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Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 145-147

Vincristine, aspirin, and prednisolone therapy in Kasabach–Merritt phenomenon: Response in 2 cases

1 Department of Dermatology, Chacha Nehru Bal Chikitsalaya, Delhi, India
2 Department of Dermatology, MIMER Medical College, Talegaon Dabhade, Maharashtra, India
3 Department of Surgery, Chacha Nehru Bal Chikitsalaya, Delhi, India
4 Department of Pathology, Chacha Nehru Bal Chikitsalaya, Delhi, India

Date of Web Publication29-Mar-2019

Correspondence Address:
Dr. Shikha Gupta
Department of Dermatology, Chacha Nehru Bal Chikitsalaya, Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_18_18

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Kasabach–Merritt phenomenon (KMP) is a severe thrombocytopenic coagulopathy which usually occurs in the presence of enlarging vascular tumors such as kaposiform hemangioendothelioma (KHE) and tufted angioma. The treatment for this potentially fatal condition is challenging without a consensus on appropriate management. The authors report two cases of KHE with KMP, wherein improvement in size of tumor and coagulopathy occurred after treatment with prednisolone, vincristine, and aspirin.

Keywords: Antiaggregant therapy, Kaposiform hemangioendothelioma, Kasabach–Merritt phenomenon, vincristine

How to cite this article:
Gupta S, Bharti S, Khan NA, Singh L. Vincristine, aspirin, and prednisolone therapy in Kasabach–Merritt phenomenon: Response in 2 cases. Indian J Paediatr Dermatol 2019;20:145-7

How to cite this URL:
Gupta S, Bharti S, Khan NA, Singh L. Vincristine, aspirin, and prednisolone therapy in Kasabach–Merritt phenomenon: Response in 2 cases. Indian J Paediatr Dermatol [serial online] 2019 [cited 2020 Oct 27];20:145-7. Available from: https://www.ijpd.in/text.asp?2019/20/2/145/255196

  Introduction Top

Kasabach–Merritt phenomenon (KMP) is characterized by a severe thrombocytopenic coagulopathy that occurs due to platelet trapping in a vascular tumor such as kaposiform hemangioendothelioma (KHE) or tufted angioma and can lead to life-threatening multiorgan hemorrhage.[1],[2] Most of the patients are diagnosed based on clinical, imaging, and laboratory findings (profound thrombocytopenia and elevated serum D-dimer levels).[3]

The treatment options are multitude, thus illustrating a lack of consensus in the management of KHE associated with KMP.[2]

  Case Reports Top

Case 1

A 3-month old female child weighing 4.5 kg was referred to our center with a rapidly expanding reddish lesion over the right arm. It was noticed shortly after birth and 1 month back, it rapidly increased in size, turned bluish at few places, and child cried on touching the area. There was no history of fever or bleeding from any site. She had been diagnosed as cellulitis and had been prescribed antibiotics at a private center with no response. Gestational and perinatal history was unremarkable. On examination, right arm and upper forearm were swollen with bright red erythema [Figure 1]a. The swelling was tender, warm to touch, and indurated. Lymph nodes were not palpable. Routine investigations revealed decreased hemoglobin (9 g/dL) and platelet count (30,000/mm3). Coagulation studies were normal. Skin biopsy already performed at an outside center reported collections of spindle-shaped cells with vascular channels throughout dermis. MRI of right arm reported hypointense signal intensity areas on T1W images from right shoulder joint till mid forearm, consistent with vascular tumor. Therefore, a diagnosis of KMP in association with KHE was made, and patient was started on daily prednisolone 2 mg/kg and propranolol 7.5 mg (2 mg/kg). However, at the end of 4 weeks, there was a continuing decline in platelet count as well as hemoglobin (4000/mm3 and 7.2 g/dL, respectively). International normalized ratio was 2.0.
Figure 1: (a) Erythematous indurated lesion over right arm, forearm. (b) Response to treatment with vincristine, prednisolone, and aspirin after 6 months

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Thus, injection vincristine 0.2 mg/week (0.05 mg/kg/week) intravenously and daily oral aspirin 50 mg (10 mg/kg) were added. Weekly monitoring of blood counts, liver and kidney function, bleeding time, clotting time, and coagulation profile along with a regular systemic examination was carried out. There was a marked clinical response after five cycles of therapy and rise in platelets (42,000/mm3) and hemoglobin (10.0 g/dL).

