Thrombotic thrombocytopenic purpura medical therapy: Difference between revisions

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{{Thrombotic thrombocytopenic purpura}}
{{Thrombotic thrombocytopenic purpura}}
{{CMG}}
{{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]].
==Medical Therapy==
==Medical Therapy==
===Plasmapheresis===
*[[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>
Since the early 1990s, [[plasmapheresis]] has become the treatment of choice for TTP.<ref>{{cite journal |author=Zheng XL, Kaufman RM, Goodnough LT, Sadler JE |title=Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura |journal=Blood |volume=103 |issue=11 |pages=4043–9 |year=2004 |pmid=14982878 |doi=10.1182/blood-2003-11-4035}}</ref> This is an [[exchange transfusion]] involving removal of the patient's [[blood plasma]] through [[apheresis]] and replacement with donor plasma ([[fresh frozen plasma]] or cryosupernatant); the procedure has to be repeated daily to eliminate the inhibitor and ablate the symptoms. Plasma infusion may be necessary and preferred if plasma exchange is not readily available.  Exchange tranfusion has a complete response rate of 76%; plasma exchange allows ~80% of acquired ADAMTS13 autoantibody mediated TTP patients to survive an episode(compared to ~80% mortality without it), usually with minimal organ damage.  FFP is the replacement fluid of choice in TTP and an exchange of a single plasma volume is the standard initial treatment.  All patients should receive folate.  [[Lactate dehydrogenase]] levels are generally used to monitor disease activity. The serum level increases as erythrocytes are destroyed.  Plasmapheresis may need to be continued for 1-8 weeks before patients with idiopathic TTP cease to consume platelets and begin to normalize their hemoglobin.  No single laboratory test (platelet count, LDH, ADAMTS13 level, or inhibitory factor) is indicative of recovery; research protocols have used improvement or normalization of LDH as a measure for ending plasmapheresis.  Although patients may be critically ill with failure of multiple organ systems during the acute illness, including renal failure, myocardial ischemia, and neurologic symptoms, recovery over several months may be complete in the absence of a frank myocardial infarct, stroke, or CNS hemorrhage. Platelet transfusions are contraindicated unless there is life-threatening hemorrhage.  
*[[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>
===TTP treatment===


===Pharmacotherapy===
**'''Plasma therapy'''
Many TTP patients need additional [[immunosuppressive]] therapy, with [[glucocorticoid]] [[steroid]]s (e.g. [[prednisolone]] or [[prednisone]]), [[vincristine]], [[cyclophosphamide]], [[splenectomy]] or a combination of the above.  [[Rituximab]], a [[monoclonal antibody]] targeting CD20 on [[B cell]]s, has been successfully used to treat patients with refractory disease.
*** [[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 [[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)
**'''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>'''


Children with Upshaw-Schulman syndrome receive plasma every three weeks prophylactically; this maintains adequate levels of functioning ADAMTS13.
==References==
{{Reflist|2}}


==References==
{{reflist|2}}
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[[Category:Disease]]
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[[Category:Autoimmune diseases]]
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[[Category:Rare diseases]]
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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

TTP treatment

References

  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.
  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.
  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.
  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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