Tyrosine Kinase Inhibitor-induced Cerebrovascular Occlusion Presenting with Moyamoya Disease-like Stenosis of the Circle of Willis.

Vascular occlusive events are notable adverse effects of tyrosine kinase inhibitors (TKIs), which are promising treatments for chronic myeloid leukemia (CML). We herein report the case of a patient with CML who developed cerebrovascular occlusion of the circle of Willis during TKI treatment. Our patient did not meet the diagnostic criteria for moyamoya disease due to the insignificant development of moyamoya vessels. The lack of moyamoya vessel development may be explained by the suppression of tyrosine kinases that are responsible for angiogenesis. Cerebrovascular occlusion of the circle of Willis, without significant development of moyamoya vessels, may be an important phenotype of TKI-associated vasculopathy.

Temporal variation in environmental radioactivity and radiation exposure doses in the restricted areas around the Fukushima Daiichi Nuclear Power Plant.

Temporal variation and fluctuation in environmental contamination in Futaba town and Okuma town, the location of the Fukushima Daiichi Nuclear Power Plant (FDNPP), were evaluated based on a car-borne survey conducted from October 2021 to November 2022. Although the environmental radioactivity in the interim storage facility area (ISF) was higher than that in open areas (i.e., the evacuation order lifted areas in Futaba town and the Specific Reconstruction and Regeneration Base area [SRRB] in Okuma town), only minor temporal changes were seen in the ambient dose and detection rate of radiocesium (the proportion of radiocesium detected points per all measuring points) in those areas, respectively. These findings suggest that the observed variations may result from physical decay and environmental remediation. Resuspension caused by human activities and weather could also affect the detection rate of radiocesium. The annual external effective doses in Futaba town and Okuma town were estimated to be at a limited level (< 1 mSv/year). Nevertheless, to help ensure the safety and future prosperity of residents and communities in the affected areas around the FDNPP, long-term follow-up monitoring of temporal exposure dose levels during the recovery and reconstruction phases is extremely important.

IgE Plasma Cell Leukemia Harboring t(11;14) and 1q Amplification.

IgE plasma cell neoplasm is the rarest subtype of plasma cell neoplasms and is known for its poor prognosis and high incidence of t(11;14). However, t(11;14) has been classified as a standard-risk rather than high-risk cytogenetic abnormality in multiple myeloma. We have been unable to explain the discrepancy that the hallmark of IgE plasma cell neoplasm with a poor prognosis is a standard-risk cytogenetic abnormality. Here, we report a case of IgE primary plasma cell leukemia with extramedullary lesions of the liver, stomach, and lymph nodes. Plasma cell infiltration was pathologically confirmed in each organ. Cytogenetic analysis of plasma cells revealed t(11;14) and amplification of 1q21. Chemotherapy, with immunomodulatory imide drugs, proteasome inhibitors, and CD38 antibodies, was unsuccessful. In IgE plasma cell neoplasm, coexistence of other cytogenetic abnormalities with t(11;14) may be important. Investigating the presence of cytogenetic abnormalities coexisting with t(11;14) is not only useful for evaluating prognosis but also important for understanding the pathogenesis of the disease. Recently, venetoclax, an oral BCL2 inhibitor, has demonstrated promising efficacy in plasma cell neoplasm patients harboring t(11;14). Development of an effective venetoclax-based regimen for treating aggressive IgE plasma cell neoplasm with t(11;14) is expected.

[Delayed diagnosis of hydroa vacciniforme-like lymphoproliferative disorder in a patient with skin rashes].

Chronic active Epstein-Barr virus (CAEBV) infection is characterized by persistent EBV infection and can lead to fatal conditions such as hemophagocytic syndrome and malignant lymphoma through the clonal expansion of EBV-infected T or natural killer (NK) cells. Hydroa vacciniforme lymphoproliferative disorder (HV) and hypersensitivity to mosquito bites (HMB) have been identified as skin diseases in EBV-associated T- or NK-cell lymphoproliferative diseases. We present the case of a 33-year-old man. The patient had frequent episodes of a facial rash for three years before he visited our hospital, he visited several dermatologists but did not receive a diagnosis of HV. He was referred to the hematology department of our hospital for assessment of atypical lymphocytes in peripheral blood. Based on routine blood and bone marrow test we were unable to diagnose HV. However, when the patient's liver function deteriorated six months later, we considered the possibility of HV after reevaluating the skin rash. After performing EBV-related tests, we were able to definitively diagnose CAEBV with HV. It is crucial to be able to connect clinical observations to EBV-related tests when diagnosing CAEBV. Hematologists must be knowledgeable of the EBV-associated skin conditions of HV and HMB.

