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Lymphoproliferative disorder (LPD) associated with viral reactivation is a known risk of immunocompromised patients. With development of novel cellular therapies utilizing lymphodepletion regimens in advanced cancer, the risk of LPDs should be a consideration. Here, we report a case of a 61-year-old treated male with history of metastatic synovial sarcoma and multiple treatment lines treated with cell therapy (lymphodepleting chemotherapy and afami-cel, formerly ADP-A2M4, T-cell treatment) on clinical study that developed Epstein Barr virus-positive LPD. Patient was treated with rituximab and achieved a complete response. New cellular therapies present promising treatment options for patients and adverse events should be monitored carefully.

Cold agglutinin disease (CAD) is a difficult-to-treat autoimmune hemolytic anemia and B cell lymphoproliferative disorder associated with fatigue, acrocyanosis, and a risk of thromboembolic events. Cold-induced binding of autoantibody agglutinates red blood cells and triggers the classical complement pathway, leading to predominantly extravascular hemolysis.
This review summarizes clinical and experimental antibody-based treatments for CAD and analyzes the risks and benefits of B cell and complement directed therapies, and discusses potential future treatments for CAD.
Conventional treatment of CAD includes a B cell targeted treatment approach with rituximab, yielding only limited treatment success. The addition of a cytotoxic agent (e.g. bendamustine) increases efficacy, but this is accompanied by an increased risk of neutropenia and infection. Novel complement directed therapies have emerged and were shown to have good efficacy against hemolysis and safety profiles but are expensive and unable to address circulatory symptoms. Complement inhibition with sutimlimab may be used as a bridging strategy until B cell directed therapy with rituximab takes effect or continued indefinitely if needed. Future antibody-based treatment approaches for CAD involve the further development of complement directed antibodies, a combination of rituximab and bortezomib, and daratumumab. Non-antibody based prospective treatments may include the use of Bruton tyrosine kinase inhibitors.

A 37-year-old, previously healthy woman presented during her first trimester of pregnancy with a two-week history of rapidly progressive proptosis in the left eye. Clinical examination revealed limited left supraduction and diplopia in upward gaze. Orbital magnetic resonance imaging showed a medial orbital mass adjacent to the globe with secondary proptosis. Pathologic examination of a biopsied specimen of the orbital mass and subsequent immunophenotyping by flow cytometry revealed an extranodal marginal zone B-cell lymphoma. Clinical and histological features as well as a review of the literature are described.

UNASSIGNED: A 36-year-old man presented with a palpable mass in the right axillary tail for four months. He was referred to breast imaging for diagnostic work-up. He does not have a family history of breast cancer.
UNASSIGNED: Breast imaging work-up for diagnosis of lymphoma is unusual and even more so in a male patient.
UNASSIGNED: After Breast Mammography and targeted Ultrasound of the axillary tail and axilla, Magnetic Resonance Imaging (MRI) was performed and suggested lymphoproliferative disorder. Excisional biopsy was performed after the breast MRI with removal of right axillary tissue measuring 15.0 × 5.5 × 2.0 cm and containing multiple lymph nodes. Excisional biopsy revealed Classic Hodgkin lymphoma of nodular sclerosis type. Staging [18F]-FDG PET/CT revealed early stage of disease.
UNASSIGNED: The presentation and diagnostic elements of Hodgkin Lymphoma are described in this case report emphasizing the significance of breast imaging in multiple populations.

Castleman disease is a rare lymphoproliferative disease commonly occurring as a benign localized mass of lymph nodes in the mediastinum. Given that Castleman disease presents as asymptomatic or through non-specific thoracic symptoms, detection is considered complex. Ultimately, surgical resection is the preferred course of action with a greater than 90% relapse-free survival and no malignant transformation reported. In this article, we describe the case of a 34-year-old male with an unclear smoking history who was diagnosed with hyaline-vascular Castleman disease. We focus on optimizing diagnosis and management through the application of radiological imaging modalities, including computed tomography scans.

