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CASE REPORT
Year : 2010  |  Volume : 55  |  Issue : 3  |  Page : 281-283
Kasabach merritt syndrome: Management with interferon


Department of Dermatology, Amala Institute of Medical Sciences,(AIMS), Thrissur, Kerala, India

Date of Web Publication25-Sep-2010

Correspondence Address:
Sandhya Acharya
Professor, Department of Dermatology, Amala Institute of Medical Sciences, Thrissur
India
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DOI: 10.4103/0019-5154.70705

PMID: 21063527

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   Abstract 

Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. The objectives of treatment of KMS are to prevent bleeding from consumptive coagulopathy and induce vascular tumor regression. A 14-month old female child was brought with a reddish lesion on the left scapular area noticed at birth, which suddenly increased in size since 3 days. Hemogram revealed anemia severe thrombocytopenia, prolongation of bleeding, clotting time and increased fibrin degradable products, suggestive of KMS. Coagulopathy was managed by transfusing fresh frozen plasma and platelets. Oral prednisolone up to 5mg/kg/day for four weeks yielded no effect on thrombocytopenia or regression of tumor size. Embolization of feeding artery was attempted but not feasible. We used Interferon -alpha- 2b (IFN α 2b), in a dosage of 3million IU/m2 /day subcutaneously. Within a month the platelet count increased and the vascular tumor started regressing. This case signifies the importance of step wise management of KMS.


Keywords: Kasabach Merritt syndrome, interferon α 2b, coagulopathy


How to cite this article:
Acharya S, Pillai K, Francis A, Criton S, Parvathi V K. Kasabach merritt syndrome: Management with interferon. Indian J Dermatol 2010;55:281-3

How to cite this URL:
Acharya S, Pillai K, Francis A, Criton S, Parvathi V K. Kasabach merritt syndrome: Management with interferon. Indian J Dermatol [serial online] 2010 [cited 2014 Apr 18];55:281-3. Available from: http://www.e-ijd.org/text.asp?2010/55/3/281/70705



   Introduction Top


Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. It is characterized by a rapidly enlarging vascular anomaly, consumptive coagulopathy, thrombocytopenia, prolonged PT and APTT, hypofibrinogenemia, the presence of D dimer and fibrin split products with or without microangiopathic hemolytic anemia. [1] Kasabach Merritt Syndrome was first noted by Kasabach and Merritt in 1940. [2] It's clinical characteristics and histopathology are distinct from those of hemangiomas. Histopathology usually reveals a tufted angioma or Kaposiform hemangioendothelioma. [1],[3],[4] Prognosis is poor due to few treatment options.

A variety of treatments have been used to induce tumor regression, including prednisolone, dipyridamole, pentoxyfylline, ticlopidine, and heparin, interferon (IFN), vincristine, radiotherapy, and embolization of the feeding vessel to the tumor. We followed a step-wise regimen as evaluated by S. Wananukul et al. [5]


   Case Report Top


A 14-month-old female child came with history of sudden increase in size of lesion over left shoulder, back and chest wall associated with severe pain. This lesion was present from birth [Figure 1]. On clinical examination the baby had petechiae over the face, a large hemangioma over left scapular area extending on to the left axilla and left mammary area [Figure 2]. The lesion was warm, tender with peau de orange appearance. There was no bruit over the lesion. Her systemic examination was normal. Our presumptive diagnosis was KMS and we investigated accordingly [Table 1].
Table 1: Investigations

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Figure 1: Hemangioma at eight months of age

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Figure 2: Hemangioma at one year two months of age

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A hemogram revealed anemia with severe thrombocytopenia and prolongation of bleeding and clotting time and the presence of increased fibrin degradation products. Skin biopsy of the lesion showed dilated irregular thin walled vascular channels lined by flattened endothelial cells in the dermis. Ultrasonography of the chest revealed large ill defined heterogeneous solid diffuse lesion noted over the left side of the upper chest wall extending from front to back and axilla, involving skin and subcutis. Color Doppler showed feeding arterial supply from the intercostals vessels.

