- The case of Kazabah—Merritt syndrome in a child
The case of Kazabah—Merritt syndrome in a child
SOVREMENNAYA PEDIATRIYA.2018.7(95):47-50; doi 10.15574/SP.2018.95.47
Makieieva N., Odinets Yu., Poddubnaya I.
Kharkiv National Medical University, Ukraine
Communal non–profit enterprise «Municipal Clinical Children's Hospital No.16» of the Kharkiv City Council, Ukraine
Kazabah—Merritt syndrome is usually combined with a large or even «giant» hemangioma and is accompanied by high mortality (37%). The most frequent localization of hemangiomas is the neck, face, head, trunk, less often internal organs, mainly the liver and brain. Unequivocally effective therapy of this disease does not exist, the use of this or that type of therapy depends on the location of the hemangioma, its size and response to therapy.
Aim: To attract the attention of pediatricians to the diagnosis of rare hematological syndromes.
Materials and methods. An extended examination of the child and a thorough differential diagnosis with other vascular anomalies has been performed.
Results. Based on the evaluation of history, clinical examination and laboratory and instrumental methods of examination, a diagnosis of Kazabah—Merritt syndrome was made.
Conclusions. The diagnosis of Kazabah—Merritt syndrome requires an extended examination of the child and careful differentiation with other vascular anomalies.
Key words: hemangiomas, thrombocytopenia, consumptive coagulopathy, children.
1. Behfar Eivazi, Jochen A Werner. (2013). Management of vascular malformations and hemangiomas of the head and neck — an update. Current opinion in otolaryngology – head and neck surgery. 21(2):157–63. https://doi.org/10.1097/MOO.0b013e32835e15a9; PMid:23486379
2. Jin Ah Kim, Young Bae Choi, Eun Sang Yi et al. (2016). Excellent outcome of medical treatment for Kasabach—Merritt syndrome: a single-center experience. Blood Res. 51(4):256–260. https://doi.org/10.5045/br.2016.51.4.256; PMid:28090488 PMCid:PMC5234231
3. Ping Wang, Wei Zhou, Li Tao et al. (2014). Clinical analysis of kasabach— merritt syndrome in 17 neonates, BMC Pediatrics. 14:146–152. https://doi.org/10.1186/1471-2431-14-146; PMid:24920221 PMCid:PMC4088914
4. Priya Dhruv Dhandore, Narendra Narayan Hombalkar, Chandrakant Kamlesh Pancholi. (2016). Kasabach Merritt syndrome: Is there a role of surgery? Journal of Clinical Neonatology. 5 (1):58–60. https://doi.org/10.4103/2249-4847.173280
5. Radovic SV, Kolinovic M, Ljubic D. (2017). Propranolol in the preoperative treatment of Kasabach—Merritt syndrome: a case report. J Med Case Rep. 11(1):308–311. https://doi.org/10.1186/s13256-017-1475-0; PMid:29073926 PMCid:PMC5658992
6. Run-Song Jiang, Zheng-Yan Zhao. (2017). Multimodal treatment of Kasabach—Merritt syndrome arising from tufted angioma: A case report. Oncology Letters. 13:4887–4891. https://doi.org/10.3892/ol.2017.6064; PMid:28599491 PMCid:PMC5453004
Article received: May 07, 2018. Accepted for publication: Oct 29, 2018.