Progression of hemolysis in a patient with hereditary spherocytosis after the second dose of COVID-19 mRNA vaccine.

Herein, we report the case of a 22-year-old woman with hereditary spherocytosis (HS) whose condition worsened after administration of the coronavirus disease 2019 (COVID-19), mRNA vaccine 'BNT162b2 Pfizer-BioNTech.' The woman had been diagnosed with HS in 2005, and her condition remained stable until February 2021. In March 2021, she received the first dose of the above vaccine and experienced pain at the injection site. After the second dose in April 2021, she developed fever and general malaise. Investigations revealed progression of hemolysis, which improved after a few days. To the best of our knowledge, this is the first report of progression of hemolysis in a patient with HS after administration of the mRNA vaccine COVID-19, BNT162b2 'Pfizer-BioNTech.'

Suspected Immune Thrombocytopenic Purpura Induced by Lenalidomide for the Treatment of Myelodysplastic Syndrome with Deletion of Chromosome 5q: A Case Report.

Lenalidomide (LEN), one of the key drugs in the treatment of myelodysplastic syndromes (MDS) with 5q deletion, as well as multiple myeloma (MM), has various immunomodulatory effects and has been associated with autoimmune diseases, including immune thrombocytopenic purpura (ITP). A 78-year-old man presented with pancytopenia and was diagnosed with MDS with 5q deletion and other chromosomal abnormalities. Two cycles of LEN therapy (one cycle: 10 mg/day for 21 days) resulted in a transient improvement in anemia, followed by MDS progression with severe thrombocytopenia (4 × 109/L) refractory to platelet transfusions. As other non-immune and alloimmune causes of transfusion-refractory thrombocytopenia were excluded, and the level of platelet-associated immunoglobulin G was extremely high compared with the level before treatment with LEN, the diagnosis of ITP was highly suspected. Despite treatment with prednisolone (PSL), eltrombopag, and repeated platelet transfusions, his platelet count did not increase, and he died of a gastrointestinal hemorrhage. Several cases of ITP induced by LEN used to treat MM had been reported, but the platelet count recovered after administration of PSL in these previous cases. However, we should be mindful of using LEN for patients with MDS because its treatment may become extremely difficult if ITP develops.

Feasibility and effectiveness of cardiac auscultation training with a cardiology patient simulator for first-year medical students.

BACKGROUND: The effectiveness of cardiac auscultation training with a cardiology patient simulator for medical students is still unclear. Starting such training earlier may help students improve their proficiency. We investigated whether cardiac auscultation training using a simulator for first-year students is feasible and effective. METHODS: A total of 43 first-year medical students (5-12 in each year, 2015-2019) participated in three 1.5-hour extra-curricular classes comprising mini-lectures, facilitated training, two different auscultation tests (the second test closer to clinical setting than the first), and a questionnaire. The test results were compared with those of 556 fourth-year medical students who participated in a compulsory 3-hour cardiac auscultation class in 2016-2019. RESULTS: The accuracy rate of all heart sounds and murmurs was higher in the first-year students than in the fourth-year students in both the first (85.8 vs. 79.4 %, p = 0.001) and second (71.3 vs. 61.2 %, p = 0.02) tests. That of second/third/fourth sounds was also higher in the first-year students than in the fourth-year students in both the first (86.0 vs. 79.7 %, p = 0.01) and second (70.9 vs. 53.9 %, p = 0.002) tests. The accuracy rate of murmurs was higher in the first-year students than in the fourth-year students in the first test (85.5 vs. 78.9 %, p = 0.04), but not in the second test (72.1 vs. 75.7 %, p = 0.58). All the first-year students and 65 % of them agreed that they had received sufficient knowledge and built sufficient skills, respectively. All the first-year students and 93 % of them agreed that they were satisfied with the program, and that the program was suitable for first-year students, respectively. CONCLUSIONS: Although training time was different between the two groups and it is possible that only motivated first-year students participated in the program, these results suggest that our cardiac auscultation training is feasible and effective for first-year medical students.

Clinical efficacy of belimumab in patients with systemic lupus erythematosus and headache. A report of two cases.

