00:00 So what then happens to your RBC? It gets spiculated. 00:06 In other words, your RBC looks like it has little needle projections from the RBC. 00:12 So you definitely know that the RBC is misshapen. 00:14 And most likely, something to do with the decreased production of ATP because you’re stuck at? Well, you’re stuck at phosphoenolpyruvate, right? And whenever you have a misshapen RBC, where does it go for destruction? Hereditary spherocytosis, hereditary elliptocytosis, sickle cell. 00:34 All of them are going to go to the spleen, resulting in? Extravascular hemolysis. 00:38 Now, this is a child. 00:40 Whenever you have an enzyme deficiency, you’re born with a disorder. 00:45 Serum methemoglobin is elevated. 00:47 Why? NADH is inadequate. 00:53 Almost, well, most common error in glycolysis. 00:56 However, in terms of a disease though, it’s quite rare, but make sure that you know in greater detail all of the different points that we made here about pyruvate kinase deficiency. 01:07 What are you going to find? This RBC is going to take on bizarre shape. 01:11 And this bizarre shape is then known as an echinocyte, most likely due to as we said, inadequate amounts of ATP, having some type of influence in a negative manner on the sodium potassium pump in which you end up finding here on a peripheral blood smear are these weird spiculated type of echinocytes.
The lecture Pyruvate Kinase Deficiency: Morphology and Clinical Pathology by Carlo Raj, MD is from the course Hemolytic Anemia – Red Blood Cell Pathology (RBC).
Pyruvate kinase is responsible for converting which of the following chemical compounds into pyruvate?
The oxygen–hemoglobin dissociation curve is shifted to the right as a result of which of the following parameters?
The oxygen–hemoglobin dissociation curve is shifted to the left as a result of which of the following conditions?
Which of the given cell types is associated with pyruvate kinase deficiency?
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