bluuebunny/crossref_metadata_2025_split huggingface dataset:
TAFRO syndrome: A case report with biopsy findings from three sites
Authors: Jadhav N C , Freeman J 
Published: October 2021

Abstract

Introduction/Objective
TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) is a rare systemic inflammatory disorder. First reported in 2010, the majority of cases are from Japan, where it is currently regarded as a subtype of HHV8-negative Multicentric Castleman Disease.

Methods/Case Report
We report a case of TAFRO occurring in a 17-year old from Puerto Rico. She originally presented with abdominal pain, vomiting, and fever, and was also found to have splenomegaly, elevated sedimentation rate and C-reactive protein. Over the course of the next several days she developed respiratory distress, requiring ventilatory support, and anuric renal failure. Renal biopsy demonstrated evidence of thrombotic microangiopathy, which was interpreted as atypical hemolytic syndrome, requiring dialysis and treated with eculizumab. Because other diagnostic considerations at the time also included hemophagocytic lymphohistiocytosis and catastrophic antiphospholipid syndrome, bone marrow examination was performed which demonstrated increased megakaryocytes, without fibrosis or hemophagocytosis. Over the next two months, she developed anasarca with ascites, pleural effusion, pericardial effusion, multiple enlarged axillary and cervical lymph nodes, and persistence of splenomegaly. Biopsy of a left cervical lymph node demonstrated features suggestive of Castlemans disease, plasma cell variant. The constellation of symptoms, laboratory investigations and biopsy diagnoses support the diagnosis of TAFRO syndrome. The patient received rituximab, high dose steroids, and hemodialysis with clinical improvement. At 16 months follow up, the patient is in remission.

Results (if a Case Study enter NA)
NA

Conclusion
From a pathology standpoint, the case provides biopsy findings of three distinct organ systems all with relatively unusual findings that together are characteristic of TAFRO syndrome. It highlights the risk of accepting “biopsy proven” diagnoses such as atypical hemolytic uremic syndrome that also require significant laboratory and clinical correlation for true confirmation, and points out the importance and opportunity for pathologists to see the forest along with the trees in order to recognize rare diseases such as TAFRO.
Weakened IgG4 Staining and Positive PLA2R staining Indicate Resolving Primary Membranous Glomerulopathy (MGN)
Authors: Al-Othman Y , Li W , Kanaan H , Zhang P 
Published: October 2021

Abstract

Introduction/Objective
IgG4 related disease, a systemic autoimmune inflammatory disorders, can be identified by high% of IgG4 positive plasma cells, thus IgG4 staining in paraffin embedded tissue is widely available in the most of pathology labs. IgG4 staining has been found useful to identify primary MGN (PLA2R and/or THSD7A positive) by others and us. This study was to scrutinize the findings of primary vs secondary MGN needed for IgG4 staining as a screening tool in our renal pathology practice over pasts 5 years

Methods/Case Report
IgG4 staining in paraffin embedded tissue was performed in 45 primary MGN and 43 secondary MGN after the clinical history was reviewed and a possibility of primary MGN cannot be excluded. In addition, both groups of cases were also stained for PLA2R. Detail correlation with clinical history was analyzed.

Results (if a Case Study enter NA)
Totally 82 % (37/45) of primary MGN was found diffuse positive for IgG4 staining at 2+ intensity in the glomeruli. Seven out of eight remaining primary MGN cases with either negative or weak IgG4 stained MGN were found to have diffuse resolving features by electron microscopy but there was still positive PLA2R staining in the glomeruli. All secondary MGN were stained negatively for both IgG4 and PLA2R and we found that etiologies of the secondary MGN included membranous lupus nephritis, infection, GVHD, or variants of cancers.

Conclusion
Our data indicate that IgG4 staining along (without IgG1-3 staining) is a reliable screening tool to confirm the majority of primary MGN vs secondary MGN in paraffin embedded tissue. As both PLA2R+ and THSD7A+ primary MGN are both IgG4 related, the IgG4 staining may potentially represent a wider range of scope in identifying primary MGN. In addition, negative/weak IgG4 staining in PLA2R-positive MGN most likely represents a primary MGN with resolving features.
Red Blood Cell Alloimmunization in Sickle Cell Disease: Advantage of Ethnic Heritage between Donors and Patients in Jeddah, Saudi Arabia
Authors: Anwar F , Almohammadi M , Garni A , Jamallail S , Alsamkari W , Alsafi A 
Published: October 2021

Abstract

Introduction/Objective
Red blood cell (RBC) transfusion is frequently required for patients with sickle cell disease (SCD). Development of alloantibodies in these patients complicates the blood bank process needed to identify these antibodies and to find compatible RBC units. The rate of alloimmunization has been reported as high as 47% in one study and 34.2% in another study from Eastern region of Saudi Arabia. The purpose of this study was to determine incidence and rate of RBC alloimmunization in the Saudi population in the Western region in SCD.

