Thrombotic thrombocytopenic purpura medical therapy: Difference between revisions

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Latest revision as of 17:02, 31 March 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

TTP is a medical emergency and requires prompt treatment. The mainstay of treatment for TTP is plasma exchange therapy. Surgery is recommended among all patients who develop TTP with no responsr to medical therapy.

Medical Therapy

Patients with TTP are treated with daily plasma therapy.^[1]
Patients with TTP are treated with rituximab to prevent complications.^[1]
Vincristine (VCR) is a agent to treat TTP patients resistant to conventional plasma exchange (PEX) and pharmacologic therapy.^[2]
Cyclosporine A ^[3]

TTP treatment

- Plasma therapy
  - Fresh frozen plasma(FFP) for patients who have inherited TTP
  - Plasma exchange(PEX) for patients who have acquired TTP
  - (1.53 plasma volume exchange for the first procedures, followed by 1.03 patient plasma volume thereafter)
- Steroids
  - Treatment of acquired TTP is high dose methylprednisolone
    - Methylprednisolone (10 mg/kg/day for 3 days and then 2.5 mg/kg/day), this dose is more efficacious than standard dose (1 mg/kg/day)
- Rituximab
  - Standard treatment(375 mg/m2 in 4 weekly doses)^[4]

References

↑ ^1.0 ^1.1 Joly, Bérangère S.; Coppo, Paul; Veyradier, Agnès (2017). "Thrombotic thrombocytopenic purpura". Blood. 129 (21): 2836–2846. doi:10.1182/blood-2016-10-709857. ISSN 0006-4971.
↑ Bobbio-Pallavicini E, Porta C, Centurioni R, Gugliotta L, Vianelli N, Tacconi F, Billio A, Ascari E (April 1994). "Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma-exchange. The Italian Cooperative Group for TTP". Eur. J. Haematol. 52 (4): 222–6. PMID 8005232.
↑ Medina PJ, Sipols JM, George JN (September 2001). "Drug-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome". Curr. Opin. Hematol. 8 (5): 286–93. PMID 11604563.
↑ Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ (August 2012). "Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies". Br. J. Haematol. 158 (3): 323–35. doi:10.1111/j.1365-2141.2012.09167.x. PMID 22624596.

Template:WH Template:WS

[JolyCoppo2017-1] 1.0 ^1.1 Joly, Bérangère S.; Coppo, Paul; Veyradier, Agnès (2017). "Thrombotic thrombocytopenic purpura". Blood. 129 (21): 2836–2846. doi:10.1182/blood-2016-10-709857. ISSN 0006-4971.

[pmid8005232-2] Bobbio-Pallavicini E, Porta C, Centurioni R, Gugliotta L, Vianelli N, Tacconi F, Billio A, Ascari E (April 1994). "Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma-exchange. The Italian Cooperative Group for TTP". Eur. J. Haematol. 52 (4): 222–6. PMID 8005232.

[pmid11604563-3] Medina PJ, Sipols JM, George JN (September 2001). "Drug-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome". Curr. Opin. Hematol. 8 (5): 286–93. PMID 11604563.

[pmid22624596-4] Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ (August 2012). "Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies". Br. J. Haematol. 158 (3): 323–35. doi:10.1111/j.1365-2141.2012.09167.x. PMID 22624596.

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[2]

[3]

[4]

