Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals - 20/12/12
, Marilyn G. Liang, MD 3, Harry P. Kozakewich, MD 4, Ahmad I. Alomari, MD 2, Steven J. Fishman, MD 5, John B. Mulliken, MD 6, Cameron C. Trenor, MD 1
Reprint requests: Stacy E. Croteau, MD, MMS, Vascular Anomalies Center, Children’s Hospital Boston, 300 Longwood Ave, Boston, MA 02115.Abstract |
Objective |
To examine the presentation characteristics of patients with Kaposiform hemangioendothelioma (KHE) to describe the spectrum of disease and risk factors for Kasabach-Merritt phenomenon (KMP).
Study design |
A retrospective review of 163 patients referred to the Vascular Anomalies Center at Children’s Hospital Boston for KHE between 1991 and 2009 identified 107 patients with sufficient data for inclusion.
Results |
The prevalence of KHE in Massachusetts is 
0.91 case per 100
000 children. KHE manifested in infancy in 93% of cases, with 60% as neonates. Common presenting features included enlarging cutaneous lesion (75%), thrombocytopenia (56%), and musculoskeletal pain or decreased function (23%). Cutaneous KHE favored the extremities, especially overlying joints. In our cohort, 71% developed KMP (11% after initial presentation), and 11% of patients lacked cutaneous findings. Retroperitoneal and intrathoracic lesions, though less common, were complicated by KMP in 85% and 100% of cases, respectively. Compared with superficial lesions, KHE infiltrating into muscle or deeper was 6.3-fold more likely to manifest KMP and 18-fold higher if retroperitoneal or intrathoracic. KHE limited to bone or presenting after infancy did not manifest KMP.
Conclusion |
An enlarging cutaneous lesion is the most common presenting feature of KHE in infancy. Older patients with KHE or those lacking cutaneous manifestations present with musculoskeletal complaints or atypical symptoms. The risk of KMP increases dramatically when tumor infiltrates muscle or when KHE arises in the retroperitoneum or mediastinum.
Le texte complet de cet article est disponible en PDF.Keyword : KHE, KMP
Plan
| Supported by a Lovejoy Resident Research and Education Award and an American Society of Hematology Trainee Research grant (to S.C.) and by the National Institutes of Health/National Heart, Lung, and Blood Institute (K08 HL089509 to C.T.). The authors declare no conflicts of interest. |
Vol 162 - N° 1
P. 142-147 - janvier 2013 Retour au numéro
Article précédent
- Prospective Randomized Crossover Evaluation of Three Anesthetic Regimens for Painful Procedures in Children with Cancer
- Doralina L. Anghelescu, Laura L. Burgoyne, Lane G. Faughnan, Gisele M. Hankins, Matthew P. Smeltzer, Ching-Hon Pui
- Hepatitis C Virus-Specific Cell-Mediated Immune Responses in Children Born to Mothers Infected with Hepatitis C Virus
- Samer S. El-Kamary, Mohamed Hashem, Doaa A. Saleh, Sayed F. Abdelwahab, Maha Sobhy, Fatma M. Shebl, Michelle D. Shardell, G. Thomas Strickland, Mohamed Tarek Shata
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?