Aplastic anaemia is a condition where the bone marrow fails to produce sufficient blood cells, leading to low counts of red blood cells, white blood cells, and platelets in the bloodstream (pancytopenia). It can be idiopathic (over 50% of cases) or secondary to viral infections, radiation/chemical exposure, drugs, or genetic conditions like Fanconi anemia. Symptoms include weakness, pallor, fatigue, bruising, bleeding, and frequent infections. Diagnosis is based on blood tests and bone marrow examination showing a hypocellular bone marrow. Treatment options include bone marrow transplantation, immunosuppressive drugs, blood transfusions, antibiotics, and growth factors.
1. APLASTIC ANAEMIA
Dr. MA Hassan Chowdhury
Associate professor
Department of Medicine
Chittagong Medical College
2. Definition
It is defined as the failure of the marrow to
produce adequate number of circulating blood
cells giving rise to peripheral pancytopenia.
3. Actio-pathogenesis
• Idiopathic (> 50% cases)
• Secondary: Related to viral infections
(hepatitis, EBV, HIV, parvovirus), exposure to
radiation and chemicals (benzene), drugs and
elements (chloramphenicol phenylbutazone,
and gold).
• Congenital (e.g. Fanconi anaemia).
4. Clinical manifestations
The cardinal clinical features of bone marrow
failure includes-
• Weakness, pallor and fatigue
• Petechiae, purpura and mucosal bleeding
• Frequent severe infections or mouth and
pharyngeal ulcerations
7. Differential Diagnosis
Parameters Acute ITP Acute leukemia Aplastic anaemia
Skin/gum/nose
bleeding
Present Present Present
Fever Usually absent Usually absent Usually absent
Appearnce Normal Sick, irritable Sick
Pallor Insignificant Severe Severe
Lymphadenopathy Absent Present Absent
Bony tenderness Absent Present Absent
Liver size Normal Enlarged Normal
Spleen size Normal Enlarged Normal
Haemoglobin Normal or slightly reduced Low Low
WBC count Normal Usually very high Low
Platelet counts Low Low Low
Blood film Normal Blast cells Normal
Bone marrow Increased megakaryocytes Blast cells >25% Pancytopenia
8. Investigations
• Complete blood count with PBF.
– Usually pancytopenia (anaemia, leucopenia and
decreased platelet count).
– Occasionally bicytopenia or monocytopenia
• Bone marrow study –
– Hypocellular bone marrow with empty specules
– Increased fat space
– Increased lymphocyte or plasma cells
• Test to find out aetiology –
– Chromosomal study- e.g. Fanconi’s anaemia.
– Viral markers.
9. Treatment
• Counseling parents about the disease.
A. Bone marrow transplantation is the treatment of
choice.
B. Immunotherapy:
• Cyclosporine – A, Anti-thymocyte globuline (ATG), Anti-
lymphocyte globuline (ALG) (ALG)
• Steroids e.g. IV Methyleprednisolone or Oral Prednisolone
C. Supportive treatment
• Transfusion of blood & blood products
• Parenteral broad spectrum Antibiotics
• Growth factor administration e.g. Colony stimulationg factor
(G-CSF).
10. Multiple choice questions
• Blood picture of acute leukaemia includes –
___a) Leucocytosis ___b) thrombocytopenia
___c) Immature WBC (blast cells) ___d) target
cells ___e) nucleated RBC
• Thrombocytopenia is found in –
___a) ITP ___b) haemophilia ___c) von
Willebrand disease ___d) Henoch-Schonlein
purpura ___e) acute leukaemia
11. Multiple Choice question
• Normal fractions of haemoglobin of a child include –
___a)HbA ___b) HbA2 ___c) HbF ___d) HbS ___e) Hb Gower
• The blood picture of beta thalassaemia major are –
___a) Microcytic hypochromic anaemia ___b) target cells ___c) nucleated RBC
___d) normal
Reticalocyte count ___e) high TIBC
• The recommended iron chelators are –
___a) desferrioxamine ___b) deferiprone ___c) deferasirox ___d) hydroxyurea
___e) 5-azacytidine
12. • The pathologcal consequences of thalassaemia major are –
___a) haemolysis ___b) excessive medullary and extramedullary haemopoiesis
___c) iron overload
___d) iron dposition to vital organs ___e) organ dysfunction
• In iron deficiency anaemia serum iron profile shows-
___a) low serum iron ___b) low serum ferritin ___c) high % saturation of iron
___d) low total iron
binding capacity ___e) low marrow sideroblast
• Pharmaco-therapy in thalassaemia causes increased production of –
___a) alpha chain ___b) beta chain ___c) gamma chain ___d) delta chain ___e)
theta chain