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Purpose: To clarify the clinical features of patients with Double seronegative (DS) ocular myasthenia gravis (OMG). Methods: Sixty-one patients diagnosed with DS OMG at the Department of Ophthalmology, Hyogo Medical University Hospital over a 5-year period from 2017 were included. Patients were classified into three groups based on the initial examination findings: group P (ptosis alone), group M (ocular motility disorder alone), and group PM (combination of both). We retrospectively reviewed the patients and clarified their clinical features. Results: There were 32 males and 29 females, with a mean age of 49.8 ± 20.9:1–82 years. Twenty-one patients (34.4%) were in group P, 23 (37.7%) in group M, and 17 (27.8%) in group PM. The proportion of males (73.9%) was significantly higher in group M compared with the other two groups. The diagnosis was proven by detection of neuromuscular junction (NMJ) disorder in 73.8%, oral pyridostigmine trial test in 13.1%, and eight patients (13.1%) in group M were diagnosed after surgical treatment. The clinical symptoms were resolved by oral pyridostigmine treatment in 54.1% of cases. Conclusion: About 30% of patients with DS OMG had no obvious NMJ disorder, and an oral pyridostigmine trial test was necessary to diagnose these patients. Although DS OMG is often considered as the mildest form of MG, its prognosis is not optimistic and it requires aggressive therapeutic intervention. Trial registration: Trial registration number: 202104–750, "2016/4/18," retrospectively registered. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to assess the role of liver stiffness measurement (LSM), evaluated using transient elastography (TE), for the diagnosis of sinusoidal obstruction syndrome (SOS)/veno-occlusive disease (VOD), a complication of hematopoietic stem cell transplantation (HSCT). Methods: In this retrospective study, ultrasonography (US) and LSM were performed on 86 adult patients (55 men and 31 women) undergoing HSCT between January 2016 and December 2022. Characteristics and changes in liver stiffness (LS) were compared between patients with and without SOS/VOD. Results: Of the 86 patients, 14 were diagnosed with SOS/VOD. A significant increase in LS (ranging from 12.6 to 55.1 kPa, median 23.8 kPa) compared to pre-HSCT values was observed in all patients who developed SOS/VOD. The area under the receiver operating characteristic curve (AUROC) for the diagnosis of SOS/VOD was 0.9663 (0.933–0.995) for LS ≥ 17.4 kPa after HSCT. Post-transplant LS exceeded 17.4 kPa in all 14 patients in the SOS/VOD group (100%) and in seven patients in the non-SOS/VOD group (9.7%). The sensitivity and specificity were 100% and 90.3%, respectively. AUROC for the diagnosis of SOS/VOD was 0.973 (0.943–1.000) for LS increase ≥ + 12.6 kPa from baseline after HSCT. The change of ≥ + 12.6 kPa from baseline was observed in all 14 patients in the SOS/VOD group (100%) and in four patients in the non-SOS/VOD group (5.6%). The sensitivity and specificity were 100% and 94.4%, respectively. Conclusion: LSM using TE may contribute to establishing the diagnosis of SOS/VOD after HSCT. [ABSTRACT FROM AUTHOR]

Purpose: The number of patients with acute acquired comitant esotropia (AACE) has been increasing in Japan. The purpose of this study was to investigate the changes in the number and characteristics of patients with AACE examined in our institution during a 12-year period. Methods: We retrospectively reviewed the medical records of patients with AACE aged < 30 years who suddenly developed diplopia or esotropia and were examined in Hyogo College of Medicine Hospital from January 2008 to December 2021. We investigated the association of the yearly changes in the number of patients with the age category, refractive error category, AACE type, esotropia type, and use or nonuse of smartphones. Results: The total number of patients with AACE was 171, and this number significantly increased each year (Pearson correlation coefficient, 0.9450; p < 0.0001). Significant increases were found among students in junior high school and beyond, patients with myopia, patients with Bielschowsky type AACE, and patients with basic esotropia (p < 0.0001 for all). We compared two age groups, elementary school students and below versus junior high school students and above, and found that the rate of increase was significantly higher in the junior high school students and above (estimate, 1.951; p < 0.0001), and the non-myopia group and myopia group and found that the rate of increase was significantly higher in the myopia group (estimate, 1.891; p < 0.0001). Excessive use of smartphones was confirmed in 82 of 133 patients, and the rate of the increase in the number of patients with AACE was significantly greater among patients with than without excessive use of smartphones (estimate, 1.098; p = 0.0009). Conclusion: This study confirmed a significant increase in the number of patients with AACE in recent years. The excessive use of smartphones may be associated with the increase in AACE. [ABSTRACT FROM AUTHOR]

