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Published on: August 25, 2017
[Kaposiform haemangioendothelioma associated with B-cell acute lymphoblastic leukemia]
F Fichel1, C Eschard, D Zachar
1Service de dermatologie, hôpital Robert-Debré, CHU de Reims, avenue du Général-Koenig, 51092 Reims cedex, France. fanny-fichel@hotmail.fr
Insights
This study reports the first case of kaposiform hemangioendothelioma (KHE) in an infant with acute B-lymphoblastic leukemia (B-ALL). The infant
Area of Science:
- Pediatric Oncology
- Hematology
- Dermatology
Background:
- Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor.
- Acute B-lymphoblastic leukemia (B-ALL) is a common childhood cancer.
- Kasabach-Merritt syndrome (KMS) is a rare complication of vascular tumors.
Purpose of the Study:
- To report the first documented case of KHE associated with B-ALL.
- To describe the clinical presentation, diagnosis, and management of this rare co-occurrence.
- To discuss the potential mechanisms linking KHE and B-ALL.
Main Methods:
- Case report of a five-month-old infant with KHE and suspected KMS.
- Diagnostic workup including skin biopsy, blood tests, and bone marrow examination.
- Treatment with chemotherapy (INTERFANT 2006 protocol) and hematopoietic stem cell transplantation.
Main Results:
- The infant presented with KHE, anemia, thrombocytopenia, and hyperleukocytosis with 90% blasts.
- Histopathology confirmed KHE with dense B-lymphoblast infiltration.
- Treatment led to complete hematological remission and near-complete regression of KHE.
Conclusions:
- KHE mimicking KMS can be an initial presentation of B-ALL.
- The lymphoblastic infiltrate in KHE may be due to passive contamination or active recruitment.
- This case highlights the importance of thorough investigation in infants with KHE and hematologic abnormalities.
Background:
Herein, we report the first case of kaposiform haemangioendothelioma (KHE) associated with acute B-lymphoblastic leukemia (B-ALL).
Patients And Methods:
A five-month-old infant presented a plaque of angiomatous appearance on the forearm that had increased in volume since birth, as well as pallor and cutaneous haematomas. Kasabach-Merritt syndrome (KMS) was evoked despite hepatomegaly and considerable splenomegaly. Laboratory tests revealed severe anaemia and thrombocytopenia as well as major hyperleukocytosis with 90% blasts. Skin biopsy revealed vast vascular lobules containing cohesive fusiform endothelial cells not expressing Glut1, bound up in a dense infiltrate of B-lymphoblast cells. It was in fact KHE associated with B-ALL confirmed by the myelogram. The child was treated with the INTERFANT 2006 protocol followed by allograft of haematopoietic stem cells, which resulted in complete haematological remission. At the same time, almost total regression of KHE was noted.
Discussion:
In this infant, KHE had an inflammatory appearance and was associated with thrombocytopenia, evocative of KMS. Analysis of blood and marrow samples resulted in a diagnosis of B-ALL. Histopathological examination of the angioma revealed a typical appearance of KHE associated with dense lymphoblastic proliferation. This appearance could have resulted either from passive contamination by circulating blast cells or from active recruitment of tumor cells at the KHE site.
Conclusion:
HK mimicking KMS may reveal B-ALL.
