Clinical features and images of malignant lymphoma localized in the pancreatic head to differentiate from pancreatic ductal adenocarcinoma: a case series study.

BACKGROUND: Pathological examination by endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA) has been reported to be useful in diagnosing pancreatic malignant lymphoma (ML), but some ML cases are difficult to be differentiated from pancreatic ductal adenocarcinoma (PDAC). METHODS: This retrospective study included 8 patients diagnosed with ML that had a pancreatic-head lesion at initial diagnosis and 46 patients with resected PDAC in the pancreatic head between April 2006 and October 2021 at our institute. ML and PDAC were compared in terms of patients' clinical features and imaging examinations. RESULTS: The median tumor size was larger in ML than in PDAC (45.8 [24-64] vs. 23.9 [8-44] mm), but the median diameter of the caudal main pancreatic duct (MPD) was larger in PDAC (2.5 [1.0-3.5] vs. 7.1 [2.5-11.8] mm), both showing significant differences between these malignancies (both, P < 0.001). In the analysis of covariance, MLs showed a smaller caudal MPD per tumor size than PDACs, with a statistical difference (P = 0.042). Sensitivity and specificity using sIL-2R ≥ 658 U/mL plus CA19-9 < 37 U/mL for the differentiation of ML from PDAC were 80.0% and 95.6%, respectively. CONCLUSIONS: Diagnosing pancreatic ML using cytohistological examination through EUS-FNA can be difficult in some cases. Thus, ML should be suspected if a patient with a pancreatic tumor has a small MPD diameter per tumor size, high serum sIL-2R level, normal CA19-9 level. If the abovementioned features are present and still cannot be confirmed as PDAC, re-examination should be considered.

Effectiveness of a targeted primary preventive intervention in a high-risk group identified using an efficiency score from data envelopment analysis: a randomised controlled trial of local residents in Japan.

OBJECTIVE: To determine whether a minimal intervention based on the data envelopment analysis (DEA)-identified efficiency score effectively prevents hypertension. DESIGN: Randomised controlled trial. SETTING: Takahata town (Yamagata, Japan). PARTICIPANTS: Residents aged 40-74 years belonged to the information provision group for specific health guidance. Participants with a blood pressure ≥140/90 mm Hg, those taking antihypertensive medication, or those with a history of cardiac diseases were excluded. Participants were consecutively assigned based on their health check-up visit at a single centre from September 2019 to November 2020 and were followed up at the check-up in the following year, until 3 December 2021. INTERVENTION: A targeted approach using minimal intervention. Target was identified using DEA and 50% of participants with higher risk were targeted. The intervention was notifying the results of their risk of hypertension according to the efficiency score obtained by the DEA. PRIMARY OUTCOME MEASURES: A reduction in the proportion of participants who developed hypertension (≥140/90 mm Hg or taking antihypertensive medication). RESULTS: A total of 495 eligible participants were randomised, and follow-up data were available for 218 and 227 participants in the intervention and control groups, respectively. The risk difference for the primary outcome was 0.2% (95% CI -7.3 to 6.9) with 38/218 (17.4%) and 40/227 (17.6%) events in the intervention and control group, respectively (Pearson's χ2 test, p=0.880). The adjusted OR of the effect of the intervention was 0.95 (95% CI 0.56 to 1.61, p=0.843), and that of the efficiency score (10-rank increase) was 0.81 (95% CI 0.74 to 0.89, p<0.0001). CONCLUSIONS: Minimal intervention to a high-risk population stratified by DEA was not effective in reducing the onset of hypertension in 1 year. The efficiency score could predict the risk of hypertension. TRIAL REGISTRATION NUMBER: UMIN000037883.

Metastatic pancreatic ductal adenocarcinoma followed by a fatal diffuse large B-cell lymphoma: A rare case report and literature review.

RATIONALE: Recently, the incidence of polyoncosis has been increasing due to advancements in treatment, such as antitumor therapy, which led to a prolonged survival. However, few patients with metastatic pancreatic ductal adenocarcinoma (PDAC) develop second tumors, which render a poor prognosis. We report a rare case of PDAC, which is metachronous with a fatal malignant lymphoma (ML). PATIENT CONCERNS: A 68-year-old woman who had been monitored due to liver cirrhosis secondary to hepatitis C virus infection presented with a 10-mm pancreatic head cancer with lung metastasis and had started an anticancer therapy with gemcitabine. Approximately 18 months after diagnosis, lymphadenopathies around the pancreas were noted, which eventually spread to the entire body over time. DIAGNOSIS: Diffuse large B-cell lymphoma was diagnosed using biopsies from cervical lymph nodes. INTERVENTIONS AND OUTCOMES: The patient started a gemcitabine + rituximab regimen; however, the patient died from cachexia-associated lymphoma progression, not PDAC. LESSONS: ML should be considered when intra-abdominal lymphadenopathies are detected in patients with pancreatic cancer, and ML should be differentiated from lymph node metastasis of pancreatic cancer.

With M-protein positive, could transthyretin amyrodosis be easily excluded? Not necessarily! Wild-type transthyretin amyrodosis with Waldenström's macroglobulinaemia: a case report.

