Pulmonary Hypertension Associated With Pulmonary Occlusive Vasculopathy After Allogeneic Bone Marrow Transplantation

Seguchi, Masato; Hirabayashi, Norio; Fujii, Yasuhiko; Azuno, Yoichi; Fujita, Naoki; Takeda, Komei; Sato, Yutaka; Nishimura, Manabu; Yamada, Kôji; Oka, Yoshitomo

doi:10.1097/00007890-200001150-00030

Cited by 66 publications

(42 citation statements)

References 6 publications

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“…The interesting and speculative issue in this case is the underlying pathogenesis of the vascular damage. PAH as a complication of marrow transplantation has been reported rarely [4][5][6], and in each of these cases was attributed to intensive conditioning; none occurred in the context of GVHD. Vaksmann et al described the onset of PAH 5 months post-transplant in an 8-year-old girl conditioned with busulphan, cyclophosphamide, and cytarabine [4].…”

Section: Discussionmentioning

confidence: 99%

“…Vaksmann et al described the onset of PAH 5 months post-transplant in an 8-year-old girl conditioned with busulphan, cyclophosphamide, and cytarabine [4]. Seguchi et al reported PAH in the 3 rd month post-transplant in a heavily pretreated 20-year-old male conditioned with total body irradiation, cyclophosphamide, and cytarabine [5]. Open lung biopsy demonstrated partial to complete occlusion of small pulmonary arteries by fibrous proliferation of the intima, together with multiple veno-occlusive lesions in small pulmonary veins.…”

Section: Discussionmentioning

confidence: 99%

“…These disorders have a strong association with chronic graft-versus-host disease (GVHD), suggesting that small airways and lung parenchyma are tissues targeted by immune processes post-transplant. Early-onset pulmonary arterial hypertension (PAH) has been reported post-transplant and attributed to direct damage from chemoradiotherapy [4][5][6] but has not, to our knowledge, been described in the context of chronic GVHD. This case describes the development of PAH in a patient with chronic GVHD, suggesting that the pulmonary vasculature may also be a target for immune-mediated damage.…”

Section: Introductionmentioning

confidence: 99%

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Late‐onset pulmonary arterial hypertension in association with graft‐versus‐host disease after allogeneic stem‐cell transplantation

2005

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Pulmonary arterial hypertension (PAH) has been reported only rarely after allografting. Cases have all been in young patients, occurred within 6 months of transplant and been attributed to direct toxicity of chemoradiotherapy on the pulmonary vasculature. This case describes the late onset of PAH in a 36‐year‐old woman on a background of acute and chronic graft‐versus‐host disease (GVHD). Open lung biopsy demonstrated medial hypertrophy and intimal proliferation in small pulmonary arteries and arterioles, with no evidence of secondary causes such as thromboembolism, vasculitis, interstitial lung disease, or obliterative bronchiolitis. Therapy with bosentan produced symptomatic improvement. While the possibility of coincidental “idiopathic” PAH cannot be excluded, the clinical context suggests that pulmonary vessels may be a target for GVHD post‐allograft. Am. J. Hematol. 80:38–42, 2005. © 2005 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Late‐onset pulmonary arterial hypertension in association with graft‐versus‐host disease after allogeneic stem‐cell transplantation

2005

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“…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%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2009

European Respiratory Journal

270

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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“…However, pulmonary hypertension has been described in only a few case reports from the immediate post-treatment period. [11][12][13][14][15][16][17] Pulmonary hypertension is a progressive condition characterized by increased pulmonary artery pressure that may lead to right ventricular failure, which is associated with increased mortality without treatment. 18 Direct measurement of pulmonary artery pressure by right-heart catheterization is the gold standard for diagnosis of pulmonary hypertension.…”

Section: Introductionmentioning

confidence: 99%

Increased Tricuspid Regurgitant Jet Velocity by Doppler Echocardiography in Adult Survivors of Childhood Cancer: A Report From the St Jude Lifetime Cohort Study

Armstrong

Joshi

Zhu

et al. 2013

JCO

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A B S T R A C T PurposeTo determine the prevalence of pulmonary hypertension, a late effect of cancer therapy not previously identified in aging survivors of childhood cancer, and associations with chest-directed radiation therapy (RT) and measures of current cardiac function, lung function, and exercise capacity. Patients and MethodsCross-sectional evaluation of 498 survivors at a median age of 38.0 years (range, 20.0 to 59.0 years) and a median of 27.3 years (range, 12.2 to 46.0 years) from primary cancer diagnosis was performed. Abnormal tricuspid regurgitant jet velocity (TRV) was defined as more than 2.8 m/s by Doppler echocardiography. ResultsIncreased TRV was identified in 25.2% of survivors who received chest-directed RT and 30.8% of those who received more than 30 Gy. In multivariable models, increased TRV was associated with increasing dose of RT (1 to 19.9 Gy: odds ratio [OR], 2.09; 95% CI, 0.63 to 6.96; 20 to 29.9 Gy: OR, 3.46; 95% CI, 1.59 to 7.54; Ն 30 Gy: OR, 4.54; 95% CI, 1.77 to 11.64 compared with no RT; P for trend Ͻ .001), body mass index more than 40 kg/m 2 (OR, 3.89; 95% CI, 1.46 to 10.39), and aortic valve regurgitation (OR, 5.85; 95% CI, 2.05 to 16.74). Survivors with a TRV more than 2.8 m/s had increased odds (OR, 5.20; 95% CI, 2.5 to 11.0) of severe functional limitation on a 6-minute walk compared with survivors with a TRV Յ 2.8 m/s. ConclusionA substantial number of adult survivors of childhood cancer who received chest-directed RT have an increased TRV and may have pulmonary hypertension as a result of both direct lung injury and cardiac dysfunction. Longitudinal follow-up and confirmation by cardiac catheterization are warranted.

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Pulmonary Hypertension Associated With Pulmonary Occlusive Vasculopathy After Allogeneic Bone Marrow Transplantation

Cited by 66 publications

References 6 publications

Late‐onset pulmonary arterial hypertension in association with graft‐versus‐host disease after allogeneic stem‐cell transplantation

Late‐onset pulmonary arterial hypertension in association with graft‐versus‐host disease after allogeneic stem‐cell transplantation

Pulmonary veno-occlusive disease

Increased Tricuspid Regurgitant Jet Velocity by Doppler Echocardiography in Adult Survivors of Childhood Cancer: A Report From the St Jude Lifetime Cohort Study

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