During the 8th week, a fall in total leukocyte count to 4000/mm3 was observed. Other blood counts were unchanged, and systemic examination including muscle tone and reflexes was normal. Therefore, vincristine was stopped, tablet aspirin continued, and a single infusion of granulocyte-colony stimulating factor (G-CSF) was given. After a week, total leukocyte count increased to 11,000/mm3 and was maintained subsequently without any further intervention. Aspirin was stopped after 7 months of therapy when there was no further clinical improvement [Figure 1]b.

At 9 months of follow-up, child is healthy, gaining weight, and there is a marked reduction in swelling and erythema over right arm.

Case 2

A 2-month old male child weighing 3 kg presented to surgery department at our hospital with erythema and swelling over right thigh. He had previously undergone incision and drainage at a private center with lack of response. There was a history of preceding reddish lesion over this site since a week after birth. An incisional biopsy was performed, and the child was later referred to dermatology department. On examination, there was swelling, erythema, and induration over anterolateral aspect of upper right thigh [Figure 2]a. A fleshy mass was seen at the center of incision site. Swelling was warm to touch and nontender. There was no significant lymphadenopathy.
Figure 2: (a) Erythematous lesion over right thigh with a fleshy mass at the site of incision. (b) Response to therapy after 6 months

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Hemoglobin (8.8 g/dL) and platelet counts (5000/mm3) were low, and coagulation profile and liver and kidney function tests were normal. Biopsy report revealed nodular vascular proliferations in dermis and subcutis separated by desmoplastic tissue reaction [Figure 3]a. Nodules showed intercommunicating vascular channels of varying calibers with slit-like spaces in the periphery [Figure 3]b. Parents refused another biopsy from the fleshy mass at the center of the lesion. Therefore, keeping a diagnosis of KHE with KMP, patient was started on vincristine 0.15 mg/week (0.05 mg/kg/week), daily oral prednisolone 5 mg (2 mg/kg/day), and oral aspirin 25 mg (10 mg/kg/day) therapy.
Figure 3: (a) Tumor nodules traversing the dermis (H and E, ×10). (b) Tumor nodules show Kaposi sarcoma-like areas with spindled endothelial cells, surrounded by capillary channels (H and E, ×40)

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After 2 weeks, there was an improvement in erythema and swelling. Prednisolone was tapered off after 6 weeks of therapy. Two weeks later, after a consistent increase in hemoglobin and platelet counts (11.2 g/dL and 400,000/mm3, respectively), injection vincristine was discontinued. The child received aspirin for 6 months with marked clinical improvement [Figure 2]b. At 4 months of follow-up, there is no recurrence.

  Discussion Top

Diagnosis of KHE associated with KMP is usually delayed, and lack of experience in clinical diagnosis of vascular anomalies is one of the factors involved.[3] Both our cases were mistaken as cellulitis leading to unnecessary administration of antibiotics.

The cause of thrombocytopenia in KMP is intralesional platelet trapping.[4] Clinically significant KMP is a severe thrombocytopenia, generally below 30,000/μL.[5] Mortality rates vary between 10%–37%. Therapeutic options include steroids, interferon, embolization, vincristine, radiotherapy, and surgery.[1],[6]

In infants, glucocorticoid therapy is considered a good choice with onset of action at 1–2 weeks.[1] Our first patient did not respond to 2 mg/kg dose of oral prednisolone following which vincristine and aspirin were added.

Vincristine is a cytotoxic agent and possibly stimulates vascular regression.[7] It is also effective and safe option in combination with antiaggregant therapy.[2],[8] In general, both modalities are administered until normal platelet count is obtained, after which only antiaggregant agents are continued.[3] In a multicenter study on 15 patients with KMP treated with vincristine, the average response time was 4 weeks.[9] Our patients had an initial increase in platelet counts during 5th and 4th weeks of treatment, respectively.

