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Terminology used for immune thrombocytopenia

Term	Definition
ITP	An acquired thrombocytopenia caused by autoantibody-mediated destruction of platelets; the autoantibodies may also affect megakaryocytes and impair platelet production. ITP is a diagnosis of exclusion; the diagnosis cannot be made without investigations for other possible causes of thrombocytopenia.
Bleeding severity	Several terms are used to describe bleeding severity. These are generally qualitative, and clinical judgment is needed to make these assessments. Critical bleeding – Bleeding into a critical anatomical site or bleeding that causes hemodynamic instability or respiratory compromise. Includes intracranial, intraspinal, intraocular, retroperitoneal, pericardial, or intramuscular bleeding with compartment syndrome. Severe bleeding – Bleeding that results in a fall in hemoglobin of 2 or more g/dL or requires transfusion of 2 or more units of pRBCs but does not meet the definition of critical bleeding. Minor bleeding – Bleeding that does not meet criteria for severe or critical bleeding. Patients may have serious bleeding with more modest thrombocytopenia (platelet counts ≥20,000/microL), but in these cases it is important to identify another cause such as trauma or an anatomical lesion.
Primary versus secondary ITP	Primary ITP is ITP not triggered by an apparent associated condition. Secondary ITP is ITP associated with a predisposing condition (examples listed below).* By convention, the associated condition is noted in parentheses, as in "Secondary ITP (SLE-associated)."
Drug-induced ITP (DITP)	DITP is a drug reaction due to drug-dependent antibodies that cause platelet destruction. This syndrome should be distinguished from other, non-immune mechanisms of drug-induced thrombocytopenia such as bone marrow suppression.^¶
New, persistent, and chronic ITP	The time elapsed since diagnosis determines whether ITP is referred to as newly diagnosed, persistent, or chronic. We use the following definitions: Newly diagnosed – Up to 3 months since diagnosis Persistent – 3 to 12 months since diagnosis Chronic – More than 12 months since diagnosis
First-line versus second-line therapy	First-line generally refers to glucocorticoids, IVIG, and anti-D immune globulin; these therapies are typically used for initial treatment of newly diagnosed patients or those with recurrent thrombocytopenia following a response. Second-line generally refers to treatment for ITP that does not respond to first-line therapy or that relapses after first-line therapy is tapered. Second-line treatments include splenectomy, rituximab, and TPO-RAs.
Complete response versus partial response	A response to therapy is defined as at least doubling of the baseline platelet count and a platelet count >30,000/microL. A complete response is defined as a platelet count ≥100,000/microL; a partial response refers to a platelet count that doubles and is between 30,000 and 100,000/microL.
Relapse	A fall in platelet count below 30,000/microL following a partial or complete response.
Refractory disease	Definitions vary and include disease requiring treatment that persists or recurs after splenectomy or for which splenectomy is not an option.

ITP was previously called "idiopathic thrombocytopenic purpura" or "immune thrombocytopenic purpura" but these terms are no longer used and have been replaced by "immune thrombocytopenia" to reflect the known immunologic mechanism and absence of purpura in some individuals. ^[1,2]Some clinical trials use different definitions or criteria, especially those published before consensus criteria were developed. Refer to UpToDate for details of ITP diagnosis and management.

ITP: immune thrombocytopenia; IVIG: intravenous immune globulin; DITP: drug-induced ITP; TPO-RA: thrombopoietin receptor agonist.
* Examples of associated conditions include:

Chronic lymphocytic leukemia (CLL)
HIV
Hepatitis C virus (HCV) infection
Systemic lupus erythematosus (SLE)

¶ A separate table listing drugs associated with DITP is provided in UpToDate.

References:

Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 2009; 113:2386.
Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv 2019; 3:3829.

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