Intractable headache, one of the manifestations of neuropsychiatric systemic lupus erythematosus (SLE), is difficult to diagnose and decide on an appropriate treatment. In addition to conventional therapy based on the type of headache, the treatment should be conducted considering the disease activity of SLE rather than the headache. We report two patients with intractable headache who were successfully treated using belimumab therapy. The headaches in both patients were relieved after 2 weeks of belimumab administration. The neutralisation of B lymphocyte stimulator and reduced production of cytokines from B lymphocytes might contribute to the early effects. The potential benefits of using belimumab as an additional immunosuppressant for treating intractable headache complicated with SLE have been discussed.

Decrease of infectious complications in outpatients with autoimmune diseases from 2019 to 2020 under the COVID-19 pandemic: A single-centre, retrospective cohort study in Japan.

OBJECTIVES: To examine how the novel coronavirus disease (COVID-19) has changed infectious complications in outpatients with autoimmune diseases. METHODS: We performed a retrospective, record-linked cohort study and questionnaire about lifestyle changes in patients who visited our department in 2019 and 2020. RESULTS: We surveyed 1316 outpatients in 2019 and 1284 in 2020. The most common underlying diseases were rheumatoid arthritis (842 vs. 814) and systemic lupus erythematosus (SLE) (126 vs. 127). No significant difference in median age (66 vs. 67 years), respiratory comorbidities (30.4% vs. 32.0%), or corticosteroid use (42.2% vs. 44.3%) was found between the years. Immunomodulating agents were used more in 2020 (33.1% vs. 39.7%, p < .001). Total number of infections (28.0/100 vs. 19.4/100 person-years), pneumonia (3.6 vs. 1.6), influenza (2.1 vs. 0.1), and nonviral dermatological infections (3.8 vs. 2.1) were significantly lower in 2020. No significant difference was found for herpes zoster (2.2 vs. 1.8), urinary tract infections (3.3 vs. 3.8), or gastrointestinal infections (2.9 vs. 3.0). According to the questionnaire, 75% of the respondents became more conscious about wearing masks and 81% began to use hand sanitizer during the pandemic. CONCLUSION: Under the COVID-19 pandemic, some infectious complications have decreased in outpatients with autoimmune diseases.

Employment of color Doppler echocardiographic video clips in a cardiac auscultation class with a cardiology patient simulator: discrepancy between students' satisfaction and learning.

BACKGROUND: We have provided fourth-year medical students with a three-hour cardiac auscultation class using a cardiology patient simulator since 2010. The test results of 2010-2012 revealed that as compared with aortic stenosis murmur, students correctly identified murmurs of other valvular diseases less often. We investigated whether employment of color Doppler echocardiographic video clips would improve proficiency in identifying murmurs of aortic regurgitation and mitral regurgitation, and whether students' favorable responses to a questionnaire were associated with improved proficiency. METHODS: A total of 250 fourth-year medical students were divided into groups of 7-9 students in 2014 and 2015. Each group attended a three-hour cardiac auscultation class comprising a mini-lecture, facilitated training, two different auscultation tests (the second test being closer to clinical setting than the first) and a questionnaire. We provided each student with color Doppler echocardiographic videos of aortic regurgitation and mitral regurgitation using a tablet computer, which they freely referred to before and after listening to corresponding murmurs. The test results were compared with those in 2010-2012. The students had already completed the course of cardiovascular medicine, comprising lectures including those of physical examination, echocardiography, and valvular heart diseases, before participating in this auscultation training class. RESULTS: Most students indicated that the videos were useful or somewhat useful regarding aortic regurgitation (86.3%) and mitral regurgitation (85.7%). The accuracy rates were 78.4% (81.2% in 2010-2012) in aortic regurgitation and 76.0% (77.8%) in mitral regurgitation in the first test, and 83.3% (71.4%) in aortic regurgitation and 77.1% (77.6%) in mitral regurgitation in the second test, showing no significant differences as compared to 2010-2012. Overall accuracy rate of all heart sounds and murmurs in the first test and that of second/third/fourth sounds in the first and second tests were significantly lower in 2014-2015 than in 2010-2012. CONCLUSIONS: Referring to color Doppler echocardiographic video clips in the way employed in the present study, which most students regarded as useful, did not improve their proficiency in identifying the two important regurgitant murmurs, revealing a discrepancy between students' satisfaction and learning. Video clips synchronized with their corresponding murmurs may contribute toward improving students' proficiency.

Suggestive Diagnostic Process in a Case of Multiple Myeloma with Gastrointestinal Immunoglobulin Light-Chain Amyloidosis Accompanied by Protein-Losing Enteropathy.

