Pulmonary veno-occlusive disease following hematopoietic stem cell transplantation: a rare model of endothelial dysfunction

Bunte, Matthew C.; Patnaik, Mrinal M.; Pritzker, Marc; Burns, Linda J.

doi:10.1038/sj.bmt.1705990

Cited by 85 publications

(74 citation statements)

References 70 publications

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“…However, PVOD shares some pathological characteristics with hepatic veno-occlusive disease, which is a well-recognised complication of antineoplastic chemotherapy and ''pyrrolizidine alkaloid'' exposure (bush teas) [43]. PVOD has been reported in association with various chemotherapy regimens, including bleomycin, BCNU and mitomycin [44][45][46][47] and after bone marrow transplantation [22][23][24][25][26][27][28]48]. …”

Section: Toxic and Tobacco Exposurementioning

confidence: 99%

“…Application of this frequency to the incidence rate of idiopathic PAH yields an estimated incidence rate of ''idiopathic'' PVOD of 0.1-0.2 cases per million [17,18]. However, PVOD can also occur in patients with associated diseases, including HIV infection [19][20][21], bone marrow transplant [22][23][24][25][26][27][28], connective tissue diseases [13,14,29], sarcoidosis [30] or pulmonary Langerhans cell granulomatosis [8,31,32], suggesting that PVOD could have a much higher prevalence than indicated by these registries.…”

Section: Epidemiology and Risk Factorsmentioning

confidence: 99%

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Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2009

European Respiratory Journal

269

250

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Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.KEYWORDS: Alveolar haemorrhage, BMPR2, computed tomography, diffusing capacity of the lung for carbon monoxide, pulmonary arterial hypertension, pulmonary veno-occlusive disease P ulmonary arterial hypertension (PAH) is a severe condition characterised by elevated pulmonary artery pressure leading to right heart failure and death [1,2]. Pulmonary veno-occlusive disease (PVOD) is classified as a subgroup of PAH and accounts for 5-10% of histological forms of cases initially considered to be idiopathic PAH. Even though the first welldocumented case of PVOD was described .70 yrs ago, the characteristics and pathophysiology of this disease remain poorly understood [3,4]. While pulmonary vascular pathology of idiopathic or familial PAH is characterised by a major remodelling of small pre-capillary pulmonary arteries with typical plexiform and/or thrombotic lesions, PVOD preferentially affects the post-capillary venous pulmonary vessels [5,6]. Despite this anatomical histological difference, PVOD has a very similar clinical presentation to PAH but is characterised by a worse prognosis and the possibility that severe pulmonary oedema can develop with specific PAH therapy, justifying the importance of diagnosing this disease. A definitive diagnosis of PVOD requires histological analysis of a lung sample [7,8]; however, surgical lung biopsy is a high-risk procedure in these patients and the development of a less invasive diagnostic approach would be preferable [9][10][11]. The present manuscript will summa...

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Section: Toxic and Tobacco Exposurementioning

confidence: 99%

Section: Epidemiology and Risk Factorsmentioning

confidence: 99%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2009

European Respiratory Journal

269

250

View full text Add to dashboard Cite

show abstract

“…Several risk factors for PVOD have been proposed that may be etiologically related, such as HIV infection (8), sarcoidosis and pulmonary Langerhans cell granulomatosis (9, 10), chemotherapy (11), bone marrow transplantation and hematopoietic blood stem cell transplantation (12,13), collagen vascular disease and genetic factors (14,15). There was no indication of a possible cause for the present patient based on medical, familial history, clinical and serological findings.…”

Section: Discussionmentioning

confidence: 66%

Lengthy Diagnostic Challenge in a Rare Case of Pulmonary Veno-Occlusive Disease: Case Report and Review of the Literature

Yan

Zhang

et al. 2011

Intern. Med.

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Pulmonary veno-occlusive disease (PVOD) is a rare and usually survival poor disorder. We report a patient with a long history of progressive dyspnea of over 8 years, who with a diagnosis of chronic cor pulmonale confirmed elsewhere, was ultimately diagnosed as PVOD via histological analysis of a lung biopsy. After treatment with combined bosentan, diuretics and digoxin, his symptoms and function improved. This case highlights that PVOD is an under-recognised and often misdiagnosed disease, especially in its chronic form. Understanding its pathogenesis, its poor response to medical therapy and its dismal prognosis remain challenges for the treatment of PVOD.

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“…Most reported patients have pulmonary arterial hypertension, about 70% of transplanted patients with pulmonary hypertension, mainly involving the arteriolar tree [80]. Pulmonary veno-occlusive disease is a less common cause of pulmonary hypertension after HSCT with endothelial injury, intimal fibrosis and lumen obliteration of venules and small veins result in post-capillary hypertension [83]. Clinical presentation is non-specific similar to other causes of PH and diagnosis can be difficult with lung biopsy may be needed to prove the diagnosis [84].…”

Section: Hemopoietic Stem Cell Transplantationmentioning

confidence: 99%

Insights into etiological factors of pulmonary hypertension in cancer patients

Al-Mansouri

AL-Obaidi²

2017

Nowotwory. Journal of Oncology

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Pulmonary hypertension is a rare vascular disease that can affect patients with or surviving malignancy resulting in significant morbidity and high mortality. Malignant diseases can lead to elevated pulmonary artery pressure through different mechanisms, either directly by structural obstruction of pulmonary vessels or indirectly through hypercoagulable state or treatment toxicity culminating in high pulmonary vascular resistance. The most common causes of cancer-related pulmonary hypertension are thromboembolic diseases, tumour emboli and treatment toxicity and less commonly intravascular tumours and malignant extrinsic compression. NOWOTWORY J Oncol 2017; 67, 4: 236-242

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Pulmonary veno-occlusive disease following hematopoietic stem cell transplantation: a rare model of endothelial dysfunction

Cited by 85 publications

References 70 publications

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease

Lengthy Diagnostic Challenge in a Rare Case of Pulmonary Veno-Occlusive Disease: Case Report and Review of the Literature

Insights into etiological factors of pulmonary hypertension in cancer patients

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