BACKGROUND: Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous group of lymphoid proliferations occurring after solid organ or bone marrow transplantation. The primary aims of our study were to characterize cumulative incidence of PTLDs, clinical and pathological features according to the Epstein-Barr virus (EBV) status and survival.
METHODS: This was a retrospective cohort study on adult and pediatric patients, from January 2001 to December 2017. The cumulative incidence of PTLD was calculated by analyzing all the patients transplanted at our hospital, based on the database of the Organ Donation and Ablation Authority of Argentina (INCUCAI). The Kaplan-Meier method was used to plot the survival.
RESULTS: Fifty-eight cases of biopsy-confirmed PTLD were identified and 12 cases of clinical data were incomplete and these patients were excluded. The median age at the time of the PTLD diagnosis was 17.5 years (interquartile range [IQR] 9 - 57). The median interval between transplant and PTLD diagnosis was 39 months (IQR 9 - 113). The most commonly transplanted organ was the liver (24 cases, 52.2%), followed by kidney (20 cases, 43.5%). The Epstein-Barr encoding region in situ hybridization (EBER ISH) was positive in 29 (69.8%) of the 43 evaluable biopsies. The PTLD cumulative incidence was 1.84% (95%CI 1.77 - 1.91) for solid organ and 0.84% (95%CI 0.48 - 1.2) for bone marrow transplant patients. The overall survival rate at 5 years was 0.77 (95%CI 0.61 - 0.87). Subgroups by the EBV EBER status, transplant type, PTLD subtype and age group (adult vs. pediatric) showed no statistically significant association with the overall survival.
CONCLUSIONS: The PTLD incidence was similar to that of previous series and the EBER did not appear as a relevant factor in our patient survival.

Two major steps are identified in the pathogenesis of cold agglutinin disease; clonal B-cell lymphoproliferation and complement-mediated hemolysis. Each of these steps constitutes a target for treatment. In this focused review, we address 2 successful therapeutic approaches; the bendamustine plus rituximab combination as a highly efficacious B-cell directed therapy and the anti-C1s monoclonal antibody sutimlimab as the most extensively studied complement-targeting therapy. We describe and discuss the prospective study of bendamustine plus rituximab and 2 recent, prospective studies of sutimlimab. Bendamustine-rituximab results in a high response rate, frequent complete responses and long median response duration, and the treatment is temporary. However, this therapy is relatively slow-acting and associated with some toxicity. Sutimlimab is also highly efficacious, is far more rapidly acting, and is low-toxic. Disadvantages of sutimlimab are the lack of effect on circulatory symptoms, the probable need for indefinite treatment, and the very high costs. In cold agglutinin disease patients who require treatment, the choice should be based on an individual assessment.

BACKGROUND: Castleman disease, which was first described by Dr. Castleman in 1954, is relatively rare and represents a spectrum of heterogeneous lymphoproliferative disorders with characteristic histological features on biopsy. It is classified based on body location and histology with variable clinical presentations. Its treatment depends on the subtype, and preoperative embolization for Castleman disease has rarely been discussed in the literature.
METHODS: A 22-year old man presented to the ENT clinic with a four-week history of a mass on the left side of the neck, which was associated initially with headache, fever, and fatigue for 2 days. Contrast tomography and magnetic resonance imaging revealed a hypervascular mass located at levels two and three of the left side of the neck with feeding vessels from the external carotid artery. Preoperative embolization was planned; however, the neurointerventionist considered it a lymph node that did not need embolization. Surgical excision was performed with relatively increased operative time and bleeding. A biopsy confirmed a hyaline-vascular type Castleman disease.
CONCLUSIONS: We reviewed the evidence-based management of CD. We reviewed the available literature on the role of preoperative embolization in management.
CONCLUSIONS: Based on published articles and the hypervascular nature of the disease, we believe that preoperative embolization helps decrease morbidity.