Related Experiment Videos
Giant hemangioma with thrombocytopenia and osteolysis successfully treated with prednisone
1Department of Pediatrics, Meir General Hospital, Sapir Medical Center, Kfar Saba, Israel.
Insights
A rare case of Kasabach-Merritt syndrome in an infant, characterized by hemangioma, thrombocytopenia, and bone destruction, was successfully treated with prednisone. The treatment led to cessation of bleeding and significant bone reconstruction, with normal pelvic films by age three.
Area of Science:
- Pediatric Oncology
- Hematology
- Pediatric Surgery
Background:
- Kasabaka-Merritt syndrome is a rare condition characterized by hemangioma, thrombocytopenia, and coagulopathy.
- Complete osteolysis associated with Kasabaka-Merritt syndrome is exceptionally rare.
- This case highlights a unique presentation with significant bone destruction.
Observation:
- A 6-week-old infant presented with a hemangioma, severe thrombocytopenia, consumption coagulopathy, extensive bleeding, and destruction of the right pubic and iliac bones.
- The patient experienced life-threatening bleeding and substantial bone loss.
- Radiographic evidence showed complete disappearance of the right pubic bone and partial destruction of the right iliac bone.
Findings:
- Successful treatment with prednisone over 30 weeks.
- Bleeding resolved within days of initiating prednisone therapy.
- Bone rebuilding was observed starting at 30 days and was extensive by the end of treatment.
- Pelvic radiographs were normal by age 3 years.
Implications:
- Prednisone can be an effective treatment for Kasabaka-Merritt syndrome with associated osteolysis.
- This case demonstrates the potential for significant bone reconstruction following successful treatment.
- Highlights the importance of early diagnosis and intervention for this rare syndrome.
Abstract:
A 6-week-old girl with a hemangioma with thrombocytopenia, consumption coagulopathy, extensive bleeding, disappearance of the right pubic bone, and destruction of part of the right iliac bone was successfully treated with prednisone. Treatment lasted 30 weeks. Bleeding ceased within a few days; bone rebuilding was first observed at 30 days and was extensive at the end of treatment. When the child was 3 years old, pelvic films were normal. The occurrence of Kasabach-Merritt syndrome associated with complete osteolysis as in our patient is rare and bone reconstruction is unusual.