Immunology/Allergology Flashcards
A 8 year old male was brought to you for chronic sneezing and cough more prominent in the morning. Father and mother were known asthmatics. Upon physical examination, patient had watery nasal discharge with boggy turbinates. (+) allergic shiners. For the past 2 months, patient has been having almost daily attacks with difficulty sleeping. What is the classification of this patient? A. Mild persistent B. Moderate-severe persistent C. Mild intermittent D. Moderate-severe intermittent
B. Moderate-severe persistent
ARIA Classification
Intermittent if symptoms:
<4 days per week, OR <4 consecutive weeks
Persistent if symptoms:
>4 days per week, OR >4 consecutive weeks
Mild if ALL of the following are fulfilled:
- normal sleep
- no impairment in activities, sports, leisure
- no impairment of work/school
- symptoms present but not troublesome
Otherwise, patient is classified as having moderate-severe symptoms.
Late phase reaction in allergic rhinitis arises how many hours following allergen exposure
4-8 hours
Early phase reaction: degranulation of mast cells and release of inflammatory mediators
Treatment for patients with moderate-severe persistent allergic rhinitis
In preferred order:
- Intranasal corticosteroid
- Oral antihistamine (given sa lahat ng classification) or LTRA
Can also give: INTERMITTENT MILD: Intranasal antihistamine Decongestant Leukotriene receptor antagonist
INTERMITTENT MODERATE-SEVERE/ PERSISTENT MILD Intranasal antihistamine Decongestant Leukotriene receptor antagonist Intranasal corticosteroid
A 3 year old male patient comes to the clinic for right ear pain and fever. It started 1 day ago which prompted consult. (-) discharge, patient has had cough and colds 3 days ago. The mother says that the patient has been having repeated ear infections and colds since infancy. He was also hospitalized twice for pneumonia at ages 9 months and 18 months. He is also known to have allergies to certain milk products. Further work up revealed IgA <10mg/dL, IgM, IgE within normal limits, what is the most likely condition? A. Selective IgA deficiency B. Bruton agammoglobulinemia C. Common variable immunodeficiency D. Severe combined immunodeficiency
A. Selective IgA deficiency- mucosal surfaces are inverted?
10 warning signs of primary immunodeficiency
- Four or more new ear infections within a year
- Two or more serious sinus infections within a year
- Two or more months on antibiotics with little effect
- Two or more pneumonia in a year
- Failure of an infant to gain weight or grow normally
- Recurrent, deep skin or organ abscesses
- Persistent thrush in mouth or fungal infection on skin
- Need for intravenous antibiotics to clear infections
- Two or more deep-seated infections including septicemia
- A family history of PI
A 1 year old male patient comes to the clinic due to cough, fast breathing and fever. It started 1 day ago which prompted consult. (-) discharge, patient has had cough and colds 3 days ago. The mother says that the patient had repeated ear infections 1 at 8 months, and 11 months. He was also admitted for pneumonia at 8 months of age. At present, HR 120, RR 55, T>38.5C, (+) crackles bilateral lung fields
with decreased breath sounds over the right lung, cervical lymphadenopathies, tonsillar tissue, after antibiotic treatment, further workup was done which revealed depressed levels of IgG, IgA, IgM and IgE below that of appropriately aged matched controls.
What is the most likely diagnosis in this case?
a. selective IgA deficiency
b. bruton agammaglobulinemia
c. common variable immunodeficiency
d. severe combined immunodeficienc
b. bruton agammaglobulinemia
Bruton disease (X-linked agammaglobulinemia)
- block in the development of pre-B to B-cell due to a defect in heavy chain gene rearrangement.
- healthy infants until 6 months old: recurrent ear infections, bronchitis or pneumococcal pneumonia, and/or dermatitis
- low or absent Igs
Management:
- Gamma globulin IM once a month at interval of 2-3 weeks
A 6 month old male infant was seen for persistent diarrhea for the past 2 weeks. The patient has visible wasting, underweight, and appears weak and irritable. The mother said that the patient has been having repeated bouts of diarrhea since 4 months of age. He
also was admitted for pneumonia at 3months of age. The infant on examination has oral thrush, no visible tonsillar tissue, no palpable lymphadenopathies. Previous xrays at 3 months of age show no thymic shadow, further workup was done which revealed depressed levels of IgG, IgA, IgM and IgE below that of appropriately aged matched controls what is the most likely diagnosis in this case?
