IMMUNOTHERAPY

Preface of Immunotherapy

Immunotherapy (also called biologic therapy or biotherapy) is a type of cancer treatment designed to boost the body's natural defenses to fight the cancer.• Primary immunodeficiencies are very rare diseases and some are so

unusual that they only affect one or two people in the world.• There is no link between Primary Antibody Deficiency and the

Acquired Immunodeficiency Syndrome (AIDS).• PIDs can present clinically at any age and some improve due to

compensatory mechanisms, others get worse with age.• Secondary immunodeficiency is a result of failure of components of

the immune system that are mainly involved in protecting against infection.

What is treatment available ?

• Almost both of the screening and treatment is the same between primary and secondary immunodeficiencies• Example of screening and treatment :• DiagnosisImmunoglobulin therapyPhysiotherapyAlternative therapiesImmunisationsLifestyles

Importance of Identifying MolecularDefects in PIDs • Pre-symptomatic diagnosis of PIDs:• – based on family history• – neonatal screening for PIDs• Taylor management to gene defect specific treatments• Identify candidates for gene therapy

• Immunoglobulin Therapy• Patients who lack a number of their ownantibodies to fight infections can be

given replacement therapy via an infusion of antibodies.• This is called immunoglobulin replacement therapy• The immunoglobulins are prepared from donated blood.• Physiotherapy• Exercise of breathing to prevent chest infection• Alternative therapies• For now there is no alternative but as to improve moral of the person• Lifestyles• Important with a sensible diet and exercise • Improve quality of lifes

Example Case Study 1 – Wiskott Aldrich syndrome

• 1st-Diagnosis• Should be considered in any boy who has unusual bleeding and

bruises, congenital or early onset thrombocytopenia, and small platelets.• The characteristic platelet abnormalities including low numbers and

small platelet size are almost always present, even in the cord blood of newborns

Treatment available• Vaccines (immunisations)-They should not receive live virus vaccines since there is a possibility that a vaccine strain of the virus may cause disease because patients with WAS have abnormal T- and B-lymphocyte function,- Other “non-live” vaccinations can be given safely to patients with WAS but may not

generate protective levels of antibody. Gene Therapynormal copy of the WAS gene is delivered into the patient’s own bone marrow

cells using a virus so the blood cells coming from the bone marrow are then able to make normal WASp protein.

The initial success of gene therapy in WAS is very encouraging, but a number of problems remain to be solved before it becomes more broadly applicable.

Other treatments which is just to stop the clinical manifestations only!!

• David Vetter (9/21/71-2/22/84)• • Lived in a plastic bubble - sterile environment• - began 20 seconds after birth• • 1977 - NASA developed the Mobile Biological• Isolation System - allowed David to venture• outside the bubble• • 1983 - given bone marrow from sister – less than perfect match but

expected to work with• new methods using unmatched marrow• • Transplant seemed to work at first.• • Transplant harbored EB virus - David died of• Burkitt’s lymphoma

Primary Immune Deficiencies:“Experiments of Nature”• Dr. Robert Good• 1922 - 2003• Founder of Modern Immunology• In 1968 Performed the 1st successful human BM transplant