RBC disorders

Question 1

what are the types of microcytic anemia? (4)

Answer 1

1. Fe Defc anemia
2. anemia of chronic dz (eg. Fe locked in Mø)
3. sideroblastic anemia (∆protophoryn)
4. Thalassemia (↓ globin chain)

Question 2

what protein transports Fe thru cell membrane –> blood?

Answer 2

ferroportin

Question 3

_____ moves Fe from blood to liver and bone marrow Mø (storage)

Answer 3

transferrin

Question 4

Iron is stored intracellularly bound to ___

Answer 4

ferritin, preventing Fenton rxn (free radicals)

Question 5

Pt presents with anemia, dypshagia, and beefy red tongue. What is the dx?

Answer 5

Plummer-Vinson syndrome (Fe defc, with esophageal + atrophic glossitis)

Question 6

most common anemia in hosptialized patients?

Answer 6

anemia of Chronic Dz (dt chronic inflamm which leads to inc hepcidin from liver)

Question 7

How does hepcidine sequester iron?

Answer 7

1. limiting Fe tranfser from mø’s –> RBC precursors (↑ferritin + ↓TIBC/Hb)
2. ↓ EPO (to prevent bact from access iron for survival)

Question 8

Lab findings for Fe defc Anemia:

1. RBC chr (cytic/chromic, RDW)
2. ferritin, TIBC, serum iron, %saturation
3. FEP

Answer 8

1. microcytic, hypochromic, ↑ RDW
2. ↓ferritin; ↑TIBC; ↓serum Fe + saturation
3. ↑FEP (free RBC protoporphyrin)

Question 9

Lab findins for ACD

1. ferrtin; TIBC + serum Fe + %sat
2. FEP

Answer 9

1. ↑ ferritin; ↓ TIBC + serum Fe + % sat
2. ↑ FEP ( no proto problems)

Question 10

Congenital sideroblastic anemia caused by

Answer 10

∆ALAS enz (rate limiting step in heme production, with vitB6 cofactor)

Question 11

Acquired sideroblastic anemia caused by (3)?

Answer 11

1. Alcoholism (mt poisoning)
2. lead poisoning (denatures ALAD [2nd heme step] + ferrochetelase [last step, iron trapped in mt])
3. vitB6 defc (ALAS cofactor; eg. Isoniazid SE for TB tx)

Question 12

laboratory findings for sideroblastic anemia (hematochromatosis)

1. ferritin; serum iron; saturation
2. TIBC

Answer 12

hematochromatosis: overloaded Fe state –> free radical reaction + cell death + mø consumption

1. ↑ ferritin; serum iron; saturation
2. ↓TIBC

Question 13

Tx for Fe defc anemia

Answer 13

ferrous sulfate

Question 14

_____ caused by gene deletion on chromosome 16

Answer 14

𝛂-thalassemia

Question 15

Spectrum of severity for 𝛂-thal

1. deletion
2. genes deleted (cis/trans)
3. genes deleted
4. genes deleted

Answer 15

1. aysmptomatic
2. mild anemia + ↑risk of severe thal in offspring
   
   1. cis (worse) = Asian (spont abortion)
   2. trans = African/AfAm
3. HbH (β₄) + Severe anemia
4. HbBarts (ɣ₄) + Hydrops fatalis (fatal in utero)

Question 16

treat ACD with

Answer 16

EPO + resolve underlying problem (Fe suppplment may be harmful)