Pediatric-type follicular lymphoma

  • 文章类型: Case Reports
    背景:接受免疫抑制剂的自身免疫性疾病患者有发生非霍奇金淋巴瘤(NHL)的风险。维多珠单抗(抗α4β7整合素抗体),克罗恩病(CD)的首选治疗方法,减少炎性淋巴细胞运输到肠粘膜。这种作用被认为局限于结肠。
    方法:我们报告了一例使用维多珠单抗治疗5年的CD患者,其发展为小儿型滤泡性淋巴瘤。治疗前的检查显示,循环T淋巴细胞减少,对有丝分裂原的反应受到抑制。利妥昔单抗,环磷酰胺,长春新碱,和泼尼松化学免疫疗法导致持久的淋巴瘤缓解,维多珠单抗继续治疗.而患者的T淋巴细胞群和免疫球蛋白产生恢复,T淋巴细胞有丝分裂原反应仍然受到抑制。
    结论:该患者的NHL可能与接受抗α4β7治疗有关。进一步的研究可能有助于确定是否在接受维多珠单抗治疗的患者中对NHL和其他全身性疾病进行主动监测。
    BACKGROUND: Patients with autoimmune conditions receiving immunosuppressants are at risk of non-Hodgkin lymphomas (NHL). Vedolizumab (anti-α4β7-integrin antibody), a treatment-of-choice for Crohn\'s disease (CD), reduces inflammatory lymphocyte trafficking into the intestinal mucosa. This effect is believed to be confined to the colon.
    METHODS: We report the case of a CD patient on vedolizumab for five years who developed pediatric-type follicular lymphoma. Work-up prior to therapy revealed a reduction in circulating T-lymphocytes and their suppressed response to mitogens. Rituximab, cyclophosphamide, vincristine, and prednisone chemo-immunotherapy resulted in durable lymphoma remission, and vedolizumab treatment was continued. While the patient\'s T-lymphocyte population and immunoglobulin production recovered, the T-lymphocyte mitogen response remained suppressed.
    CONCLUSIONS: This patient\'s NHL may be linked to receiving anti-α4β7 therapy. Further research could be beneficial to determine if proactive surveillance for NHL and other systemic diseases is indicated in patients on vedolizumab.
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  • 文章类型: Case Reports
    小儿型滤泡性淋巴瘤是一种影响成人和小儿患者组的头颈部淋巴结的疾病。眼部受累非常罕见,特别是在儿科年龄组;因此,保持高度的临床怀疑是必要的。这里,我们报道了一例15岁男孩的罕见结膜小儿型滤泡性淋巴瘤,表现为左球结膜内侧进行性肿胀2个月.在检查中,质量是坚定的,mobile,封装良好,广泛的基础,并有一个负面的过渡。进行了切除活检,组织病理学检查和免疫组织化学研究显示淋巴组织CD20、CD79a阳性,BCL6和CD10;并且对BCL2和MUM1阴性。CD21和CD23阳性突出显示存在扩大的滤泡树突状细胞网。该患者被诊断为结膜小儿型滤泡性淋巴瘤,并转诊至肿瘤中心进行进一步检查和治疗。这种淋巴瘤很罕见,需要高度的临床怀疑,因此,报告病例细节对于眼科医生和普通儿科从业者都很重要和有价值。
    Pediatric-type follicular lymphoma is a disease that affects the lymph nodes of the head and neck in the adult and pediatric patient groups. Ocular involvement is exceedingly rare, especially in the pediatrics age group; therefore, keeping a high clinical suspicion is warranted. Here, we report a rare conjunctival pediatric-type follicular lymphoma in a 15-year-old boy presenting with progressive swelling over the medial aspect of the left bulbar conjunctiva for two months. On examination, the mass was firm, mobile, well encapsulated, wide-based, and had a negative transillumination. An excisional biopsy was performed, and histopathological examination and immunohistochemistry studies revealed lymphoid tissue that was positive for CD20, CD79a, BCL6, and CD10; and negative for BCL2 and MUM1. The CD21 and CD23 positivity highlighted the presence of an expanded follicular dendritic cell meshwork. The patient was diagnosed with conjunctival pediatric-type follicular lymphoma and referred to an oncology center for further examination and treatment. This lymphoma is rare, requiring high clinical suspicion, and thus, reporting the case detail is important and valuable for ophthalmologists and general pediatrics practitioners alike.
