Seventeen Years of Experience with Chronic Idlopathlc Thrombocytopenic Purpura in Childhood. Is Therapy Always Better?

Abstract

Between 1975 and 1992 450 children with idiopathic thrombocytopenic purpura (ITP) were diagnosed, and of those 100 (22%) developed the chronic form of the disease. Approximately half the patients with chronic ITP presented with mild to moderate hemorrhagic manifestations at the onset of purpura (30 cases) and/or later during the course of the disease (25 cases). The incidence of intracranial hemorrhage was 1%, and the mortality rate due to overwhelming septicemia after splenectomy was also 1%. Overall one-third of the patients received no therapy; two-thirds of them went into spontaneous remission within 8 months to 8 years from the onset of ITP. Steroids given in conventional or high doses (51 cases) achieved a transient (if any) rise in platelet count, but in no case were steroids curative. Remission related to intravenous immune globulin (IVIG) therapy was noticed in 38.5% of the children (10 of 26) after variable courses. The response rate to splenectomy was 95.0%. Ultimately the long-term outcome in children with chronic ITP was as follows: remission, 58 cases (spontaneous, 30; after IVIG therapy, 10; after splenectomy, 18); hemostatic platelet values, 22 cases (spontaneous, 16; after IVIG, 5; after splenectomy, 1). Thirteen children were lost in follow-up, and 7 remain thrombocytopenic but asymptomatic. These data indicate that chronic ITP in childhood runs a benign course in most cases and may remit with or without therapy even several years from onset. Therefore, therapeutic intervention has to be individualized, and splenectomy, which is not always safe, should be reserved for problematic cases that fail to respond to conventional therapeutic modalities.

Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines

“…Patients >10 years and/or with a platelet count ≥20 ×10 9 /l at diagnosis are at greater risk of progression towards chronic ITP [3,9]. Nevertheless, >50% of patients with chronic ITP have spontaneous remission of the disease within 4 years from the diagnosis [10,11,12]; in fact, about 10–15% of patients per year achieve normalization of their platelet count in this time period [10]. Sex, age and platelet count at diagnosis do not appear to be prognostic for the likelihood of spontaneous recovery in children with chronic ITP [10].…”

Çocukta Kroni̇k İmmun Trombosi̇topeni̇k Purpura (63 Hastada Tedavi̇ Sonuçlari)

“…Hastalarımı-zın %71,4'ünde sadece peteşi ve purpura şeklinde deri bulgularının oluşu, düşük trombosit sayısına rağmen kronik ITP'de klinik gidişin şaşırtıcı şekilde tehlikesiz olduğunu bildiren yayınlara uygunluk göstermektedir (3,6). Kronik ITP'de spontan remisyonun %66,6 ve %63,6 gibi oldukça yüksek olduğunu bildiren (1,5) görüşlere karşın Mısır'da yapılan bir çalışmada spontan remisyon oranı %5 olarak bildirilmektedir (23). Biz izlediğimiz 63 hastanın hiçbirinde spontan remisyon görmedik.…”

“…Steroid uyguladığımız bu 60 hastada kalıcı remisyon oranının düşük olduğunu (7/60, %11,6) gördük. Literatürde de kronik ITP'de steroide yanıt oranının oldukça düşükolduğu (%11) bildirilmektedir (1,5,25). Bazı klinikler steroidi, splenektomiye hazırlık döneminde trombosit sayısını geçici olarak yükselltmek amacıyla kısa süreli kulanımını önermek-tedir.…”

“…IVIG'in farklı dozda uygulanmaları arasında da sonuç açısından önemli bir fark bulunmamıştır (15). Kronik ITP'de ise IVIG tedavi sonuçları birbiri ile çelişen şe-kildedir (1,5,18). Kronik ITP'de IVIG tedavisini özel-likle infantlarda ve küçük çocuklarda splenektomiyi geciktirmede, splenektomiye yanıt vermeyen ağır hastalarda da kanama riskini azaltmak amacıyla kullanıl-ması yaygın olarak kabul edilen bir görüştür (3,18,20).…”

Intracranial Hemorrhage in Chronic Childhood Itp

“…It is therefore both startling and disturbing to read of the extraordinarily high incidence of ICH in Turkish children with chronic ITP in this issue of Pediatric Hematology and Oncology [4] and hard to reconcile the findings (9 events in a study cohort of 78) with previous published experience such as a contemporary study from nearby Greece, where the incidence of ICH in a group of 100 children with chronic ITP was 1% [ 5 ] . Even more surprising and completely unprecedented is the fact that seven (possibly eight) of the nine children had platelet counts >30 x lO"/L at the time of their ICH, inevitably giving rise to speculation that either the children had something other than simple ITP or the intracranial pathology may not have been simply a consequence of defective primary phase hemostasis-or both.…”