RBC Disorders

Two Types Anemia Polycythemia

Anemia
Definition Clinical features Diagnosis Lab Normal values

Classification of Anemia

Morphological Etiological

Morphological
Macrocytic (Megaloblastic )MCV>100 fl Macrocytic (Non megaloblastic) Microcytic (MCV <80 fl ) Normocytic (81-99 fl)

Etiological
Excessive destruction or loss of red cells 1) Blood loss a) acute b) chronic 2)Extra Corpuscular hemolytic disease a) antibodies b) infection eg. Malaria c) Drugs chemicals d) Trauma to red cells

3)Intra corpuscalr hemolytic disease Various acquired and hereditary causes of hemolytic anemia

Inadequate production f mature red cells 1) Deficiency of essential substances like iron , folic acid, vit B12 , protein and other elements like copper,cobalt etc 2) Deficiency of erythroblasts a)Aplastic anemia b)Pure red cell aplasia

3) Infiltration of bone marrow leukemia, lymphoma, carcinoma, myelofibrosis 4) Endocrine abnormalities Myxoedema, addisons disease, pitutary insufficiency 5) Chronic renal disease 6) Chronic inflammatory disease 7)Cirrhosis of liver

Microcytic Hypochromic Anemia

Iron deficiency Anemia

Iron metabolism
Amount Total body iron= 2-5 Distribution Hemoglobin 2-3gm Storage iron ( ferriin & hemosiderin ) -1gm Essential (non available) tissue iron -0.5gm Plasma or transport iron - 3-4 mgm

Transport protein transferrin (beta globulin) One mol binds one or two atomsof ferric iron normal value 1.2 2 g/l Serum iron normal value 100ug/dl TIBC It is the amount of transferrin available to bind with iron normal value 300ug/dl TIBC is normally 3 times that of serum iron % saturation is about 335

IRON
Functions as electron transporter; vital for life Must be in ferrous (Fe+2) state for activity In anaerobic conditions, easy to maintain ferrous state Iron readily donates electrons to oxygen, superoxide radicals, H2O2, OH radicals Ferric (Fe+3) ions cannot transport electrons or O2 Organisms able to limit exposure to iron had major survival advantage

IRON

Body Compartments - 75 kg man

Stores 1000mg
Absorption < 1 mg/day

Tissue 500 mg

3 mg

Excretion < 1 mg/day

Red Cells 2300 mg

IRON CYCLE
CIRCULATING RBCs Fe Fe Fe Fe Fe MONONUCLEAR PHAGOCYTES Fe

Transferrin Receptor

Fe

Ferritin

Ferritin

Fe

FeFe Fe
slow

Ferritin

Fe Fe

Hemosiderin

Fe

RBC PRECURSOR Fe

Fe

TRANSFERRIN

Iron absorption
Duodenum Proximal jejunum Influenced by rate of erythropoiesis and state of iron stores.

Factors affecting Iron Absoption

Form of iron Acids Amount of iron Rate of erythropoiesis

Iron balance
Normal absorption exceeds excretion Plasma iron pool maintained at a constant

GI ABSORPTION OF IRON
Fe
Fe Fe Fe

Fe Fe Fe Fe

Fe

Fe Fe Fe Fe Fe Fe Fe Fe Fe Fe

Fe

Ferritin

Fe

Fe

TRANSFERRIN

Causes of Iron Deficiency

Blood Loss
Gastrointestinal Tract Menstrual Blood Loss Urinary Blood Loss (Rare) Blood in Sputum (Rarer)

IRON

Increased Iron Utilization

Pregnancy Infancy Adolescence Polycythemia Vera

Malabsorption

Dietary inadequacy (almost never sole cause) Combinations of above

Tropical Sprue Gastrectomy Chronic atrophic gastritis

Iron Deficiency Anemia

Stores 0 mg
Absorption 2-10 mg/day

IRON STORES

Tissue 500 mg

3 mg

Excretion Dependent on Cause

Red Cells 1500 mg

IRON DEFICIENCY
Symptoms

Fatigue - Sometimes out of proportion to anemia Atrophic glossitis Pica Koilonychia (Nail spooning) Esophageal Web

Laboratory Findings
Blood Hb RBC WBC Platelets Red cell indices MCV MCH MCHC RDW

Blood picture anisocytosis, poikilocytosis, microcytosis and hypochromia Bone marrow Hypercellular with erythroid hypercelluar.Micronormoblast Iron stain (PERLs) absent or minimal

Biochemical test a) Serum iron Reduced b) TIBC Increased c) % Saturation Decreased d) Serum ferritin Decreased e) Red cell protoporphyrin increased

Differential Diagnosis
Thalassemia minor Anemia of chronic disorders Sideroblastic anemia

Investigation
MCV

Fe Def
decrease

ACD
Low/N

Thal
Decrease

Sidero Decrease D

MCH

decrease

MCHC
Serum Iron TIBC Ferritin

D
D D N absent N

D
Normal Normal N

D
Inc Normal I

D I D absent N

BM Iron
Blast iron HB electro

Absent Present

present
present

present
Ring form

HB A2 N increase

Treatment
Oral Parenteral Blood transfusion Response to treatment?

Plummer Vinson Syndrome

(Patterson Kelly Syndrome)

Characterized by iron deficiency, dysphagia with glossitis Occurs in middle aged or elderly women Anemia tend to be severe spleen palpable Dysphagia due to spasm at the esophageal entrance due to fine web/band formation Mucosal change may lead to carcinoma

Iron overload
Hemosiderosis Hemochromatosis Treatment of iron overload Desferrioxamine Bronze diabetes?