Thrombotic thrombocytopenic purpura natural history, complications and prognosis: Difference between revisions
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{{Thrombotic thrombocytopenic purpura}} | {{Thrombotic thrombocytopenic purpura }} | ||
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{{CMG}}; {{AE}} {{S.G.}} | |||
==Overview== | ==Overview== | ||
==Natural History== | If left untreated, >90% of patients with TTP may progress to develop [[Kidney|renal]] dysfunction, [[neurological disorders]] (mild [[headache]], onset of [[behavioural]] anomalies, [[transient]] [[sensory]] and [[Motor neuron disease|motor]] deficits, [[coma]]), [[Ischemia|ischaemic]] [[gastrointestinal]] [[complications]] ([[abdominal pain]]) and [[retinal detachment]]. | ||
== | ==Natural History, Complications, and Prognosis== | ||
== | |||
===Natural History=== | |||
*The [[Symptom|symptoms]] of TTP([[Acquired disorder|acquired]]) usually develop in the 4th decade of life, and start with no specificity of the [[Medical sign|signs]], [[symptom]]<nowiki/>s and [[laboratory]] findings; [[Symptom|symptoms]] such as [[fever]], [[Kidney|renal]] dysfunction, [[neurological disorders]] (mild [[headache]], onset of [[behavioural]] anomalies, [[transient]] [[sensory]] and [[Motor neuron disease|motor]] deficits, [[coma]]), [[Ischemia|ischaemic]] [[gastrointestinal]] [[complications]] ([[abdominal pain]]) and [[retinal detachment]].<ref name="pmid227902582">{{cite journal |vauthors=Rizzo C, Rizzo S, Scirè E, Di Bona D, Ingrassia C, Franco G, Bono R, Quintini G, Caruso C |title=Thrombotic thrombocytopenic purpura: a review of the literature in the light of our experience with plasma exchange |journal=Blood Transfus |volume=10 |issue=4 |pages=521–32 |date=October 2012 |pmid=22790258 |pmc=3496241 |doi=10.2450/2012.0122-11 |url=}}</ref> | |||
*[[Neurological]] [[symptoms]] at onset do not occur approximately more than 35% of patients. | |||
*[[Fever]] and [[Renal insufficiency|renal dysfunction]] are present among only a small [[Minority group|minority]] of [[Patient|patients]]. | |||
*The [[Symptom|symptoms]] of TTP typically develop after 40 years. | |||
===Complications=== | |||
*Common [[Complication (medicine)|complications]] of TTP([[Restriction|restrict]] [[blood]] [[flow]] to [[Organ (anatomy)|organs]] such as the [[brain]], [[Kidney|kidneys]], and [[heart]]) include:<ref name="pmid227902582" /><ref name="pmid226245963">{{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> | |||
**Early death in approximilaty of patients are not diagnosed. | |||
**[[Neurological]] problems([[personality changes]],mild [[Headache|headaches]], [[confusion]] and [[slurred speech]], [[coma]]) | |||
**Abnormal [[kidney]] [[Function (biology)|function]] | |||
**[[Heart]] problems | |||
Approximately one-third of patients experiencing a TTP episode have a relapse within 10 years following their first attack. | ===Prognosis=== | ||
* The mortality rate is approximately 90% for untreated cases, but the [[prognosis]] is reasonably favorable (80-90%) for patients with [[idiopathic]] TTP diagnosed and treated early with [[plasmapheresis]].<ref name="pmid226245963" /> | |||
* Approximately one-third of patients experiencing a TTP episode have a relapse within 10 years following their first attack. | |||
* Secondary TTP still has a dismal prognosis, with [[Mortality rate|mortality]] rates despite treatment being reported as 59% to 100%. | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
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Latest revision as of 14:09, 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
If left untreated, >90% of patients with TTP may progress to develop renal dysfunction, neurological disorders (mild headache, onset of behavioural anomalies, transient sensory and motor deficits, coma), ischaemic gastrointestinal complications (abdominal pain) and retinal detachment.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of TTP(acquired) usually develop in the 4th decade of life, and start with no specificity of the signs, symptoms and laboratory findings; symptoms such as fever, renal dysfunction, neurological disorders (mild headache, onset of behavioural anomalies, transient sensory and motor deficits, coma), ischaemic gastrointestinal complications (abdominal pain) and retinal detachment.[1]
- Neurological symptoms at onset do not occur approximately more than 35% of patients.
- Fever and renal dysfunction are present among only a small minority of patients.
- The symptoms of TTP typically develop after 40 years.
Complications
- Common complications of TTP(restrict blood flow to organs such as the brain, kidneys, and heart) include:[1][2]
- Early death in approximilaty of patients are not diagnosed.
- Neurological problems(personality changes,mild headaches, confusion and slurred speech, coma)
- Abnormal kidney function
- Heart problems
Prognosis
- The mortality rate is approximately 90% for untreated cases, but the prognosis is reasonably favorable (80-90%) for patients with idiopathic TTP diagnosed and treated early with plasmapheresis.[2]
- Approximately one-third of patients experiencing a TTP episode have a relapse within 10 years following their first attack.
- Secondary TTP still has a dismal prognosis, with mortality rates despite treatment being reported as 59% to 100%.
References
- ↑ 1.0 1.1 Rizzo C, Rizzo S, Scirè E, Di Bona D, Ingrassia C, Franco G, Bono R, Quintini G, Caruso C (October 2012). "Thrombotic thrombocytopenic purpura: a review of the literature in the light of our experience with plasma exchange". Blood Transfus. 10 (4): 521–32. doi:10.2450/2012.0122-11. PMC 3496241. PMID 22790258.
- ↑ 2.0 2.1 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.