Background: Generally, it is said that amyloid light-chain (AL) develops not only in multiple myeloma but also in Waldenström's macroglobulinemia. We experienced a case of M-protein positive and diagnosed as wild-type transthyretin amyrodosis (ATTRwt) accompanied with Waldenström's macroglobulinemia. Case summary: The patient was 72-year-old male, and the main complaint was dyspnoea in April 2020 and visited a nearby doctor. He was introduced to the Department of Haematology at our hospital for high levels of serum immunoglobulin M, M-protein positivity, and cardiac hypertrophy with a suspect of AL amyloidosis. Duodenal mucosal biopsy and abdominal skin biopsy showed no amyloid deposits, and left iliac bone marrow biopsy diagnosed Waldenström's macroglobulinemia and with no amyloid, and Kumamoto criteria score 1. Last of all, ATTRwt was diagnosed for endocardial biopsy. Discussion: This is a very rare case of ATTRwt with Waldenström's macroglobulinaemia.

Efficacy of Additional Intervention to the Specific Health Guidance in Japan: The Takahata GENKI Project.

PURPOSE: A tailored approach to individual risk factors for developing lifestyle-related diseases would help induce behavioral changes toward intervention acceptability. The addition of preventive healthcare programs to nationwide specific health guidance in Japan is adapted in a given region. PATIENTS AND METHODS: We conducted a prospective parallel-group comparison study on 195 eligible residents from Takahata, Japan, with a high risk of lifestyle-related diseases from 2014 to 2017 to examine whether such an intervention could improve the body mass index (BMI) and estimated glomerular filtration rate (eGFR). RESULTS: Of the 195 enrolled residents, 117 were assigned to the control group and 78 to the intervention group. They were ≤65 years old and had a BMI ≥25 kg/m2 and an eGFR ≤90 mL/kg/1.73 m2. We conducted certain interventions for each group, including additional blood testing, regular health guidance, and specific health guidance. After one year, neither BMI (intervention: 26.7 ± 2.17 kg/m2 vs control: 27.3 ± 2.12 kg/m2, p = 0.076) nor eGFR (intervention: 72.2 ± 11.1 mL/kg/1.73 m2 vs control: 73.1 ± 10.5 mL/kg/1.73 m2, p = 0.608) differed significantly between groups. However, after three years, the BMI in the intervention group (26.4 ± 2.05 kg/m2) was significantly reduced compared to that in the control group (27.4 ± 2.26 kg/m2; p = 0.005). CONCLUSION: The additional interventions might have contributed to a reduction in metabolic syndrome. TRIAL REGISTRATION: This study was registered in the UMIN-Clinical Trials Registry (ID:000013581). More information: https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000015868. The registration date was 31/03/2014.

Successful treatment with gilteritinib for isolated extramedullary relapse of acute myeloid leukemia with FLT3-ITD mutation after allogeneic stem cell transplantation.

Acute myeloid leukemia (AML) harboring Fms-like tyrosine kinase 3 (FLT3) internal tandem duplication (ITD) mutation is associated with shorter remission and higher relapse risk. Several FLT3 inhibitors have been used in clinical trials, but their efficacy in extramedullary disease remains unclear. In the present case, a 56-year-old man was diagnosed with FLT3-ITD mutated AML. Due to bone marrow relapse during consolidation therapy, he underwent salvage therapy and a myeloablative conditioning regimen, followed by peripheral blood stem cell transplantation (PBSCT) from a HLA-matched related donor. Acute graft-versus-host disease (GVHD) did not develop, and complete donor chimerism was confirmed on days 27 and 96 after PBSCT. On day 180, he experienced extensive chronic GVHD and had several subcutaneous tumors in his body, which were diagnosed as myeloid sarcoma by pathological examination. We considered this to be a case of isolated extramedullary relapse, as his bone marrow had maintained complete donor chimerism. Treatment with etoposide and ranimustine produced no effect, and tumor progression continued. We started administration of gilteritinib, a FLT3/AXL inhibitor, after identifying the FLT3-ITD mutation in the tumor. Subsequently, there has been a remarkable regression of the tumors. Gilteritinib can be effective in isolated extramedullary relapse after allogeneic stem cell transplantation.

Successful management of recurrent bleeding with tocilizumab in an acquired hemophilia A patient with rheumatoid arthritis.

A 61-year-old woman with rheumatoid arthritis was diagnosed as having acquired hemophilia A with extensive subcutaneous bleeding. The patient was treated with a corticosteroid, and her symptoms improved temporarily. However, these recurred during the tapering of her corticosteroid dose, and neither the re-increase in the dose nor the addition of cyclophosphamide could control her bleeding tendency. After the administration of an anti-IL-6 receptor antibody (tocilizumab), the doses of corticosteroid and cyclophosphamide could be tapered. Tocilizumab combined with another immunosuppression therapy might be effective in the treatment of acquired hemophilia A.

Successful treatment of primary advanced gastric plasmacytoma using a combination of surgical resection and chemotherapy with bortezomib: A case report.