Multiple myeloma is a type of plasma cell neoplasm that produces monoclonal immunoglobulin. Multiple myeloma is known to cause immunoglobulin light-chain (AL) amyloidosis, which frequently involves the kidney and heart. Bone pain or fractures caused by osteolytic lesions and physical disorders related to renal or cardiac AL amyloidosis are major initial symptoms in multiple myeloma. Multiple myeloma diagnosed from the gastrointestinal symptoms is rare. We report a case of an 80-year-old man with multiple myeloma accompanied by gastrointestinal AL amyloidosis and secondary protein-losing enteropathy. The diagnostic process was suggestive, in that diarrhea and refractory leg edema related to protein-losing enteropathy were the primary symptoms and the trigger for making a sequential diagnosis of gastrointestinal AL amyloidosis and underlying multiple myeloma. This case is highly suggestive, in that multiple myeloma with gastrointestinal AL amyloidosis should be considered one of the background diseases of protein-losing enteropathy.

Salvage Cord Blood Transplantation for Sustained Remission of Acute Megakaryoblastic Leukemia That Relapsed Early after Myeloablative Transplantation.

Acute megakaryoblastic leukemia (AMKL) is a rare subtype of acute myeloid leukemia accompanied by an aggressive clinical course and dismal prognosis. We herein report a case of AMKL preceded by mediastinal germ cell tumor that relapsed early after allogeneic hematopoietic stem cell transplantation with myeloablative conditioning but was successfully treated using salvage cord blood transplantation (CBT) with reduced-intensity conditioning. Although several serious complications developed, sustained remission with a favorable general condition was ultimately achieved. Although an optimal therapeutic strategy remains to be established, the graft-versus-leukemia effect of CBT may be promising, even for the treatment of refractory AMKL.

Two cases of rheumatoid arthritis complicated by organising pneumonia successfully treated with tofacitinib therapy.

Organising pneumonia (OP) complicated by rheumatoid arthritis (RA), a rare type of interstitial lung disease, is sometimes refractory and resistant to immunosuppressive therapy. We report for the first time two cases of refractory OP with RA for which tofacitinib, an inhibitor of Janus kinase, was highly effective. Two women, aged 84 and 65 years, developed refractory OP during treatment for RA with biologics, certolizumab pegol, and etanercept. A moderate amount of prednisolone was effective in both cases; however, recurrences were observed with reduced glucocorticoid dosage. When tofacitinib was administered, OP and RA were well controlled. Thus, the glucocorticoid dosage was successfully tapered low enough until no side effects were observed. Tofacitinib therapy may be a treatment option for refractory OP.

Spontaneous Regression of Blastic Plasmacytoid Dendritic Cell Neoplasm Following Sepsis by Serratia marcescens: A Case Report and Literature Review.

Spontaneous regression is rare in patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN). An 85-year-old man presented with pancytopenia and skin lesions, and the bone marrow exhibited 79.6% CD4+, CD56+, CD123+, and TCL-1+ abnormal cells, with a normal karyotype; he was thus diagnosed with BPDCN. While being followed without chemotherapy, he was admitted due to sepsis induced by Serratia marcescens, which was successfully treated with antibiotics. Notably, his blood cell counts improved, and the skin lesions disappeared. To our knowledge, this is the first reported case of spontaneous regression of BPDCN with a decrease in tumor cells in the bone marrow following sepsis.

A Medical Record Peer-Review System to Evaluate Residents' Clinical Competence: Criterion Validity Analysis.

In contrast to input evaluation (education delivered at school) and output evaluation (students' capability at graduation), the methods of outcome evaluation (performance after graduation) of medical education have not been sufficiently established. To establish a method to measure the quality of patient care and conduct outcome evaluation, we have been developing a peer review system of medical records. Here, we undertook a pilot study to evaluate the criterion validity of our system by using "evaluation by program directors (supervisors in the hospitals)" as a criterion standard. We selected 13 senior residents from three teaching hospitals. Five reviewers (general internists working in other hospitals) visited the hospitals independently and evaluated five patients' records for each resident based on the previously established sheet comprising 15 items. Independently, program directors of the senior residents evaluated their clinical performance using an evaluation sheet comprising ten items. Pearson's analysis revealed statistically significant correlation coefficients in three pairs of assessments including clinical reasoning (r = 0.5848, P = 0.0358). Bootstrap analysis revealed statistically significant correlation coefficients in additional 5 pairs including history taking (r = 0.509, 95% confidence interval: 0.034-0.847). In contrast, the correlation coefficients were low in some items: r = 0.132 (-0.393-0.639) for physical examination and r = 0.089 (-0.847-0.472) for attitude toward patients. To the best of our knowledge, this is the first study, albeit a pilot one, that investigates the criterion validity of medical record evaluations conducted by comparing the assessments of medical records with those by program directors.

