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The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon.

Vivas-Colmenares GV, Ramirez-Villar GL, Bernabeu-Wittel J, Matute de Cardenas JA, Fernandez-Pineda I - Dermatol Pract Concept (2015)

Bottom Line: Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP.Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin.Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Surgery, Virgen del Rocio Children's Hospital, Sevilla, Spain.

ABSTRACT
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.

No MeSH data available.


Related in: MedlinePlus

Right latero-cervical kaposiform hemangioendothelioma at birth (A) and 17 months later, after completion of therapy (B). (Copyright: ©2015 Vivas-Colmenares et al.)
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f1-dp0501a18: Right latero-cervical kaposiform hemangioendothelioma at birth (A) and 17 months later, after completion of therapy (B). (Copyright: ©2015 Vivas-Colmenares et al.)

Mentions: A full-term male newborn was admitted to our hospital with diagnosis of right latero-cervical vascular mass present at birth (Figure 1). He was transferred to our center one week after his birth due to a progressive increase of tumor size. No prior therapy was given. Physical exam revealed a 12×10 cm mixed (deep and superficial) plaque-like lesion with ecchymosis. A severe coagulopathy with profound thrombocytopenia (27,000×109/L) and elevated D-dimer level (84,369 mcg/L) were observed, all of which supported the diagnosis of Kasabach-Merritt phenomenon (KMP). A Doppler ultra-sound study and magnetic resonance imaging (MRI) revealed a multichanneled fast-flowing vascular tumor. Clinical appearance of the tumor, radiological findings, and the coexisting KMP supported the diagnosis of kaposiform hemangioendothelioma (KHE). Treatment was according to SEOP (Spanish Society of Pediatric Oncology) guidelines for vascular tumors complicated by KMP and included vincristine, aspirin and ticlopidine (VAT therapy). The patient received VAT therapy with vincristine: 0.05 mg/kg weekly; aspirin: 10 mg/kg/day; and ticlopidine: 10 mg/kg/day. Treatment response was based on platelet count and classified as good response (increase of >50% of platelet count) observed in obtained blood tests no longer than three weeks since the treatment was started and repeated every two weeks. Coagulation parameters normalized within one week, along with the decrease in size of the tumor. The patient required a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Twelve months after discontinuation of aspirin, the patient remains asymptomatic with no tumor regrowth and normal platelet count.


The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon.

Vivas-Colmenares GV, Ramirez-Villar GL, Bernabeu-Wittel J, Matute de Cardenas JA, Fernandez-Pineda I - Dermatol Pract Concept (2015)

Right latero-cervical kaposiform hemangioendothelioma at birth (A) and 17 months later, after completion of therapy (B). (Copyright: ©2015 Vivas-Colmenares et al.)
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4325701&req=5

f1-dp0501a18: Right latero-cervical kaposiform hemangioendothelioma at birth (A) and 17 months later, after completion of therapy (B). (Copyright: ©2015 Vivas-Colmenares et al.)
Mentions: A full-term male newborn was admitted to our hospital with diagnosis of right latero-cervical vascular mass present at birth (Figure 1). He was transferred to our center one week after his birth due to a progressive increase of tumor size. No prior therapy was given. Physical exam revealed a 12×10 cm mixed (deep and superficial) plaque-like lesion with ecchymosis. A severe coagulopathy with profound thrombocytopenia (27,000×109/L) and elevated D-dimer level (84,369 mcg/L) were observed, all of which supported the diagnosis of Kasabach-Merritt phenomenon (KMP). A Doppler ultra-sound study and magnetic resonance imaging (MRI) revealed a multichanneled fast-flowing vascular tumor. Clinical appearance of the tumor, radiological findings, and the coexisting KMP supported the diagnosis of kaposiform hemangioendothelioma (KHE). Treatment was according to SEOP (Spanish Society of Pediatric Oncology) guidelines for vascular tumors complicated by KMP and included vincristine, aspirin and ticlopidine (VAT therapy). The patient received VAT therapy with vincristine: 0.05 mg/kg weekly; aspirin: 10 mg/kg/day; and ticlopidine: 10 mg/kg/day. Treatment response was based on platelet count and classified as good response (increase of >50% of platelet count) observed in obtained blood tests no longer than three weeks since the treatment was started and repeated every two weeks. Coagulation parameters normalized within one week, along with the decrease in size of the tumor. The patient required a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Twelve months after discontinuation of aspirin, the patient remains asymptomatic with no tumor regrowth and normal platelet count.

Bottom Line: Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP.Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin.Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Surgery, Virgen del Rocio Children's Hospital, Sevilla, Spain.

ABSTRACT
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.

No MeSH data available.


Related in: MedlinePlus