The article presents a retrospective study which investigates the clinical features of hematological disorders with increased large granular lymphocytes (LGLs). It offers a description of LGLs, along with its characteristics. A large-scale clinical data of patients with hematological disorders revealed reactive LGLs in a higher number of hematological disorders, suggesting that reactive LGLs may contribute to the prognosis of patients with hematological malignancies.

We developed an ultra-sensitive method of amino acid analysis (AAA) for the absolute quantification of less than 100 ng of proteins, in solution or on polyvinylidene difluoride (PVDF) membranes using an oxygen-free chamber for protein hydrolysis. We used a pre-label method with 6-aminoquinolyl- N-hydroxysuccinimidyl carbamate for fluorescence detection, ion-pair chromatography with a reversed-phase column, and an ultra-high-pressure high-performance liquid chromatography. We optimized both handling- and instrument-dependent factors for accurate quantification and showed that the least amount of proteins to quantify was determined by handling accuracy rather than instrumental limit for quantification which was 0.6 fmol/amino acid. As a new evaluation method for the handling accuracy, we adopted the protein identification by the obtained amino acid compositions by AAA and the Swiss-Prot database search without the restriction of species. As a result, the least amount of starting material for AAA was 16 ng (0.24 pmol) for a solution of bovine serum albumin (BSA), 33 ng (0.50 pmol) for BSA on a PVDF membrane, and 44 ng (0.15 pmol) for thyroglobulin on a PVDF membrane. These results demonstrate that the ultra-sensitive AAA developed in this study is feasible for absolute quantification of biological significant protein. [Figure not available: see fulltext.] [ABSTRACT FROM AUTHOR]

Allogeneic peripheral blood stem cell transplantation (PBSCT) is an indispensable treatment option for hematological malignancy. The optimal collection day after granulocyte colony-stimulating factor (G-CSF) administration should be determined by peripheral blood pre-apheresis CD34 positive (CD34) cell percentage. However, pre-apheresis CD34 cell analysis is not available for most institutions in Japan. Prediction of the optimal collection day based on objective parameters, other than direct CD34 cell count, is thus an important matter for investigation. To identify potential predictive factors, clinical parameters in 79 related donors who received allogeneic peripheral blood stem cell (PBSC) collection were analyzed. Eight factors were significantly correlated with the number of CD34 cells per donor body weight on the fourth day (day 4) after G-CSF administration in univariate analysis. Using multi-regression analysis, we made a simple scoring system comprising age, sex, LDH on day 4 and RBC count at the baseline, which significantly predicted CD34 cell yield ( P = 0.048). This system allows us to determine the optimal PBSC collection day. When the score is 0 or 1 on day 4, starting apheresis on day 5 potentially helps avoiding the need for multiple harvests. Score 3 or 4 on day 4 is indicative of better performance if apheresis is started on day 4. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses a case study of a 35 year old man who was suffering from acute myeloid leukemia and mentions the diagnosis of mixed-type autoimmune hemolytic anemia (AIHA) and autoimmune thrombocytopenia as symptoms of graft-versus-host disease (GVHD).

Waldenström macroglobulinemia is a lymphoplasmacytic lymphoma characterized by production of the immunoglobulin M (IgM) monoclonal protein. Commonly involved sites are the bone marrow, lymph nodes, and spleen. Lymphoplasmacytic infiltration of the central nervous system (CNS), in contrast, is referred to as Bing-Neel syndrome, and is an extremely rare phenomenon. Here, we present a unique case of Waldenström macroglobulinemia with optic neuritis accompanied by monoclonal expansion of T cells, which recovered after administration of CNS-targeting chemotherapy. Although the underlying causal relationships in this case remain obscure, aberrantly expanded T cells may have contributed to the development of optic neuritis, and we should be reminded that some types of cranial neuropathy in Waldenström macroglobulinemia may be reversible. [ABSTRACT FROM AUTHOR]