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Hemolytic Anemia: Paroxysmal Nocturnal Hemoglobinuria (PNH) – Red Blood Cell Pathology

by Carlo Raj, MD
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    Let’s take a look at paroxysmal nocturnal hemoglobinuria. Everything that you want to know about this condition is actually in the name. And so let’s go ahead and dissect each part of this. First, my issue is the fact that, well, I’m not able to properly control the destruction of my RBC. Really? Mm-hmm. Now, a couple of other things as well that you want to keep in mind, remember earlier, we had our discussion of G6PD deficiency and I told you to keep it as a differential, chronic granulomatous disease. Well, here, with this picture of paroxysmal nocturnal, granted we’re going to focus upon the RBC. However, as far as you’re concerned, the clinical picture is a lot broader. What I mean by that is if you take a look at the statement here, you’ll understand that not only is my RBC being affected, as is my neutrophil, but you really, ladies and gentleman, the clinical picture of platelets becomes important. What I mean by that is it's not that you’re going to have excess destruction of your platelet. In fact, you end up having excess stimulation of platelet function. So if you have excess platelet functioning or you have excess activity of your platelets, then you’re forming tons of? Thrombi. Where? Up and down the body. That’s your first clinical picture. The patients that walk in with PNH are suffering from some kind of thrombotic episode, including what? Well, maybe there might be portal hypertension. Why? Well, the portal vein underwent thrombotic episodes. So imagine now, the portal vein, which should normally be draining your superior mesenteric vein and the splenic vein have now become thrombosed. And if it’s thrombosed, then you’re going to back up into, well, your portal hypertension. So that will be your esophageal varices,...

    About the Lecture

    The lecture Hemolytic Anemia: Paroxysmal Nocturnal Hemoglobinuria (PNH) – Red Blood Cell Pathology by Carlo Raj, MD is from the course Hemolytic Anemia – Red Blood Cell Pathology (RBC). It contains the following chapters:

    • Paroxysmal Nocturnal Hemoglobinuria - Etiology
    • Paroxysmal Nocturnal Hemoglobinuria - Pathophysiology
    • Paroxysmal Nocturnal Hemoglobinuria - Clinical Pathology
    • Paroxysmal Nocturnal Hemoglobinuria - PNH Lab Tests

    Author of lecture Hemolytic Anemia: Paroxysmal Nocturnal Hemoglobinuria (PNH) – Red Blood Cell Pathology

     Carlo Raj, MD

    Carlo Raj, MD


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