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Is there hope of improving the prognosis of pulmonary tumour thrombotic microangiopathy?

Mansouria Merad, Laurent Savale, Sami Antoun, François Vincent for the GrrrOH: Groupe de Recherche en Réanimation Respiratoire en Onco-Hématologie (Group for Research in Respiratory Intensive Care Onco-Hematology)
European Respiratory Journal 2016 47: 688-690; DOI: 10.1183/13993003.01276-2015
Mansouria Merad
1Service de Urgences et de Permanence des Soins, Gustave Roussy Cancer Campus Grand Paris, Villejuif, France
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Laurent Savale
2AP-HP, Service de Pneumologie, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
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Sami Antoun
1Service de Urgences et de Permanence des Soins, Gustave Roussy Cancer Campus Grand Paris, Villejuif, France
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François Vincent
3Réanimation Polyvalente, GHIC Le Raincy-Montfermeil, Montfermeil, France
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  • For correspondence: frncsvncnt@aol.com
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Abstract

Better pathophysiological understanding of PTTM could result in specific targeted therapies and improve prognosis http://ow.ly/U93wg

To the Editor:

We read with interest the letter by Kumar et al. [1] reporting two new cases of fatal pulmonary tumour thrombotic microangiopathy (PTTM). As discussed by the authors, ante mortem diagnosis of PTTM is difficult to confirm because of a rapid progression of the disease. The disease is characterised histopathologically by microscopic tumour emboli and remodelling of the pulmonary vasculature, leading to right heart failure, severe hypoxaemia and, ultimately, death in the very short term, within a few hours or days following admission.

However, some cases have also been reported in the literature with higher survival of a few months that were not analysed in detail by Kumar et al. [1], who cited only one of these cases [2]. After reviewing all cases published in PubMed-indexed journals, since the original description by von Herbay et al. [3], we found six additional observations of such prolonged survival after a diagnosis of PTTM (table 1) [4–9]. These cases drew our attention because they could also be interesting to highlight the physiopathological mechanisms of PTTM and identify potential targeted therapies for this fatal condition.

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TABLE 1

Observations of prolonged survival after a diagnosis of pulmonary tumour thrombotic microangiopathy

One of these cases reported prolonged survival of a patient treated for a colorectal cancer with chemotherapy including bevacizumab, an anti-vascular endothelial growth factor (VEGF) monoclonal antibody [7]. VEGF is known to have a specific role in angiogenic endothelial cells and, thereby, in promoting the proliferation of endothelium involved in embryonic development and tumour angiogenesis. A recent clinical analysis of 30 autopsy cases observed that the immunohistochemistry of tumour cells located within the tumour emboli was positive for VEGF, suggesting that this pathway could be an interesting option to treat PTTM [10]. However, the effect of anti-VEGF antibody in this condition has to be evaluated in light of side-effects of this treatment, including thromboembolic events. In addition, four other patients were treated with imatinib, a tyrosine kinase inhibitor that inhibits platelet-derived growth factor (PDGF) receptors, at different dosages [2, 7–9]. Imatinib was associated in three cases with other chemotherapies acting on the VEGF pathway. In these patients, a survival of 12 months was reported, which is exceptional in PTTM. An immunohistochemical study reported PDGF and PDGF receptor expression in cancerous cells and pulmonary endothelial cells in lesions of PTTM caused by gastric carcinoma. In two cases, imatinib treatment lead to a decrease in serum PDGF concentration, which was associated with a decrease in D-dimer concentrations, despite the absence of anticoagulation [2, 9]. Interestingly, in one case, a sustained clinical response after weaning from imatinib was observed, probably related to the efficacy of adjunct chemotherapy [2]. Again, the effect of this drug has to be evaluated in light of its side-effects. We have to remember that the risk/benefit ratio was recently considered unfavourable for the treatment of idiopathic pulmonary artery hypertension.

Based on these observations, we can hope that a better understanding of the pathophysiology of PTTM could result in specific targeted therapies that should improve prognosis of this vascular condition.

Footnotes

  • Conflict of interest: None declared.

  • Received August 15, 2015.
  • Accepted September 24, 2015.
  • Copyright ©ERS 2016

References

  1. ↵
    1. Kumar N,
    2. Price LC,
    3. Montero MA, et al.
    Pulmonary tumour thrombotic microangiopathy: unclassifiable pulmonary hypertension? Eur Respir J 2015; 46: 1214–1217.
    OpenUrlAbstract/FREE Full Text
  2. ↵
    1. Ogawa A,
    2. Yamadori I,
    3. Matsubara O, et al.
    Pulmonary tumor thrombotic microangiopathy with circulatory failure treated with imatinib. Intern Med 2013; 52: 1927–1930.
    OpenUrlCrossRefPubMed
  3. ↵
    1. von Herbay A,
    2. Illes A,
    3. Waldherr R, et al.
    Pulmonary tumor thrombotic microangiopathy with pulmonary hypertension. Cancer 1990; 66: 587–592.
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    1. Miyano S,
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    3. Takeda Y, et al.
    Pulmonary tumor thrombotic microangiopathy. J Clin Oncol 2007; 25: 597–599.
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    Pulmonary tumor thrombotic microangiopathy diagnosed antemortem and treated with combination chemotherapy. Intern Med 2012; 51: 2767–2770.
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    1. Kitamura A,
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    3. Jinta T, et al.
    A case of pulmonary tumor thrombotic microangiopathy diagnosed by transbronchial lung biopsy and treated with chemotherapy and long-term oxygen and anticoagulation therapies. Case Rep Pulmonol 2013; 2013: 259080.
    OpenUrlPubMed
  5. ↵
    1. Higo K,
    2. Kubota K,
    3. Takeda A, et al.
    Successful antemortem diagnosis and treatment of pulmonary tumor thrombotic microangiopathy. Intern Med 2014; 53: 2595–2599.
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    1. Fukada I,
    2. Araki K,
    3. Minatsuki S, et al.
    Imatinib alleviated pulmonary hypertension caused by pulmonary tumor thrombotic microangiopathy in a patient with metastatic breast cancer. Clin Breast Cancer 2015; 15: e167–e170.
    OpenUrlCrossRefPubMed
  6. ↵
    1. Minatsuki S,
    2. Miura I,
    3. Yao A, et al.
    Platelet-derived growth factor receptor-tyrosine kinase inhibitor, imatinib, is effective for treating pulmonary hypertension induced by pulmonary tumor thrombotic microangiopathy. Int Heart J 2015; 56: 245–248.
    OpenUrlCrossRefPubMed
  7. ↵
    1. Uruga H,
    2. Fujii T,
    3. Kurosaki A, et al.
    Pulmonary tumor thrombotic microangiopathy: a clinical analysis of 30 autopsy cases. Intern Med 2013; 52: 1317–1323.
    OpenUrlCrossRefPubMed
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Is there hope of improving the prognosis of pulmonary tumour thrombotic microangiopathy?
Mansouria Merad, Laurent Savale, Sami Antoun, François Vincent
European Respiratory Journal Feb 2016, 47 (2) 688-690; DOI: 10.1183/13993003.01276-2015

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Is there hope of improving the prognosis of pulmonary tumour thrombotic microangiopathy?
Mansouria Merad, Laurent Savale, Sami Antoun, François Vincent
European Respiratory Journal Feb 2016, 47 (2) 688-690; DOI: 10.1183/13993003.01276-2015
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