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Overview of Anemia

       Dr. Adnan Qureshi
           Resident Haematology
          Aga Khan University Hospital

 
Red Cell Production (Erythropoiesis)
Kidney Bone Marrow Multi-potent
stem cell

Erythroid
Epo stem cell

Oxygen
sensor
3-4
days
Blood vessel

Peripheral
blood

RBC survival
100-120 days
 
Anemia
 Definition

 Understanding anemia
• Condition - a secondary manifestation of another disease

 Causes
• Impaired red cell production
• Increased rate of destruction
• Blood loss

 
Classification of Anemia
Different modes of classification
 Clinical findings
• Acute / Chronic
• Hereditary / Acquired
 Red cell size
• Determined by MCV
 Red cell kinetics
• Determined by reticulocyte count

 
Anemia - Causes
 Blood loss  Impaired production
• Acute • Aplastic anemia
• Chronic • PRCA
• Anemia of Chronic disease
 Increased destruction
• Anemia of renal failure
• Intrinsic
• Anemia of endocrine disorders
– Membrane disorders: HS, HE …
• Iron deficiency anemia
– Enzyme deficiencies: G6PD, PK …
• Megaloblastic anemia
– Disorder of hemoglobin synthesis:
• Etc….
Hemoglobinopathies, PNH …
• Extrinsic
– Antibody mediated
– Mechanical trauma: MAHA, TTP,
DIC
– Infections: malaria
– Sequestration
 
Laboratory Evaluation of Anemia
 Commonly ordered test
• CBC
• Peripheral film
 Supplementary test (depending upon the initial findings)
• Retic count
• Coomb’s test
• HB elctrophoresis
• G6PD
• Bone marrow

 
Reticulocyte Count
 Relative reticulocyte count
• Percent of all RBC
 Corrected reticulocyte count
• Retic % x Pt. Hct
Normal Hct
 Absolute reticulocyte count
• Relative reticulocyte count x
RBC count
 Reticulocyte production index
(RPI)

 
Classification of Anemia Based on RBC Size

MCV

Microcytic Normocytic Macrocytic

< 76fl 76-96 fl > 96 fl

 
Hypochromic Microcytic Anemia:

 Causes:
• Iron deficiency
• Thalassemia
• Lead toxicity
• Sideroblastic anemia
• Long standing anemia of chronic disease

 
Causes of Iron Deficiency
Iron deficiency is a symptom, not a disease
 Increased iron requirements  Inadequate iron supply
• Blood loss • Insufficient dietary iron
– Gastrointestinal tract • Impaired iron absorption
– Genitourinary tract – Gastric surgery
– Blood donation – Intestinal malabsorption
• Pregnancy and lactation – Celiac disease

 
Iron Deficiency Anemia

 Iron profile
• Serum Iron Low

• TIBC Raised

• Serum Ferritin Low

• Transferrin Saturation Low

 
Thalassemia: Impaired Globin Gene
Production
Hgb A tetramer

α globin β globin

β globin α globin

 
Thalassemia
 Decreased production of normal globin chains
∀ α thalassemia - deficiency of α gene(s)
∀ β thalassemia - deficiency of β gene(s)

 
Alpha Thalassemia: Clinical Features
 Absence of 1-2 alpha chains
• Common
• Asymptomatic X
α2 α1 α2 α1 X
α2 α1

• Does not require therapy


α2 α1 XX
α2 α1
X
α2 α1

 Absence of 3 alpha chains


(Hgb H disease) X
α2 α1

• Microcytic anemia (Hgb 7-10) XX


α2 α1

• Splenomegaly
 Absence of 4 alpha chains XX
α2 α1
• Hydrops fetalis (non-viable) XX
α2 α1

 
Alpha Thalassemia: Laboratory Findings
Hemoglobin
α Chains Hgb (g/dl) MCV (fl) Analysis

αα/αα Normal Normal Normal


αα/-α 12-14 75-85 Normal
α-/α- or 11-13 70-75 Normal with Hgb Barts (γ4);
--/αα Hgb H (β4) - small
--/-α 7-10 50-60 Normal with Hgb Barts (γ4);
Hgb H (β4) - large
--/-- - - Not viable

 
Beta Thalassemia

Clinical Hgb
Syndrome Genotype Hgb (g/dl) Analysis
Minor (Trait) β/β+ or β/β° 10-13 ↑ Hgb A2, ↑ Hgb F

