Introduction : Treatment outcome of adult patients with acute B-cell lymphoblastic leukemia (ALL) is suboptimal even after an allogeneic hematopoietic stem cell transplantation (allo-HSCT). To maximize the efficacies of this treatment strategy, risk stratification is crucial.
Methods : We retrospectively collected clinical data of the adult patients with allo-HSCT for ALL at a single insitiution in Japan between 2003 and 2022. Univariate and multivariate analyses were performed to identify risk factors for overall survival (OS), progression-free survival (PFS) and GVHD-free-and-relapse-free survival (GRFS) at 3 years.
Results : A total of 58 patients were included with 34 females and a median age of 39. Sixty-two percent of patients harbored high-risk cytogenetic features or Philadelphia chromosome (Ph). Hematologic complete response (CR) rate was 93％ after a first induction, but 75.9％ were in CR at allo-HSCT. Blinatumomab was used in 1.7％ of patients. A chimeric mRNA had been detected in 4 of 26 patients at allo-HSCT. The 3-year OS, PFS and GRFS were 72.7％, 54.7％ and 46.2％, respectively. Pre-transplantation CR was an independent risk factor.
Discussion/Conclusions : Our results imply that a better OS may potentially be achieved by improved pretransplantation CR rate with more frequent application of novel agents.

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A 15-year-old female was referred to our hospital because of fever and fatigue for 1 month. C-reactive protein elevation was observed (8.2mg/dL), and imaging studies including ¹⁸F-fluoro-D-glucose-positron emission tomography/computed tomography (FDG-PET/CT) showed no abnormal findings. Prednisolone (PSL) was started, and her fever went down subsequently. When PSL was tapered, fever and headache recurred ; so tocilizumab (TCZ) was initiated as a treatment for systemic juvenile idiopathic arthritis, and fever was resolved. Six months later she experienced pain around both knees. Multifocal abnormal bone marrow signals were observed through magnetic resonance imaging. Lesion bone biopsy revealed nonspecific inflammation without malignancy or infection. Our diagnosis was chronic recurrent multifocal osteomyelitis (CRMO), naproxen was started, and TCZ was switched to adalimumab (ADA). However, her pain around both knees and headache persisted. A year after CRMO diagnosis, ¹⁸F-FDG-PET/CT re-examination revealed FDG accumulation in the common carotid artery, subclavian artery, aortic arch, and femoral artery to the popliteal artery. Contrast-enhanced CT showed vessel wall thickening and arterial stenosis. Accordingly, the patient was diagnosed with Takayasu arteritis. PSL was increased, and azathioprine was initiated, and ADA was switched to subcutaneous TCZ. At the 10-month follow-up, headache and leg pain had decreased, and the steroid dose was tapered.
CRMO is a noninfectious, autoinflammatory disease causing recurrent sterile inflammatory bone lesions. CRMO has been described in association with other inflammatory conditions such as Takayasu arteritis. Physicians should consider that in a case of CRMO with persistent inflammation after treatment, other inflammatory diseases such as Takayasu arteritis may coexist.

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Avoidant and restrictive food intake disorder (ARFID) is an eating disorder characterized by the restriction of both the type and amount of food ingested without any desire to reduce weight or impaired weight/body shape perception. ARFID has garnered attention due to its association with autism spectrum disorder (ASD).
Herein, we report the case of a 9-year-old patient, who was expeditiously admitted to our hospital because of rapid weight loss and hypoglycemia during a short stature follow-up. Poor food intake, consequent to a phobia of abdominal pain and nausea, was observed, and the patient was diagnosed with ARFID. Additionally, a diagnosis of ASD was rendered owing to persistent deficiencies in social communication and interpersonal interactions since early childhood, along with restricted behavior and interests. Dietary guidance predicated on the individual's ASD characteristics, which engendered a forward perspective and reduced anxiety, led to increased food intake, improved nutritional status, and remediation of growth disturbances. ARFID can have serious ramifications in children, including growth disturbances and organ damage due to trace element deficiencies. For children with eating behavior problems, developmental characterization, prompt diagnosis, psychoeducation, and appropriate nutritional counseling are advised.

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Multisystem Inflammatory Syndrome in Children (MIS-C) is a new disease that develops after coronavirus disease 2019 (COVID-19) and is known to cause severe inflammation in multiple organs, gastrointestinal symptoms, respiratory distress, and shock.
The patient was an 8-year-old boy who developed MIS-C one month after COVID-19 and presented with left ventricular systolic dysfunction and hypotension. Prednisolone (PSL) and high-dose immunoglobulin therapy (IVIG) improved some symptoms and part of the laboratory findings, but ferritin and D-dimer remained elevated, suggesting a cytokine storm. After infliximab (IFX) administration, ferritin and D-dimer improved, suggesting that IFX may be useful in suppressing cytokines that were not suppressed by the combination of PSL and IVIG. NT-proBNP and troponin T were elevated the day before shock and decreased with improvement in left ventricular contractility. We suggest that measurement of NT-proBNP and troponin T help in early prediction of shock in MIS-C.

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