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Neuropathology Of Neuro-Ophthalmic Disorders
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Lymphomas can rarely involve orbit and ocular adnexal structures. Orbital lymphomas are usually seen in older age-group. Majority of the orbital lymphomas are B-cell type (97%). Among B-cell lymphomas, almost all types of lymphoma have been reported in the orbit. The low-grade B-cell lymphomas outnumber the high-grade B-cell lymphomas. Among the low-grade B-cell lymphomas in orbit, marginal zone lymphoma (MALToma) is the commonest type, followed by follicular lymphoma and mantle cell lymphoma. Low-grade B-cell lymphomas are composed of small size lymphocytes which are 1–1.5 times the size of mature lymphocytes, and they exhibit nodular to diffuse architecture. The MALTomas show presence of lymphoepithelial lesions. Among high-grade lymphomas, diffuse large B-cell lymphoma (DLBCL) is the commonest type. It shows diffuse architecture and is composed of large lymphoid cells, 2–3 times the size of mature lymphocytes containing frequent mitoses (Figure 25.19a). Immunohistochemistry is essential for exact categorization of the lymphoma, which is essential for adequate management (Figure 25.19a). MALToma and follicular lymphoma usually carry good prognosis, while mantle cell lymphoma and DLBCL are associated with poor prognosis.39,40
Haemato-Oncology
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Fine-needle aspiration (FNA) is commonly used in the initial investigation of neck lumps because it is quick, safe and diagnostic of many malignancies; but its role in lymphoma diagnosis has been controversial. Some lymphomas (especially HL) consist of small numbers of malignant cells within a reactive cellular background; in this situation FNA may yield only reactive cells and wrongly suggest an infective or inflammatory aetiology. This has led many organizations to advise against the use of FNA for suspected lymphoma, to avoid diagnostic delay or false negative results. However, increasing use of flow cytometry and immunocytochemistry has enabled many lymphomas to be accurately defined cytologically and the ability of this technique to provide fresh samples for DNA microarray analysis suggests that there may be an increasing role for FNA in the future.28 Centres with sufficient expertise in these techniques are able to use FNA for preliminary diagnosis, although excision biopsy is often performed for confirmation and uncertainty can still arise with certain low grade B-cell malignancies, such as marginal zone lymphoma, where the markers themselves are not specific and an appreciation of tissue architecture is needed.29 Overall we would still advocate proceeding directly to whole node biopsy when symptoms are highly suggestive of lymphoma, and using FNA only when there are several possible diagnoses and there is sufficient local expertise for cytology to be discriminatory.
Diagnosis of Leukemia, Lymphoma, and Myeloma
Published in Tariq I Mughal, John M Goldman, Sabena T Mughal, Understanding Leukemias, Lymphomas, and Myelomas, 2017
Tariq I Mughal, John M Goldman, Sabena T Mughal
Other indolent lymphomas, such as splenic marginal zone lymphoma, present almost exclusively with a large spleen. Variants include extranodal marginal zone lymphoma (previously known as maltoma) which presents with symptoms related to affected sites. For example, the gastric variety, which is often associated with infection by Helicobacter pylori, presents with abdominal discomfort, feeling bloated, and heartburn. Cutaneous lymphomas, such as mycosis fungoides, affect the skin and present with widespread psoriatic-like lesions which progress to plaque and tumor formation over long periods of time, sometimes several years, but in the later stages affect lymph nodes, other organs and blood (such as in Sézary syndrome) (Fig. 4.41).
Splenic marginal zone B-cell lymphoma associated with ruptured breast implants: case report and review of the literature
Published in Case Reports in Plastic Surgery and Hand Surgery, 2021
Mark G. Evans, Melissa A. Mueller, Frederik Chen, Larry S. Nichter
Splenic marginal zone B-cell lymphoma is a rare malignancy, accounting for less than 2% of all lymphoma cases [12]. It was first described in 1992 and is now considered a separate entity in the World Health Organization (WHO) classification [13]. The three types of marginal zone B-cell lymphomas are splenic marginal zone lymphoma (SMZL), nodal marginal zone lymphoma (NMZL), and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). The diagnosis of SMZL is made based on lymphocyte morphology, immunophenotype, cytogenetic abnormalities, bone marrow histology, and spleen histology if available. When microscopic examination of the spleen is not possible, clinical splenomegaly and typical morphologic and immunophenotypic blood and bone marrow findings are sufficient to make a diagnosis.
Malignancy in common variable immunodeficiency: a systematic review and meta-analysis
Published in Expert Review of Clinical Immunology, 2019
Fatemeh Kiaee, Gholamreza Azizi, Hosein Rafiemanesh, Hamed Zainaldain, Fatema Sadaat Rizvi, Mahla Alizadeh, Mahnaz Jamee, Sara Mohammadi, Sima Habibi, Laleh Sharifi, Farhad Jadidi-Niaragh, Sabahat Haghi, Reza Yazdani, Hassan Abolhassani, Asghar Aghamohammadi
It has been confirmed that AD is an integral part of immune dysregulation in a quarter of CVID patients [36]. Our comparative systematic review showed that the incidence of AD in CVID with malignancy was higher than those other CVID patients. Approximately two-thirds of CVID patients (73%) with malignancy had a concomitant diagnosis of AD, especially autoimmune cytopenia, emphasizing the role of uncontrolled inflammation in tumorigenesis of this subgroup of CVID patients. In other studies in patients with no immunodeficiency, the association between AD and malignancy is well established [37,38]. Based on the results obtained from 24 patients diagnosed with marginal zone lymphoma, 50% of them had an AD, with significantly increased rates for autoimmune cytopenia [39]. It has been hypothesized also that neo-antigens expressed by tumors initiate autoimmune response due to abnormal exposure or presentation of these antigens [40,41]. Then again, clonal B-cell proliferation might develop in response to auto-antigens exposed during tissue damage due to autoimmune stimulation in CVID patients [10].
Predicting lymphoma development in patients with Sjögren’s syndrome
Published in Expert Review of Clinical Immunology, 2019
Salvatore De Vita, Saviana Gandolfo
Although the clinical phenotype may be different in subsets of patients with pSS, the autoimmune and lymphoproliferative involvement of MALT sites, and in particular of the salivary and the lachrymal glands, is a common denominator in disease pathobiology, and leads to the key symptoms of dryness [2–4,27]. This MALT background is typical of pSS, and not of other CTDs. Thus, it is not surprising that the MALT histotype or, in general, the marginal zone histotype, is detected in the vast majority of B cell NHLs arising in pSS. The second, but rarer lymphoma histotype is diffuse large B cell lymphoma, which may also derive from the transformation of a MALT lymphoma [24,28,29]. The very high prevalence of marginal zone lymphoma (84.2%; De Vita S., personal observation) was recently confirmed in a large tri-centric Italian and Greek cohort from Udine, Pisa and Athens (UPA), involving 140 pSS-NHLs [30]. Strikingly, lymphoma localization is often salivary, i.e., a crucial localization of pSS itself.