With a confirmed diagnosis of KMS, the patient was started on transfusions of platelets and fresh frozen plasma as she had oral bleeds. Oral prednisolone was started 2mg/kg body weight to suppress the hemangioma. Although the coagulopathy reverted to normal in a few days, platelets started decreasing and so she was referred to higher centre for thrombosing the feeding vessels of the hemangioma. However, she was referred back to us as it was not feasible. In spite of increasing the dose of steroids to 5mg /kg body weight, the platelet count continued to drop. It was then that we started her on interferon α2b in a dose of 3million IU/m2 /day subcutaneously. Within four weeks her platelet counts became normal and the hemangioma started regressing [Figure 3] and [Figure 4].
Figure 3: Two months after steroids and interferon

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Figure 4: One year after interferon therapy

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   Discussion Top


KMS is a rare syndrome. The objectives of treatment of KMS are to prevent bleeding from thrombocytopenia and consumptive coagulopathy and to induce vascular tumor regression. Thrombocytopenia in KMS is due to peripheral destruction, as platelets are trapped in these vascular tumors. Platelet transfusions can also exacerbate bleeding. [7] Platelet transfusions should therefore be used only when bleeding is clinically apparent as in our patient.

Tumor regression can be induced by various treatment modalities, including prednisolone, [1],[3],[5] dipyridamole, pentoxyfylline, [1] ticlopidine, [1] and heparin. [1] IFN, [9] vincristine, radiotherapy, and embolization of the feeding vessel to the tumor. [6-8] Steroid was used as first line therapy because it is available at a relatively low cost. IFN α 2b was used as second line therapy because of its known efficacy, although the cost of the treatment was considerably higher than that of the first line drugs.

Our patient had complications on high dose prednisolone, particularly hypertension and cushingoid appearance. Nevertheless, because of its low cost and ease of administration, steroid may still be most appropriate first line drug for the initial few weeks of therapy.

IFN has been used in the treatment of KMS since 1992 due to its antiproliferative and antiangiogenic effect. [9] In some series, IFN has been recommended as first line therapy The two forms of IFN α 2a and IFN α 2b, are probably identical in efficacy, and both forms have been used in KMS and life- threatening hemangiomas of infancy at a dosage of 3 million IU/m2 /day, with favorable results.

The known adverse effects of IFN therapy include fever, neutropenia, mild anemia, mild elevation of liver function test, and, most deleteriously, spastic diplegia in infancy. [10] In conclusion, KMS may be treated in a stepwise approach. High dose steroid is probably the most cost effective, but does not result in a high rate of response and is not tolerated well. Response to IFN is more favorable, but life threatening adverse events can occur.

 
   References Top

1.Enjolras O, Wassef M, Mazoyer E, Frieden IJ, Rieu PN, Drouet L, et al. Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr 1999;130:631-40.  Back to cited text no. 1      
2.Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child 1940;59:1063-70.  Back to cited text no. 2      
3.Hall GW. Kasabach-Merritt syndrome; pathogenesis and management. Br J Haematol 2001;118:851-62.  Back to cited text no. 3      
4.Beaubien ER, Ball NJ, Storwick GS. Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol 1998;38:782-99.  Back to cited text no. 4      
5.Wananukul S, Nuchprayoon I, Seksarn P. Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon. Int J Dermatol 2003;42:741-8.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Phillips WG, Marsden JR. Kasabach-Merritt syndrome exacerbated by platelet transfusion. J R Soc Med 1993;86:231-2.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Larsen EC, Zinkham WH, Eggleston JC, Zitelli BJ. Kasabach-Merritt syndrome: therapeutic considerations. Pediatrics 1987;79:971-80.  Back to cited text no. 7  [PUBMED]    
8.Shin HY, Ryu KH, Ahn HS. Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome. Pediatr Int 2000;42:620-4.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Ezekowitz RA, Mulliken JB, Folkman J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992;326:1456-63.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Dubois J, Hershon L, Carmant L, Bιlanger S, Leclerc JM, David M. Toxicity profile of interferon alfa-2b in children: A prospective evaluation. J Pediatr 1998;135:782-5.  Back to cited text no. 10      


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]

This article has been cited by
1 Neonatal thrombocytopenia: Etiology and management (Chapter )
Paes, B., Nagel, K., Chan, A.
Thrombocytopenia: Causes, Diagnosis and Treatment. 2012; : 125-195
[Pubmed]



 

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    Abstract
    Introduction
    Case Report
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