The association between AB blood group and neonatal disease.

BACKGROUND: Numerous studies have examined the association between ABO blood groups and adult disease states, but very few have studied the neonatal population. The objective of this study was to determine the relationship between AB blood group and the occurrence of common neonatal disorders such as neutropenia at birth, sepsis, respiratory distress syndrome (RDS), intraventricular hemorrhage (IVH), retinopathy of prematurity (ROP), and patent ductus arteriosus (PDA) compared to all other blood groups. METHODS: We performed a retrospective review on 3,981 infants born at 22 0/7 to 42 6/7 weeks' gestational age and compared the relative risk of neonatal diseases in infants with AB blood group to that of infants with all other blood groups (A, B, and O). RESULTS: When compared to all other blood groups, AB infants demonstrated an increased risk for developing negative clinical outcomes. AB blood group was significantly associated with a 14-89% increased risk of neutropenia at birth, sepsis, RDS, and ROP. Risks for IVH and PDA were not significant. CONCLUSION: We hypothesize that the phenotypic expression of A and B antigens, rather than the antigens themselves, in the AB group may reveal an enhanced susceptibility to injury at the endothelial level resulting in an increased risk for disease development.

Decreased survival in necrotizing enterocolitis is significantly associated with neonatal and maternal blood group: the AB isoagglutinin hypothesis.

OBJECTIVE: To determine the effect of neonatal and maternal blood group on the mortality risk from necrotizing enterocolitis (NEC). STUDY DESIGN: Retrospective chart review of all neonates admitted to the neonatal intensive care unit over 24 years. Data on birth date, gestational age, maternal/neonatal blood group, number of transfusions, and survival time (defined as date of birth to date of death/discharge) were collected on those with NEC. RESULT: 276 neonates with Bell stage II-III NEC were analyzed. AB neonates had a significantly higher risk of mortality from NEC compared with other blood groups (HR 2.87; 95% CI 1.40 to 5.89; P=0.003). Multivariate analysis showed AB blood group to be an independent risk factor for mortality from NEC. CONCLUSION: Neonatal and maternal blood groups are significantly associated with a neonate's survival from NEC. The increased mortality of AB neonates may be related to factors such as neonatal blood group antigens and/or transplacental transfer of isoagglutinins.

Highly successful living donor kidney transplantation after conversion to negative of a previously positive flow-cytometry cross-match by pretransplant plasmapheresis.

Between July 2001 and November 2003, 16 patients with a positive flow-cytometry crossmatch to their potential living donor for kidney transplant were treated with desensitization protocol based on plasmapheresis and low-dose IVIg starting 1 week before the scheduled transplant. Twelve patients (75%) converted to negative crossmatch and were successfully transplanted. Immunosuppression consisted of induction with thymoglobulin, tacrolimus, mycophenolate mofetil, and steroids. Plasmapheresis and IVIg were continued on alternate days for the first postoperative week. The 1-year patient and graft survival was 100%. The rate of acute rejection was 41% (16% cellular and 25% humoral). All of the rejection episodes resolved with treatment. Combination of plasmapheresis and IVIg allows successful conversion from positive to negative flow-cytometry crossmatch in 75% of cases; after conversion, kidney transplant can be carried out with a high rate of success.

Severe hemolytic anemia due to passenger lymphocytes after living-related bowel transplant.

BACKGROUND: Hemolytic anemia following solid organ transplant may be caused by 'passenger' lymphocytes producing antibodies against erythrocytes. This phenomenon has never been described after intestinal transplant. MATERIALS AND METHODS: We report a case of severe, immune-mediated hemolysis due to symptomatic passenger lymphocyte syndrome (PLS) in a 4-yr-old recipient of living donor small bowel transplant. The Coombs'-positive hemolysis was caused by anti-A,B antibodies derived from donor lymphocytes in an ABO-compatible donor-recipient pair (O into A). RESULTS: This complication was successfully and efficiently treated by the novel combined use of group O RBC transfusion, plasmapheresis and rituximab (anti-CD20). CONCLUSIONS: A severe hemolytic anemia due to PLS can occur in bowel transplantation. This complication should be considered when performing ABO-incompatible bowel transplant with a blood group O donor and an A or B recipient. Treatment with plasmapheresis, blood group O transfusion and rituximab has proved successful in our case.

Surge of anti-SS-A antibody associated with fulminant thrombotic thrombocytopenic purpura in pregnancy.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon clinical syndrome and is rarely associated with systemic lupus erythematosus (SLE). Diagnosis of TTP in patients with SLE, especially those who are pregnant, is challenging. We report the case of a pregnant woman with a high level of anti-SS-A antibody (162,143 U/mL) and fulminant TTP. The patient responded to plasma exchange treatment. Recent studies indicate that patients with SLE and another serologic abnormality, such as the presence of antiphospholipid antibody, may be at high risk for TTP. We explore the possible pathogenesis of acute TTP in patients with SLE and summarize the risk factors for acute TTP in patients with SLE and the current treatments for SLE-associated TTP.

