Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease (PVOD) is an extremely rare form of pulmonary hypertension, affecting mostly in children and young adults as a result of a progressive obstruction of small pulmonary veins that leads to elevation in pulmonary vascular resistance and right ventricular failure.
B. Risk factors
If you are male, you are at increased risk to develop Pulmonary veno-occlusive disease (PVOD), according to the study by research team lead by Wagenvoort CA(13).
2. Genetic abnormality
PVOD is characterized by alterations of circulating cytotoxic cell subpopulations and by epigenetic dysregulation within the GNLY gene, according to the study by the Department of Pneumology and Intensive Care, Hôpital Antoine Béclère, Assistance Publique(14). Other study reported a patient with documented PVOD whose mother had severe pulmonary hypertension. Sequencing of the patient’s BMPR2 coding region revealed a del44C mutation in Exon 1 that is predicted to encode for a truncated proteindevelopment of PVOD(14a)
Injury may also have caused the arterialization of the venous walls, a common finding that cannot always be explained by distal narrowing of larger veins. Although the etiology of pulmonary veno-occlusive disease is obscure, it seems increasingly likely that multiple noxious agents may induce this condition(15).
4. Fenfluramine exposure
There is a report of a case of PVOD in a patient with a history of fenfluramine exposure, therefore suggesting a possible association between anorexigen exposure and PVOD(16).
5. Chemotherapy and bone marrow transplantation
PVOD has been reported in association with various chemotherapy regimens including bleomycin, bis-chloronitrosourea and mitomycin(17)
6. After bone marrow transplantation
There is areport of a case of pulmonary veno-occlusive disease (PVOD) following allogeneic bone marrow transplantation (BMT) for the treatment of acute myeloid leukemia (AML) from an HLA mismatched mother using a reduced-intensity conditioning (RIC) regimen including gemtuzumab ozogamicin(18).
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