Intrathoracic hemangioendothelioma presenting as refractory unilateral pleural effusion and thrombocytopenia.
10.4168/aard.2016.4.6.453
- Author:
Hyo Won KIM
1
;
Yun Jung CHOI
;
Kyung Taek HONG
;
Hyoung Jin KANG
;
Kyung Duk PARK
;
Sehui KIM
;
Young Hoon CHOI
;
Woo Sun KIM
;
Dong In SUH
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. dongins0@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Kasabach-Merrit syndrome;
Pleural effusion;
Thrombocytopenia;
Vascular neoplasms
- MeSH:
Autoimmune Diseases;
Biopsy;
Chest Tubes;
Diagnosis;
Diagnosis, Differential;
Diaphragm;
Female;
Hemangioendothelioma*;
Humans;
Infant;
Pleural Effusion*;
Thoracic Wall;
Thorax;
Thrombocytopenia*;
Vascular Neoplasms
- From:Allergy, Asthma & Respiratory Disease
2016;4(6):453-457
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A variety of diseases are associated with the development of unilateral pleural effusion. Although unilateral pleural effusion is common, refractory unilateral pleural effusion is rare. It is important to make an accurate diagnosis using proper diagnostic tools. Thrombocytopenia is one of the rare conditions occurring from various diseases such as severe infection or autoimmune diseases. It can be life-threatening if accurate diagnosis and treatment are delayed and be a clue to accurate diagnosis in differential diagnosis from refractory pleural effusion. Kasabach-Merrit syndrome (KMS) is often accompanied by extensive vascular tumors and characterized by consumptive coagulopathy with profound thrombocytopenia. It is also important to have a high index of suspicion for the diagnosis. We report a case of KMS in a 2-month-old female infant with a vascular tumor on her left intrathoracic cage, who had presented refractory unilateral pleural effusion and thrombocytopenia. Initially, the patient was diagnosed as having complications of severe infection, and a chest tube was inserted for aggressive treatment. However, her unilateral pleural effusion persisted, and thrombocytopenia and hypofibrinogenemia were refractory. Chest imaging revealed an infiltrating large vascular tumor involving the cardiac border, diaphragm, and chest wall. The patient’s unilateral pleural effusion was misidentified as an infectious condition at the initial stage. As a result of the ultrasonography-guided biopsy, it was revealed to be Kaposiform hemangioendothelioma. The patient was cured after treatment for KMS.