Multilineage Lymphoblastic Lymphoma as an Initial Presentation of Mixed Phenotype Acute Leukemia.

Mixed phenotype acute leukemia (MPAL) is characterized by leukemic blasts that express markers of multiple lineages. Compared with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL), MPAL is considered to have a poor treatment outcome. We report a case of MPAL T/myeloid not otherwise specified that was initially presented as multilineage lymphoblastic lymphoma and subsequently developed into leukemic MPAL. An acute lymphoblastic leukemia-based treatment regimen was ineffective, but azacitidine and venetoclax therapy resulted in hematological complete remission. Our case suggests that multilineage lymphoblastic lymphoma should be considered to be the same disease as MPAL, albeit with different clinical presentations. Optimal treatment for MPAL has not been established yet, but azacitidine and venetoclax therapy may be a potential approach.

Assessment of Radiation Risk Perception and Interest in Tritiated Water among Returnees to and Evacuees from Tomioka Town within 20 km of the Fukushima Daiichi Nuclear Power Plant.

In this study, we aimed to investigate radiation risk perception, mental health, and interest in tritiated water among evacuees from and returnees to Tomioka town, Japan, as well as to evaluate the intention to return (ITR) among evacuees living inside and outside Fukushima Prefecture. Of the 1728 respondents, 318 (18.4%) and 1203 (69.6%) participants reported living outside and inside Fukushima Prefecture, and 207 (12.0%) reported living in Tomioka. The ITR was not significantly different between those who lived inside and outside the prefecture among the evacuees. Similarly, there were no significant differences in radiation risk perception, mental health, and interest in tritiated water. However, the evacuees were independently associated with a motivation to learn about tritiated water (OR = 1.242, 95%Cl: 1.041-1.438, p = 0.016), reluctance to consume food from Tomioka (OR = 1.635, 95%Cl: 1.372-1.948, p < 0.001), and concern that adverse health effects would occur because of the Fukushima Daiichi Nuclear Power Plant accident (OR = 1.279, 95%Cl: 1.055-1.550, p = 0.012) compared to returnees, according to logistic regression analysis. Interestingly, the returnees were found to have better mental health but lower life satisfaction than the evacuees. These findings suggest the importance of ongoing risk communication about radiation exposure and tritiated water among residents regardless of their place of residency.

Intention to Return and Perception of the Health Risk Due to Radiation Exposure Among Residents in Tomioka Town, Fukushima Prefecture, Stratified by Gender and Generation.

OBJECTIVE: The aim of this study was to clarify residents' intentions to return (ITR) to Tomioka town, Fukushima Prefecture; and their risk perception of the health effects of radiation exposure as stratified by gender and generation. METHODS: Of almost 8000 residents who were 20 years of age or older and who had lived, prior to the accident, in the zones of Tomioka town where residents were permitted to return, 1860 were included in the analysis. For the analysis, the residents were divided into 4 groups which were stratified by gender and age: older males (over 50 years), young males (20-49 years), older females (over 50 years), and young females (20-49 years). RESULTS: ITR was found to be significantly lower in young males, young females, and older females than in older males. Conversely, young and older females had significantly higher levels of anxiety about drinking tap water and consuming food collected in Tomioka town. Young and older females also had greater concerns about adverse health effects on themselves and their offspring due to living in Tomioka town. CONCLUSION: Our results showed that special attention should be paid to younger residents, particularly to young females, with regard to recovery from a nuclear disaster.

Risk Perception of Health Risks Associated with Radiation Exposure among Residents of Okuma, Fukushima Prefecture.

Ten years have passed since the Great East Japan Earthquake and the subsequent Fukushima Daiichi nuclear power plant accident on 11 March 2011. Okuma is a town hosting the Fukushima Daiichi nuclear power plant. The evacuation order for Okuma was partially lifted in April 2019. To clarify factors associated with risk perceptions of radiation among the residents of Okuma, we conducted a questionnaire survey in January 2021. Our results revealed that resident anxieties regarding the health effects of radiation exposure from living in Okuma were independently associated with positive PCL-Specific score, recognition of the consultation services with radiation experts in the municipal government of Okuma, and planned request for consultation service regarding radiation exposure by radiation experts, along with being female and living with a child. It is important for radiation experts to promote periodic communication of risks with individuals on a small scale to provide accurate information about the health effects of radiation and to provide maternal and child healthcare services and support regarding child-rearing and radiation exposure, to reduce concerns about radiation exposure and facilitate healthy living and wellbeing in Okuma.

