暂无摘要。

Background/Objectives: Gaucher disease type 1 (GD1) is characterized by hepatosplenomegaly, thrombocytopenia, and disabling bone manifestations requiring regular MRI monitoring. The EIROS study assessed the real-world impact of velaglucerase alfa on GD1 bone disease, using MRI data collected in French clinical practice. Methods: MRIs collected retrospectively from treatment initiation and prospectively during follow-up (12-months) were analyzed centrally by a blinded expert radiologist to evaluate bone infiltration using the Bone Marrow Burden (BMB) score and a qualitative method (stable, improved or worsened for the spine and femur). Abdominal MRIs were also centrally analyzed to assess hepatosplenomegaly. Bone manifestations, hepatosplenomegaly, and hematologic parameters were analyzed from medical records. Results: MRI data were available for 20 patients: 6 treatment-naive patients and 14 patients who switched to velaglucerase alfa from another GD treatment. Interpretable MRIs for BMB scoring were available for seven patients for the spine and one patient for the femur. Qualitative assessments (n = 18) revealed stability in spine and femur infiltration in 100.0% and 84.6% of treatment-switched patients (n = 13), respectively, and improvements in 80.0% and 60.0% of treatment-naive patients (n = 5), respectively; no worsening of bone infiltration was observed. Liver, spleen, and hematologic parameters improved in treatment-naive patients and remained stable in treatment-switched patients. Conclusions: The qualitative real-world data support findings from clinical trials suggesting the long-term effectiveness of velaglucerase alfa on GD1 bone manifestations. When MRI assessment by radiologists with experience of GD is not possible, a simplified qualitative assessment may be sufficient in clinical practice for monitoring bone disease progression and treatment response.

BACKGROUND: To evaluate the efficacy of PET/CT using18F-FDG (18F-fluorodeoxyglucose) as a radiotracer compared to conventional bone marrow biopsy (BMB) in detecting infiltration to bone marrow (BM) in pediatric patients with lymphoma at the time of initial diagnosis.
METHODS: 66 pediatric patients with lymphoma (47Hodgkin\'s and 19non-Hodgkin\'s lymphoma) were referred for initial staging by18F-FDG PET/CT study. All patients underwent bilateral iliac BMB and 18F-FDG PET/CT scan with no more than 2 weeks interval in-between. Follow-up for at least 6 months was used as a reference standard to compare diagnostic performance between two modalities in detecting bone marrow infiltration (BMI).
RESULTS: Sensitivity, specificity, accuracy, as well as positive and negative predictive values of 18F-FDG PET/CT in detecting BMI were (80%, 86%, 85%, 63%, and 94%) in contrast to BMB (80%, 53%, 59%, 33%, and 90%) respectively. 18F-FDG PET/CT was concordant to BMB in 39/66 patients (59%).
CONCLUSIONS: 18F-FDG PET/CT was more accurate and specific, with higher predictive values than BMB in detecting BMI during initial staging of pediatric lymphoma. In most pediatric lymphoma patients, 18F-FDG PET/CT can be used instead of BMB to determine BMI during their initial staging process.

UNASSIGNED: Conventional computed tomography (CT) has low sensitivity for the diagnosis of bone marrow infiltration in nonosteolytic multiple myeloma (NOL-MM). This study aimed to compare the performance of the two-material decomposition technique of spectral CT with the removal of X-ray absorption components of calcium (Ca) versus that of hydroxyapatite (HAP) for diagnosis of NOL-MM.
UNASSIGNED: From October 2022 to March 2023, a total of 41 consecutive patients with MM without focal bone lesions undergoing chest spectral CT and thoracic spine magnetic resonance imaging (MRI) in Fujian Medical University Union Hospital were prospectively enrolled; meanwhile, another set of 41 age- and sex-matched healthy consecutive participants were selected as a comparison group. Based on MRI findings, patients with MM were classified with a diffuse infiltration pattern MM (DP-MM) or a normal pattern MM (NP-MM). Regions of interest (ROIs) were manually drawn on vertebrae. CT values of 70-keV images and basic material density within the ROIs were stored. The basic two-material pairs included a Ca-related pair (Ca-X) and an HAP-related pair (HAP-X), with X referring to fat, water, or muscle. Material density values DCa(X), DX(Ca), DHAP(X), and DX(HAP) were each used to diagnose MM, and the area under the receiver operating characteristic curve (AUC) was used to assess diagnostic performance.
UNASSIGNED: The 41 patients with NOL-MM included 30 with DP-MM and 11 with NP-MM. CT value, DCa(X), and DHAP(X) were comparable between the NOL-MM, DP-MM, NP-MM, and comparison groups. DX(HAP) was better than DX(Ca) for distinguishing the NOL-MM group from the comparison group {AUC [95% confidence interval (CI)], 0.874 (0.800, 0.949) vs. 0.737 (0.630, 0.844); P=0.02}, the DP-MM group from the comparison group [AUC (95% CI), 0.933 (0.878, 0.989) vs. 0.785 (0.677, 0.894); P=0.01], the NP-MM group from the comparison group [AUC (95% CI), 0.714 (0.540, 0.888) vs. 0.605 (0.429, 0.782); P=0.03], and the DP-MM group from the NP-MM group [AUC (95% CI), 0.809 (0.654, 0.964) vs. 0.736 (0.566, 0.907); P=0.049]. The diagnostic performance of DX(HAP) and DX(Ca) was influenced only by the removed material, while the X material had no influence.
UNASSIGNED: The spectral CT two-material decomposition technique with removal of X-ray absorption components of HAP is useful for diagnosis of NOL-MM, irrespective of the paired material.

