UNASSIGNED: Primary gastrointestinal follicular lymphoma is a subtype of follicular lymphoma that originates directly from the gastrointestinal tract. Pathologically, it exhibits substantial similarities with the secondary gastrointestinal involvement observed in nodal follicular lymphoma. However, primary gastrointestinal follicular lymphoma presents clinically distinct features, necessitating divergent considerations in treatment selection compared with nodal follicular lymphoma.
UNASSIGNED: This narrative review focused on recent articles (2018-2023) regarding the long-term prognosis and treatment options for gastrointestinal follicular lymphoma. In addition, a brief overview of gastrointestinal follicular lymphomas is provided.
UNASSIGNED: Patients with primary gastrointestinal follicular lymphoma often present with a low tumor burden. Lymphoma lesions typically remain asymptomatic for several years or may undergo spontaneous regression without immediate treatment. Therefore, a \'watch and wait\' approach is justified. Conversely, when large tumor masses are identified in the gastrointestinal tract, the potential for tumor bleeding or intestinal obstruction requires timely therapeutic interventions.

Primary gastrointestinal non-Hodgkin lymphoma, while rare, most often presents as diffuse large B-cell lymphoma located in the stomach or ileocecal region. Presenting symptoms include abdominal pain, gastrointestinal bleeding, weight loss, or obstructive symptoms. Imaging can reveal ileitis or obstruction. We report a case of a man from Honduras with latent tuberculosis and chronic hepatitis B who presented with features of Crohn\'s disease through clinical, radiologic, and endoscopic findings but was ultimately diagnosed with diffuse large B-cell lymphoma by histology. We emphasize the importance of maintaining a broad differential for ileitis and the importance of histologic sampling when evaluating ileitis.

Primary extranodal non-Hodgkin lymphoma (NHL) is a rare manifestation of lymphoid malignancies and typically arises in tissues outside the lymph nodes and can involve various organs and anatomical sites presenting unique challenges in diagnosis and treatment. Multifocal primary extranodal NHL, characterized by simultaneous involvement of multiple extranodal sites, presents a diagnostic and therapeutic challenge due to its uncommon presentation and varied clinical manifestations. Despite advances in diagnostic modalities and treatment strategies for NHL, multifocal involvement poses unique clinical dilemmas requiring a multidisciplinary approach for accurate diagnosis and optimal therapeutic intervention. In this case report, we describe a rare case of multifocal primary extranodal NHL in a 29-year-old female, highlighting the complexities associated with its diagnosis and management. Through this case presentation, we aim to underscore the importance of recognizing and addressing the intricacies of multifocal primary extranodal NHL to enhance clinical outcomes and improve patient care. It also highlights the diagnostic complexity and clinical challenges associated with multifocal primary extranodal NHL. A multidisciplinary approach involving haematologists, oncologists, radiologists, and pathologists is crucial for the accurate diagnosis and optimal management of such cases. Additionally, further research is warranted to better understand the underlying pathogenesis and improve treatment strategies for this rare presentation of NHL.

The sigmoid colon is an uncommon site for the origin of primary malignant lymphomas in the GI tract. Additionally, immunosuppressive agents, widely used in treating autoimmune diseases, have been associated with the induction of malignancies, including lymphoproliferative disorders. In this report, we present a rare case of GI perforation suggesting a link between immunosuppressive therapy, particularly tacrolimus treatment, and diffuse large B-cell lymphoma (DLBCL). A 75-year-old female patient presented with abdominal pain to our ER. She had a medical history of polymyositis and interstitial pneumonia, treated with the immunosuppressant tacrolimus. An abdominal CT scan revealed free gas in the abdominal cavity, leading to a diagnosis of GI perforation. The patient exhibited generalized peritonitis and underwent emergency surgery the same day. During surgery, a perforation in the sigmoid colon was identified, and a Hartmann procedure was performed. Postoperative pathology showed CD20+, CD30+, CD5-, CD10-, BCL6+, MUM1+, and MIB-1 LI of 50-60%. The diagnosis of DLBCL was confirmed, classified as EBV-positive diffuse large B-cell lymphoma, not otherwise specified (NOS), in the sigmoid colon, with positive EBER-ISH, LMP-1, and EBNA2 findings. Given her history of immunosuppressant use, she was categorized as having other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPD), according to the WHO Classification of 2017. This case highlights the importance for clinicians to consider the risk of oncogenesis associated with the prolonged use of immunosuppressive agents.

BACKGROUND: Although anti-CD19 chimeric antigen receptor (CAR) T cell therapy was approved as a very effective salvage strategy in relapsed/refractory (R/R) B cell lymphoma, the experience in R/R gastrointestinal (GI) lymphoma is still insufficient.
METHODS: We summarized the efficacy and side effects of anti-CD19 CAR T-cell therapy in 12 patients with R/R GI lymphoma. Based on literature, the R/R GI lymphoma patients were divided into subgroups with different characteristics: Bulky/No bulky disease, Gastric/Gastrointestinal involvement, Gastrointestinal/Combined extra-gastrointestinal lesions, Ulcer/Lumps or nodules type, With/without gastrointestinal bleeding.
RESULTS: The objective response rate (ORR) was 66.67% in these 12 patients. The ORR was 83.33% in no bulky disease group, 80.00% in gastric involvement group, 100.00% in ulcer type group, and 80.00% in no gastrointestinal bleeding group. The CR rate was 33.33% in these 12 patients. The CR was 50.0% in no bulky disease group, 60.00% in gastric involvement group, and 80.00% in ulcer type group. The PFS and OS rate of the 12 patients at 6 months after infusion were 54.55% and 58.33%, respectively. The overall survival (OS) at 6 months was higher in no bulky disease group. There was no difference of the OS or the progression free survival (PFS) at 6 months between the other groups. The mean peak of CAR-T cells and Cytokine Release Syndrome (CRS) grade were higher in gastrointestinal lesions group. The mean peak of IFN-γ and CRS grade were higher in gastrointestinal bleeding group. Four out of six patients in group of gastrointestinal lesions group were patient with high tumor burden. Patients with gastrointestinal involvement only were at higher risk for gastrointestinal bleeding.
CONCLUSIONS: The ORR and CR of high tumor load, gastrointestinal involvement, lumps or nodules type and gastrointestinal bleeding group were lower. The CRS grade was higher in gastrointestinal lesions group and in gastrointestinal bleeding group. Patients with gastrointestinal involvement only were at higher risk for gastrointestinal bleeding.

