Burkitt lymphoma in the modern era: real-world outcomes and prognostication across 30 US cancer centers.

We examined adults with untreated Burkitt lymphoma (BL) from 2009 to 2018 across 30 US cancer centers. Factors associated with progression-free survival (PFS) and overall survival (OS) were evaluated in univariate and multivariate Cox models. Among 641 BL patients, baseline features included the following: median age, 47 years; HIV+, 22%; Eastern Cooperative Oncology Group (ECOG) performance status (PS) 2 to 4, 23%; >1 extranodal site, 43%; advanced stage, 78%; and central nervous system (CNS) involvement, 19%. Treatment-related mortality was 10%, with most common causes being sepsis, gastrointestinal bleed/perforation, and respiratory failure. With 45-month median follow-up, 3-year PFS and OS rates were 64% and 70%, respectively, without differences by HIV status. Survival was better for patients who received rituximab vs not (3-year PFS, 67% vs 38%; OS, 72% vs 44%; P < .001) and without difference based on setting of administration (ie, inpatient vs outpatient). Outcomes were also improved at an academic vs community cancer center (3-year PFS, 67% vs 46%, P = .006; OS, 72% vs 53%, P = .01). In multivariate models, age ≥ 40 years (PFS, hazard ratio [HR] = 1.70, P = .001; OS, HR = 2.09, P < .001), ECOG PS 2 to 4 (PFS, HR = 1.60, P < .001; OS, HR = 1.74, P = .003), lactate dehydrogenase > 3× normal (PFS, HR = 1.83, P < .001; OS, HR = 1.63, P = .009), and CNS involvement (PFS, HR = 1.52, P = .017; OS, HR = 1.67, P = .014) predicted inferior survival. Furthermore, survival varied based on number of factors present (0, 1, 2 to 4 factors) yielding 3-year PFS rates of 91%, 73%, and 50%, respectively; and 3-year OS rates of 95%, 77%, and 56%, respectively. Collectively, outcomes for adult BL in this real-world analysis appeared more modest compared with results of clinical trials and smaller series. In addition, clinical prognostic factors at diagnosis identified patients with divergent survival rates.

Primary Cutaneous B-Cell Lymphoma: Management and Patterns of Recurrence at the Multimodality Cutaneous Lymphoma Clinic of The Ohio State University.

BACKGROUND: The increasing incidence of primary cutaneous B-cell lymphomas (PCBCLs) presents new challenges for clinicians. Despite advances in the clinical and pathologic characterization of PCBCL, the significance of the current staging approach as a risk profiling tool and the effect of various treatments on outcome remain unclear. MATERIALS AND METHODS: We retrospectively reviewed patients who presented with a diagnosis of PCBCL seen at The Ohio State University between 1998 and 2012. We reviewed the initial presentation and treatment modality. We then assessed whether the treatment modality (conservative skin-directed vs. definitive radiation with or without systemic therapy), stage (T1 or ≥T2), or histologic subtype (primary cutaneous follicle center lymphoma [PCFCL] vs. primary cutaneous marginal zone B-cell lymphoma [PCMZL]) affected the risk of recurrence. RESULTS: We identified 67 patients referred with an initial diagnosis of PCBCL. After imaging, 12 did not meet the criteria for PCBCL and were classified as having systemic B-cell lymphoma with cutaneous involvement. The remaining 55 patients included 25 with PCMZL, 24 with PCFCL, 2 with primary cutaneous large B-cell lymphoma leg type, and 4 with unclassifiable disease. According to the International Society of Cutaneous Lymphoma-European Organization for Research and Treatment of Cancer staging, 30 cases were T1 (55%), 14 T2 (25%), and 11 T3 (20%). Comparing the time to first recurrence (TFR) by indolent PCBCL subtypes, we found no difference in the recurrence risk for either stage (T1, p = .51 vs. T2/T3, p = .30). Comparing TFR by treatment modality, we found no difference in TFR within T1 patients (p = .34) or T2/T3 patients (p = .44). CONCLUSION: Our limited analysis highlights the importance of complete staging at diagnosis and suggests that the treatment modality does not affect the risk of recurrence in T1 indolent PCBCL.

Extranodal Marginal Zone Lymphoma-like Presentations of Angioimmunoblastic T-Cell Lymphoma: A T-Cell Lymphoma Masquerading as a B-Cell Lymphoproliferative Disorder.

Angioimmunoblastic T-cell lymphoma (AITL) is the second most common type of peripheral T-cell lymphoma worldwide, and in some countries, it is the most common form. Clinically, AITL usually presents with systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly, and common laboratory abnormalities such as hypergammaglobulinemia. Rashes are seen in 50%-80% of patients. AITL derives from follicular T-helper cells (TFH), that express germinal center markers and produce hyperactivation of B-cells seen in AITL. Although the histological features of AITL in the skin could be similar to pathological findings present in lymph node biopsies, herein, we present 2 cases of AITL with histological and immunophenotypic features that were somewhat suggestive of extranodal marginal zone lymphoma. Caution is urged to exclude the possibility of a systemic T-cell lymphoma such as AITL in cutaneous and lymph node B-cell proliferations.

Rare presentation of post-transplant lymphoproliferative disorder isolated to gastroesophageal junction.

Post transplant lymphoproliferative disorder (PTLD) represents a life threatening disorder occurring after transplantation, ranging from a polyclonal mononucleosis like illness to a monomorphic high grade neoplasm with cytologic and histopathologic evidence indicative of transformation to lymphoma. PTLD of diffuse large B-cell lymphoma (DLBCL) subtype, isolated to the esophagus is a rare diagnosis. We describe the first case of an immunocompromised adult patient diagnosed with DLBCL-PTLD limited to his esophagus without an associated mass or locoregional lymphadenopathy on imaging since the institution of the revised Cheson criteria, which includes positron emission tomography-computed tomography as the standard staging modality. Even more unique to our case was the suggestion of underlying cytomegalovirus (CMV) gastritis leading to a hypothesis about a less well understood relationship between CMV and Epstein Barr virus (EBV). In the post transplant setting, immunocompromised state, or EBV positive state, upper gastrointestinal symptoms should prompt investigation with an upper endoscopy (EGD). Additionally, specific to our case, the fact that the patients' presentation was suspicious for CMV gastritis raises the possibility that the CMV infection predated his PTLD increasing his risk of acquiring PTLD. This reemphasizes the importance and diagnostic utility of early screening with EGD in patients after transplantation.