thrombotic thrombocytopenic purpura

And first of all, you know, the fact that you found this organization 28 years ago and you've had an audience of more than 1,500 patients and families.

But the citation reads, Joanna has given more than 20 years of dedicated service to supporting people affected by a rare and life-threatening blood condition thrombotic thrombocytopenic purpura known as ttp i'm glad hopefully i got that right i'm glad i could kind of get through saying that it's quite a bit of a mouthful but ttp After being diagnosed herself, you set up the TTP network, which now supports over 600 people in the UK and around the world.

Through your work, you've helped influence national decisions on treatment and given a voice to patients within the NHS.

Alongside this, you're the founder, trustee of a charity providing free legal advice to victims of domestic abuse.

You do have to consider other MAHA-type presentations for these patients, though.

Typically, you most want to tell this apart from TTP or HUS.

And for that, you might need to test for ADAMTS13 enzyme deficiencies, because this is what's really going to differentiate it.

And if you treat one as the other, it's not going to work very well.

The treatments are very different.

So you do want to make that differentiation, though it's probably more likely going to be HELP syndrome than it is TTP.

Now to further disrail the conversation that we're having about hypertension in pregnancy is that up to 25% of patients who have eclampsia will not have hypertension.

Now we often consider this to be sort of a spectrum between preeclampsia and eclampsia, but it's not always that way.

Anytime I see anemia and thrombocytopenia, my first thought is, does this patient have a TMA? We need to rule out TMAs.

There's many TMAs, TTP being the prototypical one, but DIC, um,HUS, right? And, you know, we don't seem like we're hemolyzing here, and there's no schistocytes in the smear.

So we can take a pause, relax.

Can't relax too much 'cause there's a lot going on in this case, but we don't think that this patient has, likely has a TMA.

This refers to the combination of thrombocytopenia and schistocytosis that you see with hemolytic uremic syndrome and thrombotic thrombocytopenic purpura.

Could do a forty-six minute seminar on all this stuff, okay? So you get schistocytes, helmet cells, fragmented RBCs.

So whether you have HUS or TTP, it's schistocytes, not acanthocytes.

Wrong fucking answer.

Choice C, sepsis, wrong fucking answer.

And although these are uncommon, so is help syndrome.

The first is TTP or thrombotic thrombocytopenic purpura.

This is really something you should suspect when really your main thing is that you're seeing that the platelets are profoundly low, often less than 30,000.

But these patients can also have neurologic features and they may have some mild renal dysfunction.

So who should be interested in plasma exchange? There is such growing evidence for so many reasons, but let's start with the strongest science first, patients with established medical indications for TPE.

And depending upon the exact disease and clinical situation, TPE has established... or guideline-supported roles in conditions such as Guillain-Barre syndrome, myasthenia gravis, particularly in certain acute or severe circumstances, CIDP, which is chronic inflammatory demyelinating polyneuropathy, neuromyelitis optica spectrum disorder, certain severe or treatment-resistant multiple sclerosis attacks, anti-GBM disease, good pasture disease, thrombotic thrombocytopenia purpura, or TTP, certain antibody-mediated neurological diseases, certain paraproteinemic neuropathies, even transplant-related antibody-mediated conditions.

And that's where I really heard about it a lot when I was working transplant and other specific autoimmune neurologic and hematologic disorders.

But there's an incredibly important distinction here.

Um, so this album, Donuts, is his second studio album, and the final one during his lifetime.

Uh, he died just three days after its release, after a long battle with TTP, a rare and incurable blood disease, and Lupus throughout the preceding decade.

Uh, in Dilla's own words, Di- "Donuts is just a compilation of the stuff that I thought was a little too much for the MCs.

That's basically what it is, me flipping repor- records that people didn't really know how to rap on, but they want to rap on." So, um, uh, very much already the case there-

Um, so according to the popular version, uh, J Dilla worked on the album at the Cedars-Sinai Medical Center.

According to this narrative, most or at least a small part of Donuts was recorded at the hospital that he was based.

Um, oh, just to preface that, um, so he was diagnosed with TTP in 2002, went in and out of hospitals, moved to LA, uh, which is where the Cedars-Sinai Medical Center is.

Uh, and then after his condition worsened, uh, uh, he was diagnosed with lupus in 2005, and then he moves into the medical center.