Mycobacterial lymphadenitis without granuloma formation in a patient with anti-interferon-gamma antibodies.

A previously healthy 49-year-old Japanese woman presented with cervical lymph node swelling and tenderness. Lymph node biopsy revealed reactive lymphadenitis without granulomas. No malignant cells were found, and no acid-fast positive bacilli were identified by Ziehl-Neelsen staining. She was treated unsuccessfully with various antibiotics, and it was very challenging to reach a diagnosis. 18F-Fluorodeoxyglucose (18F-FDG) uptake in bones was evaluated using positron emission tomography-computed tomography (PET-CT), and disseminated mycobacterial infection was suspected. The interferon-gamma (IFN-γ) release assays QuantiFERON (QFT) and T-SPOT were used to diagnose tuberculosis infection. On testing, a difference in mitogen response was found between these assays. The response was low for QFT but adequate for T-SPOT, suggesting the presence of anti-IFN-γ antibodies. This difference depended on whether the patient's plasma (including anti-IFN-γ antibodies) was used within the assay system. Mycobacterium abscessus was isolated from lymph node cultures, and plasma anti-IFN-γ antibodies were confirmed. The patient was diagnosed with disseminated M. abscessus infection with underlying adult-onset immunodeficiency caused by anti-IFN-γ antibodies. Granulomas are a pathological hallmark of mycobacterial infection, but may not fully form in immunodeficient patients. Clinicians should be aware of the possibility of mycobacterial infection without granuloma formation due to anti-IFN-γ antibodies.

Risk perception of the pre-distribution of stable iodine to guardians of children living around the Genkai Nuclear Power Plant, Saga Prefecture, Japan.

Iodine thyroid blocking (ITB) is effective for preventing childhood thyroid cancer when radioactive iodine is released into the environment during a nuclear power plant accident. Japan employs the pre-distribution of stable iodine (PDSI) to residents living near nuclear power plants; however, the number of residents who have actually received stable iodine to date remains limited. The aim of this study was to evaluate the profile of guardians of children living around the Genkai Nuclear Power Plant (GNPP) in Japan. We distributed self-administered questionnaires regarding perception of risks associated with administration of stable iodide to approximated 400 guardians of children aged 0-6 in 10 kindergartens located in four municipalities. We obtained responses from 286 guardians, and after excluding invalid responses, 247 were included in the analysis. Logistic regression analysis revealed that living within 5 km of the GNPP (odds ratio [OR] = 4.48, 95% confidence interval [CI]: 2.43-8.24), awareness of preferential implementation of ITB to children (OR = 3.33, 95%CI: 1.78-6.22), and awareness of the prophylaxis booklet published by the local government (OR = 2.53, 95%CI: 1.37-4.68) were independently associated with PDSI for children. The main reasons for not receiving PDSI were "anxiety about the side effects of stable iodine" (40.2%), "distrust of the effectiveness of SI" (23.5%), "complicated procedures for receiving stable iodine" (15.7%) and "missed the date for receiving stable iodine" (8.8%). In the case of ITB implementation during a nuclear emergency, it is necessary to clarify the risk perceptions of guardians and adapt risk communication accordingly.

Experiences of crisis communication during radiation emergency and risk communication for recovery of the community in Fukushima.

Since 2011, Nagasaki University (Nagasaki, Japan) has been assisting the reconstruction efforts of Kawauchi Village (Fukushima Prefecture), which was the first village to decide to return to their home town after the evacuation due to the accident at the Fukushima Daiichi Nuclear Power Station. In April 2013, Nagasaki University and the Kawauchi Government Office concluded an agreement concerning comprehensive cooperation toward the reconstruction of the village. Furthermore, we began comprehensive support for the residents of Tomioka who returned to their hometown in 2017, and of Ohkuma town in 2020. On the basis of the experiences in Kawauchi, Tomioka and Ohkuma, it is clear that the cooperation of residents, local authorities and specialists is essential for the recovery of areas affected by the accident at the Fukushima Daiichi Nuclear Power Station. Accumulated experiences and practices should be carefully evaluated and recorded to prepare for unexpected nuclear disasters in the future.

Risk perception regarding implementation of iodine thyroid blocking during a nuclear disaster of mothers living near a nuclear power station in Japan.

