Microcytic, Hypochromic Anemia Olsow

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MCV in microcytic, hypochromic anemia

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<80

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Microcytic, hypochromic anemia is seen in these 5 conditions

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1.Iron deficiency
2.Alpha thalassemia
3.Beta thalassemia
4.Lead poisoning
5.Sideroblastic anemia

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T/F: There is iron deficiency in pregnancy

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True

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Triad of Plummer-Vinson syndrome

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Iron deficiency anemia, esophageal web, and atrophic glossitis.

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Alpha thalassemia is prevalent in these 2 populations

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Asian and African populations

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What is the defect in alpha thalassemia

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alpha-globin gene mutations leading to a decrease in alpha-globin synthesis

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The deletion which is incompatible with life in alpha-thalassemia

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Deletion of 4 genes

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In alpha thalassemia, deletion of 4 genes leads to the formation of what

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Hb Barts (γ4)

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The formation of Hb Barts in alpha thalassemia causes

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Hydrops fetalis

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HbH disease(β4) in alpha thalassemia results from

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The deletion of 3 genes

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T/F: Deletion of 1-2 genes in alpha thalassemia is not associated with significant anemia

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True

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Hb Barts is formed in alpha thalassemia when

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4 genes are deleted

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T/F: Hb Barts is compatible with life

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False. Incompatible

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HbH in alpha thalassemia is caused by

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Deletion of 3 genes

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Beta thalassemia is prevalent in this population

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Mediterranean populations

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4 types of beta thalassemia

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1.Major(Homozygote)
2.Minor(Heterozygote)
3.Both major and minor
4.HbS/β-thalassemia heterozygote

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T/F: β chain is present in β thalassemia major

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False absent

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Describe the anemia in β thalassemia major

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Severe anemia requiring blood transfusion

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Blood transfusion in β thalassemia major predisposes the patients to

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Secondary hemochromatosis

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What happens to the bone marrow in β thalassemia major

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There is marrow expansion

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Term used in describing marrow expansion on skull x ray in beta thalassemia major

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"Crew cut" on skull x ray

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T/F: Marrow expansion in beta thalassemia major does not cause skeletal deformities

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False. Causes skeletal deformities

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Chipmunk facies is seen in what type of thalassemia

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Beta thalassemia major

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Bone marrow expansion is seen in what type of thalassemia

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Beta thalassemia major

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Crew cut on skull x ray is seen in what type of thalassemia

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Beta thalassemia major

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Severe anemia requiring blood transfusion is seen in what type of thalassemia

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Beta thalassemia major

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T/F: Beta thalassemia major is heterozygote

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Homozygote

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The beta thalassemia in which the β chain is underproduced is

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Beta thalassemia minor

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The usually asymptomatic beta thalassemia is

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Beta thalassemia minor

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How is the diagnosis of beta thalassemia minor confirmed

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Increased HbA2 (>3.5%) on electrophoresis

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A characteristic of a combination of both major and minor beta thalassemias

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Increased HbF (α2γ2)

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A characteristic of HbS/β-thalassemia heterozygote

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Mild to moderate sickle cell disease depending on amount of beta globin production

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Which of the thalassemias is confirmed by increased HbA2 on electrophoresis

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Beta thalassemia minor

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Increased HbF is seen in which thalassemia

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A combination of both major and minor beta thalassemias

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What kind of hemoglobin is used for the confirmation of beta thalassemia minor on electrophoresis

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HbA2

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What is the underlying defect in beta thalassemia

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Point mutations in splicing sites and promoter sequences

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Lead inhibits these 3 things

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Ferrochelatase, ALA dehydratase and rRNA degradation

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Why the decreased heme synthesis in lead poisoning

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Because of the inhibition of ferrochelatase and ALa dehydratase by lead

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Why the basophilic stipling in lead poisoning

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Aggregation of ribosomes due to inhibition of rRNA degradation by lead

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The 2 enzymes in heme synthesis inhibited by lead

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Ferrochelatase and ALA dehydratase

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T/F: Lead promotes rRNA degradation

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False. Inhibits the degradation

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The kind of anemia caused by a defect in heme synthesis

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Sideroblastic anemia

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T/F: Sideroblastic anemia can be hereditary

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True

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The defect in hereditary sideroblastic anemia

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X-linked defect in δ-aminolevulinic acid synthase gene

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2 reversible etiologies of sideroblastic anemia

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Alcohol and lead

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Pharmacological treatment of sideroblastic anemia

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Pyridoxine (B6) therapy

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Describe the levels of iron, TIBC, and ferritin in sideroblastic anemia

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Increased iron and ferritn; Normal TIBC

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The pathological RBC seen in sideroblastic anemia

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Ringed sideroblasts (with iron-laden mitochondria)

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X-linked defect in δ-aminolevulinic acid synthase gene will cause

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Hereditary sideroblastic anemia

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In lead poisoning, lead lines are seen in these 2 areas

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The gingivae and on epiphyses of long bones in x ray

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T/F: Lead poisoning causes encephalopathy

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True

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T/F: Lead poisoning causes sideroblastic anemia

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True

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The abdominal symptom in lead poisoning is

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Abdominal colic

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What happens to the wrist and foot in lead poisoning

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Wrist and foot drop

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1st line treatment of lead poisoning

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Dimercaprol and EDTA (Ethylenediaminetetraacetic acid)

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Treatment of lead poisoning in kids

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Succimer

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Another name for lead lines

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Burton's lines

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Succimer is used in the treatment of lead poisoning for which population

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Kids

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In the diagnosis of beta thalassemia minor, what is the % of HbA2 on electrophoresis

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>3.5%