Obstetric and Newborn Weak D-Phenotype RBC Testing and Rh Immune Globulin Management Recommendations: Lessons From a Blinded Specimen-Testing Survey of 81 Transfusion Services.

CONTEXT.­: Modern RHD genotyping can be used to determine when patients with serologic weak D phenotypes have RHD gene variants at risk for anti-D alloimmunization. However, serologic testing, RhD interpretations, and laboratory management of these patients are quite variable. OBJECTIVE.­: To obtain interlaboratory comparisons of serologic testing, RhD interpretations, Rh immune globulin (RhIG) management, fetomaternal hemorrhage testing, and RHD genotyping for weak D-reactive specimens. DESIGN.­: We devised an educational exercise in which 81 transfusion services supporting obstetrics performed tube-method RhD typing on 2 unknown red blood cell challenge specimens identified as (1) maternal and (2) newborn. Both specimens were from the same weak D-reactive donor. The exercise revealed how participants responded to these different clinical situations. RESULTS.­: Of reporting laboratories, 14% (11 of 80) obtained discrepant immediate-spin reactions on the 2 specimens. Nine different reporting terms were used to interpret weak D-reactive maternal RhD types to obstetricians. In laboratories obtaining negative maternal immediate-spin reactions, 28% (16 of 57) performed unwarranted antiglobulin testing, sometimes leading to recommendations against giving RhIG. To screen for excess fetomaternal hemorrhage after a weak D-reactive newborn, 47% (34 of 73) of reporting laboratories would have employed a contraindicated fetal rosette test, risking false-negative results and inadequate RhIG coverage. Sixty percent (44 of 73) of laboratories would obtain RHD genotyping in some or all cases. CONCLUSIONS.­: For obstetric and neonatal patients with serologic weak D phenotypes, we found several critical problems in transfusion service laboratory practices. We provide recommendations for appropriate testing, consistent immunohematologic terminology, and RHD genotype-guided management of Rh immune globulin therapy and RBC transfusions.

The role of therapeutic plasma exchange in clinically amyopathic dermatomyositis with MDA-5 antibody: A case report and review of the literature.

Clinically amyopathic dermatomyositis (CADM) is a rare, aggressive variant of dermatomyositis associated with interstitial lung disease (ILD) and refractoriness to immunosuppressants. Antibodies against melanoma differentiation-associated gene 5 (MDA-5) are often found in patients with CADM. We report a patient with advanced CADM with ILD and MDA-5 antibodies who failed to improve with immunosuppressants. We performed 2 TPE over 3 days, using 5% albumin as replacement fluid. Although five total TPE were planned, he was transferred for lung transplant evaluation after the second TPE; he died 16 days after transfer without receiving a transplant. A literature review identified four patients with CADM and MDA-5 antibodies treated with TPE; all experienced symptomatic improvement of their ILD. We attribute our patient's outcome to the advanced nature of his disease rather than a failure of TPE. Additional research may indicate a possible reclassification of CADM with MDA-5 antibodies in future ASFA guidelines.

Correlating Transfusion Medicine In-Service Examination Scores With Outcomes of the American Board of Pathology Transfusion Medicine Subspecialty Certifying Examination.

OBJECTIVES: The Transfusion Medicine In-Service Examination (TMISE) is offered twice a year to transfusion medicine (TM) fellows. We examined the relationship between TMISE scores and outcomes of the American Board of Pathology (ABP) TM subspecialty certifying examination (TM boards). METHODS: TM fellowship programs were contacted to provide anonymous data about TM fellows, their scores on TMISE, and outcome of TM boards. RESULTS: Of 48 TM fellowship programs contacted, 24 (50%) responded with data for 170 fellows. Average TMISE score of fellows who passed their first TM boards attempt was 71.3, while the average TMISE score of fellows who failed their first TM boards attempt was 64.3 (P = .009). CONCLUSIONS: TMISE scores correlated with passing TM boards on the first attempt. Fellows who took the TM boards the same year that they graduated from TM fellowship had a significantly higher first-time pass rate than fellows who delayed taking TM boards.

Dilution is not the solution: acute hemolytic transfusion reaction after ABO-incompatible pooled platelet transfusion.

CONCLUSIONS: The short shelf life of platelets makes providing ABO-compatible platelets a challenge, and many institutions issue ABO-incompatible platelets when compatible units are not available. It is presumed that ABO antibodies that exist in donor plasma are diluted when platelets from multiple donors are combined to make a pooled product for transfusion. We present a case of a hemolytic transfusion reaction in a 73-year-old man with myelodysplastic syndrome who received an ABO-incompatible pooled platelet unit. This case report demonstrates that the dilution theory is not always true for pooled platelet units, and any patient receiving ABO-incompatible platelet transfusions must be closely monitored for potential hemolytic transfusion reactions.

