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Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine.

Publication ,  Journal Article
Haisley-Royster, C; Enjolras, O; Frieden, IJ; Garzon, M; Lee, M; Oranje, A; de Laat, PCJ; Madern, GC; Gonzalez, F; Frangoul, H; Le Moine, P ...
Published in: J Pediatr Hematol Oncol
2002

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.

Duke Scholars

Published In

J Pediatr Hematol Oncol

DOI

ISSN

1077-4114

Publication Date

2002

Volume

24

Issue

6

Start / End Page

459 / 462

Location

United States

Related Subject Headings

  • Vincristine
  • Thrombocytopenia
  • Retrospective Studies
  • Platelet Count
  • Oncology & Carcinogenesis
  • Male
  • Infant, Newborn
  • Infant
  • Humans
  • Hemangioma, Capillary
 

Citation

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MLA
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Haisley-Royster, C., Enjolras, O., Frieden, I. J., Garzon, M., Lee, M., Oranje, A., … Adams, D. M. (2002). Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol, 24(6), 459–462. https://doi.org/10.1097/00043426-200208000-00010
Haisley-Royster, Camille, Odile Enjolras, Ilona J. Frieden, Maria Garzon, Margaret Lee, Arnold Oranje, Peter C. J. de Laat, et al. “Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine.J Pediatr Hematol Oncol 24, no. 6 (2002): 459–62. https://doi.org/10.1097/00043426-200208000-00010.
Haisley-Royster C, Enjolras O, Frieden IJ, Garzon M, Lee M, Oranje A, et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002;24(6):459–62.
Haisley-Royster, Camille, et al. “Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine.J Pediatr Hematol Oncol, vol. 24, no. 6, 2002, pp. 459–62. Pubmed, doi:10.1097/00043426-200208000-00010.
Haisley-Royster C, Enjolras O, Frieden IJ, Garzon M, Lee M, Oranje A, de Laat PCJ, Madern GC, Gonzalez F, Frangoul H, Le Moine P, Prose NS, Adams DM. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002;24(6):459–462.

Published In

J Pediatr Hematol Oncol

DOI

ISSN

1077-4114

Publication Date

2002

Volume

24

Issue

6

Start / End Page

459 / 462

Location

United States

Related Subject Headings

  • Vincristine
  • Thrombocytopenia
  • Retrospective Studies
  • Platelet Count
  • Oncology & Carcinogenesis
  • Male
  • Infant, Newborn
  • Infant
  • Humans
  • Hemangioma, Capillary