High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency.

Marginal zone lymphoma represents about 10% of all non-Hodgkin lymphomas (NHLs). 33% of patients with acquired angioedema (AAE) due to acquired C1-inhibitor (C1-INH) deficiency (C1-INH-AAE) have or will develop NHLs. C1-INH-AAE is a rare condition. We report the follow-up of 72 C1-INH-AAE patients, followed for a median of 15 years (range 1-24). Median age was 71 (range 64-79) years; median age at onset of angioedema symptoms was 57·5 (range 50-66) years and it was 63 [range 45-80) years at diagnosis]. Twenty patients were diagnosed with low-grade non-follicular B-cell lymphomas (75% were splenic MZL), one with follicular and three with high-grade lymphomas (two diffuse large B-cell lymphomas and one mantle cell lymphoma). Fifteen NHLs were diagnosed at onset of AAE or thereafter (3 months to 7 years), eight had already been diagnosed at onset of angioedema. Two of 24 patients remain on watchful wait. Thirthen of 24 received chemotherapy, two received rituximab. Three underwent splenectomy. All 18 patients receiving therapy for NHL experienced post-treatment reduction in AAE symptoms. Our study suggests that clonal B-cell proliferation is the pathology underlying AAE leading to production of C1-INH-neutralizing autoantibodies and to NHLs. The post-germinal centre origin of NHL suggests that immune stimulation may contribute to lymphomagenesis.

Splenic Lymphomas: A Tertiary Care Center Experience and Review of Literature.

Primary splenic lymphomas are rare with the majority of lymphomas in spleen being secondary to an extra-splenic lymphoma. We aimed to analyze the epidemiological profile of the splenic lymphoma and review the literature. This was a retrospective study including all splenectomies and splenic biopsies from 2015 to September 2021. All the cases were retrieved from Department of Pathology. Detailed histopathological, clinical and demographic evaluation was done. All the lymphomas were classified according to WHO 2016 classification. A total of 714 splenectomies were performed for a variety of benign causes, as part of tumor resections and for the diagnosis of lymphoma. Few core biopsies were also included. A total of 33 lymphomas diagnosed in the spleen, primary splenic lymphomas constituted 84.84% (n = 28) of the cohort with 5 (15.15%) having the primary site elsewhere. The primary splenic lymphomas constituted 0.28% of all the lymphomas arising at various sites. Adult population (19-65 years) formed the bulk (78.78%) with a slight male preponderance. Splenic marginal zone lymphomas (n = 15, 45.45%) comprised of major proportion of cases followed by primary splenic diffuse large B-cell lymphoma (n = 4, 12.12%). Splenectomy was the main course of treatment for SMZL with a good overall outcome, with chemotherapy ± radiotherapy forming the mainstay in other lymphomas. Lymphomas in spleen can be infiltrative or a primary, hence proper clinic-radiological and pathological evaluation is required. Appropriate management is guided by the precise and detailed evaluation by the pathologist, requiring understanding of the same.

Ileal mucosa-associated lymphoid tissue lymphoma diagnosed after emergency surgery: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, also called MALT lymphoma, is one of the entities of marginal zone lymphomas. These lymphomas are originated from indolent B-cell lymphomas and involve many organs such as the gastrointestinal tract, salivary gland, skin, lung, thyroid or breast. Ileal MALT lymphoma is relatively rare and clinical symptoms are usually atypical. CASE PRESENTATION: We report a case of a 99-year-old man who admitted to the emergency department with increasing and colicky periumbilical pain, vomiting and constipation. Non-contrast-enhanced computed tomography suggested small bowel obstruction due to phytobezoar. Intraoperatively, surgeon discovered the tumor at the site of phytobezoar. Histologically, there was a diffuse infiltration comprised of small to medium sized lymphocytes with monocytoid features. Immunohistochemical result confirmed CD20 positive B-lymphocytes and the Ki-67 proliferation index was 10%. Ileal mucosa-associated lymphoid tissue lymphoma was diagnosed based on histological findings and immunohistochemistry. DISCUSSION: MALToma of the gastrointestinal tract is related to chronic antigenic, inflammatory bowel disease and malabsorption syndromes. However, the etiology of ileal MALToma is unclear. Moreover, symptom of ileal MALToma is really not typical and overleaped in the context of small intestinal obstruction. It should be differentiated small intestinal MALToma from immunoproliferative small intestinal disease and an alpha heavy chain disease. CONCLUSION: Ileal MALT lymphoma remains little known in many previous studies. It is really difficult to preoperatively diagnose. The combination of clinical presentation, postoperative histology and immunohistochemistry contribute to diagnosis and carry out appropriate management.