UNASSIGNED: To determine diagnostic accuracy of 18F-FDG PET - CT imaging in determining Bone marrow involvement in pediatric HL by taking bone marrow biopsy as standard.
UNASSIGNED: This descriptive cross-sectional study was conducted in the Department of Pediatric Hematology/Oncology, Indus Hospital and Health Network, Karachi from July 2021 to December 2022. Treatment naïve histologically proven pediatric HL patients of both gender and aged between two to 16 years with both 18F-FDG PET - CT and bone marrow biopsy imaging were included. Basic demographics such as age, gender, height, weight, as well as classification and staging of HL was obtained. Results were assessed by expert reviewers who were blinded to clinical outcome. Sensitivity, specificity, positive and negative predictive value, and diagnostic precision were assessed. The data was analyzed via SPSS 26.0.
UNASSIGNED: Total 131 participants were included with a male predominance i.e. 104 (79.6%). The mean (±SD) age was 8.7 ± 3.4 years. The present study reported PET/CT to have a sensitivity, specificity diagnostic accuracy, PPV and NPV of 94.1%, 92%, 92%, 64% and 99% respectively.
UNASSIGNED: Our findings support the idea that BMB should not be routinely conducted in all patients but rather can be reserved exclusively for patients with dubious 18F-FDG bone marrow findings, as this test has strong diagnostic potential for evaluating BMI involvement in HL.

UNASSIGNED: Diffuse large B-cell lymphoma (DLBCL) is often associated with bone marrow infiltration, and 2-deoxy-2-(18F) fluorodeoxyglucose positron emission tomography/computed tomography ( 18F-FDG PET/CT) has potential diagnostic significance for bone marrow infiltration in DLBCL.
UNASSIGNED: A total of 102 patients diagnosed with DLBCL between September 2019 and August 2022 were included. Bone marrow biopsy and 18F-FDG PET/CT examinations were performed at the time of initial diagnosis. Kappa tests were used to evaluate the agreement of 18F-FDG PET/CT with the gold standard, and the imaging features of DLBCL bone marrow infiltration on PET/CT were described.
UNASSIGNED: The total detection rate of bone marrow infiltration was not significantly different between PET/CT and primary bone marrow biopsy ( P = 0.302) or between the two bone marrow biopsies ( P = 0.826). The sensitivity, specificity, and Youden index of PET/CT for the diagnosis of DLBCL bone marrow infiltration were 0.923 (95% CI, 0.759-0.979), 0.934 (95% CI, 0.855-0.972), and 0.857, respectively.
UNASSIGNED: 18F-FDG PET/CT has a comparable efficiency in the diagnosis of DLBCL bone marrow infiltration. PET/CT-guided bone marrow biopsy can reduce the misdiagnosis of DLBCL bone marrow infiltration.

