Thrombotic thrombocytopenic purpura medical therapy: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{ | {{Thrombotic thrombocytopenic purpura}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} {{S.G.}} | ||
==Overview== | ==Overview== | ||
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]]. | |||
The mainstay of treatment for [ | |||
==Medical Therapy== | ==Medical Therapy== | ||
* | *[[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> | ||
*[[Patient|Patients]] with TTP are treated with [[rituximab]] to [[Prevention|prevent]] [[Complication (medicine)|complication]]<nowiki/>s.<ref name="JolyCoppo2017" /> | |||
*[[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> | |||
*[[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> | |||
*Patients with TTP are treated with rituximab to prevent | ===TTP treatment=== | ||
===TTP treatment | |||
** | **'''Plasma therapy''' | ||
*** (1.53 plasma volume exchange for the first procedures, followed by 1.03 patient plasma volume thereafter) | *** [[Fresh frozen plasma]]([[Fresh frozen plasma|FFP]]) for [[Patient|patients]] who have [[inherited]] TTP | ||
*** [[Plasma]] [[Exchange transfusion|exchange]](PEX) for [[Patient|patients]] who have [[Acquired disorder|acquired]] TTP | |||
*** (1.53 [[plasma]] [[volume]] [[Exchange transfusion|exchange]] for the first procedures, followed by 1.03 [[patient]] [[plasma]] [[volume]] thereafter) | |||
**** | **** | ||
**''' | **'''Steroids''' | ||
***Treatment of acquired TTP is high | ***Treatment of [[acquired]] TTP is high [[dose]] [[methylprednisolone]] | ||
**** [[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) | **** [[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) | ||
**''' | **'''Rituximab''' | ||
*** [[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>''' | |||
==References== | ==References== | ||
Latest revision as of 17:02, 31 March 2019
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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
- 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)
- Treatment of acquired TTP is high dose methylprednisolone
- Rituximab
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.