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Revista Mexicana de Pediatría

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2020, Number 3

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Rev Mex Pediatr 2020; 87 (3)

Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon, literature review

Medina-López YM, Sánchez-Grijalva CI, García-López JS, Campos-Félix LB, Calderón-Alvarado AB
Full text How to cite this article 10.35366/94842

DOI

DOI: 10.35366/94842
URL: https://dx.doi.org/10.35366/94842

Language: Spanish
References: 6
Page: 111-114
PDF size: 238.64 Kb.


Key words:

Haemangioendothelioma, Kasabach-Merritt, disseminated intravascular coagulation, newborn.

ABSTRACT

Introduction: Kaposiform hemangioendothelioma is a rare, fast growing vascular tumor. Its main complication is the Kasabach-Merritt phenomenon, which is characterized by severe thrombocytopenia, consumption coagulation disorder, and purpura. Case report: We present a male newborn in whom it was detected that the left upper limb was very thickened from birth. Using magnetic resonance imaging, the diagnosis was reached. Management with steroids and chemotherapy was started, however the evolution was torpid and he died in the first week of life. The diagnosis was confirmed at autopsy. Conclusions: Patients with a rapidly growing vascular tumor should look for hematological alterations its extent in order to give timely treatment.


REFERENCES

  1. Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB et al. Kaposiform hemangioendothelioma: atypical features and risk of a Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 2013; 162(1): 142-147.

  2. O’Rafferty C, O’Regan G, Irvine AD, Smith OP. Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon. Br J Haematol. 2015; 171(1): 38-51.

  3. Cotran RS, Kumar V, Robbins S. Robbins Patología Estructural y Funcional. 6a ed. Madrid: McGraw-Hill Inc./Interamericana de España; 2005.

  4. Adams DM, Brandão LR, Peterman CM, Gupta A, Patel M, Fishman S et al. Vascular anomaly cases for the pediatric hematologist oncologists-An interdisciplinary review. Pediatr Blood Cancer. 2018; 65(1).

  5. Arabi, J, López F. Hemangiomatosis neonatal difusa. Presentación de un caso y revisión de la literatura. Rev Mex Pediatr. 2000; 67(6): 270-273.

  6. Rodríguez N, Benavides P. Sirolimus (rapamicina) en pacientes con hemangioendotelioma kaposiforme. Caso clínico. Rev Chil Pediatr. 2013; 84(5): 537-544.




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C?MO CITAR (Vancouver)

Rev Mex Pediatr. 2020;87