Erythropoietic protoporphyria

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/73642/1/j.1537-2995.2004.04058.x.pd

Nontransfusion hazards of autologous blood donation

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/72837/1/j.1537-2995.2001.41010152.x.pd

A hot option for a cold subject

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/91340/1/j.1537-2995.2011.03474.x.pd

A case of extravascular hemolysis with Tk‐activation

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/108281/1/ccr380.pd

On the high probability that a perceived lack of value of obtaining a p value will be detrimental to patient care

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/92406/1/j.1537-2995.1997.37897424414.x.pd

The impact of recent vincristine on human hematopoietic progenitor cell collection in pediatric patients with central nervous system tumors

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/108370/1/trf12574.pd

Persistence of cefotetan on red blood cells

Cefotetan can cause severe immune hemolytic anemia that may persist long after the drug is discontinued. To study the binding of cefotetan to RBCs, patients who received cefotetan were followed and tested for the presence of antibody to cefotetan. STUDY DESIGN AND METHODS:  Patients receiving cefotetan were identified from pharmacy and nursing records. Blood samples obtained for routine hematology tests were analyzed. Cefotetan binding to patients’ RBCs was tested using a previously characterized high-titer anticefotetan serum by gel technique. To determine the minimum amount of drug necessary for binding to occur, RBCs were incubated with serial dilutions of cefotetan at pH 7.4. RESULTS:  Sixty patients receiving 1 to 25 g IV (median, 2 g) of cefotetan were followed for 1 to 123 days (median, 18 days). All were initially positive, for cefotetan on RBCs. Positivity persisted for up to 98 days after the last dose of drug. Fifteen patients became negative during follow-up. The first negative sample occurred at Day 30 to 123. Using the midpoint between the last positive and first negative to estimate of the duration of positivity, we estimate that cefotetan remains RBC-bound for 16.5 to 92 days (median, 67.5 days). During the follow-up period, five patients developed anticefotetan detectable in the serum. Twenty patients receiving other cephalosporin antibiotics showed no specific reactivity of their RBCs with anticefotetan. In vitro studies showed a minimum necessary drug concentration of 1 µmol/L at physiologic pH, which was not significantly altered by RBC pretreatment with ficin, sialydase, or DTT. CONCLUSIONS:  Cefotetan is tightly bound to RBCs after intravenous administration and remains detectable for weeks after the last dose. Antibodies to cefotetan may occur in about 8 percent of patients receiving the drug. The minimum necessary concentration for RBC binding is low compared to an estimated plasma concentration of 240 µmol/L from a single IV dose of 1 g.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/72360/1/j.1537-2995.2004.03360.x.pd

Incidence and risk factors for patellofemoral dislocation in adults with Charcot-Marie-Tooth disease: An observational study

Background and Purpose: Patellofemoral (PF) dislocation is frequently encountered in clinical practice among people with Charcot-Marie-Tooth disease (CMT), but the frequency and risk factors for PF dislocation in adults with CMT are unknown. This study aimed to establish the incidence of PF dislocation in adults with CMT and to explore the risk factors associated with PF dislocation. Methods: This is a cross-sectional study involving adults with a diagnosis of CMT, attending their outpatient clinics at a specialist neuromuscular centre in the United Kingdom. Eighty-one individuals were interviewed about any PF dislocation and underwent a lower-limb assessment, with a focussed knee examination, to identify possible risk factors for PF dislocation. The incidence of PF dislocation was expressed as a percentage (number of individuals with a positive history of patellar dislocation/overall sample) and the association between different risk factors and PF dislocation was explored using logistic regression analysis. Results: The incidence of PF dislocation was 22.2% (18/81). PF dislocation was associated with a younger age at the time of the assessment (p = 0.038) and earlier disease onset (p = 0.025). All people bar two who dislocated had CMT1A (88.9%), but there was no difference in terms of CMT distribution with the non-dislocation group (p = 0.101). No association was found between PF dislocation and CMT severity measured by CMTSS (p = 0.379) and CMTES (p = 0.534). Patella alta (p = 0.0001), J-sign (p = 0.004), lateral patellar glide (p = 0.0001), generalised joint hypermobility (p = 0.001) and knee flexors weakness (p = 0.008) were associated with an increased risk of dislocation. Patella alta (p = 0.010) and lateral patellar glide (p = 0.028) were independent PF dislocation predictors. Conclusions: PF dislocation was common in this cohort with CMT and was associated with multiple risk factors. Future studies should be conducted to confirm the present findings so that the identified risk factors may be addressed by clinicians through preventive, supportive and corrective measures

