Transcription
Hi, this is Médicos. Is perfection, Alice? Welcome to my hematology lectures. We have been talking about poikilocytes for a lot of time. High poikilocytes are abnormally shaped red blood cells. One of the poikilocytes are elliptocytes, because they are elliptical in shape. A normal red blood cell is like this; it's circular, it's nice. This one is elongated; it's called an elliptocyte. These cells are elliptical or cigar-shaped, elongated, oval red blood cells. They are seen in many different cases. One of them is hereditary elliptocytosis; it's HE, pardon. Also, iron deficiency anemia, thalassemia, megaloblastic anemia, myelodysplastic syndrome, or MDS. We have talked about hereditary elliptocytosis in a previous video. Just a recap: it's an autosomal dominant disorder of the red blood cell. There is a problem in the membrane of the RBC, especially in some proteins such as spectrin and band 4.1, band 3.1. That was hereditary spherocytosis, not elliptocytosis.
So what's the mechanism of these elliptocytes? Normally, these red blood cells will pass through the capillary bed. When they pass through the capillary bed, they get somewhat stretched or elongated, but normally they will go back to their nice biconcave shape, because they have elastic recoil, because their membrane has the necessary proteins such as spectrin and band 4.1. So they can go back from this shape to this shape, and that's normal. However, in hereditary elliptocytosis, there is a defect in spectrin and band 4.1, so these elongated red blood cells don't have elastic recoil to go back to the normal shape. They are stuck with this shape, and this will lead to hemolysis, as you know. Please go back and watch my previous video on hereditary elliptocytosis.
So let's try and answer this: A 23-year-old male feels tired all the time, and he says that he, quote, "is turning yellow." But on physical exam, there is jaundice, left upper quadrant enlargement, like in the abdomen; the left upper quadrant is large, maybe this is splenomegaly. On the lab results, we have hemoglobin of 8.2—of course, this is low; hematocrit 27—this is low; reticulocyte count 7.2—this is way too high; unconjugated bilirubin is high; haptoglobin is low; LDH 260 is high. So low hemoglobin and hematocrit—this is anemia; reticulocytosis increased; unconjugated bilirubin; let's have two globin more; LDH—all of this means there is hemolysis. So both of them together, it's a hemolytic anemia. Now, on peripheral smear or blood film, we have elongated, cigar-shaped erythrocytes; these are the elliptocytes. Also, we have spherical, dense erythrocytes; these are what, these are the spherocytes, and there is no central pallor. Both of them are abnormal. Why do we have these spherocytes here? Because it's a hemolysis.
Now, question number one: What's the mode of inheritance of this disease? So what's the disease? This is hereditary elliptocytosis. So we pick which one? It's autosomal dominant. Question number two: What else could be found in this patient? Howell-Jolly bodies? Yes, maybe, because there is a hemolytic anemia. How about smudge cells? No, this is chronic lymphocytic leukemia, which we'll talk about later. Bite cells? This is G6PD deficiency with the Heinz bodies and bite cells. Target cells has a lot of causes, such as B12 deficiency, maybe. So here we pick Howell-Jolly bodies. That's it. If you liked this video, please give it a like and please subscribe to my YouTube channel. And also, I'm posting a lot of questions and answers on Facebook, so I would like to see you there. Thank you so much. I'll see you in the next video. I'm uploading new medical videos every single week. Goodbye.