난치성 편측 흉수와 불응성 혈소판 감소증으로 나타난 흉강내 혈관내피종 1예
Intrathoracic hemangioendothelioma presenting as refractory unilateral pleural effusion and thrombocytopenia
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초록
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.
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.
참고문헌 (15)
- Hooper C, "Investigation of a unilateral pleural effusion in adults: British Thoracic Society Pleural Disease Guideline 2010", Thorax, 65(Suppl 2), pp.ii4~ii17 (2010)
- Buckley MF, "A novel approach to the assessment of variations in the human platelet count", Thromb Haemost, 83, pp.480~484 (2000)
- Sato T, "Differential diagnosis of pleural effusions", Jpn Med Assoc J, 49, pp.315~319 (2006)
- McGrath EE, "Diagnosis of pleural effusion: a systematic approach", Am J Crit Care, 20, pp.119~127 (2011)
- Broderick SR, "Hemothorax: Etiology, diagnosis, and management", Thorac Surg Clin, 23, pp.89~96 (2013)
- Varsano S, "Bilateral and unilateral spontaneous massive hemothorax as a presenting manifestation of rare tumors", Respiration, 70, pp.214~218 (2003)
- Kumar S, "Hemorrhagic pleural effusion secondary to sarcoidosis: a brief review", Ann Thorac Med, 4, pp.27~31 (2009)
- Nanaware S, "Hemorrhagic pleural effusion due to pleural hemangioma", J Assoc Physicians India, 51, pp.623~625 (2003)
- Croteau SE, "Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals", J Pediatr, 162, pp.142~147 (2013)
- San Miguel FL, "Kaposiform hemangioendothelioma: a rare cause of spontaneous hemothorax in infancy. Review of the literature", J Pediatr Surg, 43, pp.E37~E41 (2008)
- DeFatta RJ, "Kaposiform hemangioendothelioma: case report and literature review", Laryngoscope, 115, pp.1789~1792 (2005)
- 이정원, "A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol", Clinical Pediatric Hematology-Oncology, 22(2), pp.161~166 (2015), 대한소아혈액종양학회
- Hall GW, "Kasabach-Merritt syndrome: pathogenesis and management", Br J Haematol, 112, pp.851~862 (2001)
- Fernández Y, "Kaposiform hemangioendothelioma", Eur J Intern Med, 20, pp.106~113 (2009)
- Kelly M, "Kasabach-Merritt phenomenon", Pediatr Clin North Am, 57, pp.1085~1089 (2010)