AnemiaKristine Krafts, M.D.

Background facts about bloodAnemia: general informationAnemia: specific typesAnemia Outline

Background facts about bloodAnemia Outline

Normal blood cells

Complete Blood Count (CBC)RBC Hemoglobin Hematocrit

MCVMCHCmicrocyticnormocyticmacrocyticnormochromichypochromicComplete Blood Count (CBC)

Size variationAdditional Red Blood Cell PropertiesShapeanisocytosispoikilocytosis

Normal red blood cells

Background facts about bloodAnemia: general information

Anemia Outline

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

Symptoms of AnemiaPale skin, mucous membranesJaundice (if hemolytic)TachycardiaBreathlessnessDizzinessFatigue

Background facts about bloodAnemia: general informationAnemia: specific typesAnemia Outline

Lose bloodDestroy too much bloodExtracorpuscular reasonsIntracorpuscular reasonsMake too little bloodToo few building blocksToo few erythroblastsNot enough roomThree Ways to Get Anemic

Lose blood

Three Ways to Get Anemic

Anemia of Blood LossCause: traumatic, acute blood lossAt first, hemoglobin is normal!After 2-3 days, see reticulocytesChronic blood loss is different (it causes iron deficiency anemia).Things you must know

Reticulocytes

Lose bloodDestroy too much bloodExtracorpuscular reasonsIntracorpuscular reasonsThree Ways to Get Anemic

Intracorpuscular vs. extracorpuscularChronic vs. acuteSigns of destruction: bilirubin, LDH, haptoglobinSigns of production: reticulocytes, nucleated red cells in bloodHemolytic Anemias

Reticulocytes (supravital stain)

Lose bloodDestroy too much bloodExtracorpuscular reasonsThree Ways to Get Anemic

Microangiopathic Hemolytic AnemiaPhysical trauma to red cellsSchistocytesFind out why!Things You Must Know

Red cells snagged on fibrin strand

Schistocytes

Triangulocyte

Artificial heart valveMalignancyObstetric complicationsSepsisTraumaCauses of MAHA

Warm AIHAIgGSpleenSpherocytesCold AIHAIgM, complementIntravascular hemolysisAgglutinationAutoimmune Hemolytic AnemiaThings You Must Know

Warm AIHA

Warm AIHA

Warm AIHA

Cold AIHA

Cold AIHA

patient red cells+AHG=agglutinationDirect antiglobulin test (DAT)

Lose bloodDestroy too much bloodExtracorpuscular reasonsIntracorpuscular reasonsThree Ways to Get Anemic

Hemoglobinopathy (qualitative defect in hemoglobin)Single amino acid substitution in beta chain of hemoglobinCan be heterozygous or homozygousSickle cells are nasty:Fragile (burst easily)Get stuck in vesselsSickle Cell AnemiaThings You Must Know

Hemoglobin

Point mutation in chain gene

abnormal chains (substitution of valine for glutamate)

Hgb S Aggregates and polymerizes on deoxygenation Red cell becomes sickle shaped Sickles clog up vessels plus, they are fragileNasty!

Sickle cell anemia

Sickle cell anemia: foot lesion

Sickle cell anemia: spleen

Clinical Findings in Sickle Cell AnemiaBlacks (8% are heterozygous)Severity of disease is variableChronic hemolysis, vaso-occlusive disease, and infections (autosplenectomy)Treatment: prevent triggers, vaccinate, transfuse

ThalassemiaQuantitative defect in hemoglobinCant make enough or chainsVariable disease severityHypochromic, microcytic anemia with increased RBC and target cellsThings You Must Know

Hemoglobin chain developmentbirthHgb F = 22Hgb A2 = 22Hgb A = 22

Thalassemia

Thalassemia: Medullary expansion

Hereditary SpherocytosisTons of spherocytesSpectrin defectSplenectomy is curativeThings You Must Know

z

Hereditary spherocytosis

Splenomegaly in hereditary spherocytosis

Glucose-6-Phosphate Dehydrogenase Deficiency G6PD peroxides cell lysisOxidant exposureBite cells (removal of Heinz bodies)Self-limitingThings You Must Know

Some patients asymptomaticOthers have episodic hemolysisTriggers: broad beans (favism), drugs (antibiotics, aspirin)Spontaneous resolutionClinical Findings in G6PD Deficiency

Child with G6PD deficiency: jaundiced sclera

They cant reduce nastiesNasties attack hemoglobin bondsHeme breaks away from globinGlobin denatures, sticks to red cell membrane (Heinz body)Spleen bites out Heinz bodiesWhy Do G6PD-Deficient Red Cells Die?

G6PD deficiency: Heinz bodies

G6PD deficiency: bite cells

Lose bloodDestroy too much bloodExtracorpuscular reasonsIntracorpuscular reasonsMake too little bloodToo few building blocks

Three Ways to Get Anemic

Iron-Deficiency AnemiaThings You Must Know Most important cause: GI bleeding Microcytic, hypochromic anemia Must find out why patient is iron deficient!

Hemoglobin

Iron-deficiency anemia

Atrophic glossitis in iron-deficiency anemia

Koilonychia in iron-deficiency anemia

Decreased iron intakebad dietbad absorptionIncreased iron lossGI bleedmenseshemorrhageIncreased iron requirementpregnancyCauses of Iron Deficiency

Anemia of Chronic DiseaseInfections, inflammation, malignancyIron metabolism disturbedNormochromic, normocytic anemiaAnemia usually mildThings You Must Know

Megaloblastic AnemiaThings You Must KnowDefective DNA synthesisNuclear/cytoplasmic asynchrony B12/folateMacrocytic anemia with oval macrocytes and hypersegmented neutrophils

FH4methylene FH4FH2methyl FH4dUMPdTMPDNAB12Need B12 to make DNA!

retarded DNA synthesis unimpaired RNA synthesis

BIG cells! immature nucleusmature cytoplasmMegaloblastic Anemia

Megaloblastic anemia

Megaloblastic anemia

Atrophic glossitis in megaloblastic anemia

homocysteine homocysteine

endothelial damage

atherosclerosisthrombosisWhat else is B12 good for?methionine methionine

myelin damage

subacute combined degeneration

Lose bloodDestroy too much bloodExtracorpuscular reasonsIntracorpuscular reasonsMake too little bloodToo few building blocksToo few erythroblasts

Three Ways to Get Anemic

Aplastic AnemiaPancytopeniaEmpty marrowMost are idiopathicThings You Must Know

Blood smear in aplastic anemia

Empty bone marrow in aplastic anemia

Empty bone marrow in aplastic anemia

IdiopathicDrugsVirusesPregnancyFanconi anemiaCauses of Aplastic Anemia

Lose bloodDestroy too much bloodExtracorpuscular reasonsIntracorpuscular reasonsMake too little bloodToo few building blocksToo few erythroblastsNot enough roomThree Ways to Get Anemic

Bone marrow full of fibrosis

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