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Medication

Medication Summary

The treatment of immune thrombocytopenia (ITP) requires considerable individualization.^[100]Medications used for ITP include the following:

Corticosteroids
Immunoglobulins
Thrombopoieting receptor agonists (TPO-RAs)
Spleen tyrosine kinase (SYK) inhibitor
Rituximab
Immunosuppressive agents

Prednisone (Deltasone, Orasone, Sterapred)

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Oral corticosteroid that is used most frequently because of its relatively low cost, known adverse effects, and long-term clinical record. DOC for initial treatment of ITP in children and adults. For aggressive treatment, may be combined with IV RhIG or IVIG. In emergency, replace PO prednisone with IV methylprednisolone.

Methylprednisolone (Solu-Medrol)

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DOC for the initial management of severe bleeding tendency in ITP. IV is recommended when the most rapid and reliable treatment of ITP is required. In this situation, combine with IV RhIG in qualified Rh(D)-positive patients or IVIG in Rh(D)-negative patients or unqualified Rh(D)-positive patients.

Dexamethasone (Baycadron, Decadron DSC, Dexamethasone Intensol)

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Alternative corticosteroid for the initial management of severe bleeding tendency in ITP. IV is recommended when the most rapid and reliable treatment of ITP is required.

IV RhIG (WinRho SDF)

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Specialized immunoglobulin product manufactured from pools of plasma from Rh(D)-negative persons and alloimmunized to D blood group antigen. Subjected to anion-exchange column chromatography to permit IV infusion and solvent-detergent treatment and nanofiltration to reduce infectivity by lipid-enveloped viruses. Induces immune RBC hemolysis in Rh(D)-positive recipients, decreasing function of mononuclear macrophages (reticuloendothelial blockade) and sparing immunoglobulin-coated platelets from splenic destruction.

IVIG (Gamimune, Gammagard, Sandoglobulin)

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Large dose of 1 g/kg induces decreased function of mononuclear macrophages (reticuloendothelial blockade), sparing immunoglobulin-coated platelets from splenic destruction. Used with IV methylprednisolone to manage acute ITP in children. Decreased time to an increased platelet count compared with IV RhIG, but the difference does not appear to be clinically significant. Compared with IV RhIG, associated with more adverse effects, longer infusions, and increased cost, causing many hematologists to prefer IV RhIG as a supplement to corticosteroids, at least for Rh(D)-positive patients.

Azathioprine (Imuran)

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May be effective in some patients with ITP whose conditions do not or no longer have response to corticosteroids, IV RhIG, or IVIG. May be used with prednisone to reduce dose of prednisone or as another PO medication to delay splenectomy.

Cyclophosphamide (Cytoxan)

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May be useful in some patients whose conditions do not or no longer have a response to corticosteroids, IV RhIG, IVIG, or splenectomy. Induces less of a decrease in platelet count than other immunosuppressive alkylating agents.

Danazol (Danocrine)

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May impair the clearance of immunoglobulin-coated platelets and decreases autoantibody production. Increased platelet counts in 40-50% of patients, particularly postmenopausal women.

Rituximab (Rituxan)

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Chimeric monoclonal antibody directed against the CD20 antigen on the surface of normal and malignant B lymphocytes. Antibody is IgG kappa immunoglobulin with murine light- and heavy-chain variable sequences and human constant region sequences.

Avatrombopag (Doptelet)

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Second-generation orally administered thrombopoietin receptor agonist (TPO-RA). Stimulates proliferation and differentiation of megakaryocytes from bone marrow progenitor cells, resulting in an increased production of platelets. It is indicated for thrombocytopenia in adults with chronic immune thrombocytopenia (ITP) who have had an insufficient response to a previous treatment.

Romiplostim (Nplate)

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An Fc-peptide fusion protein (peptibody) that increases platelet production through binding and activation of the thrombopoietin (TPO) receptor, a mechanism similar to endogenous TPO. Indicated for chronic immune thrombocytopenia (ITP) in adults who are newly diagnosed or those who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy.

It is also indicated in children aged ≥1 year with ITP for ≥6 months who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy.

Eltrombopag (Promacta)

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Oral thrombopoietin (TPO) receptor agonist. Interacts with transmembrane domain of human TPO receptor and induces megakaryocyte proliferation and differentiation from bone marrow progenitor cells. Indicated for thrombocytopenia associated with chronic idiopathic thrombocytopenic purpura in patients experiencing inadequate response to corticosteroids, immunoglobulins, or splenectomy. Not for use to normalize platelet counts, but used when clinical condition increases bleeding risk.

Fostamatinib (Tavalisse)

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Fostamatinib is a spleen tyrosine kinase (SYK) inhibitor. The major active metabolite of fostamatinib (ie, R406) inhibits signal transduction of Fc-activating receptors and B-cell receptor, thereby reducing antibody-mediated destruction of platelets. It is indicated for thrombocytopenia in patients with chronic immune thrombocytopenia (ITP) who have had an insufficient response to a previous treatment.

Questions

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Media Gallery

Peripheral blood smear from a patient with immune thrombocytopenia (ITP) shows a decreased number of platelets, a normal-appearing neutrophil, and normal-appearing erythrocytes. ITP is diagnosed by excluding other diseases; therefore, the absence of other findings from the peripheral smear is at least as important as the observed findings. This smear demonstrates the absence of immature leukocytes (as in leukemia) and fragmented erythrocytes (as in thrombotic thrombocytopenic purpura) and no clumps of platelets (as in pseudothrombocytopenia).

