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49-year-old female complains of large bruises, headache, confusion, malages, and arthralogies. She has low hemoglobin and low platelets on labs. What are some differential diagnoses we could have? How about hemolytic uremic syndrome linked to shingallotoxin, E. coli, DIC? We have a high D domer and low fibroindrogen if we had follow-up lapse. If the patient was pregnant, we can consider help syndrome with hemolysis, elevated liver enzymes, low platelets, which resolves with delivery. Now, in this case, yeah, it'd be a little bit difficult, but we could say immune thrombocytopenia ITP, but the confusion probably wouldn't be there and a few other things wouldn't be there, but potential. There would be no schistocytes if we did a blood smear. Malignancy can cause this too. This picture, HIV, lupus, and you can always put syphilis in, causing this presentation. So what is the disease the patient has? TTP. What's the pathophysiology? Abnormal activation of platelets and endothelial cells with deposition of fibrin within mass microvasculature, and peripheral destruction of RBCs and platelets. So you have abnormal activization, peripheral destruction, fibrin deposition, and microvasculature obstruction. So let's talk more about TTP. You can also have findings of fever, acute renal failure, neurological findings such as confusion. Well, just think about it. If you have a microvascular obstruction, you can have confusion. You're not getting blood flow perfusion to the brain, along with microangiopathic hemolytic anemia and thrombocytopenia. Most common causes, deficiency in protease, protease, atoms 13. That cleaves the high molecular weight multimer of von Willebrand factor. And these multimers bind to the masses of platelets, causing vascular occlusion and thrombocytopenia. Now let's talk about the famous pentad for TTP: renal dysfunction, fever, thrombocytopenia, microangiopathic hemoginemia, and neurological dysfunction. This is only in patients, about 5 to 10% of patients will present with the TTP triad. And then the platelet count we're looking at in thrombocytopenia is usually less than 30,000, is what we're looking at. So only about 5% of the 5 to 10% of patients get this famous bored TTP triad. So what are some diagnostic criteria for TTP? Must have low platelets, makes sense. Roughly, we'll start looking at less than 30,000, but it can be above that, of course. Microangiopathic hemolytic anemia without malignant hypertension or scleroderma renal crisis. So you're ruling these out to get to thrombos thrombotic, thrombocytopenic, perpa, TTP. Diagnosis. Plasmic score can be helpful. And usually we're looking at here as we discussed platelets less than 30,000. So let's go through the plasmic score. And you can go and have there's different calculators that have it in, but basically we're looking at platelets less than 30,000, hemolysis, no active malignancy, no history of solid organ or stem cell transplant, MCV less than 90,000, INR less than 1.5, creatinin less than 1.5. So if you get a low plasmic score, consider an alternative diagnosis. So low would be less than four. And you can get multiple calculators that you can use to calculate the plasmic score. Intermediate risk, five, send Adams-13 testing, close observation. If you have the ability to get a hematology consult and consider plasmic exchange if no other cause has been identified. Now greater than six, you send for an Adams 13, get your expert consult, and immediately starting plasma exchange. Now, why do we need to do these plasmic scores? Well, sometimes the Adams 13 testing doesn't come back right away. And you need to, this is if we don't act quickly, these patients can have harm. So this is if we can't get to Adams 13, or maybe you don't have Adams 13 testing capability where you are, and you need to ship this patient to a place they can, or waiting for outside labs to come in, who knows, holidays. These are patients that you need to take care of right away. So if you had a high risk and a plasmic score greater than or equal to six, then you would consider right away getting doing plasma exchange. So evidence of microangiopathic hemilmonemia, schistocytes and a peripheral smear, schistocytes, elevated LDH, increased indirect bilirubin, decreased hapticobin, and a negative Coombs test. So you have normal or mildly abnormal coagulation studies, that'd be your PT and your PTT. So Adam-13 activity less than 10% is confirms the disease. But you should not wait for do not delay treatment. As soon as you send the test or you have conf uh suspicion, go ahead and start the treatment right away. Remember, Adams 13 activity less than 10% confirms the diagnosis, but you should not delay treatment. It's fatal in 90% of cases without therapy. The treatment is plasma exchange. Remember, do not transfuse platelets because it worsens the microvasculature involvement. Therapeutic plasma exchange removes the antibody to atoms 13 and provide himself atom 13 via donor plasma. So it removes the bad and puts in the good. That's TTP.