IgG4-related disease and B-cell malignancy due to an IKZF1 gain-of-function variant.

Background: Monoallelic loss-of-function IKZF1 (IKAROS) variants cause B-cell deficiency or combined immunodeficiency, whereas monoallelic gain-of-function (GOF) IKZF1 variants have recently been reported to cause hypergammaglobulinemia, abnormal plasma cell differentiation, autoimmune and allergic manifestations, and infections.

Objective: We studied 7 relatives with autoimmune/inflammatory and lymphoproliferative manifestations to identify the immunologic disturbances and the genetic cause of their disease.

Methods: We analyzed biopsy results and performed whole-exome sequencing and immunologic studies.

The Cardiovascular Event Risk Associated with Tyrosine Kinase Inhibitors and the Lipid Profile in Patients with Chronic Myeloid Leukemia.

Background: Second- and third-generation tyrosine kinase inhibitors (TKIs) are now available to treat chronic-phase chronic myeloid leukemia (CP-CML) in the first and second line. However, vascular adverse events (VAEs) have been reported for patients with CML treated with some TKIs.

Methods: We retrospectively evaluated the cumulative incidence (CI) and cardiovascular risk for 210 patients included in the Canarian Registry of CML.

[Osteonecrosis of the jaw in patients with multiple myeloma during and after treatment with zoledronic acid].

Background And Objective: To analyze the incidence, the clinical features, and the factors associated with the development of osteonecrosis of the jaw (ONJ) in patients with multiple myeloma (MM) treated with zoledronic acid.

Patients And Methods: Sixty-four patients diagnosed with MM and treated with zoledronic acid between August 1996 and March 2006 were included. Demographic data, predisposing factors, the type of antineoplastic therapy received and the infusions of biophosphonate were recorded.