A case of myelodysplastic syndrome with t(10;18)(q26;q21).

An 82-year-old male was admitted. Pancytopenia, a slightly low white blood cell count (3400/µL), and low levels of red blood cells (2.65 × 106/µL), hemoglobin (10.4 g/dL), and platelets (118,000/µL) were observed. Bone marrow aspiration was performed, revealing hypocellular bone marrow and normal blast levels (0.6%) with no dysplasia. G-banding chromosome analysis revealed the karyotype 45,X,-Y[3]/45, idem, t(10;18)(q26;q21)[13]/46,XY[4]. The patient was diagnosed with myelodysplastic syndrome, unclassified (MDS-U). This is the first case report demonstrating a patient with the chromosomal translocation, t(14;18)(q32;q21), which is extremely rare. This chromosomal aberration was critical for the diagnosis of MDS in this patient.

A case study of t(14;22)(q32;q11) involving immunoglobulin heavy and light chain in follicular lymphoma.

Chromosome 14 is the most frequently rearranged chromosome in non-Hodgkin lymphoma (NHL), with aberrations particularly involving the heavy-chain immunoglobulin gene (IGH) in the chromosome band 14q32. Several translocation partners have been described: t(14;18)(q32;21)/IGH-BCL2 in follicular lymphoma (FL), t(11;14)(q13;q32)/CCND1-IGH in mantle cell lymphoma, and t(8;14)(q24;q32)/MYC-IGH in Burkitt lymphoma. The chromosomal locus 22q11 contains two important genes associated with leukemia and lymphoma; one is BCR, which fuses with ABL from 9q34 in chronic myeloid leukemia, and the other is the immunoglobulin lambda gene (IGL), which is rarely involved in the translocations observed in B-cell NHL. The t(14;22)(q32;q11) translocation has been previously reported in 8 cases of B-cell NHL; however, the translocation between IGH and IGL has been experimentally confirmed using fluorescence in situ hybridization (FISH) for only 4 cases. Here, we describe the first case of FL with a t(14;22)(q32;q11)/IGH-IGL translocation confirmed using FISH analysis. The patient in our case report was immunocompromised and was treated for aplastic anemia with cyclosporine A (CsA). The patient was diagnosed with follicular lymphoma, most likely caused by CsA.

Variation in effectiveness of a cardiac auscultation training class with a cardiology patient simulator among heart sounds and murmurs.

BACKGROUND: Effectiveness of simulation-based education in cardiac auscultation training is controversial, and may vary among a variety of heart sounds and murmurs. We investigated whether a single auscultation training class using a cardiology patient simulator for medical students provides competence required for clinical clerkship, and whether students' proficiency after the training differs among heart sounds and murmurs. METHODS: A total of 324 fourth-year medical students (93-117/year for 3 years) were divided into groups of 6-8 students; each group participated in a three-hour training session using a cardiology patient simulator. After a mini-lecture and facilitated training, each student took two different tests. In the first test, they tried to identify three sounds of Category A (non-split, respiratory split, and abnormally wide split S2s) in random order, after being informed that they were from Category A. They then did the same with sounds of Category B (S3, S4, and S3+S4) and Category C (four heart murmurs). In the second test, they tried to identify only one from each of the three categories in random order without any category information. RESULTS: The overall accuracy rate declined from 80.4% in the first test to 62.0% in the second test (p<0.0001). The accuracy rate of all the heart murmurs was similar in the first (81.3%) and second tests (77.5%). That of all the heart sounds (S2/S3/S4) decreased from 79.9% to 54.3% in the second test (p<0.0001). The individual accuracy rate decreased in the second test as compared with the first test in all three S2s, S3, and S3+S4 (p<0.0001). CONCLUSIONS: Medical students may be less likely to correctly identify S2/S3/S4 as compared with heart murmurs in a situation close to clinical setting even immediately after training. We may have to consider such a characteristic of students when we provide them with cardiac auscultation training.

Causes of macrocytic anemia among 628 patients: mean corpuscular volumes of 114 and 130 fL as critical markers for categorization.