Methods/Case Report
A retrospective analysis of the immunohematological and transfusion history of a total of 161 SCD patients was reviewed, of which there 95 males and 66 females. All patients had erythrocytapheresis, ranging from one to 24 full red cell exchange sessions. A total of 490 red cell exchanges were performed and 4,914 units of blood were used. Extended compatibility to RhCcEe and K antigen was performed. Patient who developed alloimmunization to any of RhCcEe and K antigen were matched for Kidd, Duffy and MSN antigens for subsequent RBC requirements.

Results (if a Case Study enter NA)
The RBC alloimmunization incidence was 18% with a rate of 0.6 antibodies per 100 RBC transfusions. Alloimmunization in females was significantly higher than in the patients. Eighteen (11.2%) female patients demonstrated antibodies as compared to eleven (6.8%) male patients. Twelve patients (7.4%) had a history of at least one alloantibody and 17 (10.6%) had more than one. Antibodies found were directed against E (7.4%), K (5.6%), and D, C, c, S, M, Lea, Jk a, Chido/Rodgers, Fy a. Seven (4.3%) patients also had warm autoantibodies.

Conclusion
RBC alloimmunization incidence and rate in our study was lower to those reported in less heterogeneous population of donors and patients. Nonetheless, RBC alloimmunization still occurs in patients with SCD, often due to Rh variants or lack of consistency in the application of prophylactic antigen matching between institutions. Therefore, we believe that this rate can still be further reduced if all centers in the region establish transfusion programs to include at least RhCcEe and K phenotypic compatibility and communication mechanisms between major treating centers and transfusion centers in smaller cities to minimize the risks of exposing the patient to different RhCcEe and K phenotype and of developing RBC alloimmunization.
The Immunohematologic and Clinical Evaluation of Increased Risk for Red Blood Cell Alloimmunization among Multi-Transfused Pediatric Patients in a Tertiary Medical Institution in Central Luzon, Philippines
Authors: Ebreo C C , Castillo R M , Dizon M D , Domasian A D , Floresta G P , Gallestre M C , Hernaez A M , Kasilag K V , Sison R G , Nieto X A 
Published: October 2021

Abstract

Introduction/Objective
Multiple blood transfusion is a therapeutic treatment for patients with hematologic disorders. However, patients who undergo repeated blood transfusions are at higher risk of developing red blood cell alloimmunization. This may further delay the availability of compatible blood and pose a higher risk for hemolytic transfusion reactions.

Methods/Case Report
A total of 55 patients, with an age range of 2-19 years, were included in the study. All patients had hematologic disorders and underwent chronic blood transfusion. In this retrospective study, Student’s t-test and Fisher’s exact tests were used to compare the means and percentages, respectively, under 5% level of significance. R ver 4.0.3 was utilized in the analyses.

Results (if a Case Study enter NA)
Out of the 55 pediatric patients, 10 (18.2%; CI95%: 9.1 to 30.9%) were found to be alloimmunized. Two patients had multiple antibodies whereas the others had one specificity, having a total of 12 detected alloantibodies. The most prevalent alloantibody was anti-E (60%), followed by anti-Jka (20%), anti-Mia (20%), anti-S (10%), and anti-K (10%). All obtained alloantibodies showed no correlation (p&gt;0.05) with the age group of the patients. Subsequently, it was not evident that average blood transfusion (p=0.949) and packed red blood cells transfused per year (p=0.782) significantly differ between alloimmunized and non-alloimmunized patients. Similarly, there was no sufficient evidence that the age group (p=0.723), sex (p=1.000), blood group (p=1.000) and hematologic disorder (p=0.949) were associated with their alloimmunization status.

