Table 4.

Hepatic Veno-Occlusive Disease with Immunodeficiency: Targeted Therapies

TreatmentComment
IVIG or SCIG Adjust dose to maintain a trough IgG concentration >6 g/L.
Defibrotide Used for acute mgmt of VOD-assoc liver disease at doses recommended by prevailing literature & local institutional treatment guidelines.
HSCT Inclusion of defibrotide prophylaxis in HSCT regimen may improve survival. Omission of a second alkylating agent in the conditioning regimen may ↓ risk for transplant-related hVOD. 1

HSCT = hematopoietic stem cell transplantation; hVOD = hepatic veno-occlusive disease; Ig = immunoglobulin; IVIG = intravenous immunoglobulin; SCIG = subcutaneous immunoglobulin; VOD = veno-occlusive disease

1.

From: Hepatic Veno-Occlusive Disease with Immunodeficiency

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