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, myelofibrosis4) Endocrine abnormalities Myxoedema, addison’s disease,

pitutary insufficiency 5) Chronic renal disease6) Chronic inflammatory disease7)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

IRONBody Compartments - 75 kg man

Stores1000mg

Tissue500 mg

Red Cells2300 mg

3 mgAbsorption < 1 mg/day

Excretion < 1 mg/day

IRON CYCLE

Fe

Fe

FeFeFe Ferritin

Hemosiderinslow

Fe

Fe

Fe FeFe

Fe

Fe Fe

Fe

Ferritin Ferritin

Tra

nsfe

rrin

Rec

epto

r

RBC PRECURSOR

CIRCULATING RBCs

Fe Fe

TRANSFERRIN

MONONUCLEARPHAGOCYTES

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

FeFe

FeFe

Fe FeFe

FeFe

Fe

Fe

Fe

Fe

Fe

Fe

FeFe

Fe

Fe

Fe

Ferritin

Fe Fe

TRANSFERRIN

IRONCauses of Iron Deficiency

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

Increased Iron Utilization Pregnancy Infancy Adolescence Polycythemia Vera

Malabsorption Tropical Sprue Gastrectomy Chronic atrophic gastritis

Dietary inadequacy (almost never sole cause) Combinations of above

IRON STORESIron Deficiency Anemia

Stores0 mg

Tissue500 mg

Red Cells1500 mg

3 mgAbsorption 2-10 mg/day

Excretion Dependent on Cause

IRON DEFICIENCYSymptoms

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.MicronormoblastIron stain (PERL’s) – 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 ß minor Anemia of chronic

disorders Sideroblastic anemia

Investigation

Fe Def ACD Thal Sidero

MCV decrease

Low/N Decrease

Decrease

MCH “ D D

D

MCHC decrease

D D D

Serum Iron D D Normal Inc

TIBC I D Normal Normal

Ferritin D N N I

BM Iron Absent

Present present present

Blast iron absent absent present Ring form

HB electro N N HB A2 increase

N

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 womenAnemia tend to be severe –spleen palpableDysphagia due to spasm at the esophageal

entrance due to fine web/band formationMucosal change may lead to carcinoma

Iron overload

Hemosiderosis HemochromatosisTreatment of iron overload DesferrioxamineBronze diabetes?