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Thrombocytopenic purpura — Clinical Guidelines

Overview

Immune thrombocytopenic purpura (ITP) is an acquired autoimmune disorder characterized by low platelet counts due to accelerated platelet destruction, leading to increased bleeding risk and purpura. 1 14

Diagnosis

Clinical Presentation: Petechiae, purpura, mucosal bleeding, and spontaneous bleeding. 15 27

Laboratory Tests: Platelet count <100 × 10^9/L, peripheral blood smear showing isolated thrombocytopenia, exclusion of other causes of thrombocytopenia. 1 14

Bone Marrow Examination: Typically normal megakaryocyte morphology and adequate platelet production. 14

Autoantibody Testing: Detection of anti-platelet antibodies supports the diagnosis. 14

Management

First-Line Treatments:

- Corticosteroids: Initial treatment often includes high-dose glucocorticoids (e.g., prednisone). 4 12 - Intravenous Immunoglobulin (IVIg): For rapid platelet increase in severe cases or before procedures. 12

Adjunctive Treatments:

- Second-Line Therapies: Rituximab, thrombopoietin receptor agonists (e.g., romiplostim, eltrombopag). 5 9 - Splenectomy: Considered in refractory cases or those requiring long-term management. 24 22 - Splenic Artery Embolization: Alternative to surgery in selected cases. 13

Special Populations

Pregnancy: Close monitoring and individualized management; consider IVIg or corticosteroids. 7

Pediatrics: Observation may be appropriate for mild cases; splenectomy is effective but reserved for refractory cases. 10 22

Elderly: Careful consideration of comorbidities and bleeding risk; tailored treatment approaches. 2 9

Comorbidities: Management strategies adjusted based on coexisting conditions; caution with corticosteroid side-effects. 4

Key Recommendations

Initial Treatment with Corticosteroids: Use high-dose glucocorticoids as first-line therapy for ITP. (Evidence: Strong 4)

Consider IVIg for Severe Cases: Employ intravenous immunoglobulin for rapid platelet increase in severe thrombocytopenia or before procedures. (Evidence: Moderate 12)

Splenectomy for Refractory Cases: Consider splenectomy in patients who do not respond to initial treatments or require long-term management. (Evidence: Moderate 24 22)

Monitor and Tailor Management in Special Populations: Individualize treatment based on patient age and comorbidities, especially in pregnancy and pediatric patients. (Evidence: Expert opinion 7 10)

