Transcription
Another day, another video from medical professionals about hematology. In the previous videos, we have talked about hereditary angioedema and acquired angioedema. The patient is young; the patient is older. It's a hereditary autosomal dominant problem; it's an acquired, owed-to-end, by this problem. Now let's get started. [Music]
Hurry, sorry. Angioedema is an autosomal dominant condition leading to a deficiency of C1 inhibitor, at least in type 1. Originally Angelina, because type 2 is an abnormal function, not a decreased amount, leading to increased collection due to loss of inhibition, which will lead to increased, very common, leading to though all of these crazy stuff. So, chance bronchoconstriction leading to dry cough, increased vessel permeating, and you'd even increase pain due to inflammation leading to abdominal pain, which mimics colitis or pancreatitis. Natural races and vasodilation lead to hypotension; that's why bradykinin is pro-inflammatory.
Clinically, we have episodic attacks of angioedema and pain, trigger fall by prodrome followed by symptoms. Remember, no rash, no itching, no pitting edema. Diagnosis: C1 is normal, C2 is decreased, C1 inhibitor is decreased. That's the definition of the condition. Bradykinin is high; C3 is low; high molecular weight kininogen is low. Why is C3 low? Because all of it has been converted into kallikrein. Why is high molecular weight kininogen low? Because all of it has been converted into bradykinin pool.
Treatment: Do give C1 inhibitor infusion; do use Icatibant; and there's another crazy drug called Ecallantide. Icatibant is a bradykinin receptor blocker; the Ecallantide is a kallikrein inhibitor. Don't give steroids, antihistamine, or epinephrine. Why? Because this is not an allergy; this is an autosomal dominant bradykinin kind of problem; it's not a histamine kind of problem. Never ever give ACE inhibitors. Why? Because ACE inhibitors increase bradykinin, leads to angioedema, and all of this crazy but stuff. Don't add fuel to the fire.
Some crazy mnemonics: Etiology of hereditary angioedema. We have type 1 when C1 inhibitor is none, decreased amount. Type 2: the amount is normal, but C1 esterase inhibitor is too weak. Type 3: amount is normal, function is normal, but factor XII, which Roman numeral has three digits, is abnormal. Hereditary angioedema: H – high molecular weight kininogen decreased, and there is hypotension; E – episodic attacks; they're common attacks; A – autosomal dominant; D – deficiency of C1 inhibitor; D – dental procedures can trigger the symptoms; and angioedema. ACE inhibitors are contraindicated. Never ever. Oh ouch, it hurts because bradykinin produces pain. D – dry cough, and A – airway obstruction.
Treatment of hereditary angioedema: Hereditary angioedema does not respond to antihistamine, steroids, or epinephrine because it's not an allergic problem; it's not a histamine problem; it's a bradykinin problem. ACE inhibitors are contraindicated because they increase bradykinin: don't add fuel to the fire. During the acute attack, infuse the C1 INH, Icatibant, Ecallantide, racemic epinephrine. If you don't have any of these, fresh frozen plasma. If you don't have any of this, don't use fresh frozen plasma in acute attacks that much; try to leave them for the prophylaxis. And if all of these are not available, of course, you secure the airways first. It's stupid, idiot.
Prevention of future attacks, also nice prophylaxis: use Ana's, C1 inhibitor, and fresh frozen plasma, because fresh frozen plasma will contain the missing C1 inhibitor. Don't miss my 50 hematology cases; go to Patreon. Gosh, what are you waiting for? patreon.com/minicourses. These cases are just great. We're done with hereditary angioedema. Let's turn our attention to acquired angioedema. It's not a hereditary problem; it's an acquired problem. It's not a deficiency of C1; there are autoantibodies against the C1s esterase inhibitor. It's not hereditary, so there is no family history. It's less common than hereditary angioedema; manifests later in life; the patient is older; affects patients with monoclonal gammopathy, also known as paraproteinemia or myeloproliferative neoplasms, as well as malignancies such as lymphoma.
Diagnosis: C1 function here on level are decreased. This is different from hereditary angioedema because in hereditary angioedema C1 was normal. C2 and C4 are decreased. This is similar to hereditary angioedema because in both of them C2 and C4 are decreased because they are consumed by the activation of the freaking classical complement pathway. C1 inhibitor is decreased; that's the definition of the whole thing. Treatment: treated the same as hereditary angioedema.
Let's put them in a table and get out of here. Hereditary angioedema: autosomal dominant condition; there is a family history. Acquired angioedema: not hereditary, of course, no family history. Get your head out of your helmet. Hereditary angioedema: deficiency of C1 inhibitor. Acquired: autoantibodies against C1 inhibitor. More common; less common in hereditary. The patient is younger; it's called hereditary, come on, and it's usually an adolescent. Acquired: the patient is older and probably having paraproteinemia or lymphoma. Cool labs: C1 level and function normal; C1 level and function decreased; C2 and C4 decreased in both of them; and C1 inhibitor is decreased in both of them. That's the definition of the whole thing. They have the same symptoms, plus acquired angioedema may have symptoms of paraproteinemia, such as multiple myeloma or MGUS or whatever, or symptoms of lymphoma. Treatment is the same, plus acquired angioedema will require treatment of the underlying condition, being the paraproteinemia or the freaking lymphoma. Guys, it can never get easier than that.
Thank you so much for watching. Please support Medicosis by going to patreon.com/minicourses. Appreciate guys, thank you very much. I'll see you soon. As always, be safe, stay happy, and study hard.