Transcription
Hey guys, it's Medicosis Perfectio. We're continuing our playlist, our series of lectures about bleeding and coagulation disorders.
In the previous video, we talked about thrombopoietin, the famous TPO, not to be confused with Tim Tebow. Today, we'll talk about inherited thrombocytopenia. By the way, we have talked about thrombocytopenia before. We will talk about the inherited, their inner causes of thrombocytopenia. Thrombocytopenia, which theoretically is played at countless than 150,000, but clinically practically is platelet count less than 50,000. With that being said, now let's get started. Look at this batik. [Music]
Why is the normal platelet count from 150,000 to 400,000, and you don't care about thrombocytopenia except if it's less than 50,000 instead of less than 150,000? Because it's in normal distribution curve; there is a mean, median, mode, and then there is a standard deviation. We don't care about thrombocytopenia unless we pass, we are in the lower 2.5 percentile. We don't care about thrombocytosis unless we are in the highest 2.5 percentile. And I've explained this before. Translation: I'm not a medical person. However, if your platelet count is 140,000 and you don't have any symptoms, shut up, you're fine. Again, I'm not a doctor; please talk to a doctor.
We've talked about hematopoiesis before. Don't forget that the megakaryocytes are of myeloid lineage. Thrombocytes are the platelets. Decreased number called thrombocytopenia; increased number called thrombocytosis or thrombocythemia. In my hematology lectures, I've talked about thrombocytosis before. When I have increased platelet count, usually more than 750,000, two types could be primary or essential, one of the myeloproliferative neoplasms, or secondary or reactive. 100% of cases in kids are secondary. 80% of cases in adults are secondary; 20% are primary myeloproliferative neoplasm called essential thrombocytosis. So if you have a baby and you play odds, it's gonna be secondary. If you have an adult and you play odds, it's gonna be again secondary. Again, only 20% of cases are primary.
Thrombocytopenia is decreased platelet count. We have pseudothrombocytopenia and true thrombocytopenia due to decreased production, increased destruction, or splenic sequestration. As economics says, decreased supply, increased demand. We have talked about pseudothrombocytopenia before. It's an artifact due to the stupid, but machine called analyzer. Either stupid put machine, the stupid about machine counts those like seven plates as only one because of platelets agglutination. However, if you count them manually or if you use another test tube that doesn't contain the EDTA, in other words, don't use the purple, use the green or blue; this problem will disappear because it's a stupid artifact. If the test tube is purple, it will interfere with platelet coagulation functionally to pull a flat inclusion easily into pea pseudos thrombocytopenia. That's the mnemonic. True thrombocytopenia again, under production, over destruction, splenic sequestration. Under production could be inherited or acquired. Today's topic is the inherited true thrombocytopenia. It doesn't matter if it's acquired or inherited; the clinical picture is the same. We have superficial bleeding, skin bleeding, and mucosal bleeding. Again, as I've told you, there's a mistake here. Ecchymosis are the largest; they are larger than one centimeter, and these are three to ten millimeters. So the smallest is petechiae; in between we have those purpura, and the largest is the ecchymosis.
Inherited thrombocytopenia; it could be autosomal dominant, autosomal recessive, or X-linked. Large playlist because it's a sign of immaturity. Bigger isn't always better, and I've told you before many times during hematopoiesis, the cells start big, then they become smaller. If you have a problem maturing these cells, called improper maturation, you are stuck with bigger, more immature cells. Thrombocytopenia diseases include May-Hegglin anomaly, Epstein's anomaly, Sebastien anomaly, what's that, Fichtner anomaly. Okay, so those are some jerks who wanted to get their names in the textbook. That's about it. Congenital amegakaryocytic thrombocytopenia, also known as CAMT, not to be confused with the COMT in Parkinson's disease. Thrombocytopenia with absent radii; you have thrombocytopenia, and both of your radial bones are missing. Wiskott-Aldrich syndrome, a GATA1 mutation, one of the most like bored-favor diseases of all time. You have thrombocytopenic purpura, eczema, and recurrent infections. Several students got my Perfect Nails Ultimate Notebook +20 Lymphoma cases +25 Bleeding cases. Those can be yours if you go to patreon.com/4/medicosis.
Let's talk about congenital amegakaryocytic thrombocytopenia. It's a bad disease. You have a low number of megakaryocytes and a low number of platelets because it's an autosomal recessive defect. Decreased megakaryocytes are leading to decreased platelets. Could be caused by TPO receptor gene mutation, and I've told you before the TPO receptor is called MPL. It may involve the CNS, the cerebrum and cerebellum. You cannot think clearly, and you cannot balance well. Treatment: bone marrow transplantation is the only solution. Complications can lead to aplastic anemia. So not only decrease the platelets, you will also have decreased red blood cells and decreased white blood cells.
Summary: Thrombocytopenia is decreased platelet count. Theoretically, like your professor might like it this way: less than 150,000; less than 90,000, the bleeding time starts to increase or prolong; less than 50,000, clinical picture of bleeding; less than 30,000, spontaneous bruising, spontaneous without being even hit or traumatized; less than 20,000, severe bleeding; less than 10,000, transfuse platelets prophylactically, even if the patient is asymptomatic because like five seconds later he will be asymptomatic; less than 5,000, extremely severe bleeding with spontaneous hemorrhage. This is awful. If you are preparing a patient for a major surgery, provided that he or she doesn't have any symptoms, a platelet count greater than 50,000, you are safe to operate most of the time, again provided that the patient has no other symptoms, the vision has infection. I'm gonna be 15; we're gonna aim higher. Thank you for watching. Please subscribe and join the tribe. Follow me on Facebook; I have lots of cases there. Go to patreon.com/smashmedicosis to get my notes and my cases and my perfectionist ultimate notebook. Be safe, stay happy, and study hard. This is Medicosis Perfectio, where medicine makes perfect sense. Medicine and sense, two words that don't go together like a pragmatic professor.