@@ Line 1: / Line 1: @@
 __NOTOC__
-{{Xyz}}
+{{Thrombotic thrombocytopenic purpura}}
-{{CMG}}; {{AE}}
+{{CMG}}; {{AE}} {{S.G.}}
 ==Overview==
-There is no treatment for [disease name]; the mainstay of therapy is supportive care.
+TTP is a [[medical]] [[emergency]] and requires [[prompt]] treatment. The mainstay of treatment for TTP is [[Blood plasma|plasma]] exchange [[therapy]]. Surgery is recommended among all patients who develop TTP with no responsr to [[medical]] [[therapy]].
-OR
-Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
-OR
-The majority of cases of [disease name] are self-limited and require only supportive care.
-OR
-[Disease name] is a medical emergency and requires prompt treatment.
-OR
-The mainstay of treatment for [disease name] is [therapy].
-OR
-The optimal therapy for [malignancy name] depends on the stage at diagnosis.
-OR
-[Therapy] is recommended among all patients who develop [disease name].
-OR
-Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
-OR
-Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
-OR
-Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
-OR
-Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
 ==Medical Therapy==
-*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
+*[[Patient|Patients]] with TTP are treated with daily [[Plasma|plasm]]<nowiki/>a [[Therapy|therap]]<nowiki/>y.<ref name="JolyCoppo2017">{{cite journal|last1=Joly|first1=Bérangère S.|last2=Coppo|first2=Paul|last3=Veyradier|first3=Agnès|title=Thrombotic thrombocytopenic purpura|journal=Blood|volume=129|issue=21|year=2017|pages=2836–2846|issn=0006-4971|doi=10.1182/blood-2016-10-709857}}</ref>
-*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
+*[[Patient|Patients]] with TTP are treated with [[rituximab]] to [[Prevention|prevent]] [[Complication (medicine)|complication]]<nowiki/>s.<ref name="JolyCoppo2017" />
-*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
+*[[Vincristine]] (VCR) is a agent to treat TTP [[Patient|patients]] resistant to [[Conventional medicine|conventional]] [[plasma]] [[Exchange transfusion|exchange]] (PEX) and [[pharmacologic]] [[therapy]].<ref name="pmid8005232">{{cite journal |vauthors=Bobbio-Pallavicini E, Porta C, Centurioni R, Gugliotta L, Vianelli N, Tacconi F, Billio A, Ascari E |title=Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma-exchange. The Italian Cooperative Group for TTP |journal=Eur. J. Haematol. |volume=52 |issue=4 |pages=222–6 |date=April 1994 |pmid=8005232 |doi= |url=}}</ref>
-*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
+*[[Cyclosporine]] A <ref name="pmid11604563">{{cite journal |vauthors=Medina PJ, Sipols JM, George JN |title=Drug-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome |journal=Curr. Opin. Hematol. |volume=8 |issue=5 |pages=286–93 |date=September 2001 |pmid=11604563 |doi= |url=}}</ref>
-===Disease Name===
+===TTP treatment===
-* '''1 Stage 1 - Name of stage'''
-** 1.1 '''Specific Organ system involved 1'''
-*** 1.1.1 '''Adult'''
-**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''
-**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
-**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
-**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
-**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
-**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
-*** 1.1.2 '''Pediatric'''
-**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
-***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
-***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
-***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
-***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
-***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
-****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
-***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
-***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
-***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
-***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
-** 1.2 '''Specific Organ system involved 2'''
-*** 1.2.1 '''Adult'''
-**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
-*** 1.2.2  '''Pediatric'''
-**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
-* 2 '''Stage 2 - Name of stage'''
+**'''Plasma therapy'''
-** 2.1 '''Specific Organ system involved 1 '''
+*** [[Fresh frozen plasma]]([[Fresh frozen plasma|FFP]]) for [[Patient|patients]] who have [[inherited]] TTP
-**: '''Note (1):'''
+*** [[Plasma]] [[Exchange transfusion|exchange]](PEX) for [[Patient|patients]] who have [[Acquired disorder|acquired]] TTP
-**: '''Note (2)''':
+*** (1.53 [[plasma]] [[volume]] [[Exchange transfusion|exchange]] for the first procedures, followed by 1.03 [[patient]] [[plasma]] [[volume]] thereafter)
-**: '''Note (3):'''
+****
-*** 2.1.1 '''Adult'''
+**'''Steroids'''
-**** Parenteral regimen
+***Treatment of [[acquired]] TTP is high [[dose]] [[methylprednisolone]]
-***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
+**** [[drug name|Methylprednisolone]] (10 mg/kg/day for 3 days and then 2.5 mg/kg/day), this dose is more [[efficacious]] than [[standard]] [[dose]] (1 mg/kg/day)
-***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
+**'''Rituximab'''
-***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
+*** [[Standard]] treatment(375 mg/m2 in 4 weekly [[Dose|doses]])'''<ref name="pmid22624596">{{cite journal |vauthors=Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ |title=Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies |journal=Br. J. Haematol. |volume=158 |issue=3 |pages=323–35 |date=August 2012 |pmid=22624596 |doi=10.1111/j.1365-2141.2012.09167.x |url=}}</ref>'''
-**** Oral regimen
-***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
-***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
-***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
-***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
-***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
-***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
-*** 2.1.2 '''Pediatric'''
-**** Parenteral regimen
-***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
-***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
-***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
-**** Oral regimen
-***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
-***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
-***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
-***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
-***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
-***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
-** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
-**: '''Note (1):'''
-**: '''Note (2)''':
-**: '''Note (3):'''
-*** 2.2.1 '''Adult'''
-**** Parenteral regimen
-***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
-***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
-***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
-**** Oral regimen
-***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
-***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
-***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
-***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
-***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
-***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
-*** 2.2.2 '''Pediatric'''
-**** Parenteral regimen
-***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
-***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
-***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
-**** Oral regimen
-***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
-***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
-***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
-***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
-***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
-***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
 ==References==