Sideroblastic Anemia: Etiology, Pathogenesis and Iron Studies

by Carlo Raj, MD

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    00:01 Sideroblastic.

    00:02 Alcohol, most common.

    00:03 Alcohol poison resulting in sideroblastic.

    00:06 Another cause, lead.

    00:08 Not only would lead inhibit the enzyme called what? D – dehydratase.

    00:15 But then also in the other side, meaning as you’re getting near to producing your heme, you’re also going to then inhibit ferrochelatase.

    00:25 Knocking out those two enzymes.

    00:26 How in the world are you supposed to form heme? You’re not.

    00:30 And what about this child? Oh, abdominal pain.

    00:31 Sure.

    00:33 Maybe lead encephalopathy, not doing well in school, low IQ, the lead might then actually accumulate in your epiphyseal growth plate.

    00:43 And so therefore, there’s lack of endochondral ossification.

    00:46 That child is not going to grow very tall.

    00:47 So they’ll be short-statured.

    00:49 In addition, at some point in time, later on in life, there might be lead type of neuropathies.

    00:55 And lastly, I told you about you something like lead nephropathy.

    00:58 Keep that in mind from head to toe.

    01:01 And the other thing that we’ll take a look at later is basophilic stippling.

    01:03 And what that basically means is there is going to be accumulation of the ribosomes and RNA particles and such in your cells because of poisoning of these enzymes.

    01:15 Next, what if your patient was taking INH without the B6? May result in lack of activity of what enzyme? Good.

    01:25 ALA synthase.

    01:25 Do not forget that another name for B6 is pyridoxine.

    01:28 Also keep in mind, B6 deficiency, ladies and gentlemen, could also result in peripheral neuropathy.

    01:35 And do not forget, I wish to introduce this you and when we talk about WBC pathology, I’ll be going into myelodysplastic syndrome in much greater detail and talk to you about a subtype, where it’s called refractory anemia of ring sideroblasts.

    01:50 Four major different causes of more or less, acquired type of sideroblastic anemia.

    01:58 If there’s a deficiency of that enzyme, you then call that you X-linked recessive ALA synthase deficiency.

    02:05 Each of these inhibit different parts of the mitochondria, heme synthesis, that we talked about via different mechanisms in that previous picture.

    About the Lecture

    The lecture Sideroblastic Anemia: Etiology, Pathogenesis and Iron Studies by Carlo Raj, MD is from the course Microcytic Anemia – Red Blood Cell Pathology (RBC).

    Author of lecture Sideroblastic Anemia: Etiology, Pathogenesis and Iron Studies

     Carlo Raj, MD

    Carlo Raj, MD

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