title: Kaposiform Haemangioendothelioma-spectrum Lesions with Kasabach-Merritt Phenomenon: Retrospective Analysis and Long-term Outcome.
abstract: Kasabach-Merritt phenomenon (KMP) is a rare life-threatening vascular condition of infancy. Prognosis factors and long-term follow-up data are lacking. We retrospectively analysed the records of 24 infants (10 females, 14 males) treated for KMP in the Department of Dermatology of Necker-Enfants Malades Hospital, Paris, France, from 1984 to 2012. Mean duration of thrombocytopaenia (2,000-38,000 platelets/mm3, mean 10,500/µl) was 8.8 months (range 3 days-84 months), which correlated with tumour infiltration depth on imaging. D-dimer levels were always elevated, even before KMP onset. Each patient received a mean of 4.8 different treatments (range 1-10). Median follow-up was 6.5 years (range 2 months-22 years). All infants had residual cutaneous lesions, along with inflammatory manifestations (n = 9), elevated D-dimer (n = 5) and orthopaedic sequelae (n = 5). The permanent coagulopathy (elevated D-dimer) even after resolution of KMP suggests the presence of chronic low-grade platelet trapping, with possible sudden worsening, and raises the possibility of prophylactic anti-platelet therapy.
authors.affiliation: Department of Dermatology, Hopital Universitaire Necker-Enfants Malades, FR-75000 Paris, France. olivia.boccara@nck.aphp.fr.
authors.forename: Olivia
authors.initials: O
authors.lastname: Boccara
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.forename: Dominique
authors.initials: D
authors.lastname: Lasne
authors.forename: Juliette
authors.initials: J
authors.lastname: Fontaine
authors.forename: Valérie
authors.initials: V
authors.lastname: Bughin
authors.forename: Dominique
authors.initials: D
authors.lastname: Hamel-Teillac
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.forename: Francis
authors.initials: F
authors.lastname: Brunelle
authors.forename: Yves
authors.initials: Y
authors.lastname: de Prost
authors.forename: Smail
authors.initials: S
authors.lastname: Hadj-Rabia
authors.forename: Christine
authors.initials: C
authors.lastname: Bodemer
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D001777:Blood Coagulation; D003131:Combined Modality Therapy; D004621:Embolization, Therapeutic; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000378:metabolism; D006390:Hemangioendothelioma / Q000097:blood / Q000175:diagnosis / Q000628:therapy*; D006776:Hospitals, Pediatric; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D059885:Kasabach-Merritt Syndrome / Q000097:blood / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D009361:Neoplasm Invasiveness; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use*; D010976:Platelet Count; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000970:Antineoplastic Agents; D015415:Biomarkers; D005338:Fibrin Fibrinogen Degradation Products; D010975:Platelet Aggregation Inhibitors; C036309:fibrin fragment D