We investigated the diagnostic performance of dual-energy CT (DECT) virtual non-calcium (VNCa) and Rho/Z images for bone marrow infiltration of primary malignant bone tumors (PMBTs).
We retrospectively analyzed 65 patients with PMBT who underwent DECT and MRI within 2 weeks. DECT was used to evaluate the presence and extent of marrow involvement surrounding PMBTs using the SCT, VNCa, and Rho/Z images. MRI was used as the reference standard for measurements. CT values of normal and involved bone marrow areas were measured on VNCa images, and Zeff values were measured on Rho/Z images. The statistical methods used were the 2*C chi-square test, ANOVA test, paired samples t test, and diagnostic performance of the different variables were evaluated using receiver operating characteristic curves.
VNCa and Rho/Z images showed higher accuracy (91%, 92% vs. 67%) and sensitivity (90%, 92% vs. 69%) than SCT images for diagnosing bone marrow infiltration in patients with PMBT. The maximum longitudinal diameter of tumor involvement measurements was statistically different between VNCa and SCT, Rho/Z and SCT, MRI, and SCT (all p < 0.05, p = 0.047, p = 0.049, and p = 0.023, respectively). The maximum transverse diameter was statistically significant between SCT and MRI, VNCa and MRI, Rho/Z and MRI (all p < 0.05, and p = 0.015, and p = 0.044, and p = 0.047, respectively). The HU or Zeff values based on the area of interest of VNCa and Rho/Z images differed significantly between the normal and infiltrated bone marrow area (p < 0.001). Receiver operating characteristic curve analysis revealed area under the curves of 0.995 and 0.988, respectively, with cut-off values of -31.57 HU and 7.8, and the sensitivity of both was 96.9%.
DECT-VNCa and Rho/Z images have good diagnostic value when evaluating bone marrow infiltration in PMBTs.

Gastric cancer (GC) is a worldwide health condition of major concern, with gastric carcinoma with signet ring cell features being increasingly reported. A 61-year-old woman was admitted to the Emergency department with back pain, gastrointestinal complaints, and weight loss. A lumbar and hip computed tomography (CT) was performed and revealed multiple suspicious secondary bone lesions. Laboratory test results reported anemia, thrombocytopenia, and elevated alkaline phosphatase. On thoracic-abdominal-pelvic CT, multiple bone lesions suggestive of metastases were visible on the vertebral spine, ribs, pelvic bones, and proximal femurs, but no identifiable primary or visceral lesions were described. Upper endoscopy identified a gastric adenocarcinoma, and both gastric and bone lesions, especially bone lesions, contained a relevant amount of signet ring cells. The patient was referred to the Medical Oncology department, however, her condition evolved unfavorably. GC with restricted bone metastasis is rare at presentation and has a poor prognosis. Despite its infrequency, clinicians should consider GC involvement when evaluating secondary suspicious bone lesions.

Non-Hodgkin\'s lymphoma and acute myeloid leukemia are both hematological malignancies that rarely coexist at the time of initial diagnosis. We present a case of non-Hodgkin lymphoma and acute myeloid leukemia diagnosed on the first admission.
Lymphoma and leukemia, both malignant hematological cancers, are primarily different diseases, with a majority of cases originating independently. The co-occurrence of lymphoma and leukemia at the time of the first diagnosis is extremely rare, and few relevant reports exist in the medical literature. We describe a case of a patient with non-Hodgkin\'s lymphoma and acute myeloid leukemia, a very rare occurrence.
A 57-year-old man complained of fatigue and neck tumors. A physical examination revealed several enlarged superficial lymph nodes throughout the body. On admission, routine blood tests revealed anemia, thrombocytopenia, and normal counts of white blood cells. Cytology of two cervical lymph nodes indicated non- Hodgkin\'s lymphoma, 18F-PET/CT: multiple enlarged lymph nodes with hypermetabolism, diffuse hypermetabolism of the bone marrow, suggesting lymphoma infiltration in the bone marrow, and a bone marrow biopsy revealed acute myeloid leukemia. Ultimately, the patient was diagnosed with non-Hodgkin\'s lymphoma and acute myeloid leukemia.
Primary bilineage hematological malignancies are rare, and the mechanism underlying their incidence is unknown. Infiltration of the bone marrow by lymphoma or leukemia can result in diffuse hypermetabolism, mostly diagnosed via bone marrow biopsy.

BACKGROUND: Over the past 20 years, we have gained a deep understanding of the biological heterogeneity of diffuse large B cell lymphoma (DLBCL) and have developed a range of new treatment programs based on the characteristics of the disease, bringing us to the era of immune-chemotherapy. However, the effectiveness and molecular mechanisms of targeted-immunotherapy remain unclear in DLBCL. Targeted-immunotherapy may be beneficial for specific subgroups of patients, thus requiring biomarker assessment.
METHODS: Here, we report a case of MCD subtype DLBCL with MYD88L265P and CD79B mutations, considered in the initial stage as lymphoplasmic lymphoma (LPL) or Waldenstrom macroglobulinemia (WM). Flow cytometry supported this view; however, the immunohistochemical results of the lymph nodes overturned the above diagnosis, and the patient was eventually diagnosed with MCD subtype DLBCL. The presence of a monoclonal IgM component in the serum and infiltration of small lymphocytes with a phenotype compatible with WM into the bone marrow led us to propose a hypothesis that the case we report may have transformed from LPL/WM.
CONCLUSIONS: This highlights the possible transformation from WM to DLBCL, CD79B mutation may be a potential biomarker for predicting this conversion.