Abstract
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 immunoglobulin (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.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Schulze SM, Moser RL, Bhattacharyya N. A rare case of diffuse neonatal hemangimatosis. Am Surg 2006;72:359–362.
Shiran A, Naschitz JE, Yeshurun D, Misselevitch I, Boss JH. Diffuse hemangiomatosis of the spleen: splenic hemangiomatosis presenting with giant splenomegaly, anemia, and thrombocytopenia. Am J Gastroenterol 1990;85:1515–1517.
Balaci E, Sumner TE, Auringer ST, Cox TD. Diffuse neonatal hemangiomatosis with extensive involvement of the brain and cervical spinal cord. Pediatr Radiol 1999;29:441–443.
Poetke M, Jamil B, Müller U, Berlien HP. Diffuse neonatal hemangiomatosis associated with Simpson-Golabi-Behmel syndrome: a case report. Eur J Pediatr Surg 2002;12:59–62.
Abbott RM, Levy AD, Aguilera NS, Gorospe L, Thompson WM. From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics 2004;24:1137–1163.
Ontachi Y, Asakura H, Omote M, Yoshida T, Matsui O, Nakao S. Kasabach-Merritt syndrome associated with giant liver hemangioma: the effect of combined therapy with danaparoid sodium and tranexamic acid. Haematologica 2005;90(Suppl):ECR29.
Ontachi Y, Asakura H, Arahata M, Kadohira Y, Maekawa M, Hayashi T, et al. Effect of combined therapy of danaparoid sodium and tranexamic acid on chronic disseminated intravascular coagulation associated with abdominal aortic aneurysm. Circ J 2005;69:1150–1153.
About I, Capdeville J, Bernard P, Lazorthes F, Boneu B. Unresectable giant hepatic hemangioma and Kasabach-Merritt syndrome. Rev Med Interne 1994;15:846–850.
Vogel T, Lammers B, von Herbay A, Kunz BM, Donner AJ, Fürst G, et al, Kasabach-Merritt syndrome in giant hemangioma of the liver. A case report. Chirurg 2002;73:729–732.
Hanna BD, Bernstein M. Tranexamic acid in the treatment of Kasabach-Merritt syndrome in infants. Am J Pediatr Hematol Oncol 1989;11:191–195.
Ibbotson T, Perry CM. Danaparoid: a review of its use in thromboembolic and coagulation disorders. Drugs 2002;62:2283–2314.
Steininger H, Pfofe D, Marquardt L, Sauer H, Markwat R. Isolated diffuse hemangiomatosis of the spleen: case report and review of literature. Pathol Res Pract 2004;200:479–485.
Schulz AS, Urban J, Gessler P, Behnisch W, Kohne E, Heymer B. Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas. Eur J Pediatr 1999;158:379–383.
Bravo M, Aldunate G, Las Heras J, Pumarino G. Hemangioma of the spleen in a newborn infant. Postsplenectomy course and splenic autotransplantation. Rev Chil Pediatr 1989;60:40–43.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Tang, JY., Chen, J., Pan, C. et al. Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child. World J Pediatr 4, 227–230 (2008). https://doi.org/10.1007/s12519-008-0042-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12519-008-0042-6