Aplastic Anaemia (Bone Marrow Failure)

exp date isn't null, but text field is

Aplastic anaemia is defined as pancytopenia resulting from aplasia of the bone marrow. it can be  inherited  (Fanconi  anaemia)  or  acquired  (idiopathic,  chemical  or  drug  induced).  It  is  one  of  haematological emergency. 

Clinical presentation 

Vary with severity but include;  

  • Anaemia, easy bruising/bleeding, recurrent infection;  
  • splenomegaly is not a feature. 

Diagnostic Criteria 

  • Pancytopenia,  
  • Bone marrow hypocellularity of < 30% hematopoietic cells. 

Investigations 

  • FBP, peripheral smear, reticulocyte count,  
  • Viral screening (HIV, Hepatitis B and C),  
  • Bone marrow aspiration and trephine biopsy. 

Pharmacological Treatment Supportive treatment 

B: blood transfusion (preferred irradiated, leucodepleted) when Hb<7g/dl 

OR 

S: platelet transfusion if bleeding or if platelet count is <10 X109/L. see dosage under  
blood transfusion section 3.6 

AND 

Prophylactics antibiotics, hygiene, isolation of the patient, and use of masks to prevent neutropenic  sepsis in patients with ANC of less than 0.5 X 109/L. 

Note: Culture  and  sensitivity  in  patients  with  neutropenic  sepsis.  Refer  to  management  of  neutropenic  sepsis under malignant diseases chapter. 

Definitive treatment 

Allogeneic haematopoietic stem cell transplantation is indicated in patients younger than 45yrs.   Immunosuppressive Therapy 

S: anti-thymocyte globulin (ATG) (IV) 40mg/kg/daily 4-10days 

AND 

S: cyclosporine (PO)2-10mg/kg/day 12hourly for 6-24months 

AND 

S: eltrombopag (PO): Children <5years; 2.5mg/kg 24hourly, 6-11years; 75mg 24hourly, >12 years; 150mg 24hourly for 6months 

For  patients  who  develop  cyclosporine  toxicity  (nephrotoxicity,  hypertension,  gingival  hypertrophy  and hirsutism), the following drugs may be used although the response rate is low. 

D: methylprednisolone (PO) 5–10mg/kg for 3–14days 

OR 

S: cyclophosphamide (IV) 45mg/kg /day for 4days 

OR 

S: danazol (PO) 5mg/kg/day for 6months 

Note: Give  supportive  therapy  and  refer  patients  to  higher  health  facility  with  adequate  expertise  and  facilities. Monitor blood counts bi-monthly for outpatients, and as needed for admitted patients.