A.pathoma, Rbc Disorders (Incomplete)

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Macro, Micro, Normo-cytic Anemias (classification)

Back 1

microcytic (MCV < 80 μm3)
normocytic (MCV = 80-100 μm3)
macrocytic (MCV > 100 μm3)

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Microcytic Anemia

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Due to decreased Hgb production, divides 1 extra time to maintain Hgb conc. of RBC.
1. Iron Deficiency Anemia (low heme, low hgb)
2. Anemia of Chronic Disease (ACD)
3. Sideroblastic Anemia (decr protoporphyrin production, low heme, low hgb)
4. Thalassemia (low/no globin, low hgb)

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heme =

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Fe + protoporphyrin

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Fe Deficiency Anemia

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-Fe absorb in duodenum (key regulator: ferroportin on enterocytes)
-transferrin: Fe transport in blood (to liver/macrophages)
-ferritin: Fe storage, prevent free radicals (Fenton rxn)

Front 5

Fe Labs

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-serum Fe
-TIBC: transferrins in blood (bound&unbound)
-% saturation: %transferrin bound w/ Fe (nl = 33%)
-serum ferritin: Fe stores in liver and macrophages

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Stages of Fe Deficiency

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1. Storage iron is depleted—↓ ferritin; ↑ TIBC
2. Serum iron is depleted— ↓ serum iron; ↓ % saturation
3. Normocytic anemia—Bone marrow makes fewer, but normal-sized, RBCs
4. Microcytic, hypochromic anemia—Bone marrow makes smaller and fewer RBCs

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Lab Findings of Fe Def Anemia

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1. Microcytic, hypochromic RBCs with ↑ red cell distribution width (RDW)
2. ↓ ferritin; ↑ TIBC; ↓ serum iron; ↓ % saturation
3. ↑ Free erythrocyte protoporphyrin (FEP)*
(Heme = Fe + protoporphyrin)

Front 8

Plummer-Vinson Syndrome

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Fe Def Anemia w/ esophageal web & atrophic glossitis
-Presents with anemia, dysphagia, and beefy-red tongue

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Anemia of Chronic Dz

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-starts as normocytic, becomes microcytic
-(most common anemia inpatient)
-chronic inflamm/CA -> hepcidin:
1. limit Fe transfer fr macrophages
2. suppresses EPO

low Fe, low heme, low Hgb = microcytic anemia

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Lab Findings of ACD

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1. ↑ ferritin, ↓ TIBC
2. ↓ serum iron, and ↓ % saturation
3. ↑ Free erythrocyte protoporphyrin (FEP)

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Tx of ACD

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-tx underlying cause (eg. if autoimmune)
-CA pt's: give EPO

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Sideroblastic Anemia

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-defective protoporphyrin synthesis.
↓ protoporphyrin, ↓ heme,↓ hemoglobin = microcytic anemia
-Fe trapped in mito of RBC precursor (binds protoporphyrin to make heme there)
-ringed sideroblast: Fe-laden mitochondria forms ring around nucleus of RBC precursor

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Etiologies of Sideroblastic Anemia

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1. Congenital (involves ALA enzyme)
2. Acquired: ETOH, lead poisoning, VitB6 Def.

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Lab Findings of Sideroblastic Anemia

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↑ ferritin, ↓ TIBC, ↑ serum iron, and ↑ % saturation
(iron-overloaded state)

Front 15

Thalassemias

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defective SYNTHESIS of globin chains of Hgb
↓ globin, ↓ hemoglobin = microcytic anemia

Front 16

Normal Hgb's

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Normal types of hemoglobin are:
1. HbF (α2 γ 2) = FETAL Hb
2. HbA (α 2 β 2)
3. HbA2 (α 2 δ2)

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α-Thalassemia

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α-Thalassemia is usually due to gene deletion; normally, 4 alpha genes are present.

1. ONE gene deleted: asx

2. TWO genes deleted: mild anemia with ↑ RBC count
a. Cis deletion (Asians)
b. Trans deletion (Africans)

3. THREE deleted: severe anemia; β chains form tetramers (HbH) that damage RBCs

4. FOUR deleted: lethal in utero (hydrops fetalis); γ chains form tetramers (Hb Barts) that damage RBCs

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β-Thalassemia

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usually due to gene mutation; normally 2 beta genes are present
-African and Mediterranean descent
varying degrees:
1. TWO β genes present: absent (β0) or diminished (β+) production of the β-globin chain

2. β-thalassemia minor (β/β+): mildest form, asx, elevated RBC
-slightly decreased HbA with increased HbA2
-Microcytic hypochromic anemia with target cells

3. β-Thalassemia major (β0/β0): most severe, severe anemia a few months after birth, high HbF (α2γ2) at birth is temporarily protective.
-α tetramers aggregate and damage RBCs, resulting in ineffective erythropoiesis and extravascular hemolysis (splenic ΜΦ’s)
-Massive erythroid hyperplasia: hematopoiesis expands into skull, extramedullary hematopoiesis w/ HSM, risk of aplastic crisis with parvovirus B19 infx
-chronic transfusions -> hemachromatosis
-microcytic, hypochromic RBCs with target cells and nucleated red blood cells
-little or no HbA with increased HbA2 and HbF.