Statistical Literacy Among Academic Pathologists: A Survey Study to Gauge Knowledge of Frequently Used Statistical Tests Among Trainees and Faculty.

CONTEXT: -Statistical literacy can be defined as understanding the statistical tests and terminology needed for the design, analysis, and conclusions of original research or laboratory testing. Little is known about the statistical literacy of clinical or anatomic pathologists. OBJECTIVE: -To determine the statistical methods most commonly used in pathology studies from the literature and to assess familiarity and knowledge level of these statistical tests by pathology residents and practicing pathologists. DESIGN: -The most frequently used statistical methods were determined by a review of 1100 research articles published in 11 pathology journals during 2015. Familiarity with statistical methods was determined by a survey of pathology trainees and practicing pathologists at 9 academic institutions in which pathologists were asked to rate their knowledge of the methods identified by the focused review of the literature. RESULTS: -We identified 18 statistical tests that appear frequently in published pathology studies. On average, pathologists reported a knowledge level between "no knowledge" and "basic knowledge" of most statistical tests. Knowledge of tests was higher for more frequently used tests. Greater statistical knowledge was associated with a focus on clinical pathology versus anatomic pathology, having had a statistics course, having an advanced degree other than an MD degree, and publishing research. Statistical knowledge was not associated with length of pathology practice. CONCLUSIONS: -An audit of pathology literature reveals that knowledge of about 12 statistical tests would be sufficient to provide statistical literacy for pathologists. On average, most pathologists report they can interpret commonly used tests but are unable to perform them. Most pathologists indicated that they would benefit from additional statistical training.

Does therapeutic plasma exchange have a role in the treatment of prosthetic hip-associated cobalt toxicity? A case report and literature review.

BACKGROUND: Prosthetic hip-associated cobalt toxicity (PHACT) is an uncommon, but potentially devastating, complication for patients with metal-on-metal hip implants (MoMs). Clinical management of PHACT is poorly defined, with primary intervention being MoM explant followed by chelation therapy. Therapeutic plasma exchange (TPE) in cobalt toxicity has not been previously described. Given that cobalt is predominantly albumin bound, it should theoretically be removed by TPE. Here we report a case of PHACT and our experience using TPE to lower plasma cobalt levels. CASE REPORT: A 61-year-old woman developed deafness, blindness, ambulatory dysfunction, and endocrinopathies after MoM implant. Cobalt levels on admission were greater than 1500 µg/L. In an attempt to rapidly lower cobalt levels before MoM explant, hemodialysis and TPE were performed. Hemodialysis removed negligible amounts of cobalt. One session of TPE temporarily removed approximately two-thirds of measurable cobalt, but levels rebounded to pre-TPE values after 8 hours. It was only after MoM removal that cobalt levels plateaued below 300 µg/L and clinical symptoms improved. DISCUSSION: TPE removed cobalt from a PHACT patient, but a durable decrease in cobalt was only achieved after MoM explant. These findings are comparable to reports where chelation was employed in PHACT patients before MoM explant. The observed rebound phenomenon is likely from rapid equilibration between the immense extravascular tissue source (the MoM) and the intravascular compartment. CONCLUSION: TPE may serve as adjunctive therapy for PHACT patients whose cobalt levels remain high after explant, especially in patients with renal failure, in whom chelation is contraindicated.

Transfusion-related acute lung injury in an era of TRALI risk mitigation.

Transfusion-related acute lung injury (TRALI) is a rare complication of transfusion, for which the true incidence remains obscure, since there are a number of factors that may lead to misdiagnosis. Despite this, it continues to be the leading cause of transfusion-associated mortality. Here we present a historical case of TRALI in an elderly female who received group AB plasma and discuss how current mitigation strategies would likely have prevented its occurrence. It is important to remember that both immune and non-immune factors play a role in TRALI pathogenesis, and although current preventative strategies may decrease TRALI's incidence, they likely will not eliminate it.

Conversion to low transfusion-related acute lung injury (TRALI)-risk plasma significantly reduces TRALI.

BACKGROUND: Transfusion-related acute lung injury (TRALI) is an uncommon but serious transfusion reaction. Studies have shown that the transfusion of HLA and HNA antibodies in donor plasma can lead to TRALI. Female donors are more likely to have such antibodies due to alloantigen exposure during pregnancy. Many blood suppliers have now implemented various TRALI risk reduction strategies to unknown effect. A retrospective analysis of TRALI reactions in plasma recipients before and after the conversion to low-TRALI-risk plasma (all-male donor plasma, male-predominant plasma, nulliparous female plasma, and HLA antibody-tested plasma) is reported. STUDY DESIGN AND METHODS: Transfusion reaction reports at three large hospitals 16 months before and 16 months after the conversion to low-TRALI-risk plasma were analyzed. Respiratory reactions were categorized as TRALI, possible TRALI, or other (e.g., transfusion-associated circulatory overload or allergic reactions). Reactions were reported as a percentage of total units transfused and rates for the two time periods were compared. Trends in reaction rates for other components were also compared. RESULTS: A total of 2156 transfusion reactions in association with 461,598 transfused blood components were reviewed. The incidence of combined TRALI or possible TRALI reactions, due to the transfusion of plasma, decreased from 0.0084% to zero (p = 0.052). The rate of TRALI or possible TRALI reactions in red blood cell and platelet recipients did not change significantly. CONCLUSION: The conversion to low-TRALI-risk plasma has reduced the incidence of TRALI reactions in plasma recipients.

