UNASSIGNED: Mogamulizumab is an anti-C-C chemokine receptor 4 antibody that is increasingly being used to treat T-cell malignancies such as cutaneous T-cell lymphoma, adult T-cell leukemia-lymphoma, and peripheral T-cell lymphoma. Because CCR4 is expressed on both malignant T-cells and regulatory T-cells (Tregs), mogamulizumab can be associated with increased immune-related adverse events (irAEs). While there is abundant literature on mogamulizumab-associated rash (MAR) and graft-versus-host disease (GVHD), other reported irAEs have not been collated into a single review.
UNASSIGNED: This narrative review covers irAEs associated with mogamulizumab in patients with T-cell lymphomas, focusing on events other than MAR and GVHD. We searched PubMed and Google Scholar for case reports, case series, chart reviews, and clinical trials published from inception to March 2024. Identified events include alopecia, vitiligo, arthritis, psoriasis, myocarditis, myositis/polymyositis, hepatitis, and others.
UNASSIGNED: Mogamulizumab\'s ability to augment the host immune response through Treg depletion adds to its efficacy but has wide-ranging implications for autoimmunity across multiple organ systems, similar to immune checkpoint inhibitor therapy. Occurrence of irAEs may be associated with improved overall clinical response, although long-term follow-up studies are needed.

The patient presented to the clinic with painful muscle swelling in the right lower extremity, which improved with immunosuppressive therapy. Initially, the condition was diagnosed as polymyositis but recurred soon after. After imaging and biopsy, the final diagnosis was primary skeletal muscle peripheral T-cell lymphoma, not otherwise specified (PSM-PTCL, NOS). In this report, we discuss the challenges in diagnosing and treating this aggressive malignancy and review the literature on PSM-PTCL, NOS. Key Points • To date, there are few reports of PSM-PTCL, NOS, and our case is the tenth. • It is crucial to consider PSM-PTCL, NOS, when presenting with localized muscle edema and unexplained pain. • Histopathological examination is likely the most effective method for diagnosing this rare disease.

Intravascular cutaneous anaplastic large cell lymphoma (ALCL) is an extremely rare non-Hodgkin lymphoma that proliferates in the lumen of small blood vessels and has a propensity to manifest in the skin. Most reported cases of intravascular lymphoma described in the literature are of large B-cell lymphomas, making T-cell lymphomas incredibly rare. As such, we present the case of an 87-year-old male with primary cutaneous intravascular anaplastic large T-cell lymphoma that initially presented with an erythematous, subcutaneous nodule on the right mid-abdomen. We report the immunohistochemical results showing lymphoma cells staining positively for CD3 and CD30 and lacking expression of anaplastic lymphoma kinase, pan-cytokeratin, CD10, CD20, and SOX10. We also review and compare previously reported cases of intravascular ALCL with primary cutaneous involvement.

Composite lymphoma (CL) involving B-cell lymphoma and T-cell lymphoma is extremely rare. Herein, we report three such cases using immunohistochemistry, flow cytometry, and the next-generation sequencing (NGS) to identify the pathological and molecular characteristics of CL. In the first case, the patient was admitted to hospital for generalized pruritic maculopapular rash over the whole body. An excisional biopsy of the skin lesions showed T-cell lymphoma. At the same time, the staging bone marrow (BM) biopsy revealed a diffuse large B-cell lymphoma (DLBCL). After R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapies, the patient produced a good response with substantial dissipation of the rashes and relief of skin. The other two patients were admitted to hospital due to lymphadenopathy and were diagnosed with DLBCL and follicular lymphoma (FL) after core needle biopsy of lymph nodes, BM biopsy, BM aspiration, and flow cytometry. Following R-CHOP and R-COP (rituximab, cyclophosphamide, vincristine, and prednisone) therapies, they achieved complete remission unconfirmed (CRu) and complete remission (CR). However, one or two years later, they suffered a relapse of lymphadenopathy. The shocking fact was that re-biopsy of lymphadenopathy revealed peripheral T-cell lymphoma (PTCL) and angioimmunoblastic T-cell lymphoma (AITL). NGS findings identified DNA methyltransferase 3a (DNMT3a), isocitrate dehydrogenase 2 (IDH2), Ras homolog gene family, member A (RHOA), splicing factor 3B subunit 1 (SF3B1), and tumor protein p53 (TP53) mutations. After immunochemotherapy, these patients achieved CRu and CR again. Nevertheless, they suffered a second relapse of T-cell lymphoma. Finally, they died due to progression of disease. We found that the occurrence of CL is associated with Epstein-Barr virus infection and DNMT3a, IDH2, and TP53 mutations, and the prognosis of the disease is closely related to the T-cell lymphoma components.
复合性B细胞和T细胞淋巴瘤发病率很低。为了研究复合性淋巴瘤的临床、病理和分子学特征，本文报道了三例复合性B细胞和T细胞淋巴瘤患者，并通过免疫组化、流式细胞术和二代测序检测分析患者的病理和分子学特征。第一例患者通过皮肤活检、骨髓活检和流式细胞术明确诊断为皮肤T细胞淋巴瘤和骨髓弥漫大B细胞淋巴瘤。另外两例患者通过淋巴结粗针穿刺活检和骨髓活检明确诊断为B细胞淋巴瘤，但疾病复发后再次经过病理活检明确诊断为T细胞淋巴瘤，同时二代测序检测发现了DNA甲基转移酶3a（DNMT3a）和肿瘤蛋白p53（TP53）等基因突变。在此基础上，本文回顾了复合性淋巴瘤的相关文献，并总结了复合性B细胞和T细胞淋巴瘤的临床、病理和分子特征。我们发现复合性淋巴瘤的发病和EB病毒感染，以及DNMT3a、异柠檬酸脱氢酶2（IDH2）和TP53突变等相关，同时该疾病的预后与侵袭性更高的T细胞淋巴瘤成分密切相关。.

