EHA Library - The official digital education library of European Hematology Association (EHA)

IMMUNE MEDIATED THROMBOCYTOPAENIA IN CHILDREN: WHAT HAPPENS AFTER THE REFERRAL? : A 7 YEAR RETROSPECTIVE REVIEW IN A TERTIARY PAEDIATRIC HAEMATOLOGY CENTRE
Author(s): ,
Rincy Koshy
Affiliations:
Paediatric Haematology,Our Lady's Hospital For Sick Children,Dublin ,Ireland
Beatrice Nolan
Affiliations:
Paediatric Haematology,Our Lady's Hospital for Sick Children,Dublin,Ireland
(Abstract release date: 05/17/18) EHA Library. Koshy R. 06/14/18; 216813; PB2355
Rincy Koshy
Rincy Koshy
Contributions
Abstract

Abstract: PB2355

Type: Publication Only

Background

Immune thrombocytopaenia (ITP) develops secondary to the production of auto-antibodies against platelets leading to isolated thrombocytopaenia. The cause is unknown in most cases, although ITP may be triggered by a viral illness . It is characterised by spontaneous bruising, petechiae and mucosal bleeding. ITP resolves in approximately 90% of children without intervention. However, a minority will continue to have low platelet counts for over 1 year ( chronic ITP) . Treatment is indicated for severe or prolonged bleeding.  The majority of children do not require treatment . This condition can result in considerable concern from diagnosing clinicians and parents alike in both acute and chronic settings.

Aims

This study aims to perform a retrospective review of all children referred /attending our haematology department with a diagnosis of ITP and report on outcomes, treatment tolerability and describe the relationship, if any, between chronic ITP and the progression to development of auto-immune disease in a cohort of paediatric patients.   

Methods

A retrospective review of patients referred to the Haematology Department  between 2010-2017 with a platelet count <100x 10(9) platelets per litre. Demographics, disease-related data, relevant auto-immune history and laboratory results were collected from medical records entered and analysed using Excel. 

Results

Demographics: 140 patients were identified, 71 male and 69 female. At diagnosis, 78 were between 0 and 4 years of age, 35 between 5 and 10 years and 27 between 10 and 15 years. 

Preceding events: 3 patients had history of recent vaccinations . 6 patients had Epstein Barr Virus associated ITP. 

Course of thrombocytopenia : In 79 patients , platelet count recovered in less than 6 months. 43 patients had chronic ITP : 19/78 between 0 and 4 years of age, 13 / 35 between 5 and 9 years of age, and  11 / 27 between 10 and 15 years of age. Platelet count returned to normal in 17 patients who had ITP for more than 12 months.  3 patients developed recurrent ITP after initial recovery of platelet count.

Bleeding: 27 patients received treatment for bleeding : 24 for epistaxis, 1 for haematuria, 2 for GI bleeds. 4 patients received precautionary treatment following trauma, 7 received treatment for dental/surgical procedures. 7 of 37 patients who received intravenous immunoglobulin developed signs and symptoms suggestive of aseptic meningitis. 

Two patient received platelets (one: Poland, one: Letterkenny) No patients received red cells. 

Treatment of thrombocytopenia. 8 patients received Prednisolone with non sustained increase in platelet count. 3/8 received Rituximab treatment.

Auto-immune disease. 5 patients had co-existing / progression to autoimmune conditions : 1 hyperthyroidism, 1 mixed connective tissue disease and auto-immune hepatitis, 1 systemic lupus erythematosus ,1 autoimmune neutropaenia, IgA deficiency and coeliac disease and 1 evolving connective tissue disease/SICCA syndrome. 

Conclusion

In conclusion, this data illustrates the diversity of ITP in children.  Data here may not reflect the true history of ITP.  Data may be skewed to chronic or atypical patients referred to one tertiary centre.  We would recommend that a national registry be established to capture data on all children presenting with ITP in Ireland.

Session topic: 33.  Platelets disorders

Keyword(s): Children, ITP, Platelet count

Abstract: PB2355

Type: Publication Only

Background

Immune thrombocytopaenia (ITP) develops secondary to the production of auto-antibodies against platelets leading to isolated thrombocytopaenia. The cause is unknown in most cases, although ITP may be triggered by a viral illness . It is characterised by spontaneous bruising, petechiae and mucosal bleeding. ITP resolves in approximately 90% of children without intervention. However, a minority will continue to have low platelet counts for over 1 year ( chronic ITP) . Treatment is indicated for severe or prolonged bleeding.  The majority of children do not require treatment . This condition can result in considerable concern from diagnosing clinicians and parents alike in both acute and chronic settings.

Aims

This study aims to perform a retrospective review of all children referred /attending our haematology department with a diagnosis of ITP and report on outcomes, treatment tolerability and describe the relationship, if any, between chronic ITP and the progression to development of auto-immune disease in a cohort of paediatric patients.   

Methods

A retrospective review of patients referred to the Haematology Department  between 2010-2017 with a platelet count <100x 10(9) platelets per litre. Demographics, disease-related data, relevant auto-immune history and laboratory results were collected from medical records entered and analysed using Excel. 

Results

Demographics: 140 patients were identified, 71 male and 69 female. At diagnosis, 78 were between 0 and 4 years of age, 35 between 5 and 10 years and 27 between 10 and 15 years. 

Preceding events: 3 patients had history of recent vaccinations . 6 patients had Epstein Barr Virus associated ITP. 

Course of thrombocytopenia : In 79 patients , platelet count recovered in less than 6 months. 43 patients had chronic ITP : 19/78 between 0 and 4 years of age, 13 / 35 between 5 and 9 years of age, and  11 / 27 between 10 and 15 years of age. Platelet count returned to normal in 17 patients who had ITP for more than 12 months.  3 patients developed recurrent ITP after initial recovery of platelet count.

Bleeding: 27 patients received treatment for bleeding : 24 for epistaxis, 1 for haematuria, 2 for GI bleeds. 4 patients received precautionary treatment following trauma, 7 received treatment for dental/surgical procedures. 7 of 37 patients who received intravenous immunoglobulin developed signs and symptoms suggestive of aseptic meningitis. 

Two patient received platelets (one: Poland, one: Letterkenny) No patients received red cells. 

Treatment of thrombocytopenia. 8 patients received Prednisolone with non sustained increase in platelet count. 3/8 received Rituximab treatment.

Auto-immune disease. 5 patients had co-existing / progression to autoimmune conditions : 1 hyperthyroidism, 1 mixed connective tissue disease and auto-immune hepatitis, 1 systemic lupus erythematosus ,1 autoimmune neutropaenia, IgA deficiency and coeliac disease and 1 evolving connective tissue disease/SICCA syndrome. 

Conclusion

In conclusion, this data illustrates the diversity of ITP in children.  Data here may not reflect the true history of ITP.  Data may be skewed to chronic or atypical patients referred to one tertiary centre.  We would recommend that a national registry be established to capture data on all children presenting with ITP in Ireland.

Session topic: 33.  Platelets disorders

Keyword(s): Children, ITP, Platelet count

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies