Correct!
3. Pulmonary arterial thrombotic microangiopathy due to malignancy
The pulmonary CTA largely excluded the possibility of venous thromboembolism, although there are uncommon cases of small pulmonary arterial emboli, or progressive pulmonary arterial thrombosis that occur at the small vessel level, unaccompanied by central, visible thromboembolic disease. Such patients are typically inoperable, and have a poor prognosis, and have a pulmonary CTA appearance similar to this patient- bilateral geographic areas of mosaic perfusion due to obstruction of small pulmonary vessels. While the imaging is consistent with this potential diagnosis, the patient’s clinical presentation is not- chronic venous thromboembolic disease does not adequately address the bloodwork abnormalities in this patient. While congenital heart disease can produce pulmonary hypertension and could explain some of the patient’s clinical presentation, congenital cardiovascular disease was not detected at the non-invasive and invasive imaging studies, nor would it account for the bloodwork abnormalities in this patient. Pulmonary vasculitis could potentially account for the elevated inflammatory markers detected in this patient, but no imaging features to suggest vasculitis were found, nor did extensive rheumatological evaluation disclose evidence supporting such a disorder and no pulmonary hemorrhage occurred. The patient’s chemotherapy treatment for her breast malignancy was years prior to her presentation, and no evidence of pneumonitis was seen at imaging, making progressive interstitial pneumonitis due to pulmonary toxic medication an unlikely explanation for the patient’s presentation and rapid clinical deterioration. The widespread fibrocellular intimal hyperplasia affecting small pulmonary arteries that occurs with thrombotic microangiopathy due to malignancy can present at imaging in a fashion similar to very small pulmonary emboli- bilateral geographic areas of mosaic perfusion due to obstruction of small pulmonary vessels. Clinically such patients may present with progressive shortness of breath, hypoxia, and pulmonary hypertension, as in the case of the patient presented.).
The patient was prepared for immediate plasmapheresis. During the procedure, the patient became increasingly restless, with worsening hypoxia. Her neurological status deteriorated progressively until she became unresponsive. She developed agonal respirations and cardiopulmonary resuscitation was initiated, but was unsuccessful. Autopsy was performed and showed diffuse, bilateral, microangiopathic metastatic breast carcinoma associated with thrombotic-type pulmonary hypertensive arteriopathy. No evidence of thromboembolic disease was noted.
Diagnosis: Pulmonary hypertension due to breast malignancy-induced thrombotic microangiopathyIf you are a member of the Arizona, New Mexico, Colorado or California Thoracic Societies or the Mayo Clinic and wish to receive 0.25 AMA PRA Category 1 Credit(s)™, click here.
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