Kerala State Drug Formulary.pdf

Haemolytic Anaemias
HAEMOLYTIC ANAEMIAS
Autoimmune hemolytic anemia(AIHA)
Warm AIHA: Glucocorticoids : prednisone 1 mg/kg can be tapered
over 2-3 months;Rituximab 375 mg/m 2 BSA, IV weekly for 4 doses.IV IG is less
effective than in ITP with a response rate of about 40%.
Idiopathic cold (AIHA): Rituximab 375 mg/m 2 BSA,IV weekly for 4
doses
A/E: serious adverse events, which can cause death and
disability include severe infusion reactions,cardiac
arrest,tumor lysis syndrome causing acute renal
failure,infections,hepatitis B reactivation,other viral
infections progressive mutlifocal leukoencephalopathy
(PmL),immune toxicity, with depletion of B cells in 70%
to 80% of patients with Non Hodgkin’s lymphoma,
pulmonary toxicity
Paroxysmal nocturnal hemoglobinuria(PNH)
Eculizumab: is a humanized monoclonal antibody against complement
protein C5,administered IV at twice weekly intervals. Folic Acid supplements:
3 mg/day
Hemolytic disease of the new born
Exchange transfusion with Rh negative group ‘O’ blood if say Bilirubin
is > 20 mg% in term babies and >15 mg% in pre-term babies. (150ml/kg body
weight of compatible blood is required for an exchange transfusion)
HAEMOGLOBINOPATHIES
Sickle Cell Syndromes
Antibiotic prophylaxis appropriate for splenectomized patients during
dental or other invasive procedures.
Vigorous oral hydration during or in anticipation of periods of extreme
exercise, exposure to heat or cold, emotional stress or infection.
Pneumococcal and Haemophilus influenza vaccines are less effective
in splenectomized individuals. Thus, patients with Sickle Cell Anemia should
be vaccinated early in life.Bone marrow transplantation can provide definitive
cures.Gene therapy is also useful.
Hydroxyurea
I: Myeloproliferative disorders (primarily polycythemia
vera) not responding to venepuncture and essential
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