condition in which the formed elements of the blood are simultaneously depressed. Also known as Bone Marrow Failure Syndrome Characterized by Pancytopenia or the triad of Profound Anaemia Leucopenia Thrombocytopenia Hypoplastic Anaemia is the condition where only RBC production is profoundly decreased but normal or slightly decreased WBC and Platelets.
Etiology and Classification:
Aplastic Anaemia can be 1. Primary or Congenital (Inherited) Fanconi’s Anaemia Dyskeratosis Congenita Reticular Digenesis Diamond Blackfan (Pure Red Cell) Anaemia Miscellaneous: Familial Aplastic Anaemia, Monosomy, Down’s Syndrome etc. 2. Secondary or Acquired A.Idiopathic B.Secondary Drugs: Cytotoxic Drugs, Antiepileptic Drugs, Chloramphenicol, Gold Radiation Chemicals: Benzene, Dyes Viruses: Hepatitis, HIV, Human para viruses Pregnancy Paroxysmal Nocturnal Hemoglobinurea (PNH) Clinical Manifestation 1. Thronbocytopenia: Bleeding Manifestation- Mucosa, Skin, Brain 2. Neutropenia: Infection, PUO 3. Fatigue, Breathlessness, Puffiness of Face, CCF Pathophysiology Seed and Soil Theory a. Seed theory: Aplastic Anaemia is caused by lack of Pluripotent Stem Cell (Evidence-Success of BMT) b. Soil theory: Aplastic Anaemia is caused by lack of Surroundings microenvironment / Stroma of the bone (Evidence-Success of Immuno-suppression) Laboratory Investigations: I. Peripheral Blood Smear II. Bone Marrow Cytology III. Chromosomal Study- Rule out Fanconi’s Anaemia IV. Liver Function Test Treatment: A.Measures to increase Blood Components 1. Blood / Packed Cell Transfusion- Maintain Se. Hb >8 – 10 gm/dl 2. Platelets Transfusion: Maintain > 20000 to prevent spontaneous bleeding 3. Bleeding Control- Local Pressure, Dental Care, Restricted use of NSAID 4. Steroid- Stabilizes capillary wall Prevent bleeding, Promote Erythropoiesis Tab. Prednisolone- 0.5 -1 mg / kg / day B.Promote Erythropoiesis 1. Growth Factor 2. Androgenic Steroid: Inj. Nandronolone enanthate- 2 – 5mg / kg / day / 10 days apart Inj. Methylprednisolone- 15 – 20 mg / kg / day Tab. Prednisolone- 0.5 -1 mg / kg / day C.Immunosuppression 1. Antilymphocyte Globulin (AMG) 2. Antinomocyte Globulin (AMG): 10-15 mg / kg / day for 4 days 3. Cyclosporin: 8 -10 mg /kg / day for 6 months D.Antibiotics: Broad Spectrum as per culture report E.Nursing Care: 1. Prevention from infection 2. Prevention from Trauma 3. Dental Care 4. Hydration 5. Care of Central Line