Document Detail

Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine.
MedLine Citation:
PMID:  12218593     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of vincristine in the treatment of KMP.
METHODS: We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe.
RESULTS: All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability.
CONCLUSION: Vincristine presents a safe and sometimes effective treatment option in the management of KMP.
Camille Haisley-Royster; Odile Enjolras; Ilona J Frieden; Maria Garzon; Margaret Lee; Arnold Oranje; Peter C J de Laat; Gerard C Madern; Francisco Gonzalez; Hayder Frangoul; Philippe Le Moine; Neil S Prose; Denise M Adams
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Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  24     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:    2002 Aug-Sep
Date Detail:
Created Date:  2002-09-09     Completed Date:  2002-12-09     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  459-62     Citation Subset:  IM    
Duke University Medical Center, Durham, NC, USA.
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MeSH Terms
Antineoplastic Agents, Phytogenic / therapeutic use*
Fibrinogen / metabolism
Hemangioendothelioma / complications,  drug therapy*,  pathology
Hemangioma, Capillary / complications,  drug therapy*,  pathology
Infant, Newborn
Platelet Count
Retrospective Studies
Thrombocytopenia / complications,  drug therapy*,  pathology
Vincristine / therapeutic use*
Reg. No./Substance:
0/Antineoplastic Agents, Phytogenic; 57-22-7/Vincristine; 9001-32-5/Fibrinogen
Erratum In:
J Pediatr Hematol Oncol 2002 Dec;24(9):794

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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