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Let's talk about a 32-year-old female with past medical history of two miscarriages. She's noted for anemia and thrombocytopenia. And I always want you to think about differentials. Now, what if this patient was in the hospital for five days? What are you thinking? Well, I would think a hit. This patient wasn't in the hospital. You're looking at this patient as an outpatient. So what do we look at? We're looking at something that's very difficult to diagnose and difficult to treat: antiphospholipid syndrome. This even scares hematologists. It has a high morbidity. You can have arterial clots. It's not clean cut. You can have weird examples of people in their clinical scenarios that have antiphospholipid syndrome. It's not very easy. It's not clean cut. It's a snake in the grass. So what do you start thinking about patients who have antiphospholipid syndrome? What would trigger you to think that they may have it? Odd clot locations, so they're having clots in weird places. We got clots in weird places, multiple pregnancies with losses? How about a young person having a stroke or an MI, and there's no reason why? What about a patient who's having recurrent clots and they're on a DOAC and they're taking the DOAC? Antiphospholipid syndrome is an autoimmune disorder that causes acceleration in the clotting process. When should you be concerned about antiphospholipid syndrome? Clotting well on anticoagulation, clotting at unusual sites. Well, you've been clotting in wrong places, brother. History of an autoimmune disorder. There's high frequency of false positives in testing. What can cause this? Inflammation or the patient being on anticoagulation. A lot of these cannot be tested in the setting of a provoked clot. And we'll talk about it. And this is a very hard one, and it actually has to be tested twice, and we'll explain why. This is the autoimmune disorder in which the body produces antibodies that recognize certain self-plotting clotting proteins. The antibodies cause destruction of the self-proteins they recognize. However, in antiphospholipid antibodies, they have a special property that causes them to activate these clotting proteins, resulting in an acceleration of the clotting process. You need one or more of the antiphospholipid antibodies. Now we're going to talk about antibodies that are against different types of phospholipids in our cell membrane. Lupus anticoagulant. Now, this is a bad name. It's probably one of the worst names in medicine. It should be called lupus procoagulant. It's not anticoagulant, it's procoagulant. You cannot test this when someone's on anticoagulation. Anticardioliptin antibodies, you can get the IgG or IgM in medium high tider. These can be tested while in anticoagulation. It's still recommended that you are not on anticoagulation when you're testing, but in some cases, the world isn't perfect and you have to test things even though you don't want to. Or it's not the perfect scenario. Antibeta to glycoprotein 1 antibodies, IgG or IgM and any titer, they can be tested while on anticoagulation. Plus either of the following. Vascular thrombosis, deep vein thrombosis, and/or pulmonary embolism, and or arterial clots, stroke, myocardial infarction, pregnancy, mortality, morbidity presenting as miscarriage of a normal fetus at less than equal to 10 weeks gestation, birth of one or more premature babies at less than 32 weeks gestation because of a clampsia, pre-clampsia, or placenta insufficiency. Multiple miscarriages greater than or equal to three at less than 10 weeks gestation. Let's continue our discussion of diagnosis of antiphospholipid syndrome. Now you're not done when you just do that testing. You have to do retesting because other conditions can be transly associated with antiphospholipid antibodies. The antibodies must be present on two separate occasions, at least 12 weeks apart for diagnosis of antiphospholipid syndrome. There could be a high false positive rate. Up to 10% healthy people can have positive antibodies. That's why you got to test them again at least 12 weeks apart. What is not included in the criteria to diagnosis, but other findings that are associated with antiphospholipid syndrome include liverido reticularis, which is the blue net-like discoloring of the skin, sterile cardiac vegetations, Leibman sacs, endocarditis, vegetations can be on both the ventricular and arterial sides of the valves. Most affected are in the left chambers, mitral is number one, followed by aortic. Thrombocytopenia, this could be another thing that could be found in antiphospholipid syndrome, prolongation of the PTT caused by lupus anticoagulant. Remember, this is a bad name. Okay, it's lupus procoagolin. What is happening is the antibodies affecting the clotting SA and falsely falsely prolongs the PTT. So in labs you'd have an elevated PTT and a normal PT, elevated PTT and a normal PT. So now we jump through the hoops and we have a diagnosis of antiphospholipid syndrome. How do we treat it? Asymptomatic? No treatment. No treatment at all. Asymptomatic. Thrombitic episode, heparin, followed by warfarin. And we have an INR of two to three. How good are DOAX? No, we do not use DOACs. DOACs are not used. That would be a wrong answer. Wrong answer. Chronic treatment of APS. Treat the underlying disease if APS is secondary. And then anticoagulation lifelong if the primary disease is there forever. So basically, if it's chronic APS, treat the underlying disease if APS is secondary. And if it's a chronic disease, you're going to stay on it forever. During pregnancy, no prior abortion, no treatment. History of pregnancy with loss, aspirin press prophylactic heparin or low molecular heparin. History of prior thrombosis, full anticoagulation with either heparin drip or low molecular weight heparin. Let's talk about antiphospholipid syndrome treatment. If you have breakthrough clots on warfarin, what do you do? Add aspirin to the warfarin. Switch warfarin to noxoperin. Consider addition of hydroxychloroquine. If they're hospitalized with severe clotting and they've been compliant with or adherent to their medication, steroids, plasma exchange, IVIG, echoluzimab, which inhibits complement. Burging for procedures, hold warfarin, and transition to an oxyparin.