Kris+ne

Kra,s, M.D. | November 5, 2012

Anemia

Anemia Outline

Background facts about blood Anemia: general informa+on Anemia: specic types

Anemia Outline

Background facts about blood

Normal blood cells

Complete Blood Count (CBC)

RBC
Hemoglobin Hematocrit

Complete Blood Count (CBC)

MCV MCHC

microcy+c

normocy+c

macrocy+c

hypochromic normochromic

Addi+onal Red Blood Cell Proper+es

Size varia+on Shape

anisocytosis

poikilocytosis

Normal red blood cells

Anemia Outline

Background facts about blood Anemia: general informa+on

An (without) -emia (blood): a reduc+on below normal in hemoglobin or red blood cell number.

Symptoms of Anemia
Pale skin, mucous membranes Jaundice (if hemoly;c) Tachycardia Breathlessness Dizziness Fa;gue

Anemia Outline

Background facts about blood Anemia: general informa+on Anemia: specic types

Three Ways to Get Anemic

Lose blood Destroy too much blood Extracorpuscular reasons Intracorpuscular reasons Make too liXle blood Too few building blocks Too few erythroblasts Not enough room

Three Ways to Get Anemic

Lose blood

Anemia of Blood Loss

Things you must know
Cause: trauma+c, acute blood loss At rst, hemoglobin is normal! A,er 2-3 days, see re+culocytes Chronic blood loss is dierent

(it causes iron deciency anemia).

Re+culocytes

Three Ways to Get Anemic

Lose blood Destroy too much blood
Extracorpuscular reasons Intracorpuscular reasons

Hemoly+c Anemias

Intracorpuscular vs. extracorpuscular Chronic vs. acute Signs of destruc+on: bilirubin, LDH,

haptoglobin
Signs of produc+on: re+culocytes,

nucleated red cells in blood

Re+culocytes (supravital stain)

Three Ways to Get Anemic

Lose blood Destroy too much blood
Extracorpuscular reasons

Microangiopathic Hemoly+c Anemia

Things You Must Know

Physical trauma to red cells Schistocytes Find out why!

Red cells snagged on brin strand

Schistocytes

Triangulocyte

Causes of MAHA

Ar+cial heart valve Malignancy Obstetric complica+ons Sepsis Trauma

Autoimmune Hemoly+c Anemia

Things You Must Know
Warm AIHA IgG Spleen Spherocytes Cold AIHA IgM, complement Intravascular hemolysis Agglu+na+on

Warm AIHA

Warm AIHA

Warm AIHA

Cold AIHA

Cold AIHA

patient red cells

AHG

agglutination

Direct an+globulin test (DAT)

Three Ways to Get Anemic

Lose blood Destroy too much blood
Extracorpuscular reasons Intracorpuscular reasons

Sickle Cell Anemia

Things You Must Know
Hemoglobinopathy (qualita+ve defect in

hemoglobin)

Single amino acid subs+tu+on in beta

chain of hemoglobin

Can be heterozygous or homozygous Sickle cells are nasty: Fragile (burst easily) Get stuck in vessels

Hemoglobin

Point muta+on in chain gene abnormal chains (subs+tu+on of valine for glutamate)

Aggregates and polymerizes on deoxygena+on Red cell becomes sickle shaped Sickles clog up vessels plus, they are fragile

Nasty!

Hgb S

Sickle cell anemia

Sickle cell anemia: foot lesion

Sickle cell anemia: spleen

Clinical Findings in Sickle Cell Anemia

Blacks (8% are heterozygous) Severity of disease is variable Chronic hemolysis, vaso-occlusive disease,

and infec+ons (autosplenectomy) transfuse

Treatment: prevent triggers, vaccinate,

Thalassemia
Things You Must Know
Quan+ta+ve defect in hemoglobin Cant make enough or chains Variable disease severity Hypochromic, microcy+c anemia with

increased RBC and target cells

birth Hgb F = 22 Hgb A2 = 22 Hgb A = 22

Hemoglobin chain development

Thalassemia

Thalassemia: Medullary expansion

Hereditary Spherocytosis

Things You Must Know

Tons of spherocytes Spectrin defect Splenectomy is cura+ve

Hereditary spherocytosis

Splenomegaly in hereditary spherocytosis

Glucose-6-Phosphate Dehydrogenase Deciency

Things You Must Know

G6PD peroxides cell lysis Oxidant exposure Bite cells (removal of Heinz bodies) Self-limi+ng

Clinical Findings in G6PD Deciency

Some pa+ents asymptoma+c Others have episodic hemolysis Triggers: broad beans (favism),

drugs (an+bio+cs, aspirin)

Spontaneous resolu+on

Child with G6PD deciency: jaundiced sclera

Why Do G6PD-Decient Red Cells Die?

They cant reduce nas+es Nas+es aXack hemoglobin bonds Heme breaks away from globin Globin denatures, s+cks to red cell

membrane ( Heinz body )

Spleen bites out Heinz bodies

G6PD deciency: Heinz bodies

G6PD deciency: bite cells

Three Ways to Get Anemic

Lose blood Destroy too much blood Extracorpuscular reasons Intracorpuscular reasons Make too liXle blood Too few building blocks

Iron-Deciency Anemia

Things You Must Know

Most important cause: GI bleeding Microcy+c, hypochromic anemia Must nd out why pa+ent is iron decient!

Hemoglobin

Iron-deciency anemia

Atrophic glossi+s in iron-deciency anemia

Koilonychia in iron-deciency anemia

Causes of Iron Deciency

Decreased iron intake bad diet bad absorp+on Increased iron loss GI bleed menses hemorrhage Increased iron requirement pregnancy

Anemia of Chronic Disease

Things You Must Know

Infec+ons, inamma+on, malignancy Iron metabolism disturbed Normochromic, normocy+c anemia Anemia usually mild

Megaloblas+c Anemia
Things You Must Know
Defec+ve DNA synthesis Nuclear/cytoplasmic asynchrony B12/folate Macrocy+c anemia with oval macrocytes

and hypersegmented neutrophils

methyl FH4 B12 FH4

methylene FH4 dUMP

FH2 dTMP DNA

Need B12 to make DNA!

Megaloblas+c Anemia
retarded DNA synthesis unimpaired RNA synthesis

BIG cells!
immature nucleus mature cytoplasm

Megaloblas+c anemia

Megaloblas+c anemia

Atrophic glossi+s in megaloblas+c anemia

What else is B12 good for?

homocysteine
homocysteine endothelial damage atherosclerosis thrombosis

methionine
methionine myelin damage subacute combined degenera+on

Three Ways to Get Anemic

Lose blood Destroy too much blood Extracorpuscular reasons Intracorpuscular reasons Make too liXle blood Too few building blocks Too few erythroblasts

Aplas+c Anemia

Things You Must Know

Pancytopenia Empty marrow Most are idiopathic

Blood smear in aplas+c anemia

Empty bone marrow in aplas+c anemia

Empty bone marrow in aplas+c anemia

Causes of Aplas+c Anemia

Idiopathic Drugs Viruses Pregnancy Fanconi anemia

Three Ways to Get Anemic

Lose blood Destroy too much blood Extracorpuscular reasons Intracorpuscular reasons Make too liXle blood Too few building blocks Too few erythroblasts Not enough room

Bone marrow full of brosis