Conclusion
The alloimmunization incidence rate of 18.2% in this study was higher compared to related literatures, and most of the detected alloantibodies were of IgG type which are commonly clinically significant. The study showed no significant correlation between the specific risk factors and the development of alloimmunization. For patient care, it is imperative to include precautions such as extended RBC phenotyping and provision of antigen matched RBCs to prevent unnecessary transfusion reactions caused by alloimmunization.
Crossmatch to Transfusion Ratios in the Operating Room with an Expanding Hospital Footprint
Authors: Virk M S 
Published: October 2021

Abstract

Introduction/Objective
The crossmatch to transfusion (CT) ratio is one of several metrics used to assess the efficiency of blood product utilization. A ratio of 2:1 has been suggested as a hospitalwide limit that indicates appropriate use of red blood cells (RBCs), and the Q Probe Study found that the average CT ratio for hospitals in the United States is 1.85:1. This ratio can vary widely from one patient location to another due to anticipatory or unknown needs within the operating room (OR) compared to laboratory or symptomatic based orders for patients on the floor. Following a recent hospital expansion at Stanford University Hospital that led to the creation of multiple adult OR locations and a move of our blood bank, we monitored the CT ratios of various OR locations and surgical subspecialties to assess the drivers of increased blood product utilization and interventions that could improve these metrics.

Methods/Case Report
This quality improvement project involved the collection of blood product order information through our LIS (SafeTrace) and clinical system (EPIC), creation of an on-demand report to provide constant updates to that data, qualitative interviews with OR staff, and process mapping for multiple subspecialties.

Results (if a Case Study enter NA)
Prior to our hospital expansion there was an appropriate CT ratio of 2.1:1 in our adult ORs. Post-expansion, this ratio has been between 3-5:1, depending on the OR location. This led us to focus our efforts on the OR locations that were furthest from the blood bank and existed prior to the hospital expansion. In discussions with our OR colleagues, we found that the major driver for this increased ratio was a longer travel path for blood products and delays in delivery which led to larger amounts of anticipatory orders. In addition, these concerns caused duplicative efforts in the OR with surgical teams and Anesthesiologists placing pre-operative orders for the same cases. To address this concern, remote blood product dispensing was implemented. To address duplicative ordering workflows, we partnered with Anesthesiology to determine the blood ordering workflow agreements with each surgical subspecialty to ensure appropriate division of roles and responsibilities. Evaluation of CT ratios after these interventions is ongoing, but early data suggests a significant reduction.

Conclusion
Post-intervention phase ongoing and will be updated with additional data & conclusions prior to conference.
Transfusion-Associated Circulatory Overload (Taco) Presentations In Pediatric Patients
Authors: Sallagonda A , Andrzejewski C 
Published: October 2021

Abstract

Introduction/Objective
Limited literature exists regarding Transfusion Associated Circulatory Overload(TACO) in children. Its clinical expressions compared to those in adults remains to be fully explored. We report two TACO cases in children &lt;18 months of age describing their clinical presentations compared to those in older patients.

Methods/Case Report
Case series

Results (if a Case Study enter NA)
Case 1: 1.13 kg 18 day old male neonate (27 weeks premature) with anemia requiring hemotherapy(HT). He received 35 ml aliquot of Red Blood Cells(RBCs) which he tolerated well on postoperative day(POD) 1 after bowel surgery. On POD 2, he was transfused RBCs(18 mls). Within 15 minutes of HT initiation, marked elevations in blood pressure(BP) were noted. Workup for a suspected transfusion reaction(STR) was initiated. Blood Bank studies revealed vital sign value(VSV) changes similarly seen in adults with TACO(Transfusion: 52; 2311, 2012). NT-proBNP levels post HT were markedly elevated (8,000 and 64,000 pg/ml).Case 2: 17 month old(11 kg) female with a three weeks prior history of COVID-19 admitted with fever/dehydration and subsequently diagnosed with multisystem inflammatory syndrome in children (MIS-C). Intravenous immunoglobulin(IVIG) infusion ordered and within 20 minutes of starting IVIG, she developed grunting. STR workup showed post HT BP/temperature elevations/chest X-ray with increased interstitial markings. Of note she had also received 1070 ml of intravenous fluids within 48 hours prior to HT. Elevated NT-proBNP levels pre/post HT were measured(17,121 pg/ml and 19, 824 pg/ml respectively). Symptoms improved with diuretics.

