Allogeneic stem cell transplantation in patients with mantle cell lymphoma: results from the MANTLE-FIRST study on behalf of Fondazione Italiana Linfomi.

The role of allogeneic stem cell transplantation (allo-SCT) in mantle cell lymphoma (MCL) is uncertain, even more in the era of bruton's tyrosine kinase inhibitors (BTKi) and chimeric antigen receptor T-cells. We retrospectively analyzed 55 patients who underwent allo-SCT for MCL relapsed or refractory (r/r) after rituximab and high-dose cytarabine within the MANTLE-FIRST project. With a median follow-up of 3.7 years, non-relapse mortality (NRM), progression-free survival, and overall survival were 23%, 53%, and 56%, respectively. NRM was significantly higher in the case of acute graft-versus-host disease, > 2 prior lines of therapy, age > 60 years. The outcome was similar for patients with early (≤24 months) and late progression of disease. The use of BTKi as a bridge to allo-SCT did not increase the toxicity and allowed a good control of disease. Our real-life experience confirms that allo-SCT still represents an option in MCL patients, especially if young and early-relapsed.

Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach.

Mastocytosis represents a heterogeneous group of neoplastic mast cell disorders. The basic classification into a skin-limited disease and a systemic form with multi-organ involvement remains valid. Systemic mastocytosis is a disease often hard to diagnose, characterized by different symptoms originating from either the release of mast cell mediators or organ damage due to mast cell infiltration. Gastrointestinal symptoms represent one of the major causes of morbidity, being present in 60-80% of patients. A high index of suspicion by clinicians and pathologists is required to reach the diagnosis. Gastrointestinal mastocytosis can be a challenging diagnosis, as symptoms simulate other more common gastrointestinal diseases. The endoscopic appearance is generally unremarkable or nonspecific and gastrointestinal mast cell infiltration can be focal and subtle, requiring an adequate sampling with multiple biopsies by the endoscopists. Special stains, such as CD117, tryptase, and CD25, should be performed in order not to miss the gastrointestinal mast cell infiltrate. A proper patient's workup requires a multidisciplinary approach including gastroenterologists, endoscopists, hematologists, oncologists, and pathologists. The aim of this review is to analyze the clinicopathological features of gastrointestinal involvement in systemic mastocytosis, focusing on the relevance of a multidisciplinary approach.

Diffuse large B-cell lymphoma occurring in an ovarian cystic teratoma: expanding the spectrum of large B-cell lymphoma associated with chronic inflammation.

Diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) is a well-recognized entity, originally recorded as pyothorax-associated lymphoma because of the association with artificial pneumothorax. Clinically, it is characterized by a mass arising in a long-standing inflammation and by a poor prognosis. Recently, DLBCL-CI has been described growing along the wall of a preexisting cyst, without forming a mass. Here we describe a case of DLBCL-CI arising in the wall of a mature cystic teratoma of the ovary. On histology, the cystic surface of the cyst was infiltrated by large lymphocytes, immunoreacting with CD20, Multiple Myeloma Oncogene-1/Interferon Regulating Factor-4 (MUM1/IRF4), and PAX5 and positive for Epstein-Barr virus. "Cystic" DLBCL-CIs usually hold an indolent behavior despite heterogeneous therapeutic approaches. Some authors understandably wonder whether patients affected by "cystic" DLBCL-CIs are at risk for overtreatment, and, consequently, DLBCL-CIs associated with cystic lesions should be classified as an entity separated from classic pyothorax-associated lymphomas.