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Thursday, March 10, 2016

Pathogenesis In A Patient With Thrombotic Thrombocytopenic Purpura



A 38-year-old woman presents with a 3-day history of fever and confusion. She was previously healthy and is taking no medications. She has not had diarrhea or rectal bleeding. She has a temperature of 38°C (100.4°F) and a blood pressure of 145/85. Splenomegaly is absent. She has no petechiae but does have evidence of early digital gangrene of the right second finger. Except for confusion the neurological examination is normal. Her laboratory studies reveal the following:
Hemoglobin: 8.7
Platelet count: 25,000
Peripheral smear: numerous fragmented RBCs, few platelets
LDH 562 (normal <180)
Creatinine: 2.7
Liver enzymes: normal
Prothrombin time/PTT/fibrinogen level: normal

What is the most likely pathogenesis of her condition?

A. Disseminated intravascular coagulation
B. Antiplatelet antibodies
C. Failure to cleave von Willebrand factor multimers
D. Verotoxin-induced endothelial damage
E. Cirrhosis with sequestration of erythrocytes and platelets in the spleen

Answer And Discussion:

The answer is C

This patient has thrombotic thrombocytopenic purpura (TTP). TTP is an acute life-threatening disorder that is characterized by the pentad of
  1. microangiopathic hemolytic anemia,
  2. nonimmune thrombocytopenia, 
  3. fever, 
  4. renal insufficiency, and 
  5. CNS involvement (confusion or multifocal encephalopathy). 
Not all patients have the full pentad; the essential features are the red blood cell fragmentation (schistocytes andhelmet cells) and the thrombocytopenia. TTP may be triggered by endothelial damage and is associated with deficiency of a plasma protein (ADAMTS 13) that breaks down multimers of von Willebrand factor. Plasma exchange (with the infusion of fresh frozen plasma to provide the missing ADAMTS 13 protein) can be lifesaving.

The hemolytic uremic syndrome (often associated with verotoxin producing strains of E coli O157:H7) is similar but is usually not accompanied by CNS changes. The renal failure is usually more severe in HUS.

Disseminated intravascular coagulation associated with sepsis can resemble TTP, but the coagulation pathway is usually activated in DIC. In TTP the prothrombin time, PTT, and fibrinogen level are normal.

Antiplatelet antibodies are associated with idiopathic thrombocytopenic purpura (ITP), but this patient has multiple abnormalities, not just thrombocytopenia. Hypersplenism can cause thrombocytopenia but rarely with a platelet count of below 50,000; it is not associated with red cell fragmentation.

2 comments:

  1. Hello, my name is Betty. i was diagnosed of HIV virus 4 years back. Before then, i was dating this Derrick guy. we had something great going on. Our love life only lasted for 8 months. the sixth and seven month of our relationship, i began feeling very weak every now and then. But i never suspected anything until I kept on experiencing different symptoms like having sore throats, constant headache, body weakness e.t.c. I decided to go for a medical check up. I was tested HIV positive. when the doctor broke the news to me, it was as if my whole world had crumbled. it dawned on me then that Derrick has been lying to me. i was very mad to an extent i almost committed suicide. But there was nothing i could do, other than weep and weep. ever since then, i have been spending heavily on medications, going from one medical clinic or the other so I can look healthy. Though since i was a kid, i love herbs a lot. i take herbal tea with grandma while growing so i am quite familiar with herbs. early last year, i was on the internet and on a particular health blog, to check for herbal medication as alternative. I saw many comments and read many testimonies. Until i came across a particular testimony on how Dr.ugo cured a patient from herpes simplex virus using pure natural herbs. i reached out to the doctor because I also was thinking Herpes had no cure. I told him about my HIV status.He encouraged me not to panic that he will help me get my deliverance. he prepared some herbs and sent them to me. The herbs were in four 70ml bottles and I took them morning and night. i used as he directed, and in less than three weeks, i started having appetite to eat plenty of food and i gained back my strength. the fourth week,I consumed all the herbal meds. He encouraged me to go for a test which i did. This time, the result was negative. I quickly contacted the doctor and informed him of the news. I immediately asked him to prepare for Derrick too. He did and sent it. Today, me and Derrick are HIV free. All thanks goes to God Almighty for healing us through Dr.Ugo. if you are having any kind of sickness at all, Dr.Ugo is the right person to talk to and i assure you, you will be glad you did. His email: ugoherbalhome@gmail.com you can also write him on WhatsApp +2348104990619.

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  2. Nice blog
    Have been totally convinced that HIV,and many others dangerous diseases can be best cured using the Herbal Medicine. Dr.Akpu the great and powerful herbalist cured me with his Herbal remedy. I'm Linda,15 years from Texas. I was born with HIV. I lost parents at my teenager age when I was 4th. I had to grew up with my mum only Sister, whom I loved so much. She was so worried about my life as my condition was her mature problem in life. I had taken lots of antiviral treatment to get ride of the virus not believing what there said HIV has no cure. I was on the internet when i came across blogs of testimony about a lady who where cured of a 7 years diagnose of Herpes by this powerful Herbalist, Dr.Akpu.I wrote down his mail @ Dr.akpupowerherbscenter@gmail.com
    I told my Aunt,luckily it was the same Dr,we where referred to few months back by my pastor in church whom members have being testifying about of his cure We did contacted him and explained my problem to him.He prepared the medicine and sent to me with details of how to use and apply. After a while, I began to feel more strong than never before, I felt so lucky and happy when my Doctor congratulated me that he could found no HIV virus again in me. I knew I was free cos my body was so strong. His herbal medicine should have saved my parents life, i said!
    Thanks so much for reading my article, you should share your testimony if you have cure as well
    Dr.akpupowerherbscenter@gmail.com
    Is the solution

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