This thesis focuses on several aspects related to the hematological outcome of infants with hemolytic disease of the fetus and newborn (HDFN) due to red blood cell alloimmunization, including... Show moreThis thesis focuses on several aspects related to the hematological outcome of infants with hemolytic disease of the fetus and newborn (HDFN) due to red blood cell alloimmunization, including pathogenesis and management of the disease. The presence of leukocytopenie and thrombocytopenia support the mechanism of suppression of thrombopoiesis and granulopoiesis in favor of the increased erythropoiesis stimulated by anemia and hypoxia. In addition to the problems caused by a shortage of platelets and white and red blood cells, the excess of the red blood cell metabolites bilirubin and iron also contribute to the morbidity of the disease. This thesis also contains a systematic review that demonstrates that there is lack of high level evidence promoting the use of intravenous immunoglobulin (IVIg) to prevent exchange transfusions in HDFN. Irrespective of the controversy concerning the use of IVIg, this thesis shows that intensive follow-up is indicated in both Rhesus D and non-Rhesus D HDFN and raised awareness about the associated morbidity is of paramount importance. Finally, this thesis also contains future research questions concerning HDFN. Show less
BACKGROUND: Neonates with Rhesus c (Rh c) hemolytic disease of the fetus and newborn (HDFN) are often managed in the same way as neonates with Rhesus D (Rh D) HDFN, although evidence to support... Show moreBACKGROUND: Neonates with Rhesus c (Rh c) hemolytic disease of the fetus and newborn (HDFN) are often managed in the same way as neonates with Rhesus D (Rh D) HDFN, although evidence to support this policy is limited. The objective of this study was to evaluate neonatal outcome in severe Rh c HDFN compared to Rh D HDFN. STUDY DESIGN AND METHODS: A retrospective study of (near-)term neonates with severe Rh c (n = 22) and Rh D HDFN (n = 103; without additional antibodies) admitted to the Leiden University Medical Center between January 2000 and October 2011 was conducted. The need for intrauterine transfusions (IUTs), phototherapy, exchange transfusions (ETs), and top-up transfusions up to 3 months of age were recorded and compared between both groups. RESULTS: Although there was a trend for a slightly more severe antenatal course for Rh D HDFN reflected by an earlier need for and higher number of IUTs (median [interquartile range], 2 [1.5-4] vs. 2 [1-2] in Rh c HDFN; p = 0.070), no significant differences were found for the postnatal course between Rh c and Rh D group in days of phototherapy (mean, Days 4.8 and 4.6, respectively; p = 0.569), need for ET (50% vs. 44%, respectively; p = 0.589), and top-up transfusions (62% vs. 78%, respectively; p = 0.128). CONCLUSION: Postnatal outcome in neonates with severe Rh c HDFN is similar compared to neonates with severe Rh D hemolytic disease in terms of days of phototherapy, need for ET, and need for top-up transfusions. These results justify a similar postnatal management of neonates with Rh D and Rh c HDFN. Show less
Rath, M.E.A.; Smits-Wintjens, V.E.H.J.; Walther, F.J.; Lopriore, E. 2011
Objective To evaluate the incidence and severity of and risk factors for thrombocytopenia at birth in neonates with red cell alloimmunization. Study design All neonates with haemolytic disease of... Show moreObjective To evaluate the incidence and severity of and risk factors for thrombocytopenia at birth in neonates with red cell alloimmunization. Study design All neonates with haemolytic disease of the foetus/newborn (HDFN) due to red cell alloimmunization admitted to our centre between January 2000 and September 2010 were included in this retrospective study. We measured platelet counts at birth and determined the incidence of thrombocytopenia (platelet count < 150 × 10(9) /l) and severe thrombocytopenia (platelet count < 50 × 10(9) /l). Risk factors for thrombocytopenia at birth were evaluated. Results Thrombocytopenia was present in 26% (94/362) of included neonates with HDFN at birth. Severe thrombocytopenia was found in 6% (20/362) of neonates. Three risk factors were found to be independently associated with thrombocytopenia at birth: treatment with intrauterine red cell transfusion (IUT) (OR 3·32, 95% CI 1·67-6·60, P = 0·001), small for gestational age (SGA) below the 10th percentile (OR 3·32, 95% CI 1·25-8·80, P = 0·016) and lower gestational age at birth (OR 1·22/week, 95% CI 1·02-1·44, P = 0·025). Conclusions Thrombocytopenia at birth occurs in 26% of neonates with HDFN due to red cell alloimmunization and is independently associated with IUT treatment, SGA and lower gestational age at birth. Show less
Objective To study the effect of a restrictive guideline for exchange transfusion (ET) on the number of top-up transfusions in neonates with Rh hemolytic disease. Study Design Retrospective study... Show moreObjective To study the effect of a restrictive guideline for exchange transfusion (ET) on the number of top-up transfusions in neonates with Rh hemolytic disease. Study Design Retrospective study of all (near)-term neonates with Rh hemolytic disease admitted to our center between 2000 and 2008. In December 2005, policy changed from using liberal ET criteria to more restrictive ET criteria. We recorded the number of ETs and the number of top-up transfusions in the group of neonates before (group I, n = 156) and after (group II, n = 27) the guideline change. Results The percentage of neonates requiring an ET decreased from 66% (103/156) in group I to 26% (7/27) in group II (P < 0 center dot 01). The percentage of neonates receiving a top-up transfusion increased from 68% (105/154) in group I to 81% (22/27) in group II (P = 0 center dot 25). The median number of top-up transfusions increased from 1 (interquartile range 0-2) in group I to 2 (interquartile range 1-3) in group II (P = 0 center dot 01). Conclusion In this study, restrictive ET criteria in neonates with Rh hemolytic disease lead to a reduction of the rate of ET but an increase in the number of top-up transfusions for neonatal anemia. Show less