Jump to comment:

• Author's reply
  Suna Emir
  Published on: 5 March 2003
• Kasabach-Merritt syndrome and interferon alpha: still a controversial issue.
  Paolo Biban
  Published on: 17 February 2003

• Published on: 5 March 2003

  Author's reply

  • Suna Emir, Pediatric Oncologist
  • Other Contributors:
    • Canan Akyüz.

  Dear Editor

  We thank Dr Biban for his comments on our article.[1] We share his concerns that our case has some interesting and unique points. As he/she stated that our case was the first reported case of a Kasabach-Merrit syndrome (KSM) associated with a lipomatous tumor. Her severe findings due to KSM and as well as her progressively enlarging mass on the shoulder were dramatically responded to interferon therapy.[...

  Show More

  Dear Editor

  We thank Dr Biban for his comments on our article.[1] We share his concerns that our case has some interesting and unique points. As he/she stated that our case was the first reported case of a Kasabach-Merrit syndrome (KSM) associated with a lipomatous tumor. Her severe findings due to KSM and as well as her progressively enlarging mass on the shoulder were dramatically responded to interferon therapy.[2] On the other hand, Dr Biban critisized that we did not adequately emphasise the neurologic side effects of interferon treatment. It has been reported that interferon alpha has been responsible for various neurologic side efects. But, there is no clear data indicating the frequency of these neurologic side effects in children although it has been reported some adverse effects related to the interferons such as spastic diplegia. Short-term interferon therapy has been safely used at our department in treating various different conditions, particularly in the complex hemangiomas for many years. No serious side effects of interferon therapy except mild fever, malaise, leukopenia and elevation of liver transaminases has been observed. These minor side effects were reversible by stopping therapy for a short period in our patients. In only one patient who received the long term interferon therapy, a peripheral neuropathy developed during the treatment.

  He was a 15-year-old boy with Hodgkin’s disease who received interferon therapy as an adjuvant immunotherapy post autologous stem cell transplant. Peripheral neuropathy developed twenty months after ÝFN treatment.[3] Prolonged administration of high dose interferon treatment has been usually considered as a major cause in the development of neurologic side effects. High cumulative dose combined with the prolonged treatment may be have important role in occuring this complication in our previous case. We concluded that the use of interferon in children affected by KSM or in children with various benign tumors containing vascular elements is a still good therapeutic alternative. If the duration of treatment and the cumulative doses of interferon are closely monitorised, severe neurologic side effects during IFN therapy would not be an important problem. When the use of interferons in various conditions gradually expands, the data related to the adverse neurologic side effects will be increased and better understood.

  References

  (1) Akyüz C, S Emir, M Büyükpamukçu, et al. Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome. Arch Dis Child 2003;88:67-68.

  (2) Biban P. Kasabach-Merrit syndrome and interferon-alpha: still a controversial issue [electronic response to Akyüz et al, Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome]. archdischild.com 2003http://adc.bmjjournals.com/cgi/eletters/archdischild;88/1/67#422

  (3) Emir S, Kutluk T, Chan KW, Varlý K, Yalçýn B, Büyükpamukçu M. Peripheral neuropathy during interferon therapy in a child with Hodgkin’s disease. Ped Hem Oncol 1999;16:557.

  Show Less

  Conflict of Interest:
  None declared.

  • Back to top
• Published on: 17 February 2003

  Kasabach-Merritt syndrome and interferon alpha: still a controversial issue.

  • Paolo Biban, paediatric intensivist

  Dear Editor

  We read with interest the paper by Akyuz and colleagues,[1] which described a 2 year old patient with a Kasabach-Merritt syndrome (KMS) secondary to an infiltrating angiolipoma, who was successfully treated with interferon alpha 2a (IFN-alpha).

  Although the case presents some points of interest, such as the previously unreported association of a KMS with a lipomatous tumor and the potential effec...

  Show More

  Dear Editor

  We read with interest the paper by Akyuz and colleagues,[1] which described a 2 year old patient with a Kasabach-Merritt syndrome (KMS) secondary to an infiltrating angiolipoma, who was successfully treated with interferon alpha 2a (IFN-alpha).

  Although the case presents some points of interest, such as the previously unreported association of a KMS with a lipomatous tumor and the potential effectiveness of IFN-alpha for these patients, the authors did not emphasise the increasing body of concerns associated with the use of IFN-alpha in children affected by KMS. Indeed, several authors have recently warned about potential adverse effects related to the use of this drug, the most worrisome being spastic diplegia.[2-4]

  Regardless the aetiology, the KMS could be a potentially fatal condition due to severe thrombocytopenic coagulopathy. Corticosteroids are still considered as the treatment of choice, but several other therapeutic options for non responding patients have been proposed, including vincristine, embolisation, surgical excision, radiotherapy. Despite IFN-alpha has also been shown to be an effective therapy for patients with KMS,[5] it may cause transient or permanent neurological disabilities.[6] Furthermore, neurotoxicity of IFN-alpha, the pathogenesis of which remains unclear, is usually detected late during the course of treatment, and early diagnosis may result very challenging particularly in young children, who appear to be at higher risk for their immature central nervous system. Although neurological complications can spontaneously reverse after discontinuation of IFN-alpha, some patients may experience permanent disabilities.

  Unfortunately, no predictive risk factors regarding either the onset of symptoms or the reversibility of neurological deficits have been identified as yet. This fact may preclude a proper counselling about the actual risk of neurological deficits associated with a long term treatment with IFN-alpha. Interestingly, we have recently observed a term newborn with life-threatening KMS due to a large haemangioma of her left cervical region. She was treated with corticosteroids without success. Fearing neurological consequences, her parents opposed the IFN-alpha treatment, then the patient underwent three cycles of selective embolisation, which resulted in a shrinking of the mass and a reduction of its feeding arterial vessels. At nearly three months of age the haemangioma was successfully excised, although bleeding was indeed a major concern during the surgical intervention.

  Would a treatment with IFN-alpha have been less invasive and risky? What about the safest and most effective therapeutic option for these patients? Further controlled studies are urgently needed in order to answer these questions.

  References

  (1) Akyüz C, S Emir, M Büyükpamukçu, et al. Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome. Arch Dis Child 2003;88:67-68.

  (2) Barlow CF, Priebe CJ, Mulliken JB, et al. Spastic diplegia as a complication of interferon Alfa-2a treatment of hemangiomas of infancy. J Pediatr 1998;132:527-30.

  (3) Worle H, Maass E, Kohler B, et al. Interferon alpha-2a therapy in haemangiomas of infancy: spastic diplegia as a severe complication. Eur J Pediatr 1999;158:344.

  (4) Vesikari T, Nuutila A, Cantell K. Neurologic sequelae following interferon therapy of juvenile laryngeal papilloma. Acta Pediatr Scand 1988;77:619-22.

  (5) Ezekowitz RAB, Mulliken JB, Folkman J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992;326:1456-63.

  (6) Vial T, Descotes J. Clinical toxicity of interferons. Drug Saf 1994; 10:115-50.

  Show Less

  Conflict of Interest:
  None declared.

  • Back to top