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Table 1. Bleeding scale for pediatric patients with ITP

Table 1. Bleeding scale for pediatric patients with ITP

Grade	Bleeding	Management approach
Grade 1 (minor)	Minor bleeding, few petechiae (≤100 total) and/or ≤5 small bruises (≤3 cm in diameter), no mucosal bleeding	Consent for observation
Grade 2 (mild)	Mild bleeding, many petechiae (>100 total) and/or >5 large bruises (>3 cm in diameter), no mucosal bleeding	Consent for observation
Grade 3 (moderate)	Moderate bleeding, overt mucosal bleeding, troublesome lifestyle	Intervention to reach grade 1 or 2
Grade 4 (severe)	Severe bleeding, mucosal bleeding leading to decrease in Hb > 2 g/dL or suspected internal hemorrhage	Intervention

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Author

Craig M Kessler, MD, MACP Professor, Department of Medicine and Pathology, Division of Hematology/Oncology, Georgetown University School of Medicine; Director, Clinical Coagulation Laboratory, Lombardi Comprehensive Cancer Center, Georgetown University Hospital

Disclosure: Received honoraria from NovoNordisk for consulting; Received grant/research funds from NovoNordisk for other; Received honoraria from Baxter-Immuno for consulting; Received honoraria from Octapharma for speaking and teaching; Received grant/research funds from Octapharma for none; Received consulting fee from Amgen for consulting; Received honoraria from Bayer for review panel membership.

Coauthor(s)

Hira Latif, MD Assistant Professor, Division of Hematology/Oncology, Marlene and Stewart Greenebaum Comprehensive Cancer Center, University of Maryland Medical Center

Hira Latif, MD is a member of the following medical societies: American College of Physicians, American Society of Clinical Oncology, American Society of Hematology, International Society on Thrombosis and Haemostasis, Pakistan Medical and Dental Council

Disclosure: Nothing to disclose.

Julia M Cunningham, MD Assistant Professor of Medicine, Division of Hematology and Oncology, Attending Physician in Hematology, Lombardi Comprehensive Cancer Center, Georgetown University Medical Center

Julia M Cunningham, MD is a member of the following medical societies: American Medical Association, American Society of Clinical Oncology, American Society of Hematology, Hemostasis and Thrombosis Research Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald A Sacher, MD, FRCPC, DTM&H Professor Emeritus of Internal Medicine and Hematology/Oncology, Emeritus Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MD, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Srikanth Nagalla, MD, MS, FACP Chief of Benign Hematology, Miami Cancer Institute, Baptist Health South Florida; Clinical Professor of Medicine, Florida International University, Herbert Wertheim College of Medicine

Srikanth Nagalla, MD, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alexion; Alnylam; Kedrion; Sanofi; Dova; Apellis; Pharmacosmos<br/>Serve(d) as a speaker or a member of a speakers bureau for: Sobi; Sanofi; Rigel.

Additional Contributors

S Gerald Sandler, MD, FCAP, FACP Professor Emeritus of Medicine and Pathology, Georgetown University School of Medicine

S Gerald Sandler, MD, FCAP, FACP is a member of the following medical societies: American Association of Blood Banks, College of American Pathologists, International Society of Blood Transfusion

Disclosure: Nothing to disclose.

Immune Thrombocytopenia (ITP) Medication

Medication Summary

Corticosteroids

Prednisone (Deltasone, Orasone, Sterapred)

Prednisone (Deltasone, Orasone, Sterapred)

Methylprednisolone (Solu-Medrol)

Methylprednisolone (Solu-Medrol)

Dexamethasone (Baycadron, Decadron DSC, Dexamethasone Intensol)

Dexamethasone (Baycadron, Decadron DSC, Dexamethasone Intensol)

Blood Products

IV RhIG (WinRho SDF)

IV RhIG (WinRho SDF)

IVIG (Gamimune, Gammagard, Sandoglobulin)

IVIG (Gamimune, Gammagard, Sandoglobulin)

Immunosuppressive Antimetabolites

Azathioprine (Imuran)

Azathioprine (Imuran)

Synthetic Antineoplastic Drugs

Cyclophosphamide (Cytoxan)

Cyclophosphamide (Cytoxan)

Androgens

Danazol (Danocrine)

Danazol (Danocrine)

Monoclonal Antibodies

Rituximab (Rituxan)

Rituximab (Rituxan)

Thrombopoietic Agents

Avatrombopag (Doptelet)

Avatrombopag (Doptelet)

Romiplostim (Nplate)

Romiplostim (Nplate)

Eltrombopag (Promacta)

Eltrombopag (Promacta)

SYK Inhibitors

Fostamatinib (Tavalisse)

Fostamatinib (Tavalisse)

Immune Thrombocytopenia (ITP) Medication

Medication Summary

Corticosteroids

Prednisone (Deltasone, Orasone, Sterapred)

Methylprednisolone (Solu-Medrol)

Dexamethasone (Baycadron, Decadron DSC, Dexamethasone Intensol)

Blood Products

IV RhIG (WinRho SDF)

IVIG (Gamimune, Gammagard, Sandoglobulin)

Immunosuppressive Antimetabolites

Azathioprine (Imuran)

Synthetic Antineoplastic Drugs

Cyclophosphamide (Cytoxan)

Androgens

Danazol (Danocrine)

Monoclonal Antibodies

Rituximab (Rituxan)

Thrombopoietic Agents

Avatrombopag (Doptelet)

Romiplostim (Nplate)

Eltrombopag (Promacta)

SYK Inhibitors

Fostamatinib (Tavalisse)

References

Tables

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