Conclusion
Children experiencing TACO can clinically manifest similarly as in adults with respect to BP elevations and pulmonary changes. Grunting may be an underappreciated manifestation of TACO in pts &lt; 18 months of age. IVIG infusions used in the treatment of patients with MIS-C may present problematic fluid challenges. Recognition of and mitigation strategies for TACO risk factors in such patients may enhance HT safety in this vulnerable patient population.
Comparison of Polybrene DAT and Conventional DAT in Cases of Suspected DAT Negative Autoimmune Hemolytic Anemia (AIHA)
Authors: Sultana S , Balbuena-Marle R , Tormey C , Gollan J , Skeret R 
Published: October 2021

Abstract

Introduction/Objective
A negative Direct Antiglobulin Test (DAT) is seen in approximately 1-5% of patients presenting with signs and symptoms of immune hemolysis. A well-performed ‘standard’ DAT detects ~100-500 molecules of bound IgG per red cell. A more sensitive polybrene DAT may be helpful in order to confirm a diagnosis of AIHA in patients whose samples have tested negative via standard DAT. However, there has been little reported on utility of the polybrene DAT in evaluating such cases with modern DAT reagents. We hypothesized that the polybrene DAT would not contribute substantially to the analysis of Coombs-negative hemolytic anemia (C-NHA), based primarily on our anecdotal observation. As such, we undertook a study to assess results of polybrene DAT in cases evaluated for possible (C-NHA).

Methods/Case Report
Two study sites were used for data analysis (Yale-New Haven Hospital, Site A, and VA Connecticut, Site B) over a five year period (2016-2021). During this time, standard DATs were performed at both study sites by the tube method using polyspecific antiglobulin and, if positive, reflex to anti-IgG and -C3. For cases of suspected C-NHA (which are reported to Blood Bank via a consult mechanism), conventioned DAT- samples are referred to our regional immunohematology laboratory (American Red Cross, Farmington, CT) for manual hexadimethrine bromide (Polybrene) DAT. Polybrene DAT is reported as negative with two sources of polyspecific AHG. Subsets of patients also underwent acid elution studies (Gamma ELU-KITII) as part of a C-NHA algorithm developed during the study period. Results of standard DAT, acId elution, and polybrene DAT were extracted from Blood Bank electronic records at both study sites.

Results (if a Case Study enter NA)
Evaluation for C-NHA was performed in 32 patients/cases over the study period. Amongst these individuals, 96.8% (31/32) underwent polybrene DAT assessment and none (0%; 0/31) demonstrated a positive polybrene DAT result. Notably, acid elution studies were performed in 90.6% (29/32) of traditional DAT negative cases. Of these, 10.3% (3/29) had reactive eluates.

Conclusion
Performance of the polybrene DAT appeared to be of no value in the assessment of suspected C-NHA. Given that nearly 10% of individuals with a negative conventional DAT had a positive acid elution, this testing step appears to be of greater value in potentially identifying an autoantibody in suspected cases of C-NHA.
RhD negative women transfused RhD positive blood: Alloimmunization prophylaxis protocol and experiences
Authors: Alkhateb R , Prajapati V , Daniels J , Greebon L 
Published: October 2021

Abstract

Introduction/Objective
Preventing allomiunization from D-mismatched transfusions, i.e., transfusion RhD positive (RhD+) red blood cells (RBC) to an RhD negative (RhD–) individual, is not well established. We describe our hospital protocol and experiences managing RhD– young women who received emergency release D-mismatched transfusions.

Methods/Case Report
The pathologists evaluate all patients who received D-mismatched blood transfusions. The inclusion criteria for alloimmunization prophylaxis protocol include: RhD– females; &lt;50 years old; No current or historical Anti-D; and received ≥1 unit of RhD+ blood.
The prophylaxis protocol depends on the RBC volume (RBCV) transfused. Patients who receive RBCV &lt;20% of their total blood volume (TBV) are eligible to receive high dose RhIg, calculated based on the volume transfused. Those who received RBCV ≥20% of TBV would be eligible for red cell exchange (RBCX) followed by RhIg.

Results (if a Case Study enter NA)
Since 2016, four eligible patients received RhIg prophylaxis protocol and none met the criteria for RBCX. All the patients started the prophylaxis protocol within 24 hours of transfusion and completed it within 72 hours. One patient developed post treatment hemolysis and significant drop in hemoglobin requiring blood transfusion. Passive anti-D post treatment was confirmed in three patients, and one had passive anti C. Three of the four patients had follow up antibody screens &gt;6 months post treatment that were negative for RhD alloimmunization.

