Paroxysmal Nocturnal Hemoglobinuria (PNH): Clinical Pathology

00:01 Further, well, episodic, that’s your paroxysmal.

00:04 What’s the second, second time that you can develop this acidotic environment? Respiratory acidosis at night.

00:13 Also, when would you have lactic acidosis? When you exercise, at some point, you fell cramps.

00:18 Well, I feel cramps all the time.

00:20 That’s because I’m out of shape.

00:22 And so when I exercise, I end up going through what? The skeletal muscle undergoes anaerobic glycolysis.

00:29 When you have anaerobic glycolysis, you’re producing more lactic acid.

00:33 So what kind of acidosis is this? Metabolic acidosis.

00:37 So take a look at the two bullet points here.

00:40 Both of these are acidotic environments.

00:43 Which means what? If a patient has PNH and is exposed to these acidotic environments, then guess what happens? Your RBCs will then become destroyed episodically.

00:55 Not all the time.

00:57 Remember I told you earlier the term episodic in pathology.

00:59 It is very important because it will help you distinguish one type of pathology from another, one that is continuous versus one that’s episodic.

01:10 Overtime, earlier I told you when you’re losing hemoglobin, and you tell me about where iron is bound to, it’s bound to the heme component, right? So if you’re urinating your hemoglobin along with it, there goes my iron.

01:25 So also look for microcytic anemia, iron deficiency.

01:28 Diagnosis, you’re going to use a flow cytometry.

01:31 And with this, ladies and gentlemen, I’m going to walk you through more detail that’s necessary in the next slide, but at this point, flow becomes very important for you and you’re going to be looking for this glycosylphosphatidylinositol proteins, those GPI-linked protein.