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  • 文章类型: Case Reports
    BACKGROUND: Pediatric-type follicular lymphoma (PTFL) is a unique pathological type in the 4th edition of hematopoiesis and lymphoid tissue tumor classification revised by World Health Organization. It is unique in clinical practice and seldom seen in adult. PTFL mainly occurs in the head and neck lymph nodes. Most of the cases are short of fever, night sweat, weight loss, and other B symptoms which substitute for lymphadenopathy as the main symptom. PTFL can be disposed of surgical resection and it can achieve long-term tumor-free survival, and it has an excellent outcome.
    METHODS: Two cases of PTFL were reported and their clinicopathological features, differential diagnosis, therapy and prognosis were discussed. PTFL showed gray-brown tough texture in general performance. The histological manifestations of PTFL were similar to that of adult-follicular lymphoma (FL). Under low power microscope, the structure of lymph nodes was destroyed in different degree, the follicles were closely arranged, expanded and irregular, and the mantle zone became thin or disappeared. In addition, the \"starry sky phenomenon\" could be seen. At high magnification, the follicles were mainly composed of single medium-sized central cells, and some of them mainly consisted of centroblastic cells to characterize scattered chromatin and inconspicuous nucleoli. Immunohistochemical showed the tumor cells expressed CD20, PAX5, CD79a and CD10, BCL6, FOXP-1, which were limited in germinal center; Ki-67 was highly expressed in germinal center. CD21 and CD23 showed nodular and expanded follicular dendritic cells. Immunoglobulin gene rearrangement was positive for IGH and IGK. The two patients underwent surgical resection with no complications. After discharge, the two patients with a close review for 18 mo and 5 mo respectively and showed no evidence of recurrence.
    CONCLUSIONS: PTFL in adult is generally supposed to be extremely rare. PTFL displayed characteristic morphological, immunophenotypic, and molecular biological changes which are a kind of neoplasm with satisfactory prognosis after surgical excision.
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  • 文章类型: Journal Article
    Pediatric-type follicular lymphomas are rare lymphomas, affecting mostly children and young adults. These are characterized by an excellent prognosis, despite their conservative therapeutic approach. Recognized as an entity in the most recent 2016 WHO classification of tumors of hematopoietic and lymphoid tissues, its diagnosis is based on the recognition of an exclusively nodular architecture, thus conditioning the possibility of a cytological diagnosis. It is thus not odd, the scant literature found on the cytological approach to these lesions. Herein we describe a case of a pediatric-type follicular lymphoma, first approached through fine needle biopsy. The case is thoroughly discussed from a cytologic, immunophenotypic, and molecular point of view. Differential diagnoses are discussed. The final diagnosis was performed on histology.
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  • 文章类型: Case Reports
    BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a relatively common tumor of the central nervous system. Although PCNSLs generally arise from the parenchyma, lymphomas arising primarily from the dura are termed dural lymphomas (DLs). Mucosa-associated lymphoid tissue is the most unique histological feature of DLs. Because of its rarity, the clinical characteristics of and treatments for DL have not been fully elucidated to date.
    METHODS: A 31-year-old man with no past medical history presented with numbness in his left upper limb. Magnetic resonance imaging revealed a dural-based tumoral lesion and cervical lymphadenopathies. The lesion was diagnosed radiologically as a meningioma, and tumor resection was planned. However, an intraoperative pathological diagnosis showed neoplastic lymphocytes, and the planned total tumor resection was halted. Histologically, the tumor was characterized by areas of poorly defined follicular architecture consisting of medium and large centroblasts. These tumor cells were immunohistologically positive for CD10 and CD20 and negative for B-cell lymphoma (BCL) 2 and BCL6. Fluorescence in situ hybridization did not show evidence of an Immunoglobulin H/BCL2 fusion. The lesion was subsequently diagnosed as a pediatric-type follicular lymphoma (PTFL). Six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) plus 2 cycles of rituximab were administered. The patient showed no evidence of relapse at 12 months after diagnosis, and follow-up was ongoing at the time of this report.
    CONCLUSIONS: This very rare case of DL was originally diagnosed as a PTFL. The tumor could be treated by immunochemotherapy alone.
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