Fernandez-Pineda et al.[2] observed 100% success rate with vincristine, aspirin, and ticlopidine therapy in treating 11 patients of KMP over 17 years. The mean duration of treatment was 3.9 months for vincristine, 13.9 months for aspirin, and 13.4 months for ticlopidine. Two patients (18.2%) developed polyneuropathy which resolved on discontinuing vincristine.[2] Furthermore, vincristine is a vesicant (care must be taken to avoid extravasation) and can cause myelosuppression, a dose-limiting side effect.[7] Our first patient developed a decrease in total leukocyte counts during 8th week of vincristine therapy which was managed with single infusion of G-CSF.

Regarding safety of aspirin in neonates and children, the American College of Chest Physicians guidelines suggest that Reye's syndrome appears to be a dose-dependent effect of aspirin and usually is associated with daily doses >40 mg/kg.[10]

Our second patient developed a mass over biopsy site which was likely a hyperproliferation of underlying vascular elements and it responded to treatment as the rest of the lesion. The authors' center lacks the facility of assessment of D-dimer and fibrinogen levels; therefore, these parameters could not be assessed.

The purpose of reporting these cases is to highlight the challenges associated with diagnosis and management of KMP and to stress on prolonged treatment with vincristine, prednisolone, and aspirin in improving the primary lesion as well as coagulopathy seen in this disorder.

Declaration of patient consent

We hereby declare that written consent has been obtained from parents of both the patients for publication of case material including various investigations and clinical photographs in a research journal. Due measures have been taken not to disclose the identity of the patients.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Fernandez-Pineda I, Lopez-Gutierrez JC, Chocarro G, Bernabeu-Wittel J, Ramirez-Villar GL. Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon. Pediatr Blood Cancer 2013;60:1478-81.  Back to cited text no. 1
Kasabach H, Merritt K. Capillary hemangioma with extenstive purpura: Report of a case. Am J Dis Child 1940;59:1063-70.  Back to cited text no. 2
Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB, et al. Kaposiform hemangioendothelioma: Atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013;162:142-7.  Back to cited text no. 3
Wang P, Zhou W, Tao L, Zhao N, Chen XW. Clinical analysis of Kasabach-Merritt syndrome in 17 neonates. BMC Pediatr 2014;14:146.  Back to cited text no. 4
Kwok-Williams M, Perez Z, Squire R, Glaser A, Bew S, Taylor R, et al. Radiotherapy for life-threatening mediastinal hemangioma with Kasabach-Merritt syndrome. Pediatr Blood Cancer 2007;49:739-44.  Back to cited text no. 5
Haisley-Royster C, Enjolras O, Frieden IJ, Garzon M, Lee M, Oranje A, et al. Kasabach-Merritt phenomenon: A retrospective study of treatment with vincristine. J Pediatr Hematol Oncol 2002;24:459-62.  Back to cited text no. 6
Fernandez-Pineda I, Lopez-Gutierrez JC, Ramirez G, Marquez C. Vincristine-ticlopidine-aspirin: An effective therapy in children with Kasabach-Merritt phenomenon associated with vascular tumors. Pediatr Hematol Oncol 2010;27:641-5.  Back to cited text no. 7
Wharton S, Soueid A, Nishikawa H, Sridhar AV. Endangering cutaneous infantile hemangioma treated with vincristine: A case report. Eur J Plast Surg 2009;32:157-61.  Back to cited text no. 8
Monagle P, Chan AK, Goldenberg NA, Ichord RN, Journeycake JM, Nowak-Göttl U, et al. Antithrombotic therapy in neonates and children: Antithrombotic therapy and prevention of thrombosis, 9th ed: American college of chest physicians evidence-based clinical practice guidelines. Chest 2012;141:e737S-801S.  Back to cited text no. 9
Vivas-Colmenares GV, Ramirez-Villar GL, Bernabeu-Wittel J, Matute de Cardenas JA, Fernandez-Pineda I. The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon. Dermatol Pract Concept 2015;5:91-3.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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