Truncating titin variants lead to pronounced cardiac alterations in mitochondrial function, with increased interstitial fibrosis and reduced hypertrophy. Those structural and metabolic alterations in TTNtv hearts go along with increased ventricular arrhythmias at long-term follow-up, with a similar survival and overall cardiac function.

Intravascular large B-cell lymphoma (IVLBCL) is a rare variant of extranodal diffuse large B-cell lymphoma (DLBCL). It is characterized by proliferation of blastic, neoplastic B cells within the lumina of small-or intermediate-sized blood vessels and capillaries. 1 IVLBCL may potentially involve any organ and is often widely disseminated. Two major patterns have been recognized. In Western patients, predominantly the skin and the central nervous system (CNS) are involved, whereas in Asian countries, the disease often involves multiple other organs and is accompanied by hemophagocytosis. 2 Additionally, a cutaneous variant, with skin-limited disease at time of diagnosis, has been described in younger, Western women. 3 Standard treatment of IVLBCL consists of rituximab-containing chemotherapeutic regimens, demonstrating an estimated 3-year overall survival (OS) of 60% to 81%. 4,5

To conclude, this study suggests that immunosuppressive therapy in infl-CMP patients results in an improved heart transplantation-free survival as compared with standard heart failure therapy alone, underscoring the urgent need for a large prospective multicenter trial.