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Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child 
 
Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child
  Jing-Yan Tang, Jing Chen, Ci Pan, Min-Zhi Yin, Min Zhu
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Shanghai, China 

Author Affiliations: Hematology/Oncology Department (Tang JY, Chen J, Pan C); Pathology Department (Yin MZ); Diagnostic Radiology Department (Zhu M), Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China

Corresponding Author: Jing-Yan Tang, MD, Hematology/Oncology Department, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China (Email: yantfk@online.sh.cn)

Background: Most cavernous hemangiomas in the spleen are small lesions that are found incidentally and patients usually present with no symptoms. Imaging is able to detect the lesions that are considered as diagnostic evidence. But some patients with diffuse cavernous hemangioma may present with anemia, thrombocytopenia, coagulopathy and bleeding, which might be misdiagnosed as idiopathic thrombocytopenia with disseminated intravascular coagulation (DIC). Splenectomy is the most effective therapy for diffuse cavernous hemangiomas with symptoms.

Methods: The history, imaging results, pathologic findings, diagnosis and treatment of a 34-month-old boy with severe petechiae were reviewed.

Results: The boy was diagnosed as having refractory idiopathic thrombocytopenia (ITP) because of low platelet count and bleeding at a local hospital. He had no response to a full-dose of corticosteroid and a high-dose of immuno-globulin (2 g/kg). Huge splenomegaly and DIC were found after 7 months. Diffuse cavernous hemangioma of the spleen was highly suspected, but it was not confirmed by B ultrasound, enhanced CT or MRI. DIC and bleeding were solved by low molecular weight heparin, supplement of fibrinogen and prothrombin complex. A diffuse cavernous hemangioma involving the whole spleen was confirmed pathologically following a successful splenectomy. The boy recovered completely without any complication after the operation.

Conclusions: Diffuse cavernous hemangioma of the spleen should be differentiated from ITP associated with splenomegaly. Radiological and overall physical examination should be emphasized for refractory ITP cases.

Key words: diffuse cavernous hemangioma; idiopathic thrombocytopenia; Kasabach-Merritt syndrome; misdiagnosis

                  World J Pediatr 2008;4(3):227-230

 
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World Journal of Pediatric Surgery

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