Childhood ITP: 12 months follow‐up data from the prospective registry I of the Intercontinental Childhood ITP Study Group (ICIS)

Imbach, Paul; Kühne, Thomas; Müller, Dominic; Berchtold, Willi; Zimmerman, Sherri A.; Elalfy, Mohsen Saleh; Buchanan, George R.

doi:10.1002/pbc.20453

Cited by 167 publications

(128 citation statements)

References 25 publications

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“…Our results concerning the acute and chronic patients were comparable with other series in terms of the incidence of clinical courses, mean/ median age at diagnosis, male/female ratio, initial PCs, and initial response rates to therapy [12][13][14][15]. At present, it is difficult to foresee at the time of diagnosis the patients who will develop a chronic course; although age older than 10 years, adoles- cence in females, and an initial PC >20x10 9 /L have been associated with a chronic course [12][13][14][15][16].…”

Section: Discussionsupporting

confidence: 77%

“…At present, it is difficult to foresee at the time of diagnosis the patients who will develop a chronic course; although age older than 10 years, adoles- cence in females, and an initial PC >20x10 9 /L have been associated with a chronic course [12][13][14][15][16]. In our study, initial PC >20x10 9 /L and initial diagnosis age >10 years were found to increase the probability of chronic outcome by at least two-fold (OR=2).…”

Section: Discussionmentioning

confidence: 69%

“…All of these authors recommended delay or omission of surgical treatment and reservation of splenectomy for patients with severe symptoms and non-responders. Imbach et al [14] and Donato et al [8] also reported that the cut-off value to define cITP should be changed from 6 months to 12 months. Our observation, too, showed that the cut-off point of cITP should be changed to 12 months since immune thrombocytopenia could persist beyond 6 months and could recover spontaneously in months to years.…”

Section: Discussionmentioning

confidence: 99%

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Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

Yaprak¹,

Atabay²,

Durak³

et al. 2010

Turk J Hematol

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Objective: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia in children. The objective of this study was to evaluate the presenting features, variation in the clinical courses, initial response rate to therapy, and long-term outcome in patients with ITP. Materials and Methods: Three hundred and fifty out of 491 newly diagnosed patients with ITP between the initial diagnosis ages of 6 months to 16 years were included in this retrospective, descriptive study. Patients with acute vs chronic ITP, acute vs recurrent ITP and chronic vs recurrent ITP were compared in terms of age at diagnosis, gender, initial platelet count, response rate to initial therapy, long-term outcome, and total duration of follow-up. Results: The clinical courses of the patients were determined as acute, chronic and recurrent in 63.8%, 29.1%, and 7.1%, respectively. Platelet count >20x10 9 /L and initial diagnosis age >10 years were found to increase the probability of chronic outcome by at least two-fold. Conclusion: It is concluded that ITP in childhood is a common disease with low morbidity and mortality. In addition to the acute and chronic form, a rare recurrent form, which accounts for about 4-7% of all ITP patients, should be considered. (Turk J Hematol 2010; 27: 147-55) Key words: Immune thrombocytopenic purpura, children, clinical course, chronic, recurrent, outcome

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

Yaprak¹,

Atabay²,

Durak³

et al. 2010

Turk J Hematol

View full text Add to dashboard Cite

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“…10 Longer follow-up of the ICIS-I cohort published in 2006 suggests that a 6 month definition of chronic ITP may be too restrictive, since 25.6% of their 308 chronic ITP patients achieved a normal platelet count between 6 and 12 months after diagnosis. 11 Adult subjects are included in the current Pediatric and Adult Intercontinental Registry on Chronic ITP (PARC-ITP), and the UK ITP registries also enroll patients of all ages.…”

Section: Natural History Of Itp and Clinical Outcome Measuresmentioning

confidence: 99%

ITP in the 21st Century

Beardsley¹

2006

Hematology

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Ten years ago the American Society of Hematology published immune (or idiopathic) thrombocytopenic purpura (ITP) practice guidelines based upon a systematic and comprehensive review of the literature.1 The authors acknowledged that hematologists regularly faced clinical management decisions with too little published scientific information about the natural history of ITP. Many of the treatments employed in ITP had been discovered empirically, and therapeutic choices were often guided by published results in case series of selected patients without a control group. Today, a decade later, we benefit from work by many investigators addressing the natural history of ITP and the pathogenic mechanisms contributing to autoimmune platelet destruction. Practice guidelines and a number of expert and consensus opinions in the literature have better defined the field.2-6 The recent era has also brought reports of new prospective clinical trials including multicenter, randomized studies that promise better guidance for the clinical hematologist in the 21st century. Recent clinical investigations of ITP assess outcome measures in addition to platelet count increments including quality of life, 7 severity of bleeding, 8 and complications of therapy. In addition, there are published reports of systematic analyses that help interpret previous literature. Hypothesis-driven studies of ITP patients and experimental animal models have also improved our understanding of the basic cellular and molecular immune mechanisms that contribute to ITP and have thus revealed new therapeutic opportunities.The present contribution will cover some of the recent basic research that has advanced our understanding of the pathogenesis of ITP and will highlight some of the clinical trials that offer insights into the diagnosis, natural history, and management of ITP in adults and children. Promising new therapeutic modalities for chronic refractory ITP will be discussed. Emphasis is placed upon explicitly designed trials. Several important clinical topics could not be included: secondary ITP, immune thrombocytopenia in pregnancy and infancy, the role of viral infection in ITP, and drug-related thrombocytopenia. Space limitations and edi-

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“…Severe bleeding is more likely to occur in children with platelet counts less than 50 × 10 9 /l. The incidence of intracranial hemorrhage in children with ITP is ~0.1-0.5% (9).…”

mentioning

confidence: 99%

B-cell hyperfunction in children with immune thrombocytopenic purpura persists after splenectomy

et al. 2015

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Background: Immune thrombocytopenic purpura (ITP) is characterized by reduced platelet count secondary to immunemediated destruction, this results in an increased bleeding risk. Autoantibodies binding to platelets tag them for premature destruction in the spleen. For this reason, splenectomy is often performed as treatment of chronic forms of disease that are resistant to pharmacological therapy. Methods: We studied 30 patients with ITP and compared them with age-matched controls. results: We show that B cells of patients with chronic ITP are intrinsically hyperreactive, producing more than normal IgG in vivo and plasma cells in vitro. In normal individuals after splenectomy, a significant depletion of memory B cells is observed, associated with loss of reactivity to CpG oligodeoxynucleotide and consequent inability to form antibody-producing cells. In Enzyme-Linked ImmunoSpot Methods, we compared three splenectomized ITP patients relapsing after surgery, 30 healthy controls, and 37 individuals splenectomized for trauma, spherocytosis, thalassemia, nonhematological tumor, and other diseases. conclusions: We confirmed that B cells of ITP patients remain hyperreactive in vitro and form high numbers of antibody-producing cells after splenectomy. Thus, chronic ITP may be associated with intrinsic B-cell hyperfunction, leading to the production of antibodies with multiple specificities including that against platelets.

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Childhood ITP: 12 months follow‐up data from the prospective registry I of the Intercontinental Childhood ITP Study Group (ICIS)

Cited by 167 publications

References 25 publications

Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

ITP in the 21st Century

B-cell hyperfunction in children with immune thrombocytopenic purpura persists after splenectomy

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