Abstract | BACKGROUND: METHODS: Here we present a 52-year-old man with hepatitis C cirrhosis who presented with an extensive, painful vascular tumor involving the right side of his head, neck, and back that initially arose within a PWS. RESULTS: He developed petechiae, severe thrombocytopenia, and a consumptive coagulopathy consistent with KMP. An incisional biopsy of his vascular tumor was compatible with a TA. Numerous treatments aimed at halting proliferation of his TA were attempted, with sustained improvement in his vascular tumor and platelet count achieved only after several courses of electron beam radiation therapy in association with corticosteroids and bevacizumab. CONCLUSION: To our knowledge, this represents the first report of KMP in an adult man associated with TA and challenges previous hypotheses arguing that KMP occurs only in children. We offer our own hypothesis concerning how, in extremely rare circumstances, this life-threatening consumptive coagulopathy may occur in adults. Electron beam radiation therapy in association with corticosteroids can be an effective treatment in these rare patients.
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Authors | Anthony P Fernandez, Aaron Wolfson, Eugene Ahn, Jennifer C Maldonad, Javier Alonso-Llamazares |
Journal | International journal of dermatology
(Int J Dermatol)
Vol. 53
Issue 9
Pg. 1165-76
(Sep 2014)
ISSN: 1365-4632 [Electronic] England |
PMID | 24697614
(Publication Type: Case Reports, Journal Article)
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Copyright | © 2014 The International Society of Dermatology. |
Chemical References |
- Angiogenesis Inhibitors
- Antibodies, Monoclonal, Humanized
- Glucocorticoids
- Bevacizumab
- Prednisone
|
Topics |
- Angiogenesis Inhibitors
(therapeutic use)
- Antibodies, Monoclonal, Humanized
(therapeutic use)
- Bevacizumab
- Combined Modality Therapy
- Glucocorticoids
(therapeutic use)
- Hemangioma
(drug therapy, radiotherapy)
- Hepatitis C, Chronic
(complications)
- Humans
- Kasabach-Merritt Syndrome
(drug therapy, radiotherapy)
- Liver Cirrhosis
(virology)
- Male
- Middle Aged
- Prednisone
(therapeutic use)
- Skin Neoplasms
(drug therapy, radiotherapy)
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