Transfusion medicine in American undergraduate medical education.

BACKGROUND: Blood transfusion is the most common procedure performed in American hospitals, and transfusions are commonly ordered by physicians without formal training in transfusion medicine. Several transfusion medicine curricula have been proposed, including those developed through the Transfusion Medicine Academic Awards (TMAA). To our knowledge, no comprehensive study has assessed how transfusion medicine is incorporated into undergraduate medical education. STUDY DESIGN AND METHODS: We conducted an online survey to determine the manner in which transfusion medicine is incorporated into American undergraduate medical education. The survey was e-mailed to administrators of medical education at all of the 129 American medical schools accredited by the Association of American Medical Colleges. RESULTS: Eighty-six (67%) of the 129 identified medical school administrators responded. Seventy-one (83%) of the 86 administrators reported that their undergraduate medical education curriculum provides didactic lectures in transfusion medicine, with 48% of medical schools providing 1 or 2 hours of lecture-based instruction. A minority reported small group sessions devoted to transfusion medicine topics. While a slim majority reported the availability of transfusion medicine electives, only one of 84 administrators reported that such a rotation is required. Seventy-six of 83 (92%) administrators were unfamiliar with either the 1989 or the 1995 TMAA transfusion medicine curricula. CONCLUSION: Transfusion medicine content in American undergraduate medical education is variable and the influence of the TMAA program on contemporary medical school curricula is questionable. Future efforts in this area should focus on standardizing and improving undergraduate medical education in transfusion medicine.

Autoimmune hepatitis in patients with primary Sjögren's syndrome: a series of two-hundred and two patients.

Based on the revised criteria of the American-European Consensus Group, we retrospectively established the diagnosis of primary or secondary Sjögren's syndrome for 202 patients referred to a Sjögren's syndrome clinic. Of these, 58 patients and 8 patients fulfilled criteria for primary and secondary Sjögren's syndrome, respectively. Of the 58 patients with primary Sjögren's syndrome, one (1.7%) had definite autoimmune hepatitis, as defined by the International Autoimmune Hepatitis Group diagnostic criteria. One additional symptomatic patient who did not fulfill criteria for primary Sjögren's syndrome had definite autoimmune hepatitis. None of the patients with secondary Sjögren's syndrome had definite autoimmune hepatitis. Two (1%) of the 194 patients with primary Sjögren's syndrome or clinical symptoms had primary biliary cirrhosis. These values are lower than those reported by prior studies with smaller patient populations and likely represent a more accurate estimate of the true prevalence of these diseases in patients with primary Sjögren's syndrome.

International variation in volunteer whole blood donor eligibility criteria.

BACKGROUND: This study assesses international variation in volunteer whole blood donor eligibility criteria. STUDY DESIGN AND METHODS: In February 2008, Web sites of major blood collection organizations in 17 countries were queried for the volume of whole blood donations and six donor eligibility criteria: allowed donation frequency, donor age, hemoglobin (Hb), weight, and deferrals for tattoo and pregnancy/breast-feeding. RESULTS: The allowed frequency of whole blood donation ranged from 56 to 120 days, some with sex- and age-specific limitations. While blood collection agencies in three countries did not have an upper age limit for donation, the remainder mandated donor retirement at ages from 60 to 81 years. The minimum Hb level was 11.5 to 12.5 g/dL for women and 12.5 to 13.5 g/dL for men. Blood collection organizations in only three countries required a minimum donor weight of less than 50 kg. Tattoo and pregnancy deferrals ranged from 4 to 12 months and 6 weeks to 12 months, respectively. The volume of whole blood donations ranged from 300 to 500 mL. The percentage of total blood volume donated, the absolute grams of Hb expected to be restored per deferral period and per day of donor deferral, and the concentration of Hb expected to be restored per deferral period were calculated. CONCLUSIONS: International volunteer whole blood donor eligibility criteria demonstrate marked variation. These differences likely cause international variation in the prevalence of adverse donor reactions and iron deficiency anemia. The reasons underlying these dissimilarities are unclear, but may include varying cultural influences and average donor body habitus.