PTTM. Pulmonary tumor thrombotic microangiopathy. EndoTODAY 이준행

[PTTM. Pulmonary tumor thrombotic microangiopathy.] - 終

PTTM (pulmonary tumor thrombotic microangiopathy)은 매우 무서운 질환입니다. 79%가 사후에 진단이 되고 근본원인인 암조차 35%가 사후에 확진이 된다고 합니다 (Godbole. Pulm Circ 2019). 이 질환이 잘 알려지기 전에는 사전 진단이 목표인 듯하니 얼마나 심한 질환인지 알 수 있습니다. 가장 흔한 원인은 위암인데 우리나라에서는 2014년 처음 보고된 것 같습니다 (Yoo. EMJ 2014). 위암환자의 부검 시 16.7%에서 PTTM의 소견이 보입니다 (Pathol Res Pract 2010).

EMJ 2014

기전은 pulmonary vasculature에 tumor cell embolization이 발생하고 이어서 coagulation cascade가 활성화되고 fibrin clot 형성과 혈관 내막의 fibrocellular proliferation이 되어 pulmonary hypertension이 발생되는 것으로 이해되고 있습니다.

폐 결절의 혈관 내 tumor emboli

호흡곤란과 기침이 가장 중요한 증상인데 상세한 임상양상에 대해서는 2019년 review(Godbole. Pulm Circ 2019)에서 PTTM을 잘 요약하고 있어 옮깁니다.

"Pulmonary tumor thrombotic microangiopathy (PTTM) is a fatal disease process in which pulmonary hypertension (PH) develops in the setting of malignancy. The purpose of this study is to present a detailed analysis of cases of PTTM reported in literature in the hopes of achieving more ante-mortem diagnoses. We conducted a systematic review of currently published and available cases of PTTM by searching the term "pulmonary tumor thrombotic microangiopathy" on the Pubmed.gov database. Seventy-nine publications were included consisting of 160 unique cases of PTTM. The most commonly reported malignancy was gastric adenocarcinoma (94 cases, 59%). Cough and dyspnea were reported in 61 (85%) and 102 (94%) cases, respectively. Hypoxemia was reported in 96 cases (95%). Elevation in D-dimer was noted in 36 cases (95%), presence of anemia in 32 cases (84%), and thrombocytopenia in 30 cases (77%). Common findings on chest computed tomography (CT) included ground-glass opacities (GGO) in 28 cases (82%) and nodules in 24 cases (86%). PH on echocardiography was noted in 59 cases (89%) with an average right ventricular systolic pressure of 71 mmHg. Common features of PTTM that are reported across the published literature include presence of dyspnea and cough, hypoxemia, with abnormal CT findings of GGO, nodules, and mediastinal/hilar lymphadenopathy, and PH. PTTM is a universally fatal disease process and this analysis provides a detailed examination of all the available published data that may help clinicians establish an earlier diagnosis of PTTM."

예후는 매우 좋지 않습니다.

삼성서울병원에서도 몇 개의 보고를 한 바 있으니 참고하시기 바랍니다.

Echocardiographic assessment of Patients with PTTM first diagnosed in the emergency department Diagnostics (Basel). 2022
Clinical course of suspected diagnosis of PTTM: A 10-year experience of rapid progressive right ventricular failure syndrome in advanced cancer patients Korean Circ J. 2023

[References]

1) Clinical course of suspected diagnosis of PTTM: A 10-year experience of rapid progressive right ventricular failure syndrome in advanced cancer patients Korean Circ J. 2023