Use of recombinant human erythropoietin (EPO-alfa) in a mother alloimmunized to the Js(b) antigen.

Erythropoietin (EPO) is a glycoprotein hormone and the principal regulator of erythropoiesis in the fetus, newborn, and adult. EPO-alfa is erythropoietin manufactured by recombinant human DNA technology (rhEPO). After counseling, a pregnant woman with anti-Js(b) in her serum was started on rhEPO (600 U/Kg, biweekly) to prevent anemia secondary to serial donations of her blood for fetal transfusions. After a total of 25 rhEPO infusions and autologous donation of 8 units of whole blood, maternal hemoglobin prior to the elective cesarean section at 37 weeks was 11.3 gm/dL. Serum EPO concentration was determined in paired maternal and fetal blood samples, before ultrasound guided intravascular transfusions, in this alloimmunized Js(b)-negative and another Rh(D) alloimmunized pregnancy to determine possible correlations between maternal and fetal serum EPO. rhEPO prevented anemia in a patient who donated 8 units of blood from 18-37 weeks of pregnancy without inducing adverse biological effects such as hypertension or thrombotic complications in the placenta. Data presented in this study suggest that EPO does not cross the human placenta.

Successful management of concurrent congenital dyserythropoietic anaemia and autoimmune haemolytic anaemia with splenectomy.

This first known case of concurrent congenital dyserythropoietic anaemia (CDA) and autoimmune haemolytic anaemia (AIHA) which occurred in a hispanic male and spanned 6 years from the age of 2. Light and electron microscopy of bone marrow erythroblasts and immunophenotyping confirmed CDA; serum/eluate warm autoantibodies and positive direct antiglobulin tests (DAT) associated with severe, episodic anaemias established AIHA. Cytogenetic analysis of bone marrow cells and peripheral blood lymphocytes ascertained sex chromosome aneuploidy (48 XY,+ Y,+ Y). Recurrent, life-threatening episodes of transfusion-dependent anaemia refractory to steroids and intravenous immune globulin, were put into stable remission at age 8 years when splenectomy successfully managed both disorders.

Practice parameter for the use of red blood cell transfusions: developed by the Red Blood Cell Administration Practice Guideline Development Task Force of the College of American Pathologists.

A practice parameter has been developed to assist physicians in the therapeutic use of red blood cell transfusions. The developers of this parameter used the best available information from the medical literature, as well as clinical experience and the extensive reality testing required by the College of American Pathologists for approval. In acute anemia, a fall in hemoglobin values below 6 g/dL or a rapid blood volume loss of more than 30% to 40% requires red blood cell transfusions in most patients. However, tissue oxygenation provides a better indication of physiologic need in situations where invasive monitoring provides this information. When these data are not available, heart rate and blood pressure measurements and the nature of bleeding (active, controlled, uncontrolled) supplement the hemoglobin value in guiding the transfusion decision. In sickle cell disease and thalassemias, red blood cells are transfused to prevent acute or chronic complications. Red blood cell transfusions are used in chronic anemias unresponsive to pharmacologic agents based on the patient's symptoms. Guidelines must be altered for neonates who require an increase in hematocrit to above 0.30 to 0.35 when respiratory distress is present. Indications for red blood cell transfusion for the pregnant or postpartum patient are similar to those for the nonpregnant patient. Risks of transfusion, particularly transmissible disease and incompatibility, remain but have been reduced. Thus, red blood cell transfusion continues to be a powerful therapeutic tool when used judiciously and carries less risk than in the recent past.

Delayed hemolytic transfusion reaction due to anti-Js(a) in an alloimmunized patient with a sickle cell syndrome.

Delayed hemolytic transfusion reactions occur via an anamnestic immune response in patients previously alloimmunized by certain RBC antigens. Conventional pretransfusion antibody screening tests and crossmatches are unable to detect certain antibodies that potentially can cause these reactions because they may be present in low concentrations or have low affinity for their respective antigen or their indicator antigen may be absent from test RBCs. We report the second case of a delayed hemolytic transfusion reaction caused by an undetectable (by routine methods) anti-Js(a) in a patient with a sickle cell syndrome (hemoglobin SC disease) and multiple alloantibodies, in whom retrospective indirect antiglobulin tests enhanced by polyethylene glycol revealed the presence of weakly reactive anti-Js(a).

Risks of transfusion and organ and tissue transplantation: practical concerns that drive practical policies.

The risks and other adverse effects associated with blood transfusion and tissue and organ transplantation were reviewed retrospectively with the use of recent US activity data and published compilations of such events for both therapeutic modalities. In addition, practices that govern blood transfusion and transplantation, in light of regulations and standards of practice, and the practical factors that cause variations in the use of both therapies were compared and contrasted. These factors include availability, preservation and viability, life-saving vs life-enhancing effects, donor selection and qualification, donor-recipient pair matching, disease marker screening and transmission potential, immunopathologic considerations, and future alternative directions.

