Diferencia entre revisiones de «Immune thrombocytopenic purpura»
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==Management== | ==Management== | ||
American Society of Hematology Recommendations <ref>Neunert C, Terrell DR, Arnold DM, Buchanan G, Cines DB, Cooper N, Cuker A, Despotovic JM, George JN, Grace RF, Kühne T, Kuter DJ, Lim W, McCrae KR, Pruitt B, Shimanek H, Vesely SK. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019 Dec 10;3(23):3829-3866. doi: 10.1182/bloodadvances.2019000966. Erratum in: Blood Adv. 2020 Jan 28;4(2):252. PMID: 31794604; PMCID: PMC6963252.</ref> | |||
===Adults=== | |||
*Patients with severe bleeding should receive high dose steroids (ie methylprednisolone 1g IV vs dexamethasone 40mg IV), IVIG (1g/kg), and platelet transfusion | |||
* | *Adults with minor mucosal or no bleeding and a platelet count <30x10<sup>9</sup>/L should receive steroids | ||
* | **Suggested regimens: prednisone 0.5-2mg/kg/day for 14 days with taper vs dexamethasone 40mg/day for 4 days | ||
* | |||
* | ===Pediatrics=== | ||
*Patients with severe bleeding should receive high dose steroids (ie methylprednisolone 30mg/kg IV vs dexamethasone 28mg/m<sup>2</sup> IV), IVIG (1g/kg), and platelet transfusion | |||
*Patients with a non-life-threatening bleed or reduced quality of life can receive prednisone 2-4mg/kg/day for up to 7 days | |||
*Patients with mild or no bleeding have no benefit with steroids or IVIG regardless of platelet count | |||
===Other Options=== | ===Other Options=== | ||
| Línea 81: | Línea 62: | ||
*[[Rituximab]] | *[[Rituximab]] | ||
**May help as ITP is autoimmune process | **May help as ITP is autoimmune process | ||
*Thrombopoietin receptor agonists (eltrombopag, romiplsotim) may be effective in steroid-refractory cases | |||
==Disposition== | ==Disposition== | ||
Revisión del 14:27 20 may 2023
This page is for adult patients. For pediatric patients, see: Immune thrombocytopenic purpura (peds).
Background
- Abbreviation: ITP
- Known as both "idiopathic" or "immune" thrombocytopenic purpura
- Acquired autoimmune disease resulting in destruction of platelets
- Because circulating platelets are functional, life-threatening bleeding only once platelet count <10K
- Chronically, the disease follows a course that is usually stable with intermittent and episodic flares, leading to clinically relevant thrombocytopenia[1]
ITP Types
| Charateristic | Acute ITP | Chronic ITP |
| Common age group | Younger children | Adults |
| Sex | Affects men/women equally | Usually women |
| Duration | Resolves in 1-2 months | Lasts > 3 months |
| Cause | Typically follows viral illness | More likely to have an underlying disease or autoimmune disorder (e.g. SLE) |
| Prognosis | Rarely remits spontaneously or with treatment |
Clinical Features
Differential Diagnosis
Thrombocytopenia
Decreased production
- Marrow infiltration (tumor or infection)
- Viral infections (rubella, HIV)
- Marrow suppression (commonly chemotherapy or radiation)
- Congenital thrombocytopenia
- Fanconi anemia
- Alport syndrome
- Bernand Soulier
- Vitamin B12 and/or folate deficiency
Increased platelet destruction or use
- Idiopathic thrombocytopenic purpura
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- Disseminated Intravascular Coagulation (DIC)
- Viral infections (HIV, mumps, varicella, EBV)
- Drugs (heparin, protamine)
- Postransfusion or Posttransplantation
- Autoimmune destruction (SLE or Sarcoidosis)
- Mechanical destruction
- Artificial valves
- ECMO
- HELLP syndrome
- Excessive hemorrhage
- Hemodialysis, extracorporeal circulation
- Splenic Sequestration
- Occurs in Sickle cell disease and Cirrhosis
Drug Induced
- sulfa antibiotics, ETOH, ASA, thiazide diuretics/furosemide
Comparison by Etiology
| ITP | TTP | HUS | HIT | DIC | |
|---|---|---|---|---|---|
| ↓ PLT | Yes | Yes | Yes | Yes | Yes |
| ↑PT/INR | No | No | No | +/- | Yes |
| MAHA | No | Yes | Yes | No | Yes |
| ↓ Fibrinogen | No | No | No | No | Yes |
| Ok to give PLT | Yes | No | No | No | Yes |
Petechiae/Purpura (by cause)
- Abnormal platelet count and/or coagulation
- Septicemia
- Idiopathic thrombocytopenic purpura (ITP)
- Hemolytic uremic syndrome
- Leukemia
- Coagulopathies (e.g. hemophilia)
- Henoch-Schonlein Purpura (HSP)
- Acute hemorrhagic edema of infancy (AHEI)
- Hypersensitivity vasculitis
- Primary vasculitides
- Wegener's
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Secondary vasculitides
- Trauma
Evaluation
- Diagnosis of exclusion
- Must differentiate acute ITP from chronic ITP, which suggests an underlying disorder
- CBC shows normal cell lines except for the platelets (may have mild anemia)
Management
American Society of Hematology Recommendations [2]
Adults
- Patients with severe bleeding should receive high dose steroids (ie methylprednisolone 1g IV vs dexamethasone 40mg IV), IVIG (1g/kg), and platelet transfusion
- Adults with minor mucosal or no bleeding and a platelet count <30x109/L should receive steroids
- Suggested regimens: prednisone 0.5-2mg/kg/day for 14 days with taper vs dexamethasone 40mg/day for 4 days
Pediatrics
- Patients with severe bleeding should receive high dose steroids (ie methylprednisolone 30mg/kg IV vs dexamethasone 28mg/m2 IV), IVIG (1g/kg), and platelet transfusion
- Patients with a non-life-threatening bleed or reduced quality of life can receive prednisone 2-4mg/kg/day for up to 7 days
- Patients with mild or no bleeding have no benefit with steroids or IVIG regardless of platelet count
Other Options
- Splenectomy if above fails (only 2/3 effective)
- Rho(D) Immune Globulin (RhoGAM)
- Will induce mild hemolysis in Rh+ patients that decreases macrophage activity
- Spleen will not destroy platelets covered in antibodies
- Rituximab
- May help as ITP is autoimmune process
- Thrombopoietin receptor agonists (eltrombopag, romiplsotim) may be effective in steroid-refractory cases
Disposition
- Admit: platelet count <20K or significant mucous membrane bleeding
- Discharge: platelet count >20K AND asymptomatic OR only minor petechiae
Complications
- Rare: more common in elderly
See Also
References
- ↑ Cines DB and Blanchette VS. Immune Thrombocytopenic Purpura. New England Journal of Medicine. Vol 346 (13); 995-1008.
- ↑ Neunert C, Terrell DR, Arnold DM, Buchanan G, Cines DB, Cooper N, Cuker A, Despotovic JM, George JN, Grace RF, Kühne T, Kuter DJ, Lim W, McCrae KR, Pruitt B, Shimanek H, Vesely SK. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019 Dec 10;3(23):3829-3866. doi: 10.1182/bloodadvances.2019000966. Erratum in: Blood Adv. 2020 Jan 28;4(2):252. PMID: 31794604; PMCID: PMC6963252.