Pre-emptive evacuation orders following the accident at the Fukushima Daiichi Nuclear Power Station (FDNPS) in March 2011 and subsequent regulatory limits regarding contaminated food, milk, and water minimized the external and internal radiation exposure doses of nearby residents. However, with regard to implementation of iodine thyroid blocking (ITB), residents were confused because no information on the matter was released by the central and/or local governments. Based on lessons learned from the FDNPS accident, many countries have revised their guidelines regarding ITB during nuclear disasters. To adequately revise such guidelines and ensure effective ITB implementation during a nuclear disaster, however, residents' perceptions of ITB must be clarified. In this study, the perception of risks associated with ITB was investigated in mothers residing near the Sendai Nuclear Power Plant (SNPP) in Kagoshima Prefecture, Japan. Of the 520 mothers surveyed, 467 (89.8%) expressed anxiety regarding the administration of potassium iodine (KI) to their children. Logistic regression analysis revealed that the mothers' anxiety regarding the administration of KI to their children was positively correlated with their wish to consult an expert about KI and their hesitation to let their children eat foods produced in Fukushima, and negatively correlated with having confidence about administering KI to their children. Careful communication of potential risks to mothers residing near nuclear power plants is thus critical for implementing effective ITB in children.

Acute compartment syndrome as the initial manifestation of chronic-phase chronic myeloid leukemia: a case report and review of the literature.

BACKGROUND: Acute compartment syndrome is an orthopedic emergency requiring urgent fasciotomy to prevent irreversible damage. In hematological malignancies, acute compartment syndrome caused by severe soft tissue bleeding is extremely rare. We present a patient with chronic-phase chronic myeloid leukemia who had acute compartment syndrome caused by severe soft tissue bleeding in her right forearm. CASE PRESENTATION: A 72-year-old Japanese woman was referred to our hospital with swelling and pain of her right forearm without a previous history of trauma. She was diagnosed with chronic-phase chronic myeloid leukemia. Extreme thrombocytosis was present, although no evidence of acquired von Willebrand disorder was found. Compartment syndrome caused by soft tissue bleeding was confirmed. An emergency fasciotomy for decompression was conducted. However, sustained postoperative bleeding occurred and required massive red cell concentrate transfusion. As her platelet count decreased by cytoreductive therapy, complete hemostasis was achieved. CONCLUSIONS: Patients with an extremely high platelet count might be at high risk for severe bleeding complications even without acquired von Willebrand disease. For the control of severe bleeding complications in patients with myeloproliferative disorder, the importance of thrombocyte reduction should be recognized.

Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites.

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy.

Stringent complete remission of primary plasma cell leukemia with reduced-dose bortezomib, lenalidomide and dexamethasone: a case report and review of the literature.

Plasma cell leukemia (PCL) is an aggressive variant of multiple myeloma characterized by a high level of plasma cells circulating in the peripheral blood. The prognosis of PCL patients treated with conventional chemotherapy remains poor. Some reports have suggested that both bortezomib and lenalidomide are effective in treating PCL. We herein report a case of primary PCL in which the patient achieved stringent complete remission after receiving combination chemotherapy with reduced-dose bortezomib, lenalidomide and dexamethasone (VRd). This regimen was very effective, and no severe adverse events were observed. A reduced-dose VRd regimen can be considered in PCL patients.

Expression of CD27 on peripheral CD4+ T-lymphocytes correlates with the development of severe acute graft-versus-host disease after allogeneic bone marrow transplantation.

Allogeneic immune responses during hematopoietic reconstitution play central roles in beneficial and adverse effects after allogeneic bone marrow transplantation (allo-BMT). Appropriate regulation of the immune responses might improve the outcome of allo-BMT. However, a useful marker for monitoring allogeneic immune responses remains to be established. We enrolled 22 consecutive patients who underwent myeloablative allo-BMT between March 2002 and March 2006 and examined the relationship between CD27 expression on peripheral blood T-lymphocytes, a possible marker for naive/effector phenotypes, and clinical events, especially acute graft-versus-host disease (aGVHD). In 8 patients with aGVHD of grades II to IV, the CD27+/CD27- ratios of CD4+ (but not CD8+) T-lymphocytes were significantly higher after allo-BMT, even at day 21, than the ratios in patients with aGVHD of grade 0 or I and remained high after day 21. In contrast, the ratios were low after day 21 following allo-BMT in 14 patients with aGVHD of grade 0 or I. Moreover, the clinical analysis suggested a relationship between the ratio and aGVHD grade. Thus, we showed that the CD27+/CD27- ratio in CD4+ T-lymphocytes may have value in predicting the development of severe aGVHD and may correlate with clinical symptoms of aGVHD.