Burkitt Lymphoma, an aggressive form of non-Hodgkin\'s lymphoma, is a rare cause of ileocolic intussusception in adult patients. We present the case of a 17-year-old male patient, with a history of non-Hodgkin\'s lymphoma in childhood, presenting with acute abdominal pain, vomiting, and diarrhea. CT and colonoscopy findings revealed ileocolic intussusception with a large ileocecal mass, leading to a diagnosis of Burkitt Lymphoma after histopathological and immunohistochemical examination. This case highlights the rarity of Burkitt Lymphoma causing intussusception in adults, a condition more commonly seen in children. The case also underscores the importance of considering Burkitt Lymphoma in patients with a history of non-Hodgkin\'s lymphoma presenting with acute abdominal symptoms. He was successfully treated with surgery without any complications. On follow-ups, he is doing well.

Monomorphic epitheliotropic T-cell lymphoma (MEITL) of gastrointestinal tract is an aggressive T-cell lymphoma that can rarely involve the brain. We present detailed descriptions of clinical and autopsy neuropathological findings of a rare case of an elderly woman who had surgery and chemotherapy for MEITL of the small intestine. Following her surgery, she progressively exhibited neurologic decline towards the end of her treatment. The patient eventually succumbed to her illness and was found to have MEITL with intracranial involvement on autopsy. Brain autopsy was performed and examination of tissues with hematoxylin-eosin staining under optical microscopy with 100 X magnification. Immunostaining for CD3, CD4, CD5, CD7, CD8, CD56, CD20, beta-amyloid, c-Myc, TCR-beta, TCR-delta, and EBER-ish was conducted on the formalin-fixed paraffin-embedded (FFPE) brain tissues. A neuropathological exam revealed multifocal friable necrotic and hemorrhagic areas in the supratentorial region. Histologically, monotonous small to medium-sized atypical lymphocytes infiltrated the brain parenchyma, prominently around the vessels. The immunophenotype of the atypical lymphocytes was positive for CD-3, CD-7, and CD-56 and negative for CD-5, CD-4, CD-8, CD-20, and c-Myc. EBER-ish was negative. The histology and immunophenotype confirmed the MEITL brain involvement. Neurologic decline and cognitive changes in patients with known MEITL can be the first clue of brain involvement upon which prompt evaluation is warranted.

The most commonly utilized protocols to treat lymphoma in cats employ vincristine, cyclophosphamide and prednisone; with additional drugs sometimes used including L-asparaginase and doxorubicin. Medical records were reviewed for 55 cats with alimentary lymphoma treated with a novel multiagent protocol using prednisolone, L-asparaginase, doxorubicin, vinblastine instead of vincristine, a higher dosage of cyclophosphamide and oral procarbazine (VAPC protocol). Outcomes evaluated were response to therapy, toxicity and progression-free survival (PFS). Grade 3 or 4 neutropenia was the most common treatment-related reason for chemotherapy dosage adjustment, occurring in 8 of 52 cats receiving vinblastine, 7 of 55 cats receiving cyclophosphamide and 1 of 40 cats receiving doxorubicin, but febrile neutropenia was identified in only two cats. Of 38 cats receiving chemotherapy for measurable disease, 26 (68.4%) achieved complete response (CR). Three cats achieved a partial response and 9 cats failed to achieve a remission. There were no identified factors influencing whether a cat was likely to achieve CR. For all 55 cats (including those receiving chemotherapy and surgery), median PFS was 184 days with 1, 2 and 3-year survival rates of 35.4%, 26.5% and 26.5%, respectively. On multivariate analysis, 40 cats that achieved CR had a median survival time of 341 days (78 days for PR, 45 days for NR); PFS times were also significantly affected by lymphocyte:monocyte L:M ratio (>3.4 = 700 days vs. ≤3.4 = 126 days) and B-cell versus T-cell phenotype (220 days vs. 42 days, respectively).

Lymphoproliferative disorders are a heterogeneous group of neoplasms with varying clinical, morphologic, immunophenotypic, and genetic characteristics. A subset of lymphomas have a proclivity for the gastrointestinal tract, although this region may also be involved by systemic lymphomas. In addition, a number of indolent lymphoproliferative disorders of the gastrointestinal tract have been defined over the past decade, and it is important to accurately differentiate these neoplasms to ensure that patients receive the proper management. Here, the authors review lymphoid neoplasms that show frequent gastrointestinal involvement and provide updates from the recent hematolymphoid neoplasm classification systems.

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