Guest speaker -IPT Immunology

Question 1

What is Immune Thrombocytopenia (ITP)?

Answer 1

An acquired autoimmune disorder in which the body’s immune system destroys healthy platelets
o Leave patient at risk for spontaneous bleeding & bruising

Leaves patient at risk for spontaneous bleeding & bruising

Question 2

What are the environmental causes of ITP?

Answer 2

• Infections (viral)
• Vaccines
• Toxins
• Stress

Question 3

What genetic predispositions are associated with ITP?

Answer 3

• Family Hx of autoimmunity
• Known gene mutations
• 1st sign of immunodeficiency

Question 4

What is loss of immune self-tolerance?

Answer 4

• Antigen mimicry
• Anti-platelet IgG antibodies
• Dysfunction of Tregs

Question 5

What are the common signs and symptoms of ITP?

Answer 5

• Purpura
• Petechiae
• Nosebleeds
• Heavy menstrual bleeding
• Bleeding in mouth/gums
• Blood in emesis/urine/stool
• Fatigue

Question 6

What laboratory findings are associated with ITP?

Answer 6

• Normal WBC
• Normal Hb
• Normal cell morphology
• Anemia only present if severe bleeding

Question 7

What are the major differentials for causes of thrombocytopenia in children?

Answer 7

• Primary ITP
• PID (primary immune deficiency or immune dysregulation disorder)
• IPD (inherited platelet disorder)

Question 8

What can ITP indicate in the context of inborn errors of immunity (IEI)?

Answer 8

ITP can be the 1st presentation of an IEI

IEI includes infections, allergy, autoimmunity, malignancy, and autoinflammation

Question 9

What are the features of immunodeficiency disorders related to IEI?

Answer 9

• Susceptible to pathogens
• Recurrent infections
• Severe infections

Question 10

What are the features of immune dysregulation disorders related to IEI?

Answer 10

• Loss of self-tolerance
• Autoimmune cytopenias (ITP)
• Cancer predisposition
• Eczema/severe allergies
• Systemic autoimmunity (Lupus)/vasculitis
• Enteropathy/inflammatory bowel disease

Question 11

What are the general treatment goals of ITP?

Answer 11

Treat bleeding symptoms – NOT platelet count

Question 12

What are the first-line treatment options for ITP?

Answer 12

• Observation
• IVIG
• Corticosteroids

Question 13

What are the second-line treatment options for ITP?

Answer 13

• TPO-agents
• Rituximab
• Splenectomy

Question 14

Why is immunology important?

Answer 14

• Understanding intricate biochemical pathways of the immune system helps better understand human disease
• Knowing which pathway/receptor/gene may be involved helps predict symptoms a patient might experience
• Every immunologic pathway has potential to be targeted by therapies that can treat human disease