Edit an article: Ophthalmologic Manifestations of Thrombotic Thrombocytopenic Purpura (TTP)

You do not have permission to edit this page, for the following reasons:

You are not allowed to execute the action you have requested.
You must confirm your email address before editing pages. Please set and validate your email address through your user preferences.

Original article author(s) EyeWiki username.

Additional contributor (who has not actually edited this page)

Select one or more categories for this page.

Articles
- Cataract/Anterior Segment
- Cornea/External Disease
- Glaucoma
- Miscellaneous
- Neuro-ophthalmology/Orbit
- Ocular Trauma
- Oculoplastics/Orbit
- Oncology/Pathology
- Pediatric Ophthalmology/Strabismus
- Refractive Management/Intervention
- Retina/Vitreous
- Techniques
- Uveitis

Reviewers

Enter the name of the assigned editor for this page.
Enter the name of the reviewer for this page.
Enter the date this page was reviewed.
Select the status of this page.

Local Videos

Meta Description

Enter a short description of the article. Used to generate the page's meta description tag.

Ophthalmologic Manifestations of Thrombotic Thrombocytopenic Purpura

Article Text

Edit article text below. You can save this page without filling in all of the sections and come back to it later.

= Disease Entity = Thrombotic thrombocytopenic purpura (TTP) or Moschcowitz disease, is an uncommon hematologic disease characterized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia with purpura, acute kidney injury (AKI), neurologic abnormalities with fluctuating mental status, and fever.<ref name="percival1">Percival SP. Ocular findings in thrombotic thrombocytopenic purpura (Moschcowitz’s disease). Br J Ophthalmol [Internet]. 1970 Feb [cited 2019 Jul 21];54(2):73–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/5462448</ref><ref name="said2">Said A, Haddad RY, Stein R. Thrombotic thrombocytopenic purpura. Disease-a-Month [Internet]. 2014 Oct 1 [cited 2019 Jul 22];60(10):500–4. Available from: https://www.sciencedirect.com/science/article/pii/S0011502914001242?via%3Dihub</ref> Though these are the classic symptoms, this article will focus on ocular manifestations, which have been reported to be present in 14-20% of cases.<ref name="bobbiopallavicini3">Bobbio-Pallavicini E, Porta C, Brocchieri A, Saporiti A, Tacconi F. Ocular involvement in acute thrombotic thrombocytopenic purpura. Haematologica [Internet]. [cited 2019 Jul 21];80(2):194–5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/7628756</ref> == Epidemiology == The incidence of TTP has been reported between 3.7 and 11 cases per million. It is more prevalent in females, patients of Afro-Caribbean origin, and obese patients.<ref name="said2"/><ref name="black4">Black RL, Terry JE. Ocular manifestations of thrombotic thrombocytopenic purpura. J Am Optom Assoc [Internet]. 1991 Jun [cited 2019 Jul 21];62(6):457–61. Available from: http://www.ncbi.nlm.nih.gov/pubmed/1813541</ref> == Etiology == TTP is generally believed to be caused by a decrease in activity of A disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13). This can occur secondary to a variety of genetic mutations that affect ADAMTS13 activity or, in cases of acquired TTP, autoimmune inhibitors of ADAMTS13 are the most common culprit. Low levels of ADAMTS13 have also been seen in sepsis, disseminated intravascular coagulopathy (DIC), liver disease, and plasmodium falciparum infection.<ref name="tsai5">Tsai H-M. Pathophysiology of thrombotic thrombocytopenic purpura. Int J Hematol [Internet]. 2010 Jan 9 [cited 2019 Jul 23];91(1):1–19. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20058209</ref> TTP has also been reported to be triggered by certain medications, including tyrosine kinase inhibitors.<ref name="demirsoy6">Demirsoy ET, Mehtap O, Atesoglu EB, Tarkun P, Eren N, Gedük A, et al. Dasatinib-induced immune mediated-thrombotic thrombocytopenic purpura. Transfus Apher Sci [Internet]. 2018 Apr [cited 2019 Jul 22];57(2):222–4. Available from: http://www.ncbi.nlm.nih.gov/pubmed/29475747</ref> == Pathophysiology == ADAMTS13 is a protease that cleaves large multimers of von Willebrand factor. When these multimers are not cleaved properly, they build up in the bloodstream in comparison to normal von Willebrand factor. This leads to platelet-rich microthrombus formation and mechanical hemolysis.<ref name="tsai5"/><ref name="zhao7">Zhao C, Qu Y, Sui R, Feng J, Gao J, Ma J, et al. Delayed visual recovery in pregnancy-associated thrombotic thrombocytopenic purpura with bilateral serous retinal detachment. Doc Ophthalmol [Internet]. 2013 Apr 4 [cited 2019 Jul 21];126(2):163–9. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23208746</ref> This can disrupt the blood supply to any organ, including the retina if the choroid or retinal vasculature is involved.<ref name="percival1"/> Involvement of cranial nerves has also been reported, thought to be secondary to thrombotic ischemia.<ref name="percival1"/> Due to concomitant kidney failure, hypertensive emergency (i.e., malignant hypertension) may accompany TTP, which can lead to papilledema.<ref name="bashir8">Bashir T, Alfishawy M, Babigumira M, Bashir T. Malignant Hypertension and Thrombotic Thrombocytopenic Purpura: False Friends. Am J Case Rep [Internet]. 2015 Jun 17 [cited 2019 Jul 21];16:374–6. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26083445</ref> = Diagnosis = TTP is diagnosed by the presence of microangiopathic hemolytic anemia (MAHA) in conjunction with thrombocytopenic purpura (the two major criteria). ADAMTS13 activity can support the diagnosis, but if the two major criteria are present, treatment should not be withheld waiting for the results.<ref name="said2"/> When ocular signs or symptoms are present, physicians should take a thorough history to ensure the patient does not have other systemic symptoms that may indicate a systemic disorder such as TTP. If other pieces of the classic pentad are present, TTP along with other unifying systemic causes should be investigated. == Signs == Ophthalmologic signs of TTP may be retinal including hemorrhage, arterial or venous occlusion, or serous retinal detachment. Anisocoria or ocular misalignment may be present if cranial nerves are involved.<ref name="percival1"/> Papilledema (optic disc edema due to increased intracranial pressure) as well as hypertensive choroidopathy, retinopathy, and optic neuropathy have also been reported.<ref name="percival1"/> == Symptoms == Symptoms of TTP can vary depending on the affected organ. Ocular symptoms may include acute loss of vision due to retinal pathology, diplopia if cranial nerves are affected, or blurring of vision secondary to papilledema. = Management = Plasma exchange is the mainstay of treatment for acquired TTP.<ref name="tsai5"/> == Prognosis == Plasma exchange is often successful as levels of ADAMTS autoimmune inhibitors tend to decrease over time5. = References = <references />
Please note that all contributions to EyeWiki may be edited, altered, or removed by other contributors. If you do not want your writing to be edited mercilessly, then do not submit it.
You are also promising us that you wrote this yourself, or copied it from a public domain or similar free resource (see EyeWiki:Copyrights for details). Do not submit copyrighted work without permission!

Cancel
Watch this page