Table 3.

Published remission rate for children and adults with chronic ITP

PublicationStudy designPopulation (n)Inclusion criteria

  • Definition of CR regarding:

  • platelet count threshold (×109/L)

  • time duration without treatment

FU periodCR rate at FU in patients with chronic ITP
Sailer et al26  Monocentric cohort, retrospective Adults (n = 59) Severe chronic ITP, nonsplenectomized (old definition) 

  • platelets >100

  • no information about time “off treatment”

 		3 y 0.5%/mo from 6 mo to 3 y 
Bourgeois et al27  Prospective Children and adults (n = 47) Chronic ITP (old definition) and refractory to splenectomy 

  • platelets >100

  • no information about time “off treatment,” except for 9 patients with no treatment after splenectomy

 		Median FU 7.5 y after splenectomy 57% 
Schifferli et al2  Prospective registry Children and adults Chronic ITP (new definition) 

  • platelets >100

  • with/without treatment

 		2 y 30% 
Medeiros et al28  Review of 9 studies Children (n = 415) Chronic ITP (old and new definition, and others) Very heterogenous, (analysis includes 100 patients with splenectomy) Heterogenous, up to 15 y 40%-79% (for patients without splenectomy) 
Blanchette et al29  Review of 7 studies Children (n = 337) Chronic ITP (old and new, and others) 

  • platelet threshold heterogenous

  • treatment stop heterogenous

 		Heterogenous up to 20 y 10%-64% 
Jayabose et al30  Retrospective Children (n = 62) Chronic ITP (old definition) 

  • platelets >100

  • no treatment for >3 mo

 		4 y 45%, CR without splenectomy (68% including patients with splenectomy) 
Donato et al31  Retrospective Children (n = 230) Nonsplenectomized chronic ITP (old definition) 

  • platelets >150

  • no information about time “off treatment”

 		6 mo to 10 y 27%, CR without splenectomy (using new definition of chronic ITP: 13.5% CR)
(33% including patients with splenectomy) 
Bansal et al, 2010 Retrospective Children (n = 270) Chronic ITP (old definition) 

  • platelets >100

  • no treatment ≥6 mo

 		Median FU, 30 mo 25% (predicted CR rate at 5 y 30%, at 10 y 44%) 
Rosthøj et al32  Prospective cohort Children (n = 96) Chronic ITP (old definition) 

  • platelets >150

  • no information about time “off treatment”

  • analysis includes 12 patients with splenectomy

 		7 -93 mo (86 patients with 5 y FU) 56% at 5 y (including patients with splenectomy) 
Neunert et al 201346  Prospective Children (n = 343) Persistent and chronic ITP (new definition) 

  • platelets >100

  • without previous splenectomy

  • with or without drug treatment

 		24 mo 29% 
Shim et al33  Retrospective Children (n = 47) Chronic ITP (new definition) 

  • platelets >100

  • no treatment for 3 mo

 		Median, 4.4 y 45% 
Kim et al34  Retrospective Children (n = 41) Chronic ITP (new definition) 

  • platelets >100

  • with/without treatment

 		2-5 y 76% 
Ducassou et al17  Prospective observational cohort Children (n = 392) Chronic ITP (new definition) 

  • Continuous CR

  • platelets >100

  • no treatment for at least 1 y

 		5-20 y 19.7%, cumulative at 5 y
39.2% cumulative at 10 y
61.2%, cumulative at 20 y 
Pincez et al13  Prospective observational cohort Children (n = 846) Chronic ITP (new definition) 

  • Continuous CR

  • platelets>100

  • no treatment for at least 1 y

 		Median FU for children 5.3 y 23% 
PublicationStudy designPopulation (n)Inclusion criteria

  • Definition of CR regarding:

  • platelet count threshold (×109/L)

  • time duration without treatment

FU periodCR rate at FU in patients with chronic ITP
Sailer et al26  Monocentric cohort, retrospective Adults (n = 59) Severe chronic ITP, nonsplenectomized (old definition) 

  • platelets >100

  • no information about time “off treatment”

 		3 y 0.5%/mo from 6 mo to 3 y 
Bourgeois et al27  Prospective Children and adults (n = 47) Chronic ITP (old definition) and refractory to splenectomy 

  • platelets >100

  • no information about time “off treatment,” except for 9 patients with no treatment after splenectomy

 		Median FU 7.5 y after splenectomy 57% 
Schifferli et al2  Prospective registry Children and adults Chronic ITP (new definition) 

  • platelets >100

  • with/without treatment

 		2 y 30% 
Medeiros et al28  Review of 9 studies Children (n = 415) Chronic ITP (old and new definition, and others) Very heterogenous, (analysis includes 100 patients with splenectomy) Heterogenous, up to 15 y 40%-79% (for patients without splenectomy) 
Blanchette et al29  Review of 7 studies Children (n = 337) Chronic ITP (old and new, and others) 

  • platelet threshold heterogenous

  • treatment stop heterogenous

 		Heterogenous up to 20 y 10%-64% 
Jayabose et al30  Retrospective Children (n = 62) Chronic ITP (old definition) 

  • platelets >100

  • no treatment for >3 mo

 		4 y 45%, CR without splenectomy (68% including patients with splenectomy) 
Donato et al31  Retrospective Children (n = 230) Nonsplenectomized chronic ITP (old definition) 

  • platelets >150

  • no information about time “off treatment”

 		6 mo to 10 y 27%, CR without splenectomy (using new definition of chronic ITP: 13.5% CR)
(33% including patients with splenectomy) 
Bansal et al, 2010 Retrospective Children (n = 270) Chronic ITP (old definition) 

  • platelets >100

  • no treatment ≥6 mo

 		Median FU, 30 mo 25% (predicted CR rate at 5 y 30%, at 10 y 44%) 
Rosthøj et al32  Prospective cohort Children (n = 96) Chronic ITP (old definition) 

  • platelets >150

  • no information about time “off treatment”

  • analysis includes 12 patients with splenectomy

 		7 -93 mo (86 patients with 5 y FU) 56% at 5 y (including patients with splenectomy) 
Neunert et al 201346  Prospective Children (n = 343) Persistent and chronic ITP (new definition) 

  • platelets >100

  • without previous splenectomy

  • with or without drug treatment

 		24 mo 29% 
Shim et al33  Retrospective Children (n = 47) Chronic ITP (new definition) 

  • platelets >100

  • no treatment for 3 mo

 		Median, 4.4 y 45% 
Kim et al34  Retrospective Children (n = 41) Chronic ITP (new definition) 

  • platelets >100

  • with/without treatment

 		2-5 y 76% 
Ducassou et al17  Prospective observational cohort Children (n = 392) Chronic ITP (new definition) 

  • Continuous CR

  • platelets >100

  • no treatment for at least 1 y

 		5-20 y 19.7%, cumulative at 5 y
39.2% cumulative at 10 y
61.2%, cumulative at 20 y 
Pincez et al13  Prospective observational cohort Children (n = 846) Chronic ITP (new definition) 

  • Continuous CR

  • platelets>100

  • no treatment for at least 1 y

 		Median FU for children 5.3 y 23% 

CR, complete remission; FU, follow-up.

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