Immune thrombocytopenic purpura: Difference between revisions

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{{AdultPage|Immune thrombocytopenic purpura (peds)}}
For pregnant patients, see [[ITP in Pregnancy]]
==Background==
==Background==
===GENERAL===
*Abbreviation: ITP
# low platelets and mucocutaneous bleeding
*Known as both "idiopathic" or "immune" thrombocytopenic purpura
# half occur in kids
*Acquired autoimmune disease resulting in destruction of platelets
# either primary or secondary to other disorder
*Because circulating platelets are functional, life-threatening bleeding only once platelet count <10K
# either acute or chronic > 6mo
*Chronically, the disease follows a course that is usually stable with intermittent and episodic flares, leading to clinically relevant thrombocytopenia<ref>Cines DB and Blanchette VS.  Immune Thrombocytopenic Purpura. New England Journal of Medicine.  Vol 346 (13); 995-1008.</ref>
# usually presents days to weeks after infection
# in kids, 70% resolve in 6 mo regardless of tx or not
# adults usually chronic, insidous onset and more likely in women than men


===PATHOPHYS===
{{Acute vs chronic ITP}}
# mediated by autoantibodies- platelets coated with igg antibodies undergo accelerated clearance in spleen and liver
# can get compensatory increase in platelet production in some pts
# in other pts, get abnormal production destruction of intermedullary antibody coated platelets
# level of thrombopoietin not increased
# methods to tx are directed at 1- antibody prodctn, 2- platelet sensitization, 3- clearance, 4- production.


===GENETICS===
==Clinical Features==
# can be seen in certain families and twins
[[File:Purpura.jpg|thumb|Petechiae in a patient with ITP.]]
# HLA type may predict response to different tx's
[[File:Petechia on the tongue.jpg|thumb|Petechiae on the tongue in a patient with ITP.]]
# no consistent assoc btwn HLA types and ITP however
[[File:Petechia lower leg2.jpg|thumb|Petechiae on the lower leg in a patient with ITP.]]
[[File:PMC4192833 TODENTJ-8-164 F1.png|thumb|Unprovoked gingival bleeding as a presenting symptom in ITP.]]
*[[Petechiae]]
*[[Epistaxis]]
*[[Gingival bleeding]]
*[[Vaginal Bleeding (Non-Pregnant)|Menorrhagia]]
*[[GI bleeding]]
*[[Intracranial hemorrhage]]


==Diagnosis==
==Differential Diagnosis==
# is dx of exclusion
{{Thrombocytopenia}}
#can get secondary ITP in SLE, antiphospholid syn, immune deficiency states, leukemia, lymphoma, HIV, Hep C, heparin and quinidine.
{{Purpura DDX}}
# duration of bleeding tells acute from chronic
#absence of systemic symptoms helps rule out secondary forms and other dxs
# family hx usually negative.
# physical exam shows platelet type bleeding-petechia, purpura, conj hem,
# if spleen very big, consider other dx
# can see anemia if have lots of blood loss
# can see large young plateltets on perif smear
# bone marrow bx- debatetable- but need it if atypical case, fever, pain, neutropenia, unexplained macrocytosis.
# dx by measuring platelet assoc antibodies- fair sensitivity, specificity and predictive value
# neg test does not rule it out


==Initial Management==
==Evaluation==
===ADULTS===
*Diagnosis of exclusion
# if plts above 50k- will dx incidentally
*Must differentiate acute ITP from chronic ITP, which suggests an underlying disorder
# plts 30k- 50k- will get easy bruising
*CBC shows normal cell lines except for the platelets (may have mild anemia)
# petechia and purpura at 1-k- 30k
#<10k at risk for internal bleeding
# adults usually require tx with prednsone at tme of presentation
# can also use Anti- D immune globulin in Rh + pt
# IV IG used for internal bleeding
# splenectomy within 3- 6mo if more than 10- 20 mg prednisone per day.


===KIDS===
==Management==
#outcome so good usually dont need to tx unless risk of intracranial hem- plts <10k- 20k
===Adults===
# usually observe and avoid sports, ASA..
*Patients with severe bleeding should receive high dose steroids (ie methylprednisolone 1g IV vs dexamethasone 40mg IV), IVIG (1g/kg), and platelet transfusion<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>
# if severe thrombocytopenia- can tx with IV IG or anti D IG. or prednisone.
*Adults with minor mucosal or no bleeding and a platelet count <30x10<sup>9</sup>/L should receive steroids<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>
**Suggested regimens: prednisone 0.5-2mg/kg/day for 14 days with taper vs dexamethasone 40mg/day for 4 days


Urgent Tx in Kids
===Pediatrics===
# neuro sxs, internal bleeding, surgery- tx with iv solumedrol and iv ig and platelet infusion
*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<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>
# consider vincristine
*Patients with a non-life-threatening bleed or reduced quality of life can receive prednisone 2-4mg/kg/day for up to 7 days<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>
# consider splenectomy
*Patients with mild or no bleeding have no benefit with steroids or IVIG regardless of platelet count<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>
# plasmapharesis not helpful
# consider antifibrinolytic/ aminocaproic acid fo mucosal bleeding
# for severe bleeding- iv ig and iv platelets


