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Recombinant interleukin-6 in the treatment of congenital thrombocytopenia associated with absent radii.

AbstractPURPOSE:
The role interleukin-6 (IL-6) in the treatment of congenital thrombocytopenias is unknown. The purpose of this case report is to describe the efficacy of IL-6 in a child with thrombocytopenia with absent radii (TAR) syndrome.
METHODS:
A 23-month-old girl with TAR syndrome was treated with recombinant IL-6 (Sigosix; Serono Laboratories, Norwell, MA) at a dose of 7 micrograms/kg subcutaneously daily. Complete blood counts were monitored weekly. The child was closely monitored for any toxicity.
RESULTS:
After 3 weeks of therapy, the patient had an increase in platelet count from a baseline of 5,000 to 8,000/microliter to a maximal level of 33,000/microliter. She was platelet transfusion-independent during IL-6 therapy. Fevers and chills, the main toxicities encountered, were controlled with acetaminophen and ibuprofen. An increase in the IL-6 dose caused anemia with no further increase in platelet count. After discontinuation of the drug, her hemoglobin rose to baseline and the platelet count returned to pretreatment levels.
CONCLUSIONS:
We conclude that IL-6 may benefit some children with TAR syndrome. The role of IL-6 and other thrombopoietic agents in the treatment of TAR and other congenital thrombocytopenias deserves further clinical study.
AuthorsV M Aquino, M M Mustafa, L Vaickus, R Wooley, G R Buchanan
JournalJournal of pediatric hematology/oncology (J Pediatr Hematol Oncol) 1998 Sep-Oct Vol. 20 Issue 5 Pg. 474-6 ISSN: 1077-4114 [Print] United States
PMID9787323 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Interleukin-6
  • Recombinant Proteins
Topics
  • Administration, Cutaneous
  • Female
  • Humans
  • Infant
  • Interleukin-6 (administration & dosage)
  • Radius (abnormalities)
  • Recombinant Proteins (administration & dosage)
  • Syndrome
  • Thrombocytopenia (drug therapy, physiopathology)

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