Delayed platelet engraftment in group O patients after autologous progenitor cell transplantation

Fucosylated glycans, including H-antigen, play critical roles in hematopoietic progenitor cell homing, adhesion, growth, and differentiation. H-active antigens are strongly expressed on CD34+ progenitor cells and committed megakaryocytic progenitors and may mediate adhesion to marrow stromal fibroblasts. We examined the possible influence of donor ABO type on platelet (PLT) engraftment after autologous peripheral blood progenitor cell transplant (PBPCT). STUDY DESIGN AND METHODS: A retrospective analysis of all patients who underwent a single autologous PBPCT between 1996 and 2000 were reviewed. Neutrophil and PLT engraftment were compared by patient ABO type and CD34+ cell dose by t test, chi-square test, analysis of variance, Kaplan-Meier probability, and log-rank test. RESULTS: Engraftment data was available in 195 patients. PLT engraftment was delayed in all patients, regardless of ABO type, at CD34+ PBPC doses of 2 × 10 6 to 3 × 10 6 per kg (p  50 × 10 9 /L) was significantly delayed in group O patients relative to all non-group O patients (32.4 days vs. 19.6 days, p < 0.001). Approximately 50 percent of group O patients required more than 40 days to achieve late PLT recovery (p < 0.005). CONCLUSIONS: A group O phenotype may be associated with delayed PLT engraftment at lower CD34 doses.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/73409/1/j.1537-2995.2005.04346.x.pd

Anti-A and anti-B titers in pooled group O platelets are comparable to apheresis platelets

Although uncommon, acute hemolytic transfusion reactions (AHTRs) have been reported after transfusion of group O single-donor apheresis platelets (SDPs) to group A, B, and AB recipients. Current methods for identifying “high-titer” SDPs include tube and gel methods. The risk of a high-titer unit is considered low with group O, poststorage, pooled platelet concentrates (PPLTs); however, data regarding anti-A and anti-B titers in PPLTs are lacking. STUDY DESIGN AND METHODS: Anti-A and anti-B titers were determined in 185 PPLTs by direct agglutination using manual gel and tube methods. PPLTs tested included 124 group O PPLTs, 25 group A PPLTs, 26 group B PPLTs, and 10 PPLTs containing a mix of either groups O plus A or groups O plus B (mixed PPLTs). The reciprocal of the highest dilution giving macroscopic agglutination was considered the agglutinin titer. RESULTS: Mean anti-A and anti-B titers in group O PPLTs were, respectively, 16 and 8 by tube and 64 and 32 by gel (p < 0.0001). Gel titers were one to two dilutions higher than tube and sensitive to reagent red cell lots. With the use of at least 64 as a critical titer, 60 percent of group O PPLTs tested by gel would be considered high-titer. In mixed PPLTs, the addition of one non-group O PLT significantly decreased or neutralized the corresponding anti-A or anti-B (p < 0.0001). CONCLUSION: Anti-A and anti-B titers in group O PPLTs are comparable to those reported in group O SDPs and significantly lower than titers reported in AHTR. A critical direct agglutinin titer of 64 for identifying high-titer units by gel is too low and should be increased to 128 or higher.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/73545/1/j.1537-2995.2008.01814.x.pd