==Management of First Relapse==
===Other Options===
===ADULTS===
*[[Splenectomy]] if above fails (only 2/3 effective)
#no tx if asymptomatic with plt 30k
*[[Rho(D) Immune Globulin (RhoGAM)]]
# splenectomy if relapse or not response to steroids, iv ig or iv anti D ig
**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


===KIDS===
# delay splenectomy as long as possible- or if symptomatic > 1yr or severe thrombocytopenia


==SPLENECTOMY==
==Medication Dosing==
# immunize against H flu, pneumoccus, and give daily prophylactic penicilin for 1 yr
{{MedicationDose
| drug = Methylprednisolone
| dose = 1g
| route = IV
| context = Severe bleeding with high-dose steroids
| indication = Immune thrombocytopenic purpura
| population = Adult
}}
{{MedicationDose
| drug = Dexamethasone
| dose = 40mg daily x 4 days
| route = IV/PO
| context = First-line steroid regimen
| indication = Immune thrombocytopenic purpura
| population = Adult
}}
{{MedicationDose
| drug = IVIG
| dose = 1g/kg
| route = IV
| context = Severe bleeding
| indication = Immune thrombocytopenic purpura
| population = Adult
}}
{{MedicationDose
| drug = Methylprednisolone
| dose = 30mg/kg
| route = IV
| context = Severe bleeding with high-dose steroids
| indication = Immune thrombocytopenic purpura
| population = Pediatric
}}
{{MedicationDose
| drug = IVIG
| dose = 1g/kg
| route = IV
| context = Severe bleeding
| indication = Immune thrombocytopenic purpura
| population = Pediatric
}}


==Chronic Refractory ITP==
==Disposition==
#30- 40% adults do not respond to splenectomy or have relapse p splenectomy
# hunt for accessory splene
# tx with prednisone, iv ig
# next step is medicine that impedes platelet clearance- Danazol, Dapsone, vinca alkaloids.
# if splenectomized, do not give ig anti D ig.
# immunesuppression if platelet <20k and no response to above tx- use azathioprine or cyclophosphamide
# Azathioprine watch LFT's- is also carcinogenic
# Cyclophoshamide- marrow suppression, hem cystitis, bladder fibrosis, alopecia, infertility, leukemia and teratogenic.


===Children===
===Adults===
# azathioprine alone or in combination with cyclophosphamide.
*Admit any patient with significant bleeding or platelet count < 20 x10<sup>9</sup>
*Patients without significant bleeding and a platelet count > 20 x10<sup>9</sup> can be managed as an outpatient with hematology
 
===Pediatrics===
*Admit patients with significant bleeding
*Patients with minor or no bleeding may be saely managed as an outpatient with hematology regardless of patient count.


==Complications==
==Complications==
# fatal bleeding from ICH- risk greatest in elderly or recurrent bleeding or no response to tx
*Rare: more common in elderly
**[[Intracerebral bleeding]]
**Severe [[GI bleeding]]


==Source ==
==See Also==
6/06 MISTRY
*[[ITP in Pregnancy]]
*[[TTP]]


==References==
<references/>
[[Category:Heme/Onc]]
[[Category:Heme/Onc]]

Latest revision as of 18:44, 20 March 2026

This page is for adult patients. For pediatric patients, see: Immune thrombocytopenic purpura (peds)

For pregnant patients, see ITP in Pregnancy

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]


Acute vs Chronic ITP

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

Petechiae in a patient with ITP.
Petechiae on the tongue in a patient with ITP.
Petechiae on the lower leg in a patient with ITP.
Unprovoked gingival bleeding as a presenting symptom in ITP.

Differential Diagnosis

Thrombocytopenia

Decreased production

Increased platelet destruction or use

Drug Induced

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)

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

Adults

  • Patients with severe bleeding should receive high dose steroids (ie methylprednisolone 1g IV vs dexamethasone 40mg IV), IVIG (1g/kg), and platelet transfusion[2]
  • Adults with minor mucosal or no bleeding and a platelet count <30x109/L should receive steroids[3]
    • 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[4]
  • Patients with a non-life-threatening bleed or reduced quality of life can receive prednisone 2-4mg/kg/day for up to 7 days[5]
  • Patients with mild or no bleeding have no benefit with steroids or IVIG regardless of platelet count[6]

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


Medication Dosing

Methylprednisolone 1g IV Dexamethasone 40mg daily x 4 days IV/PO IVIG 1g/kg IV Methylprednisolone 30mg/kg IV IVIG 1g/kg IV

Disposition

Adults

  • Admit any patient with significant bleeding or platelet count < 20 x109
  • Patients without significant bleeding and a platelet count > 20 x109 can be managed as an outpatient with hematology

Pediatrics

  • Admit patients with significant bleeding
  • Patients with minor or no bleeding may be saely managed as an outpatient with hematology regardless of patient count.

Complications

See Also

References

  1. ↑ Cines DB and Blanchette VS. Immune Thrombocytopenic Purpura. New England Journal of Medicine. Vol 346 (13); 995-1008.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ 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.
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