B-cell lymphomas with concurrent IGH-BCL2 and MYC rearrangements, also known as “double-hit” lymphomas (DHL), are rare neoplasms characterized by highly aggressive clinical behavior, complex karyotypes, and a spectrum of pathological features overlapping with Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL) and B-lymphoblastic lymphoma/leukemia (B-LBL). The clinical and pathological spectrum of this rare entity, including comparison to other high-grade B-cell neoplasms, has not been well defined. We conducted a retrospective analysis of clinical and pathologic features of 20 cases of DHL seen at our institution during a 5-year period. In addition, we performed case-control comparisons of DHL with BL and International Prognostic Index (IPI)-matched DLBCL. The 11 men and 9 women had a median age of 63.5 years (range 32-91). Six patients had a history of grade 1-2 follicular lymphoma (FL); review of the prior biopsy specimens in 2 of 5 cases revealed blastoid morphology. Eighteen patients had Ann Arbor stage 3 or 4 disease and all had elevated serum lactate dehydrogenase (LDH) levels at presentation. Extranodal disease was present in 17/20 (85%), bone marrow involvement in 10/17 (59%) and central nervous system (CNS) disease in 5/11 (45%). Nineteen patients were treated with combination chemotherapy, of whom 18 received rituximab and 14 received CNS-directed therapy. Fourteen patients (70%) died within 8 months of diagnosis. Median overall survival in the DHL group (4.5 months) was inferior to both BL (p=0.002) and IPI-matched DLBCL (p=0.04) control patients. Twelve DHL cases (60%) were classified as B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL, 7 cases (35%) as DLBCL, not otherwise specified, and 1 case as B-LBL. Distinguishing features from BL included expression of Bcl2 (p<0.0001), Mum1/IRF4 (p=0.006), Ki-67 <95% (p<0.0001), and absence of EBV-EBER (p=0.006). DHL commonly contained the t(8;22) rather than the t(8;14) seen in most BL controls (p=0.001), and exhibited a higher number of chromosomal aberrations (p=0.0009). DHL is a high-grade B-cell neoplasm with a poor prognosis, resistance to multi-agent chemotherapy, and clinical and pathological features distinct from other high-grade B-cell neoplasms. Familiarity with the morphologic and immunophenotypic spectrum of DHL is important in directing testing to detect concurrent IGH-BCL2 and MYC rearrangements when a karyotype is unavailable. The aggressive clinical behavior and combination of genetic abnormalities seen in these cases may warrant categorization as a separate entity in future classifications and call for novel therapeutic approaches.

The histologic findings of celiac disease, that is, gluten-sensitive enteropathy (GSE), are dominated by increased intraepithelial lymphocytes, villous blunting, lymphoplasmacytic infiltration of lamina propria, and crypt hyperplasia. To date, neutrophils have not been thought to constitute a significant cell type in GSE, and their presence often invokes consideration of alternative diagnoses. Thus, we sought to determine the prevalence and severity of neutrophilic infiltration in duodenal biopsies from patients with GSE. The degree of neutrophilic infiltration and features characteristic of GSE were assessed in duodenal biopsies from 267 clinically confirmed GSE patients (116 adults and 151 children). These specimens were graded by the disease activity score (DAS) and the neutrophilic activity score (NAS). Gastric antral biopsies obtained from 195 patients were also evaluated for lymphocytic gastritis. NAS was correlated with DAS and other clinicopathologic features. We found that 56% of pediatric and 28% of adult GSE patients had significant duodenal neutrophilia. NAS was higher in children than in adults (2.3 vs. 1.2, P<0.001). Multivariate regression showed that DAS, eosinophilic infiltration, and foveolar metaplasia correlated positively, and age correlated negatively with NAS. Lymphocytic gastritis was seen in 21.5% of the gastric biopsies. The presence of lymphocytic gastritis correlated positively with NAS and DAS, and in the pediatric population it correlated negatively with age. Significant duodenal neutrophilia is often found in patients with celiac disease, especially in the pediatric population, and is associated with more active disease. Thus, the findings of duodenal neutrophilia in biopsies, otherwise consistent with GSE, should not preclude the diagnosis of GSE.

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Chylomas, or chylous lymphoceles, are rare cystic formation of the thoracic duct or its tributaries which arise as an unusual complication following open neck surgery. In the few cases of cervical chyloma reported in the literature, management has varied from watchful waiting to thoracic duct ligation. This report describes the first case of postoperative cervical chyloma following parathyroidectomy and illustrates a successful surgical treatment plan for this infrequent entity.A 56-year-old female underwent a left inferior parathyroidectomy for a parathyroid adenoma at an outside institution. A complex dissection ensued which revealed the adenoma to be located deep to the recurrent laryngeal nerve on the left side. The adenoma was identified and removed and the patient experienced an appropriate drop in her parathyroid hormone level.Postoperatively, the patient developed a cystic central neck swelling. On needle aspiration of the cyst, the fluid was found to be consistent with chyle. Despite numerous attempts at needle decompression in addition to a low-fat diet, the chyle-filled cyst recurred. On presentation to our institution, a 3 x 3-cm cystic mass was noted inferior to the previous suprasternal incision. A noncontrast computed tomography scan of the neck demonstrated a 3.8 x 2.2-cm low density, well-defined midline mass at the level of the mid to inferior thyroid gland consistent with a chyloma ( Figure 1A, B).A surgical excision was performed using continuous nerve monitoring which revealed a well-circumscribed lesion filled with chylous fluid emanating from the previous surgical bed (Figure 2A).The cyst was removed in its entirety after its attachments were meticulously ligated ( Figure 2B). Pathologic examination was consistent with a chylous lymphocele ( Figure 3A,B).Postoperatively, the patient was maintained on a medium-chain triglyceride diet for one week with no evidence of chyloma recurrence both clinically and on repeat radiographic imaging. OBJECTIVES OBJECTIVES : :• To describe the surgical management of a rare case of cervical chyloma following parathyroid adenoma excision. Figure 3A). The cyst contents consisted of brisk lymphocyte infiltration with sheets of organizing fibrin and blood ( Figure 3B). Both of these findings were consistent with a chylous lymphocele. After the surgical excision, there was no further recurrence of the chyloma and the patient continues to do well in followup.We demonstrate that careful re-exploration with definitive ligation and excision of postoperative chylomas is an effective cure for this difficult lesion.