A 72-year-old Chinese male presented with unilateral left eye panuveitis, then diagnosed as bilateral T-cell primary vitreoretinal lymphoma (T-PVRL) through chorioretinal biopsy and immunohistochemistry. No CNS nor systemic involvement was found at diagnosis. Despite initiating intravenous and intrathecal chemotherapy and intravitreal methotrexate, the disease eventually spread to the fellow eye with subsequent recurrence and systemic metastasis. To our knowledge, no cases of T-PVRL treated in a silicone-filled eye were reported in the literature. T- PVRL is exceedingly rare, with most PVRL being the malignant B-cell variant. This case highlights the challenges encountered throughout the treatment course of this aggressive entity, including the administration of intravitreal methotrexate in a silicone oil-filled eye. The poor overall survival rate and grim prognosis of T-PVRL are highlighted. Therefore, we recommend prompt tissue biopsy and immediate initiation of systemic chemotherapy and intravitreal methotrexate.

Primary pancreatic lymphoma (PPL) is an extremely rare type of non-Hodgkin\'s lymphoma (NHL). It accounts for 0.1% of all lymphomas and less than 1% of pancreatic tumors. Within this subtype, T-cell lymphomas only account for up to 6.7% of pancreatic lymphomas. In this study, we present the case of a 78-year-old Hispanic man who presented with obstructive jaundice associated with a mass within the head of the pancreas; pathologic analysis of the tumor revealed a mature T-cell lymphoma, not otherwise specified (NOS).

The Epstein-Barr virus is closely linked to a lymphoproliferative disease known as natural killer/T-cell lymphoma (NKTL). Early identification of NKTL might be challenging because it can resemble other nasopharyngeal pathologies. Contrary to the presented case, T-cell lymphoma often develops in the nasal canal and spreads to the oral cavity. Here, we present the case of a 45-year-old man with an unusual presentation of NKTL presenting initially as acute follicular tonsillitis.

Lymphomas are solid tumors of the immune system and include 14% of all head and neck malignancies. Non-Hodgkin lymphomas (NHLs) are a heterogeneous group of lymphoproliferative disorders originating in B-, T-, or natural killer T-cells. They have a wide range of histological appearances and clinical features at presentation, which can make diagnosis difficult. A 58-year-old male patient presented with a 1-month history of swelling in the upper right back tooth region, which developed after extraction. On intraoral examination, there was small nodular lesion proliferation from the extracted socket. Biopsy specimen on histological examination revealed sheets of small round cells with hyperchromatic nucleus resembling lymphoblast. Immunohistochemistry (IHC) confirms the NHL of T-cell origin. This article is an attempt to correlate the clinical presentation and histological importance of small round cell tumors of the jaw and to discuss the differential diagnosis of small round cell tumors. Typically, a multimodal approach is employed, and the principal ancillary technique that have been found to be useful in classification is IHC.

Purpose Primary cutaneous T-cell lymphoma (CTCL) is a rare diagnosis, and the subset primary cutaneous peripheral T-cell lymphoma (pcPTL) is even more uncommon. Both CTCLs and pcPTLs typically occur in the head and neck. The authors aim to evaluate the literature for reports of presentation in the hand. Materials and Methods A case report of a 77-year-old man with pcPTL of the hand is presented. Oncologic workup revealed an independent diagnosis of medullary thyroid carcinoma. A review of the literature was performed using the following search terms in the PubMed database: primary, cutaneous, T-cell lymphoma, peripheral presentation, and hand. One case of pcPTL in the hand was identified and included in this study. Results One case report in the literature was identified describing a 78-year-old man with a 1-year history of a progressive hand lesion. Biopsy revealed pcPTL. Conclusion This report presents a rare presentation of pcPTL in the hand, reviews the current literature, and provides insight into management. Prompt biopsy of any unresolving lesion of the hand is crucial to expedite diagnosis and treatment of otherwise difficult to diagnose pathologies. Increased awareness of rare malignancies is important to avoid delay in patient care and to improve outcomes.

BACKGROUND: Anaplastic large cell lymphoma (ALCL) is a type of T-cell lymphoma that can be divided into two categories: anaplastic lymphoma kinase-positive (ALK+) and ALK-negative. Gastrointestinal ALK+ ALCL is rare. Multiple lymphomatous polyposis (MLP) is thought to be a representative form of gastrointestinal lesion in mantle cell lymphoma, and T-cell lymphomas seldom show this feature. Here, we report the first known case of ALK+ ALCL with gastroduodenal involvement to present with MLP.
METHODS: The patient was a 43-year-old man who was complained of a mass in the left inguinal area and was performed open biopsy. ALK+ ALCL was diagnosed pathologically. Computed tomography scan demonstrated multiple lymph node lesions in the abdomen - pelvis/inguinal region, and scattered nodular lesions in both lung fields. He did not complain of gastrointestinal symptoms. While, esophagogastroduodenoscopy identified MLP lesions from the antrum of the stomach to the descending portion of the duodenum and mild thickened folds on the corpus of the stomach, and biopsy showed invasion of ALK+ ALCL. We treated this patient with six cycles of CHOEP (Cyclophosphamide, Doxorubicin, Vincristine, Etoposide, and Prednisone) chemotherapy. At the conclusion of treatment, there was complete remission. Numerous white scars were found on the stomach, endoscopically consistent with a remission image of lymphoma. The endoscopic features of this case were thought to be similar to those of MCL.
CONCLUSIONS: The macroscopic/endoscopic features of gastrointestinal ALK+ ALCL may be more similar to those of B-cell lymphomas rather than T-cell lymphomas.