Indian Journal of Paediatric Dermatology

: 2018  |  Volume : 19  |  Issue : 1  |  Page : 57--58

Kasabach–Merritt syndrome in an infant successfully treated with a combination of propranolol and methotrexate

Thulasi Weerasinghe1, AC Ranasinghe2, Jayamini Seneviratne1,  
1 Department of Dermatology, Lady Ridgeway Hospital, Colombo, Sri Lanka
2 Department of Dermatology, Colombo North Teaching Hospital, Colombo, Sri Lanka

Correspondence Address:
Thulasi Weerasinghe
Lady Ridgeway Hospital, Colombo
Sri Lanka


Kasabach Merritt syndrome is a rare childhood vascular tumour,which develops bleeding as an acute complication. The underlying tumour is either a Kaposiform haemangioendothelioma or a tufted angioma. Tumour associated platelet trapping, activation and concomitant fibrinolysis leads to bleeding. Herein we report a case with Kasabach Merritt syndrome initially treated with a combination of drugs but subsequently successfully managed with Methotrexate.

How to cite this article:
Weerasinghe T, Ranasinghe A C, Seneviratne J. Kasabach–Merritt syndrome in an infant successfully treated with a combination of propranolol and methotrexate.Indian J Paediatr Dermatol 2018;19:57-58

How to cite this URL:
Weerasinghe T, Ranasinghe A C, Seneviratne J. Kasabach–Merritt syndrome in an infant successfully treated with a combination of propranolol and methotrexate. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Jun 2 ];19:57-58
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Full Text


Kasabach–Merritt syndrome (KMS) is a pediatric emergency characterized by rapid enlargement of vascular tumor with platelet trapping, activation, and concomitant fibrinolysis, leading to a hemorrhagic diathesis. The underlying tumor is probably a kaposiform hemangioendothelioma or tufted angioma.

Treatment options are either medical or surgical or a combination. Both modalities of treatment are associated with morbidity and mortality. Standard medical treatment consists of using steroids, propranolol, interferon alfa, vincristine, and other drugs. However, a number of patients may require a combined approach. Most of the drugs are aimed at targeting the underlying pathomechanisms mentioned above. Vincristine, which binds to platelets, targets the tumor. However, it is given intravenously with the use of a central venous line. Recurrent usage of this drug often results in symptomatic bicytopenia, namely, thrombocytopenia and neutropenia.

Herein, we describe a case of KMS successfully treated with daily propranolol combined with weekly doses of oral methotrexate.

 Case Report

An eight-month-old baby was referred to our department with a vascular tumor on the lateral side of the left thigh and a fluctuating platelet count [Figure 1].{Figure 1}

Platelet count had varied from 170,000 to 55,000. The tumor had been tender, and the mother had noticed rapid enlargement of tumor associated with severe pain recently, which prompted her to seek treatment. Examination revealed a large lesion 10 cm × 8 cm on the lateral margin of the left thigh extending to the buttock. A clinical diagnosis of Kasabach–Merritt phenomenon was made, and a full blood count performed which revealed a platelet count of 170,000. The other parameters were normal. Subsequent measurement of platelet count showed a further drop, the lowest recorded being 55,000. The patient was started on prednisolone 5 mg twice daily. Reassessment of platelet count after a week showed a nonsignificant rise. This prompted us to add oral propranolol based on the current literature. The continuation of combination therapy for a week did not yield any benefits. Instead of using intravenous (IV) vincristine which requires an insertion of central venous line, we decided to use oral methotrexate with an initial lower dose of 1.25 mg, with weekly increases up to maximum of 3.75 mg weekly. We noticed a significant increase of the platelet count reaching 288,000 after 2 weeks. In addition, we were happy to note the decline of tumor size and color [Table 1].{Table 1}


Kaposiform hemangioendotheliomas are rare, a rapidly proliferating vascular tumors which usually appear in infants. They are more prone to develop the serious complication known as Kasabach–Merritt phenomenon. This is clinically characterized by acute deterioration of the tumor leading to life-threatening thrombocytopenia and bleeding.

Principles of management of KMS involve inhibiting tumor growth and targeting the main pathomechanisms, namely, platelet activation, trapping, and fibrinolysis. Up to now, no single agent has proven more effective in patients with KMS. Most of the medications have significant side effects.

Methotrexate is a widely used drug in dermatology. It is cheap, freely available and has the added benefit of being used orally weekly. Methotrexate, which is a folic acid antagonist, inhibits DNA synthesis and arrests cell division in the S phase of the cell cycle in rapidly proliferating cells. For this reason, this drug is widely used in oncology, both in pediatric as well as adult practice.

Propranolol is a beta blocker, which gained popularity in view of its effects in the treatment of hemangioma. Recently, its use has expanded into treating KMS. The effect of propranolol on vascular tumors such as kaposiform hemangioendothelioma and KMS can be attributed to molecular mechanisms, including vasoconstriction, decreased expression of vascular endothelial growth factor, inhibition of angiogenesis, and induction of apoptosis.[1]

The use of this combination in our patient avoided the use of IV vincristine and its associated serious side effects.

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Conflicts of interest

There are no conflicts of interest.


1Oksiuta M, Matuszczak E, Dębek W, Dzienis-Koronkiewicz E, Hermanowicz A. Successful exclusive propranolol therapy in an infant with life-threatening Kasabach-Merritt syndrome. J Pediatr Surg Case Rep 2013;1:200-2.