INTRODUCTION: Extramedullary plasmacytoma (EMP) is a plasma cell neoplasm that presents as a solitary tumor. EMP in the gastrointestinal organs are extremely uncommon. PRESENTATION OF CASE: A 36-year-old man was admitted to our hospital with advanced anemia. He had no specific medical history. Gastroendoscopic findings showed an 8.0-cm submucosal tumor with ulcer on the greater curvature of the gastric body. Fine-needle aspiration was performed, and the pathologic diagnosis of the submucosal tumor was a plasmacytoma. Therefore, the patient was diagnosed with gastric plasmacytoma. A total gastrectomy was performed with lymphadenectomy. The result of intraoperative peritoneal lavage cytology was positive. Histological examination revealed serosa-exposed plasmacytoma of the stomach with lymph nodes metastasis. Additionaly the patient received a three-drug chemotherapy regimen (bortezomib, cyclophosphamide, and dexamethasone [VCD]) from 3 weeks after the operation. After 4 cycles of chemotherapy, the patient received autologous peripheral blood stem-cell transplantation (auto-PBSCT). Eighteen months after diagnosis, the patient is in complete remission with no evidence of local relapse or evolution to multiple myeloma. CONCLUSIONS: This is the first reported case of advanced gastric plasmacytoma using adjuvant chemotherapy involving bortezomib and auto-PBSCT after the resection, and the patient has maintained a good course over a year. This protocol could be a new way to treat these tumors.

Bortezomib and dexamethasone for multiple myeloma: higher AST and LDH levels associated with a worse prognosis on overall survival.

BACKGROUND: Bortezomib offers a novel approach to the treatment of multiple myeloma producing rapid control. The aim of this study was to investigate the outcomes of bortezomib and dexamethasone-treated patients with multiple myeloma. METHODS: We conducted a retrospective study of 44 consecutively-treated multiple myeloma patients with bortezomib (1.3 mg/m(2) on days 1, 4, 8, and 11 of a 21-day cycle or 1.3 mg/m(2) intravenously 1, 8, 15, and 22 of every 35-day cycle) and dexamethasone. RESULTS: The median time to progression, progression free survival time, and overall survival time in the treatment groups was 14.9, 14.9, and 38.3 months, respectively. The present study also suggests the possibility that the prognosis of patients with high levels of AST and LDH might be worse. CONCLUSIONS: Our results indicate that the treatment of multiple myeloma with bortezomib and dexamethasone is feasible.

[A case of neurotoxicity reduced with pregabalin in R-CHOP chemotherapy for diffuse large B-cell lymphoma].

When performing R-CHOP(rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone)for diffuse large B-cell lymphoma(DLBCL), neurotoxicity of vincristine(VCR)is the serious dose-limiting factor.Pregabalin is one of the first-line treatments for painful diabetic peripheral neuropathy in many countries, and we have administered it to relieve the neurotoxicity associated with adverse effects of VCR in a DLBCL patient treated with the R-CHOP regimen.A 49-year-old man with kidney DLBCL had surgery performed.Afterward, the R-CHOP regimen was introduced.In order to relieve the neurotoxicity of VCR, pregabalin was used from day 8 in the second course.The severity of sensory neurotoxicity after the administration of pregabalin was improved from CTCAE(v4.0)grade 3 to grade 1.Therefore, there is a possibility that VCR-induced neurotoxicity is relieved by pregabalin.Further trials are needed to confirm the value of pregabalin.

[Exacerbation of acute leukemia bearing isolated i(17q) along with proliferation of blasts with high BMI-1 expression].

We report a case of acute leukemia with an isolated isochromosome 17q karyotypic abnormality, which may be transformed from myeloproliferative disease (MPD)/myelodysplastic syndrome (MDS). A 69-year-old male patient with 27% of blasts in the peripheral blood underwent hematological examinations including cytochemical staining of cells such as myeloperoxydase (MPO), surface marker study on blasts, chromosomal test and bcr-abl mRNA analysis. The cytological and molecular findings (MPO-positive, myeloid marker CD13 expression (67.3%) and megakaryocytic marker CD41 expression (24.8%)) indicated that the blasts were consistent with myeloid leukemic cells partially committed to megakaryocytes. He was diagnosed as having leukemic transformation from MPD/MDS based on history of leukocytosis and thrombocytosis, isolated i(17q), bcr-abl negative, hepatosplenomegaly, increased eosinophil/basophil count and cytologic dysplasia. Positivity of BMI-1 in CD34+ blasts was 25.8% at the diagnosis and anti-leukemic drugs including anthracyclines were effective for his disease control during 6 months. However, the CD34+ cells turned out to highly express BMI-1 (83.1%), and leukemic cells started to increase progressively following which the leukemic cells failed to respond efficiently to any anti-leukemic drugs. Thus, expression of BMI-1 was well correlated with the disease progression, growth ability of blasts and resistance to anti-cancer drugs, indicating that BMI-1 positivity in CD34+blasts is an excellent molecular marker for disease progression and prognosis in such patients.