Allergy Asthma Respir Dis.  2016 Nov;4(6):453-457. 10.4168/aard.2016.4.6.453.

Intrathoracic hemangioendothelioma presenting as refractory unilateral pleural effusion and thrombocytopenia

• Kim HW
• Choi YJ
• Hong KT
• Kang HJ
• Park KD
• Kim S
• Choi YH
• Kim WS
• Suh DI

• KMID: 2361262
• DOI: http://doi.org/10.4168/aard.2016.4.6.453

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.

Keyword

Kasabach-Merrit syndrome; Pleural effusion; Thrombocytopenia; Vascular neoplasms

MeSH Terms

Autoimmune Diseases
Biopsy
Chest Tubes
Diagnosis
Diagnosis, Differential
Diaphragm
Female
Hemangioendothelioma*
Humans
Infant
Pleural Effusion*
Thoracic Wall
Thorax
Thrombocytopenia*
Vascular Neoplasms

Figure

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  Fig. 1 Chest anterior-posterior (A) and lateral (B) radiography showed large amount of unilateral pleural effusion.

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  Fig. 2 Serial changes in hematological parameters. The patient's laboratory test showed refractory anemia and thrombocytopenia until he was treated with propranolol. *Transfusion: ① antibiotics, furosemide; ② prednisolone, atenolol, intravenous immunoglobulin; ③ prednisolone; ④ propranolol.

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  Fig. 3 Chest magnetic resonance imaging revealed a vascular neoplasm involving pericardium, diaphragm, and chest wall (contrast enhanced T1 coronal image).

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  Fig. 4 Biopsy specimen showed (A) sheets of spindle cells with slit-like lumina and glomeruloid like structure (H&E, ×200) which were (B) glucose transporter- 1 negative (×200), suggestive Kaposiform hemangioendothelioma.

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