Pulmonary Vascular Disease: Student/Resident Case Report Poster - Pulmonary Vascular Disease II |

The Limitations of a Trans-Bronchial Biopsy in Evaluating Interstitial Lung Opacities and Hypoxemia: A Case of Pulmonary Tumor Thrombotic Microangiopathy FREE TO VIEW

Anastasiia Rudkovskaia, MD; Ying-Chun Lo, MD; Wassim Fares, MD
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Bridgeport Hospital/Yale New Haven Health, Trumbull, CT

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1229A. doi:10.1016/j.chest.2016.08.1338
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary tumor thrombotic microangiopathy (PTTM) is associated with the activation of the coagulation system at the surface of the tumor emboli and manifests as subacute respiratory failure (RF) with progressive right ventricular (RV) failure and sudden death. We are presenting a case of PTTM developing in a patient with gastro-esophageal (GE) junction adenocarcinoma (AC).

CASE PRESENTATION: A 49 years old man with stage IV human epidermal growth factor receptor 2 positive GE junction AC presented with complaints of cough, shortness of breath and fatigue. These symptoms coincided with the initiation of KTN3379/trastuzumab about a month earlier with rapid worsening over the last couple of days. KTN3379 is the phase 1 clinical trial medication that is a human monoclonal antibody blocking the activity of ErbB3 receptor tyrosine kinase (RTK). Upon admission, he was found to have mildly elevated troponins. A transthoracic echocardiogram (TTE) demonstrated severely increased RV cavity size with severely decreased RV systolic function. McConnell's sign was noted. A computed tomography angiography was negative for pulmonary embolism (PE) but demonstrated new bilateral pulmonary infiltrates. Due to concern for possible infection, the patient underwent bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsies (TBBx). BAL ruled out an infectious etiology. TBBx showed arteriole obliteration by smooth muscle proliferation suggestive of pulmonary vasculopathy (Figure 1). The right heart catheterization (RHC) confirmed severe pulmonary hypertension with mean pulmonary artery pressure of 70 mmHg and a pulmonary vascular resistance of 20 Wood units. Unfortunately, shortly after the RHC, the patient developed a pulseless electrical activity cardiac arrest and died after resuscitation efforts were unsuccessful. Ante-mortem cytologic examination of blood aspirated from the pulmonary artery catheter (PAC) (not in a wedged inflated balloon position) confirmed the presence of circulating tumor cells. Autopsy results showed diffuse dissemination of tumor cells in the lymphatic channels (Figure 2) and small pulmonary arterioles obliteration by tumor cells and fibromuscular proliferation with recanalization suggestive of PTTM.

DISCUSSION: PTTM is predominantly diagnosed post-mortem. In this case, TBBx were suggestive of PAH and the patient had history of being treated with RTK blocking medications implicated in its development. However, ante-mortem cytologic examination of blood aspirated from the PAC showed the presence of tumor cells.

CONCLUSIONS: In conclusion, it is important to consider PTTM as a differential diagnosis for rapidly progressive RF in patients with metastatic tumors. Lymphangitic carcinomatosis may be diagnosed with cytologic examination of blood aspirated from a wedged PAC. TBBx may be misleading.

Reference #1: Bazan IS, Fares WH. Pulmonary hypertension: diagnostic and therapeutic challenges. Ther Clin Risk Manag. 2015; 11:1221-1233.

DISCLOSURE: The following authors have nothing to disclose: Anastasiia Rudkovskaia, Ying-Chun Lo, Wassim Fares

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