Peripheral blood stem cell collection with reduced platelet loss to the patient/donor.

Apheresis procedures that optimize peripheral blood stem cell (PBSC) harvesting also result in a significant loss of platelets to the patient/donor because of their similar densities. We compared the percent drop in platelet count and hemoglobin concentration in the patients before and after PBSC collection using two different collection chambers with the CS-3000. A modified plateletpheresis procedure was utilized. Seven patients underwent 38 PBSC collections during steady state hematopoiesis using the standard A-35 collection chamber. At the end of the procedure, a second low-speed centrifugation of the PBSC concentrate was performed in the manual mode, with siphoning out and return of the PRP to the patient through a transfer pack. For 14 patients who underwent 113 PBSC collections, a small volume collection chamber (SVCC) was substituted for the A-35 chamber and the second centrifugation step was omitted. These patients were also primed with 4 g/m2 of cyclophosphamide. The percent drop in platelet count in the patients after the collection procedures was significantly less in the SVCC group (20.4 +/- 9.1 vs. 36.0 +/- 12.3, P = 0.000), even after correction for the difference in the volume of blood processed between the two groups (3.2 +/- 1.4 vs. 3.9 +/- 1.3, P = 0.006). The percent drop in hemoglobin concentration was also less with the SVCC both before (5.4 +/- 3.8 vs. 11.7 +/- 3.0, P = 0.000) and after (0.8 +/- 0.6 vs. 1.3 +/- 0.3, P = 0.000) correction for the difference in the volume of blood processed.(ABSTRACT TRUNCATED AT 250 WORDS)

Concentration of bone marrow mononuclear cells using a programmable blood cell separator.

Bone marrow was collected from adult patients with various solid tumors who consented to participate in a study of myelo-ablative chemotherapy followed by autologous bone marrow rescue. Twenty marrow suspensions were processed by using standard Procedure 3 (PRO-3) for lymphocytapheresis without modification. A modified Procedure 1 (M-PRO-1) for plateletpheresis was employed for processing 34 marrow suspensions. For PRO-3, mononuclear cell (MNC) recovery was 68 +/- 22% of the starting marrow suspension (baseline), in a concentrate volume of 234 +/- 53 ml. MNC represented 59 +/- 27% of the total WBC count of the concentrate. The residual volume of RBC was 49 +/- 47 ml. For M-PRO-1, MNC recovery was 63 +/- 22% of the baseline in a concentrate volume of 200 +/- 8 ml. MNC comprised 94 +/- 7% of the total WBC count of the concentrate. RBC contamination was 7 +/- 3 ml. Hematopoietic recovery, defined as the post-transplant days when a sustained granulocyte count of 500/microL and a platelet count of 50,000/microL were achieved, was similar in the two groups and comparable to other reports utilizing other methods and equipment for bone marrow concentration. Personnel time was significantly reduced compared to other procedures for bone marrow concentration due to increased automation. Although there was no significant difference in MNC recovery between the two groups (P greater than 0.5), M-PRO-1 was clearly superior to PRO-3 because of the consistently high degree of purity of the MNC in the concentrate and minimal RBC contamination.(ABSTRACT TRUNCATED AT 250 WORDS)

Peripheral blood-derived stem cell collections for use in autologous transplantation after high dose chemotherapy: an alternative approach.

Peripheral blood-derived hematopoietic stem cells (PBHSC) can be utilized to reconstitute hematopoiesis in patients after high dose myeloablative chemotherapy. We have performed PBHSC reinfusion in 18 patients who have or had a history of tumor involving the bone marrow or a hypocellular bone marrow. The PBHSC were collected by continuous flow leukapheresis and subsequently cryopreserved. A median of 31.9 X 10(9) (range 18 X 10(9)-67.2 X 10(9)) mononuclear cells or a median of 4.85 X 10(8) mononuclear cells/kg (range 2.7 X 10(8)-11.0 X 10(8)) were collected. Fifteen patients had in vitro assays of granulocyte-monocyte colony-forming cells (CFU-GM) performed with a median of 2.7 X 10(4) (range 0-11.5 X 10(4)) CFU-GM/kg. To date, of the 18 patients collected, all have undergone high dose chemotherapy and PBHSC reinfusion. For the evaluable patients, granulocyte recovery (greater than 500 X 10(6)/ml) has occurred in a median of 15 days and platelet recovery (greater than 50 X 10(9)/ml without transfusion support) has occurred in a median of 43 days. Four patients have had prolonged thrombocytopenia and still require platelet transfusion at 80+, 97+, 124+, and 270+ days. PBHSC collection is safe and effective for hematopoietic reconstitution after high dose chemotherapy in the majority of patients; however incomplete hematopoietic reconstitution has been observed in 4/18 evaluable patients. This procedure requires further investigation.