3.1: Anaemias due to Red Cell Disorders

3.1.1 Iron Deficiency Anaemia

This is a condition whereby a lack of iron in the body (mainly due to blood loss secondary to haemorrhage, malabsorption and hookworm infestations and pregnancy) leads to a reduction in the number of red blood cells.

Clinical presentation: fatigue, palpitation, dizziness, glossitis, koilonychias (spoon shaped nails) and pica

Diagnostic Criteria

Pallor, glossitis, koilonychias (spoon shaped nails) and pica

Investigations

• CBC-Low Hb, MCV, MCHC and raised RDW
• Peripheral smear- microcytic hypochromic red cell, pencil cells.
• Low serum Iron levels and raised Total iron binding capacity
• Stool analysis

Non-Pharmacological Treatment

• Treat the cause of blood loss, for example menorrhagia, upper GI bleeding due to peptic ulcer and lower GI bleeding secondary to hookworm infestation and malignancy.
• Blood transfusion is only indicated if it is life threatening.

Pharmacological Treatment

A: Ferrous Sulfate 200 mg (PO) 8 hourly for 3 months Children 5 mg/kg (PO) 8 hourly. Continue for 3 months after the normal hemoglobin has been achieved.

3.1.2 Megaloblastic Anemia

This is a condition whereby the bone marrow usually produces large, structurally abnormal, immature red blood cells (megaloblasts) often due to inadequate intake or malabsorption of vitamin B₁₂ or folate.

Diagnostic Criteria

Pallor, depression, hair loss, pins and needles, numbness in hands or feet, tremors and palsies, mildly jaundiced (lemon yellow tint), beefy tongue, darkening of palms and ataxic gait.

Investigations

• FBC-Low Hb, sometime pancytopenia, raised mcv but maybe low normal if coexisting with iron deficiency
• Peripheral smear-oval macrocytes, hyper segmented neutrophils
• Serum vitamin B₁₂ maybe low or normal, Serum folate level, TSH, U+Es, LFT
• Raised reticulocyte count
• Bone marrow studies may be indicated

Pharmacological Treatment

Vitamin (B₁₂ deficiency anaemia) and other macrocytic without neurological involvement A: Hydroxycobalamine, initially 1 mg IM 3 times a week for 2 weeks then 1mg every 3months

Clinically review every 2 months with or without serum B₁₂ and if clinically indicated increase the frequency to every 2 months or every month

Pernicious Anaemia (B₁₂ deficiency) with neurological symptoms and signs A: Hydroxycobalamine, initially 1 mg IM on alternate days until no further improvement (maximum reversal or neuro-psychiatric signs and symptoms are achieved) then 1mg every 2 months

NOTE: • Folic acid 5mg (PO) once daily for least 2 months this must be started simultaneously with injection vitamin B12 • Ferrous Sulphate 200mg 8 hourly for at least 3 months

3.1.3 Haemolytic Anaemia

Haemolytic anaemia results from an increase in the rate of red cell destruction in the intravascular or in the reticuloendothelial system in some pathological disorders

Clinical Features:

• Pallor, jaundice, splenomegaly
• Anaemia, Reticulocytosis, indirect hyperbilirubinemia

Note: After supportive treatment refer to higher health facility with adequate expertise and facilities

Classification of haemolytic anaemia

I. Acquired haemolytic anaemias:

a. Immune

• Autoimmune (warm antibody type, cold antibody)

b. Alloimmune:

• Haemolytic transfusion reactions
• HD
• Allograft especially marrow transplantation

Red cell Fragmentation Syndromes:

• Arterial grafts, cardiac valve
• Microangiopathic haemolytic anaemias

Others

• March haemoglobinuria
• Infections (malaria, clostridia)
• Chemicals and physical agents
• Paroxysmal nocturnal haemoglobinulia

II Hereditary Haemolytic Anaemia

o Membrane

1. Hereditary spherocytosis
2. Hereditary elliptocytosis

o Metabolism

1. G6PD deficiency
2. Pyruvate kinase deficiency

o Haemoglobin

1. Abnormal haemoglobin such as Hb S, C, Unstable Hb

Clinical Features:

• The disease may occur at any age and sex
• Patient may present with symptom and features of Anaemia
• Symptoms are usually slow in onset however rapidly developing anaemia can occur
• Splenomegaly is common but no always observed
• Jaundice

General Treatment:

1. Remove the underlying cause
2. Blood transfusion if anaemia is severe
3. Plasmapheresis

Note: After supportive treatment refer to higher health facility with adequate expertise and facilities

Pharmacological Management

Immunosuppressant’s

C: Prednisolone 1–1.5mg/kg/day (PO) for 1–3 weeks until Hb is greater than 10g/dl

AND

S: Cyclosphophamide 60mg/m2 IV

OR

S: Azathioprine 100–150mg/mg (PO) daily

OR

S: Cyclosporin 2–5mg/

OR

S: High dose immunoglobulin 400mg/kg daily IV for 5 days

A: Folic acid is 5mg (PO) daily should be given to severe cases

Surgical Management

Splenectomy may be considered in those who fail to respond