Anemia

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Anemia
Kristine Krafts, M.D. November 3, 2008
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Anemia Outline

• Background facts about blood
• Anemia general information
• Anemia specific types

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Anemia Outline

• Background facts about blood

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Normal blood cells
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Complete Blood Count (CBC)
RBC
hemoglobin
hematocrit
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Complete Blood Count (CBC)
MCV
MCHC
normochromic
hypochromic
microcytic
normocytic
macrocytic
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Additional Red Blood Cell Properties
Size variation
Shape
anisocytosis
poikilocytosis
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Normal red blood cells
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Anemia Outline

• Background facts about blood
• Anemia general information

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Definition

• An (without) -emia (blood)
• a reduction below normal
• in hemoglobin or red blood cell number.

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Symptoms of Anemia

• None! (if slow or mild)
• Fatigue
• Breathlessness
• Dizziness
• Pale skin, mucous membranes
• Jaundice (if hemolytic)
• Tachycardia

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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Extracorpuscular stuff
• Intracorpuscular stuff
• Make too little blood
• Not enough building blocks
• Not enough erythroblasts
• Not enough room

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Anemia Outline

• Background facts about blood
• Anemia general information
• Anemia specific types

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Three Ways to Become Anemic

• Lose blood

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Anemia of Blood Loss
Things you must know

• Cause acute blood loss due to trauma.
• Immediately after blood loss, hemoglobin is
  normal!
• After fluid replacement, low hemoglobin, but
  cells look normal.
• After 2-3 days, see reticulocytes.
• Chronic blood loss is different (it causes iron
  deficiency anemia).

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Reticulocytes
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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood

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Hemolytic anemias

• Intracorpuscular vs. extracorpuscular
• Chronic vs. acute
• Signs of destruction ? bilirubin, ? LDH, ?
  haptoglobin
• Signs of production ? reticulocytes, nucleated
  red cells in blood

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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Extracorpuscular stuff

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Microangiopathic Hemolytic Anemia
Things you must know

• Lots of causes, many dangerous
• Red cells get ripped up, forming schistocytes
• Need to find out why!

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Red cell snagged on fibrin strand
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MAHA schistocytes
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Microangiopathic Hemolytic Anemia
Causes

• Dumpers
• Obstetric complications
• Adenocarcinoma
• Acute promyelocytic leukemia
• Rippers
• Bacterial sepsis
• Trauma
• Vasculitis

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Autoimmune Hemolytic Anemia
Things you must know

• Warm AIHA
• IgG
• Spleen
• Spherocytes
• Cold AIHA
• IgM, complement
• Intravascular hemolysis
• Agglutination

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Warm AIHA
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Warm AIHA
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Warm AIHA
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Cold AIHA
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Cold AIHA
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patient red cells

AHG

agglutination
Direct antiglobulin test (DAT)
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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Extracorpuscular stuff
• Intracorpuscular stuff

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Sickle Cell Anemia
Things you must know

• Hemoglobinopathy (qualitative defect in
  hemoglobin)
• Single amino acid substitution in beta chain of
  hemoglobin
• Can be heterozygous or homozygous
• Sickle cells are nasty
• Fragile (burst easily)
• Get stuck in vessels

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Point mutation in ? chain gene abnormal ?
chains (substitution of valine for
glutamate) Hgb S
Nasty!
Aggregates and polymerizes on deoxygenation
Red cell becomes sickle shaped Sickles clog up
vessels plus, they are fragile
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Sickle cell anemia
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Sickle cell anemia foot lesion
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Sickle cell anemia spleen
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Sickle Cell Anemia
Clinical features

• Blacks (8 are heterozygous)
• Chronic hemolysis
• Vaso-occlusive disease (bones, lungs, limbs)
• ? susceptibility to infection (autosplenectomy)
• Treatment prevent triggers, vaccinate, transfuse

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Thalassemia
Things you must know

• Quantitative defect in hemoglobin
• Cant make enough a or ß chains
• Variable disease severity
• Hypochromic, microcytic anemia with increased RBC
  and target cells

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birth
Hgb F a2?2
Hgb A2 a2d2
Hgb A a2ß2
Hemoglobin chain development
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Thalassemia
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Thalassemia Medullary expansion
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Hereditary Spherocytosis
Things you must know

• Defect in spectrin
• Tons of spherocytes
• Splenectomy curative

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Hereditary spherocytosis
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Jaundice
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Splenomegaly in hereditary spherocytosis
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Glucose-6-Phosphate Dehydrogenase Deficiency
Things you must know

• ? G6PD ? ? peroxides ? cell lysis
• Oxidant exposure
• Bite cells
• Self-limiting

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Glucose-6-Phosphate Dehydrogenase Deficiency
Clinical features

• G6PD gene is on X chromosome
• Highest incidence in malaria regions
• After oxidant exposure, get acute hemolysis
• Self-limiting

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Glucose-6-Phosphate Dehydrogenase Deficiency
Why do the red cells die?

• They cant reduce nasties
• Nasties attack hemoglobin bonds
• Heme breaks away from globin
• Globin denatures and sticks to RBC membrane,
  forming Heinz body
• Macrophages bite out Heinz bodies, leaving cell
  fragile and deformed

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Glucose-6-phosphate dehydrogenase deficiency
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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Make too little blood

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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Make too little blood
• Not enough building blocks

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Iron Deficiency Anemia
Things you must know

• Many causes most important is bleeding from GI
  tract
• Hypochromic, microcytic anemia
• Must find out why patient is anemic! Then treat.

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Hemoglobin
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Iron-deficiency anemia
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Atrophic glossitis in iron-deficiency anemia
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Koilonychia in iron-deficiency anemia
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Iron Deficiency Anemia
Causes

• Decreased iron intake
• Chronic blood loss
• Increased iron requirement

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Anemia of Chronic Disease
Things you must know

• Infections, inflammation, malignancy
• Disturbance in iron metabolism
• Normochromic, normocytic anemia
• Anemia usually mild

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Megaloblastic Anemia
Things you must know

• Defective DNA synthesis leads to
  nuclear/cytoplasmic asynchrony
• B12/folate deficiency
• Macrocytic anemia with hypersegmented neutrophils

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Megaloblastic Anemia
retarded DNA synthesis unimpaired RNA
synthesis BIG cells! immature nucleus mature
cytoplasm
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Megaloblastic Anemia
How is B12 involved?

• B12 (and folate) are required for DNA synthesis.
• Slowed DNA synthesis means big, immature nucleus
• Cytoplasm (with RNA in it) matures just fine
• B12 is also required for conversion of
  homocysteine to methionine
• ? homocysteine atherosclerosis!
• ? methionine subacute combined degeneration

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Megaloblastic anemia
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Megaloblastic anemia
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Atrophic glossitis in megaloblastic anemia
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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Make too little blood
• Not enough building blocks
• Not enough erythroblasts

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Aplastic Anemia
Things you must know

• Pancytopenia
• Empty bone marrow
• Most are idiopathic

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Aplastic anemia
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Aplastic Anemia
Causes

• Idiopathic
• Drugs
• Viruses
• Pregnancy
• Fanconi anemia

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Three Ways to Become Anemic

• Lose blood
• Destroy too much blood
• Make too little blood
• Not enough building blocks
• Not enough erythroblasts
• Not enough room

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Bone marrow full of fibrosis