A dual-acting DNASE1/DNASE1L3 biologic prevents autoimmunity and death in genetic and induced lupus models.

A defining feature of systemic lupus erythematosus (SLE) is loss of tolerance to self-DNA, and deficiency of DNASE1L3, the main enzyme responsible for chromatin degradation in blood, is also associated with SLE. This association can be found in an ultrarare population of pediatric patients with DNASE1L3 deficiency who develop SLE, adult patients with loss-of-function variants of DNASE1L3 who are at a higher risk for SLE, and patients with sporadic SLE who have neutralizing autoantibodies against DNASE1L3. To mitigate the pathogenic effects of inherited and acquired DNASE1L3 deficiencies, we engineered a long-acting enzyme biologic with dual DNASE1/DNASE1L3 activity that is resistant to DNASE1 and DNASE1L3 inhibitors.

Diagnostic Dilemma in a Case of Necrotizing Lymphadenitis With Macrophage Activation Syndrome.

Necrotizing lymphadenitis is a histological diagnosis that can arise from various conditions, including lupus lymphadenitis (LL), Kikuchi disease (KD), and infectious causes. Distinguishing between Kikuchi disease and lupus lymphadenitis can be challenging in clinical practice. In this report, we present the clinical scenario of a young female patient with lymphadenopathy and elucidate the process through which we ultimately arrived at a diagnosis of systemic lupus erythematosus (SLE) with macrophage activation syndrome.

A rare case of B cell lymphoblastic leukemia with inv(7)(p15q34) with review of literature.

The inv(7)(p15q34) chromosomal abnormality which juxtaposes part of the HOXA gene cluster on 7p15 to the TCRβ locus on 7q34, has been described in a subset of cases of T-cell lymphoblastic leukemia, but its presence in cases of B-cell lymphoblastic leukemia is virtually unknown. Herewith, we report a case of a B-cell lymphoblastic leukemia with inv(7)(p15q34). The patient received standard induction chemotherapy, which failed to produce remission.