Intermedia β+/β+ 7-9 ↑ Hgb A2, ↑↑ Hgb F

Major (Cooleys) β+/β° or β°/β° <7 ↑ Hgb A2, ↑↑↑ Hgb F

 
Normocytic Anemia with Low Retic Count
 Decreased stimulation of RBC production in bone marrow
• Anemia of chronic disease
• Chronic renal insufficiency
 Isolated decrease in RBC precursors (red cell aplasia)
 Bone marrow damage
• Aplastic anemia
• Fibrosis
• Stem cell damage
• Infiltration with tumor/infection
 Disorder of hematopoeisis
• Myelodysplasia/sideroblastic anemia

 
Anemia of Chronic Disease
 Associated conditions
• Infections
– Viral, bacterial, TB, parasitic, fungal
• Autoimmune diseases
– RA, SLE, sarcoidosis, IBD, vasculitis
• Cancer
 Characteristics
• WBC and platelet counts are normal
• Anemia of variable severity (mild-severe)
• Low erythropoietin level
• Low reticulocyte count

 
Normocytic Anemia with High
Reticulocyte Count
 General principles
• Bleeding may have similar lab findings as hemolysis
• High reticulocyte count may lead to macrocytosis
• Classification
– Hereditary vs. acquired
– Extravascular vs. intravascular
– Immune vs. nonimmune

 
Hemolytic Anemia with Extravascular
Hemolysis
 Extravascular (reticuloendothelial system)
• Hereditary
– Hemoglobinopathies (sickle cell anemia)
– Enzymopathies (G6PD deficiency)
– Membrane defects (hereditary spherocytosis)
• Acquired
– Immune mediated
 Autoimmune hemolytic anemia
– Nonimmune mediated
 Spur cell hemolytic anemia

 
Sickle Cell Disease
 Mutation in beta globin
(β6 Glu Val)
 Inherited as autosomal
recessive

 Protection against malaria

 
Glucose-6-Phosphate Dehydrogenase
Deficiency
 G-6-PD - reduces NADP/oxidizes glucose-6-phosphate
• Detoxifies free radicals and peroxides
 Sex-linked disorder
 Effects > 200 million people
 Hemolytic anemia occurs in the presence of stress
(infection or drugs)
• African form - mild hemolysis
• Mediterranean form - more severe
– Unique sensitivity to fava beans

 
Hereditary Spherocytosis
 Autosomal dominant disorder
 Abnormality in RBC
membrane protein
 Clinical and laboratory
findings
• Splenomegaly
• Chronic hemolytic anemia
• Spherocytes on peripheral
smear
• Increased osmotic fragility

 
Acquired Hemolytic Diseases
 Immune mediated hemolytic anemia

 Non-immune mediated

 
Autoimmune Hemolytic Anemia
 Warm antibodies (IgG-mediated)
• Primary
• Secondary
– Lymphoproliferative disease
– Connective tissue disease
– Infectious disease
• Drug-induced

 Laboratory testing
• Normocytic/macrocytic anemia
• Peripheral smear – spherocytosis
• Coomb’s test - positive
 
Anti-Globulin (Coombs) Testing

Direct antiglobulin testing

+
Anti-C3d
Patients RBCs Anti-IgG

 
Hemolytic Anemia with Intravascular
Hemolysis
 Mechanical damage (Microangiopathic hemolytic
anemia)

 Chemical damage (Burns)

 Infection (Malaria)

 Transfusion reaction (ABO incompatibility)

 
Differential Diagnosis of
Microangiopathic Hemolytic Anemia
 Thrombotic thrombocytopenic purpura (TTP)
 Hemolytic uremic syndrome (HUS)
 Disseminated intravascular coagulation (DIC)
 Vasculitis
 Malignant hypertension
 Metastatic neoplasm with vascular invasion
 Preeclampsia/HELLP syndrome of pregnancy
 
Macrocytic Anemia with Low
Reticulocyte Count
 Megaloblastic anemia
• Vitamin B12 deficiency
• Folate deficiency

 Nonmegaloblastic macrocytic anemia


• Liver disease
• Hypothyroidism
• Drug-induced (DNA synthesis block)
• Myelodysplastic syndrome

 
Morphology ….

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Thank You !

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