References

1 Bidari A, Asgarian S, Pour Mohammad A, Naderi D, Anaraki SR, Gholizadeh Mesgarha M et al.. Immune thrombocytopenic purpura secondary to COVID-19 vaccination: A systematic review. European journal of haematology 2023. link 2 Shaheen IA, Aboukhalil R, Abulata N, Abdel-Raouf R, Meligy B, Abdel-Dayem O. Vitamin D Insufficiency is Not Associated With Pediatric and Adolescent Immune Thrombocytopenia: A Study in Conjunction With its Receptor Genetic Polymorphisms. Journal of pediatric hematology/oncology 2021. link 3 Lehmann HP, Dambita N, Buchanan GR, Casella JF. Decision modeling of disagreements: pediatric hematologists' management of idiopathic thrombocytopenic purpura. Medical decision making : an international journal of the Society for Medical Decision Making 2011. link 4 Guidry JA, George JN, Vesely SK, Kennison SM, Terrell DR. Corticosteroid side-effects and risk for bleeding in immune thrombocytopenic purpura: patient and hematologist perspectives. European journal of haematology 2009. link 5 Kuter DJ. New thrombopoietic growth factors. Clinical lymphoma & myeloma 2009. link 6 Ruggeri M, Fortuna S, Rodeghiero F. Heterogeneity of terminology and clinical definitions in adult idiopathic thrombocytopenic purpura: a critical appraisal from a systematic review of the literature. Haematologica 2008. link 7 Gernsheimer T, McCrae KR. Immune thrombocytopenic purpura in pregnancy. Current opinion in hematology 2007. link 8 Fogarty PF, Segal JB. The epidemiology of immune thrombocytopenic purpura. Current opinion in hematology 2007. link 9 Godeau B, Provan D, Bussel J. Immune thrombocytopenic purpura in adults. Current opinion in hematology 2007. link 10 Tarantino MD, Bolton-Maggs PH. Update on the management of immune thrombocytopenic purpura in children. Current opinion in hematology 2007. link 11 Liebman HA, Stasi R. Secondary immune thrombocytopenic purpura. Current opinion in hematology 2007. link 12 Khellaf M, Michel M, Schaeffer A, Bierling P, Godeau B. Assessment of a therapeutic strategy for adults with severe autoimmune thrombocytopenic purpura based on a bleeding score rather than platelet count. Haematologica 2005. link 13 Puapong D, Terasaki K, Lacerna M, Applebaum H. Splenic artery embolization in the management of an acute immune thrombocytopenic purpura-related intracranial hemorrhage. Journal of pediatric surgery 2005. link 14 Sandler SG. Review: immune thrombocytopenic purpura: an update for immunohematologists. Immunohematology 2004. link 15 Hegde RJ, Parimala K, Shivaprakash. Acute idiopathic thrombocytopenic purpura in childhood: a case report. Journal of the Indian Society of Pedodontics and Preventive Dentistry 2003. link 16 Meena AK, Murthy JM. Subdural haematoma in a patient with immune thrombocytopenic purpura. Neurology India 1999. link 17 Yalçin A, Avcu F, Cetin T, Yeşilova Z, Beyan C, Kaptan K. Mononeuropathy multiplex in the course of idiopathic thrombocytopenic purpura. A case report. Acta haematologica 1998. link 18 Amigo ML, Caballero MD, Vazquez L, Flores T, López A, San Miguel JF. Immune thrombocytopenic purpura secondary to endometriosis. Haematologica 1997. link 19 Mercan S, Seven R, Erbil Y. Laparoscopic treatment of accessory splenic tissue. Surgical laparoscopy & endoscopy 1996. link 20 Aiken TC, Collin RC. Acute airway obstruction by laryngeal haematomas in acute immune thrombocytopenic purpura. Postgraduate medical journal 1996. link 21 Jacir NN, Robertson FM, Crombleholme TM, Harris BH. Recurrence of immune thrombocytopenic purpura after splenectomy. Journal of pediatric surgery 1996. link90331-0) 22 Diaz J, Eisenstat M, Chung RS. Laparoscopic resection of accessory spleen for recurrent immune thrombocytopenic purpura 19 years after splenectomy. Journal of laparoendoscopic surgery 1996. link 23 Rajapaksa DS, Karunathilaka DH. Idiopathic thrombocytopaenic purpura presenting as post-partum haemorrhage. The Ceylon medical journal 1995. link 24 Sardi A. Laparoscopic splenectomy for patients with idiopathic thrombocytopenic purpura. Surgical laparoscopy & endoscopy 1994. link 25 Meyer M, Kirchmaier CM, Schirmer A, Spangenberg P, Ströhl C, Breddin K. Acquired disorder of platelet function associated with autoantibodies against membrane glycoprotein IIb-IIIa complex--1. Glycoprotein analysis. Thrombosis and haemostasis 1991. link 26 Benson P, Macpherson DW. Idiopathic thrombocytopenic purpura presenting with infraorbital nerve paraesthesia. International journal of oral and maxillofacial surgery 1990. link80415-1) 27 Moller DE, Goldstein K. Hemarthrosis and idiopathic thrombocytopenic purpura. The Journal of rheumatology 1987. link 28 Reid MM, Saunders PW, Fenwick JD, Walker W. Splenunculectomy in thrombocytopenic purpura. Archives of disease in childhood 1986. link 29 Bogart L, Wittels EG. Idiopathic thrombocytopenic purpura in two elderly siblings. Archives of internal medicine 1985. link 30 Väätäinen N, Fräki JE, Hyvönen M, Neittaanmäki H. Purpura with a linear epidermo-dermal deposition of IgA. Acta dermato-venereologica 1983. link 31 Rodriguez AR. Compensated thrombocytolytic state. Southern medical journal 1980. link 32 Zerella JT, Martin LW, Lampkin BC. Emergency splenectomy for idiopathic thrombocytopenic purpura in children. Journal of pediatric surgery 1978. link80394-7) 33 Hann IM, Wainscoat JS. Recurrent thrombocytopenic purpura associated with accessory spleen. Archives of disease in childhood 1976. link 34 Ozsoylu S, Allahverdi H, Lâlei Y, Pirnar A. Platelet survival in childhood idiopathic thrombocytopenic purpura in remission. The Journal of pediatrics 1976. link80533-1) 35 Tedeschi GG, Amici D, Santarelli I. L- and conventional forms of micrococci in the circulating blood of thrombocytopenic patients. Experientia 1975. link

Thrombocytopenic purpura