [p<0.001]. No adverse effects of HDIVIG therapy were observed. The use of HDIVIG therapy in newborns with ABO and/or Rh hemolytic disease reduces hemolysis, serum bilirubin levels, the need for blood exchange transfusion and the duration of hospitalization

Neonatal polycythemia: effects of partial exchange transfusion using fresh frozen plasma, Haemaccel and normal saline.

BACKGROUND: Neonatal polycythemia remains a significant clinical problem in Thailand. Partial exchanges transfusion (PET) with fresh frozen plasma (FFP) has been the mainstay of management for this condition in Thailand. Since FFP is difficult to find in certain areas and can cause concerns of transfusion related diseases, this study was undertaken to investigate the possibility of using plasma substitute and normal saline (NSS) for PET in the newborn infant with polycythemia. OBJECTIVE: 1. To compare the rate and duration of decrease of venous hematocrit (Hct) before and after PET with FFP, Haemaccel and NSS. 2. To compare any complications from using FFP, Haemaccel and NSS such as coagulation defect, electrolytes change, etc. in PET. METHODS AND SUBJECTS: A randomized prospective trial was conducted in Neonatal Unit, Department of Pediatrics, Ramathibodi Hospital. The first phase of study: July 1, 1993 to June 30, 1994: randomized prospective trial using FFP or Haemaccel for PET in 26 newborn infants with polycythemia. The second phase of study: July 1, 1994 to June 30, 1995: consecutive enrollment trial using NSS for PET in 38 consecutive newborn infants with polycythemia. RESULTS: There was significant decrease in Hct in both groups after PET but there was no statistically significant difference in the rate of decrease of Hct. There was no significant difference in biochemical profiles in both groups of infants 24 hours after PET. In the NSS group, there was significant decrease of Hct level after PET. There was no significant change of biochemical profiles and coagulation activity in these patients 24 hours after exchange transfusion. There were 2 patients with complications related to umbilical venous catheter and PET. CONCLUSION: Haemaccel and NSS can be safely used for PET to treat neonatal polycythemia. However, the attending physician should be aware of possible complications related to umbilical venous catheterization and PET.

Symptomatic neonatal polycythemia: comparison of partial exchange transfusion with saline versus plasma.

A prospective study to evaluate efficacy and safety of partial exchange blood transfusion (PEBT) with normal saline or plasma was conducted in 30 symptomatic polycythemic newborns. Babies were randomly assigned to receive PEBT either with normal saline or plasma. Both groups were comparable in terms of birth weight, gestational age, preexchange hematocrit and viscosity. A significant fall in hematocrit and viscosity was noticed at 6 hours following PEBT which persisted even at 24 hours (P < 0.001). Hematocrit and viscosity were comparable in the two groups at 6 and 24 hours (p > 0.05 for both). Majority of babies became asymptomatic after 24 hours of PEBT, but one baby in the saline group remained polycythemic and symptomatic requiring repeat PEBT. No complications related to the procedure were encountered in the two groups. Partial exchange with normal saline was as effective and safe as plasma in symptomatic polycythemic newborns.

Exchange transfusion therapy in severe complicated malaria.

Nine cases of severe complicated falciparum malaria treated by exchange transfusion were studied. Eight patients survived and one patient died. Multisystemic complications were found in all cases. The CNS complications, acute renal failure, pulmonary insufficiency, jaundice, bleeding, sepsis, and DIC were found in 9, 7, 5, 7, 2, 4 and 1 cases, respectively. The fatal case presented with severe multisystemic complications together with 40% parasitemia. In eight survivors, whose parasitemia ranged from 0.3%, to 90%, had milder degrees of systemic complications. With the use of blood exchange 10-15 units, the parasitemia was decreased to less than 5% within 24 hours in all expect one who had parasitemia 90%. In comparison with the other 10 matched non-exchanged patients, there was no significant difference in survival rate between these two group (89% vs 80%). However, in the patients with ARDS the survival rate in the group who received the exchange transfusion therapy was superior (75% vs 0%). The exchange transfusion therapy is therefore strongly recommended in the treatment of malarial patients who present with parasitemia > 30% and severe systemic complications, particularly those who have severe acute renal failure or have lung complications. The amount of blood used for exchange transfusion should at least 1.2 times the blood volume for rapid removal of parasites and toxic metabolites from the circulation.

Cerebral malaria treated with partial exchange transfusion

We report a 25-year-old non-immune male with cerebral malaria, successfully treated with quinine infusion and a 8 units exchange transfusion. The plasmodium falciparum parasitaemia increaswd from 8% to 15% despite the quinine infusions and scanning electron microscopy showed at the same time persistent morphological changes in the parasitised erythrocytes. Exchange transfusion was associated with a rapid decrease in the parasitaemia to < 5% with improvement of the clinical condition and haematological parameters. This supports the recommendations of exchange transfusion in cerebral malaria

Exchange transfusions via peripheral vessels.

During a 15 month period, partial exchange transfusions (ET) were done in 40 neonates with polycythemia, and double volume ET attempted in 7 neonates with hyperbilirubinemia via peripheral vessels. The procedure was effective and not associated with any complications for partial ET. During double volume ET minor complications were noted in 2 cases, both of whom recovered and subsequently successfully underwent supraumbilical ET. Of the 5 cases who had uneventful double volume exchanges, there was a significant drop in indirect serum bilirubin following the procedure. The mean pre-ET serum indirect bilirubin in these 5 cases was 334 mumol/L and mean post-ET level was 179 mumol/L with a mean drop of 155 mumol/L (46% drop). Technical difficulties in catheterization may be overcome with greater expertise and use of heparin to flush arterial catheters.

Exchange transfusions in neonatal sepsis.

Between October, 1987 and October, 1988, 53 neonates with severe or unresponsive sepsis were subjected to therapeutic exchange transfusions (ET) using 170 ml/kg of citrated blood less than 24 hours old. The procedure was repeated up to a maximum of 4 times. The success of therapy was adjudged by resolution of sclerema and/or improvement in clinical features. There were 32 low birth-weight (LBW) and 21 non-LBW infants and 51/53 subjects had sclerema. The mean time for recovery following ET was 19.6 +/- 12.4 h (range: 1-48 h). The overall survival was 77.4% and the survival rates for LBW and non-LBW infants were 73.6 and 68.2%, respectively, however, the difference was not statistically significant. No significant or fatal complications occurred during ET. The effects of other associated problems on outcome studied by multiple regression analysis showed that neurologic problems were associated with a poor chance for survival despite ET. Exchange transfusion may thus be an effective and safe therapeutic modality for severe neonatal sepsis.

Método manual de exanguinotransfusión parcial en pacientes con anemia drepanocítica / Manual method for partial exchange transfusion in patients with drepanocytic anemia

Se describe un método manual de exanguinotransfusión parcial utilizado en 21 pacientes con anemia drepanocítica. Se analizan las ventajas de este proceder, entre las que se destacan: obtención de una disminución de los valores de hemoglobina S a un 40% aproximadamente y utilización de solo 80 minutos como promedio para realizar el recambio sanguíneo, 20 días después de la exanguinotransfusión las cifras de HbS se mantienen inferiores al 50%