Abstract
Vascular tumours (VT) with Kasabach-Merritt phenomenon (KMP) are rare and aggressive tumors. In absence of evidence based treatment guidelines, we studied varied presentation and response to therapy with vincristine and steroids in VT with KMP at our center. In this retrospective observational study, infants with a symptomatic/disfiguring rapidly growing VT with features of KMP were included. Demographic, treatment and outcome data was retrieved from patient file. Complete response (CR) was defined as complete clinical regression of VT with normalization of coagulopathy and thrombocytopenia. Partial response (PR) was defined as decrease in size of VT by more than 80%, absence of clinical bleed with normalization of coagulopathy and platelet count > 50,000/cumm. Five infants (2-male, 3-female) with age range (0–7 month) treated with daily prednisolone and weekly vincristine were included. The location of VT was: face (2), hemi-thorax (2) and urinary bladder (1). Four of five infants showed PR within two months; while two of these attained CR to treatment. There were no significant adverse effects over 9–32 (range) month follow-up. Two children (one in PR, one immediately after presentation) succumbed to intra-cranial hemorrhage. Combination therapy of steroids with vincristine is effective and safe in management of VT with KMP.
Abbreviations
- VT:
-
Vascular tumor
- KMP:
-
Kasabach Merritt phenomenon
- VCR:
-
Vincristine
- TA:
-
Tufted angioma
- KHE:
-
Kaposiform hemangio-endothelioma
- PR:
-
Partial response
- CR:
-
Complete response
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Acknowledgements
Dr Vishal Puri, 166 MH and Dr Aniruddha Bhattacharjee, MH Thiruvanantapuram for help in patient management.
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All authors contributed to the study conception, design and patient management. Material preparation, data collection and analysis were performed by Sanjeev Khera and Pulkit Agarwal. The first draft of the manuscript was written by Pulkit Agarwal and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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Being a retrospective observational study for an established drug use in a particular condition, the ethics committee approval was not warranted.
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Informed consent was obtained from parents/guardians of all individual participants included in the study at the time of enrollment.
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Agarwal, P., Khera, S., Shaw, S.C. et al. Vascular Tumor with Kasabach Merritt Phenomenon Treated with Steroids and Vincristine: A Retrospective Study. Indian J Hematol Blood Transfus (2023). https://doi.org/10.1007/s12288-023-01724-4
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DOI: https://doi.org/10.1007/s12288-023-01724-4