Autologous hematopoietic stem cell transplantation in Clarkson disease

Abstract

Idiopathic systemic capillary leak syndrome (ISCLS) flares are frequently preceded by infections, particularly respiratory virus infections including coronavirus disease 2019.1,2 Hemodynamic collapse occurs at the onset of episodes, typically followed by massive peripheral edema resulting from intravenous (IV) fluid administration. Flares may be complicated by multiorgan dysfunction syndrome, vascular thrombosis due to hemoconcentration, and compartment syndrome.1 Indefinite monthly prophylaxis with high-dose intravenous immunoglobulins (IVIGs) induces disease remission but is expensive and inconvenient to administer.