Conclusion
Our therapeutic plan, the first well established protocol, involves identifying eligible patients based on set criteria and protocols. Our experience demonstrates that this protocol is effective in reducing/preventing RhD alloimmunization.
Effect of Severe Acute Respiratory Syndrome Coronavirus 2 on Blood Product Utilization in the Community Hospital Setting
Authors: Messersmith L , Mack M , Cordell J 
Published: October 2021

Abstract

Introduction/Objective
Blood products are a major expense for the laboratory, with an intricate balance between maintaining adequate supply and expiring products. Blood product wastage rates are typically less than 2%. The SARS-CoV-2 (SARS-CoV-2) pandemic has created a multifaceted challenge in blood utilization. Difficulties in predicting product usage, decreased supply, and transportation delays put hospitals at risk of increased blood product destruction. In this study, we analyze the effect of the SARS-CoV-2 pandemic on blood utilization in the community hospital setting, in order to develop improved laboratory management strategies for decreasing unit wastage.

Methods/Case Report
Quality indicators of blood utilization from 2012 to 2021 were retrospectively reviewed. Data was collected for packed red blood cells (pRBCs), platelets, and plasma. Units transfused, expired/destroyed, and total percent discarded were recorded for each month. Average transfusion and destruction rates were calculated for each product. Blood product utilization from 2020 was compared to years 2012-2019 using Pearson correlation, linear regression, and student paired t-tests.

Results (if a Case Study enter NA)
1,042 units were reviewed. For pRBCs, there was a strong correlation between 2020 and increased destruction compared to 2012-2019 (R= 0.8, p=0.005). There was no significant change in pRBC transfusions in 2020 compared to 2018 and 2019 (p= 0.3, p= 0.2). There were fewer pRBC transfusions in 2020 compared to 2016 and 2017 (p= 0.006, p=0.01). For platelets, there was increased destruction in 2020 compared to 2012-2019 (p= 0.01-0.04) and no significant change in usage (p= 0.3-0.5).

Conclusion
During the 2020 SARS-CoV-2 pandemic, there was a significant increase in blood product wastage. There was no significant increase in transfused pRBCs or platelet units, suggesting the increased destruction was not due to an overall increase in transfusions. Blood products are a major cost for laboratory and further investigation of these findings is warranted to mitigate continued wastage.
Massive Transfusion Protocol Over-Activation
Authors: Abdelmonem M , Wasim H , Abdelmonem M 
Published: October 2021

Abstract

Introduction/Objective
Massive blood transfusion protocol (MTP) is revealed in many cases, such as massive hemorrhage after surgeries, trauma settings, and labor and delivery. Patients who require blood transfusion of more than ten units of packed red blood cells in 24 hours or transfusion of more than four units of packed red blood cells (PRBCs) in one hour are the massive blood transfusion protocol candidates.

Methods/Case Report
A retrospective study was performed at a 225-bed level III trauma center in California. The overall massive blood transfusion protocol utilization, blood product emergency release, and blood product waste were recorded twelve months before and after launching an educational and collaboration program between blood banks and clinicians about the difference between massive transfusion protocol and blood emergency release.

Results (if a Case Study enter NA)
MTP utilization for the 12 months (June 2017 to June 2018) was demonstrated as 59 MTP activations: 32 MTPs from the emergency department, 4 MTPs from inpatient floors, 3 MTPs from labor and delivery, and 4 MTPs from operating rooms while the blood product emergency releases were 7 emergency releases. MTP utilization from (June 2018 to June 2019) was demonstrated as 15 MTP activations: 11 MTPs from the emergency department, 2 MTPs from inpatient floors, 1 MTPs from labor and delivery, and 1 MTPs from operating rooms, while the blood product emergency releases were 43 emergency releases. The blood product waste was reduced by 44.6% in 2018.

Conclusion
There was a significant reduction in MTP activation and blood product waste after implementing the educational program for the clinicians. The collaboration between the blood bank and the clinicians and coordinating educational sessions for clinicians about the difference between MTP and emergency release and the negative impact of the MTP over-activation on the blood product waste and the clinical laboratory scientists in the blood bank is vital in MTP utilization.