A. Di-George syndrome
B. bruton agammaglobulinemia
C.common variable immunodeficiency
D. severe combined immunodeficiency
D. severe combined immunodeficiency
Severe Combined Immunodeficiency Disorder
- Selective defect in cell-mediated immunity with susceptibility to chronic candida infection of the skin and mucous membranes w/ or w/o endocrinopathy
- failure to thrive, chronic otitis media, chronic diarrhea, oropharyngeal candidiasis, recurrent pneumonia
Enzyme deficient in 50% cases in severe combined immunodeficiency disorder
Adenosine deaminase
- due to deletion or mutation of the encoding gene -> deoxyadenosine and deoxy-ATP accumulate inside lymphocytes -> cell death
A 10 month old male patient is brought to the clinic by his mother because of a rash on his face. This started 1 week ago and he was seen by his mother to be scratching or touching the facial area. She also noticed rashes in the area over the upper extremities.
There was no use of new soaps/shampoos, no substance was applied to the skin, mother is a known asthmatic. On close examination, there is an eczematous rash over the face particularly the cheeks and the extensor surfaces of the upper
extremities. What is the most likely diagnosis?
A. irritant contact dermatitis
B. allergic contact dermatitis
C. photosensitive dermatitis
D. atopic dermatitis
E. infantile SLE
D. atopic dermatitis
Aberrant thymus or its absence occurs in the 12th week of gestation. Symptoms at birth involves abnormal facies, hypothyroidism, and congenital heart disese
DiGeorge Syndrome
“Congenital Thymic Aplasia”
Also presents with low lymphocyte count
DiGeorge Syndrome characteristics
“CATCH-22”
Cardiac defect (TOF) Abnormal facies Thymus aplasia Cleft palate Hypocalcemia 22q11.2 chromosomal deletion
Management of DiGeorge Syndrome
Fetal thymus transplant as early as possible results in reconstitution of T-cell immunity
Immunodeficiency with thrombocytopenia, infection, and eczema (T-I-E)
Normal appearing megakaryocytes but small defective platelets. Often presents as prolonged bleeding from the circumcision site or bloody diarrhea during infancy.
Wiskott-Aldrich syndrome
Treatment of Wiskott-Aldrich Syndrome
Nutrition, routine IVIG, use of killed vaccines , platelet transfusion, splenectomy
Treatment of choice: bone marrow or cord blood transplantation
Wiskott-Aldrich Syndrome
“How do you TIE a WASP”
Thrombocytopenia
Infection
Eczema
WASP mutation
Which of the following is the gold standard in the diagnosis of food allergy?
A. Patch test
B. Skin prick test
C. RAST (radioallergosorbent test)
D. Double-blind placebo controlled food challenge
D. Double-blind placebo controlled food challenge
Study primary defects of antibody production TABLE
Topnotch p. 81
Functional deficiency and main clinical features of
XLA or Bruton Agammaglobulinemia
Functional deficiency: no antibody production, lack of B cells
Clinical features: bacterial infections, small or absent tonsils, no palpable LN
Functional deficiency and main clinical features of
CVID
Functional deficiency: low IgG with normal B cells
Functional deficiency and main clinical features of
Selective IgA deficiency
Functional deficiency: low or absent IgA
Clinical features: resp, GI, urogenital tract infections
Functional deficiency and main clinical features of
Hyper IgM syndrome
Functional deficiency: failure to produce IgG, IgA, IgE antibiodies with normal or increased IgM
Clinical features: recurrent pyogenic infections
Functional deficiency and main clinical features of
X-linked lymphoproliferative (XLP) disease or DUNCAN disease
Functional deficiency: low Ig, lack of anti EBNA and long-lived T cell immunity
Clinical features: inadequate immune response to EBV, fatal infectious mononucleosis, lymphomas, acquired hypogammaglobulinemia
Treatment for B cell defects
- Stem cell transplant recommended for XLP and Hyper IgM
- antibiotics and regular IVIG for primary B cell defects
Primary defects of Antibody Production
- XLA/Bruton
- CVID
- Selective IgA deficiency
- Hyper IgM syndrome
- XLP/Duncan
