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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406 EISSN: 2008-2150
Vol. 18, Num. 4, 2008, pp. 381-385 








Iranian Journal of Pediatrics, Vol. 18, No. 4, Dec, 2008, pp. 381-385


Chronic Childhood ITP: High Dose Parenteral Dexamethasone Therapy 


Abdulah Banihashem, MD, Pediatrician; Farhad  Heydarian*, MD, Pediatrician; Simin Hyradfar, MD, Pathologist; Morteza Jafarzadeh, MD, Pediatrician


Department of Pediatrics & Pathology, Mashhad University of Medical Sciences, IR Iran


* Correspondence author;
  Address: Division of Pediatrics, Ghaem Hospital, Ahmad-abad Blved, Mashhad, IR Iran
E-mail: heydarianf@mums.ac.ir


Received: 10/02/08; Revised: 20/08/08; Accepted: 21/09/08


Code Number: pe08062 


Key words: ITP; Dexamethasone; Childhood


Chronic immune thrombocytopenic purpura (ITP) means at least 6-month duration of ITP[1]. An autoimmune process leads to production of anti platelet membrane anti bodies that destruct the platelets. Although in most cases no significant hemorrhage occurs, but bleeding in brain is possible. Also, thrombocytopenia limits the natural activity of children. 


So, it is justified to find ways to treat these patients more safely. In a study high dose dexamethasone was administrated cyclically. The results were good and it was recommended to be applied to patients with symptomatic chronic ITP[2]. In another in patients with chronic ITP who did not respond to IVIG, Danazol and Rhogam, high dose oral dexamethasone was effective[3]. 


This study was performed on 10 cases of chronic ITP aged 2 to 14 years old who were admitted in pediatric ward, Dr. Sheikh Hospital, Mashad/Iran, during the years 2002 and 2003. The patients consisted of six females and 4 males (age below 18 years). They had chronic ITP and they had no response to previous treatments including IVIG, oral glucocorticoides, Rhogam administration and/or splenectomy. 


Patients received intravenous dexa­methasone 40 mg/m2/day in divided doses, for 4 days a week, then every 4 weeks for 4 cycles. This study was confirmed by the Research center and Ethics Committee of the University of Mashad.


Among 10 patients there were 4 males and 6 femaleswith a mean age of 7.1±3.8 years (2 to 14 years). The most common symptom was epistaxis that was seen in 80% of cases. Before starting the protocol, platelet count was below 30.000 mm3(mean platelet count was 29300 ranging 4000-66000). After treatment, platelet count rose above 30.000 mm3 (mean platelet count 31.500 ranging 8.000-58.000) (SD: 22437 vs 19193) P-value: 0.87 Wilcoxan Rank test). Patients were symptom free at least 4 months after completing the therapy. 


Due to production of anti membranous anti platelet antibodies, chronic ITP results in 1 to 20% of ITP patients[1,4,5]. It is not clearly known what the predictable risk factors are, but it seems that some circumstances are predisposing to chronicity such as onset of the disorder after 10 years of age, insidious symptoms before diagnosing the disease, more platelet count at onset of symptoms and female gender. 


Occasionally, after months to a few years, spontaneous recovery occurs[1]. In comparison with no therapy, it is detected that starting treatment with IVIG or glucocorticoides is associated with some recovery[6-7]. Although splenectomy remains among the most effective measures in treating the chronic ITP[8], because of its serious complications other therapies should be tried.


In one study in which 11 cases received high dose dexamethasone in divided doses for 4 days and every 28 days for 2-4 cycles, complete or partial response in some cases revealed[9]. Also in another study on 20 children who received high dose dexamethasone, 5 cases had complete or partial remission[2]. 


 In other reports, 5 males and 7 females treated with high dose oral dexamethasone, at least half of cases went to complete or partial remission for a long time[3] and pulsed high dose therapy of dexamethasone with low side effects was reported[10].


 In our study we tried high dose dexamethasone for a few days and repeated at regular intervals. Prescription of drug was fairly successful; rise of platelet count above 30.000 mm3 was seen. It seems that it may be the result of glucocorticoides' regulation of immune system that acts via suppression of phagocytic action of reticuloendothelial system and auto antibodies production inhibition. Also, temporary lymphopenia can cause inhibition of more lymphocyte lysis through redistribution of lymphocytes to other lymphoid tissues. It can suppress preactivated T cells. And finally, it inhibits antibody binding on antigenic sites on platelets[11,12,13]. Up to now, patients are being followed. They have received no other treatment. CBC is checked periodically.


Because of short course glucocorticoide therapy, long term complications such as osteoporosis, cataract, glaucoma, decreased linear growth, Cushingoid faces and obesity were not seen. No complication is observed.      It seems that if chronicity occurs in a child with ITP, high dose dexamethasone administered parenterally can be helpful. 


References


  1. Kuhne T, Imbach P. Management of children with acute and chronic immune thrombocytopenic purpura. Transfusion Science. 1998;19(3):261-8.
    2. 
  
  2. Hedlund-Treutiger I, Henter JI, Elinder G. Randomized study of IVIg and high-dose dexamethasone therapy for children with chronic idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol. 2003; 25(2):139-44.
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  3. Wali YA, AI Lamki Z, Shah W, et al. Pulsed high-dose dexamethasone therapy in children with chronic idiopathic thrombo­cytopenic purpura. Pediatr Hematol Oncol. 2002;19(5):329-35.
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  4.  Portielje JE, Westendorp RGJ, Kluin-Nelemans HC, et al. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001; 97(9):2549-54.
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  5. Beardsly DS, Narthan DG. Platelet abnormalities in infancy and childhood. In: Nathan DG, Oski FA, editors. Hematology of Infancy and Childhood, vol.5. Philadelphia: Saunders; 1998; Pp: 1585-616.
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  6. Blannchette VS, Luke B, Andrew M, et al. A prospective, randomized trial of high–dose intravenous immune globulin G therapy, oral prednisone therapy, and no therapy in childhood acute immune thrombocytopenic purpura. J Pediatr. 1993;123(6):989-95.
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  7.  Blanchette V, Imbach P, Andrew M, et al. Randomized trial of intravenous immune globulin G, intravenous anti-D, and oral prednisone in childhood acute immune thrombocytopenic purpura. Lancet. 1994; 344(8924):703-7.
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  8. Syed NN, Adil SN, Sajid R, et al. Chronic ITP: analysis of various factors at presentation which predict failure to first line treatment and their response to second line therapy. J Pak Med Assoc. 2007; 57(3): 126-9.
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  9.  Kuhne T, Freedman J, Semple JW, et al. Platelet and immune responses to oral cyclic dexamethasone therapy in childhood chronic immune thrombo­cytopenic purpura.  J Pediatr. 1997;130(1):17-24.
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  10. Ferrara M, Borrelli B, Greco N, et al. Side effects of corticosteroid therapy in children with chronic idiopathic thrombocytopenic purpura. Hematology. 2005;10(5):401-3.
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  11. Boumpas DT, Chrousos GP, Wilder RL, et al. Glucocorticoid therapy for immune-mediated diseases: basic and clinical correlates. Ann Intern Med. 1993; 119(12): 1198-208.
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  12. Paliogianni F, Ahuja SS, Balow JP, et al. Novel mechanism for inhibition of human T cells by glucocorticoids. J Immunol. 1993;151(8):4081-9.
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  13. Mizutani H, Furubayashi T, Imai Y, et al. Mechanisms of corticosteroid action in immune thrombocytopenic purpura (ITP): experimental studies using ITP-prone mice (NZW 5 BXSB) F1. Blood. 1992;79(4):942-7.
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