CHEST Guidelines-Venoocclusive-Disease-With-Both-Hepatic-and-Pulmon

Venoocclusive-Disease-With-Both-Hepatic-and-Pulmon

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The document discusses a rare medical case involving both hepatic venoocclusive disease (HVOD) and pulmonary venoocclusive disease (PVOD) in a single adult patient, presenting post-hematopoietic stem cell transplantation (HSCT). Traditionally, HVOD and PVOD, both characterized by vein occlusion in the liver and lungs respectively, are separately documented conditions with some shared risk factors like chemotherapy and HSCT. HVOD occurs in 13% to 15% of patients after HSCT and usually manifests within 30 days post-chemotherapy, whereas PVOD is a less common complication with potential fatal outcomes.<br /><br />The case study details a 54-year-old woman who developed both conditions following HSCT. After receiving an allo-HSCT with conditioning regimens that included gemtuzumab, cyclophosphamide, busulfan, and fludarabine, she presented with symptoms of HVOD 6 days post-transplantation, managed effectively with defibrotide. Later, 10 months post-transplantation, she was diagnosed with PVOD following severe dyspnea.<br /><br />The discussion highlights the potential of shared pathophysiological mechanisms between HVOD and PVOD, particularly in association with endothelial damage from cytotoxic chemotherapy. It proposes that defibrotide, while primarily used for HVOD, may also facilitate improvement in PVOD, although the efficacy may not solely rely on it. An endothelin receptor antagonist was also used in this case, resulting in significant improvement in the patient's condition over 7 weeks.<br /><br />The authors suggest a need for further exploration into the pathogenesis and optimal treatment strategies for these diseases in the context of HSCT. They emphasize the novelty of diagnosing both PVOD and HVOD concurrently in one patient, proposing that both conditions may not be coincidental and are potentially linked by common risk factors and underpinning mechanisms.

Keywords

hepatic venoocclusive disease

pulmonary venoocclusive disease

hematopoietic stem cell transplantation

HSCT complications

defibrotide treatment

endothelial damage

gemtuzumab conditioning

chemotherapy risk factors

endothelin receptor antagonist

pathogenesis exploration