There have been no studies on the distribution of causes of macrocytic anemia with respect to mean corpuscular volume (MCV) cutoff values. We retrospectively investigated the causes of macrocytic anemia (MCV ≥100 fL) among 628 patients who visited the outpatient hematology clinic in Tohoku University Hospital. To ensure data validity, we also analyzed data from 307 patients in eight other hospitals in the Tohoku district. The leading causes of macrocytic anemia (number of patients, %) were myelodysplastic syndromes (121, 19.3 %), suspected bone marrow failure syndromes (BMF; 74, 11.8 %), aplastic anemia (51, 8.1 %), plasma cell dyscrasia (45, 7.2 %), and vitamin B12 deficiency (40, 6.4 %) in Tohoku University Hospital. We made three primary findings as follows. First, the most common cause of macrocytic anemia is BMF. Second, lymphoid and solid malignancies are also common causes of macrocytosis. Third, macrocytic anemia may be classified into three groups: Group 1 (megaloblastic anemia and medications), which can exceed MCV 130 fL; Group 2 (alcoholism/liver disease, BMF, myeloid malignancy, and hemolytic anemia), which can exceed MCV 114 fL; and Group 3 (lymphoid malignancy, chronic renal failure, hypothyroidism, and solid tumors), which does not exceed MCV 114 fL. These conclusions were supported by the results from eight other hospitals.

Number of papers published in English from the nursing departments of 42 national universities in Japan in the past ten years.

BACKGROUND: In Japan, the departments of nursing were established by 2004, and graduate school programs for master's degree were established by 2008, in 42 national universities. With these changes, a more academic mission has been pursued, and the need for writing papers in English has increased. AIM: To investigate the numbers of papers published in English from the nursing departments of national universities in Japan over the past ten years. METHODS: The lists of teachers who have nursing licenses in the departments of nursing in the 42 national universities (n=2292) were obtained from the Japan Association of Nursing Programs in Universities. The number of papers published in English by these teachers from 2004 to 2013 was counted using the SCOPUS database. RESULTS: The average number of total papers, in which at least one of the authors was a nursing teacher, and first-authored papers, in which the first author was a nursing teacher, were 211.4 and 69.9 per year, respectively; both increased approximately two-fold during the past ten years. The means and standard deviations of the number of total papers and first-authored papers were 50.3±63.8 (range: 1-382) and 18.3±23.4 (range: 0-147) according to universities, and 1.39±5.84 (range: 0-140) and 0.33±1.28 (range: 0-21) according to teachers, respectively. When journals with the highest number of papers were analyzed, 12 of the top 20 (total papers) and 12 of the top 16 (first-authored papers) were in journals whose editorial offices are in Japan. CONCLUSION: The number of papers published in English has increased over the past ten years, varied markedly depending on the universities and teachers, and many papers were published in Japanese journals. To our knowledge, this is the first report anywhere to determine the average number of nursing papers "per teacher" in a specific population.

Development of a peer review system using patient records for outcome evaluation of medical education: reliability analysis.

In addition to input evaluation (education delivered at school) and output evaluation (students' capability at graduation), the methods for outcome evaluation (performance after graduation) of medical education need to be established. One approach is a review of medical records, which, however, has been met with difficulties because of poor inter-rater reliability. Here, we attempted to develop a peer review system of medical records with high inter-rater reliability. We randomly selected 112 patients (and finally selected 110 after removing two ineligible patients) who visited (and were hospitalized in) one of the four general hospitals in the Tohoku region of Japan between 2008 and 2012. Four reviewers, who were well-trained general internists from outside the Tohoku region, visited the hospitals independently and evaluated outpatient medical records based on an evaluation sheet that consisted of 14 items (3-point scale) for record keeping and 15 items (5-point scale) for quality of care. The mean total score was 84.1 ± 7.7. Cronbach's alpha for these items was 0.798. Single measure and average measure intraclass correlations for the reviewers were 0.733 (95% confidence interval: 0.720-0.745) and 0.917 (95% confidence interval: 0.912-0.921), respectively. An exploratory factor analysis revealed six factors: history taking, physical examination, clinical reasoning, management and outcome, rhetoric, and patient relationship. In conclusion, we have developed a peer review system of medical records with high inter-rater reliability, which may enable us, with further validity analysis, to measure quality of patient care as an outcome evaluation of medical education in the future.