Autoimmune phenomena in untreated and treated marginal zone lymphoma

Abstract

The course of MZL is accompanied by a variety of hematologic and non-hematologic autoimmune disorders. Whereas some of them could be secondary and related to the course of the MZL, others may be primary and might even favor the development of MZL itself. In addition, authentic autoimmune conditions have been documented with the use of rituximab as a single agent and the nucleoside analogs. Therefore, we believe caution should be exerted with the use of these agents in MZL patients with evidence of autoimmune disorders, as exacerbation of autoimmune phenomena can be anticipated. While the heterogeneity of the MZL subtypes represents an inherent limitation, integration of emerging information from immunology research laboratories and clinical practice could translate into improved outcomes of this disease spectrum.

Autoimmune manifestations in patients with multiple myeloma and monoclonal gammopathy of undetermined significance

“…Immune dysregulation plays a key role in lymphomagenesis. Of note, chronic autoimmune inflammatory conditions have been associated with lymphoproliferative disorders such as lymphoma and chronic lymphocytic leukemia [4] , [5] .…”

Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

“…1 Recent studies documented significantly higher incidences of lymphoproliferative malignancies and solid cancers (esophageal and laryngeal cancers, lung cancers, and nonmelanoma skin cancers) in patients with PV and PF, suggesting a paraneoplastic association in pemphigus disorders beyond PNP. [2][3][4][5] In light of these emerging findings, we recommended and coordinated age-appropriate cancer screenings for our patient. This led to the diagnosis of an otherwise asymptomatic and nonpalpable stage IIA breast cancer.…”

“…1 Emerging evidence suggests that pemphigus vulgaris (PV) and pemphigus foliaceus (PF), the 2 most common types of pemphigus, may also be associated with an increased incidence of certain malignancies. [2][3][4][5] Here, we report a patient with no known medical history who presented with new-onset PF and was subsequently diagnosed with an invasive mammary carcinoma from investigation of a possible paraneoplastic etiology. This case provides supportive evidence for potential paraneoplastic associations of PF and highlights the clinical importance of screening for malignancy in patients with pemphigus.…”

Splenic Marginal Zone Lymphoma in a Patient With Antiphospholipid Syndrome Treated With Rituximab: A Case Report

“…Furthermore, in previously reported cases, patients initially diagnosed with SMZL were found to have APLAs [ 4 , 6 ]. Grabska et al suggested that the finding of autoimmune phenomena in marginal zone lymphomas (MZL) could be either secondary due to the malignancy or as a primary condition that favored the development of malignancy [ 10 ]. SMZL was never suspected in our patient with a long history of APS as there was low suspicion given her normal hematology panel in outpatient laboratory results and the patient had no manifestations of lymphoma including low-grade fever, weight loss, night sweats, and lymphadenopathy.…”

“…Splenectomy is reserved for cases refractory to rituximab therapy [ 12 , 13 ]. Grabska et al suggested caution in the use of rituximab due to its association with autoimmune neutropenia and thrombocytopenia and nucleoside analogs due to their association with autoimmune hemolytic anemia [ 10 ]. However, despite the concern regarding these drugs with autoimmune phenomena, multiple studies have found resolution of APLAs after treatment with rituximab with or without chemotherapy [ 7 , 14 , 15 ].…”