Allergy and Immunology Flashcards
What immunodeficiency is associated with an increased risk of anaphylaxis to whole blood transfusions?
Selective IgA deficiency; body has such low IgA levels that it develops antibodies to IgA when exposed to it in whole blood; minimize risk of anaphylaxis by transfusing washed blood.
Name the disorder. Telangiectasias, photosensitivity leading to malar rash, increased risk of cancers, long narrow face with protruding ears, long limbs.
Bloom syndrome AKA congenital telangiectatic erythema– impairment in DNA helicase activity leads to breaks in DNA
Diagnosis: infant with blood streaked stools after MOP switched from breast-feeding to cow’s milk protein formula feeding; otherwise asymptomatic and gaining weight well
Protein-induced proctitis
Diagnosis: infant with severe vomiting, diarrhea, blood streaked stools, bloated abdomen, several hours after trying new food/formula
Food protein-induces enterocolitis syndrome (FPIES)
SLE is associated with which complement deficiency?
C2 complement deficiency
Which disease is associated with terminal complement deficiency and properdin deficiency?
Meningococcal disease (both meningitidis and gonorrhea)-- think of C5-C9 Properdin is a protein that is part of the complement cascade.
What does DRESS syndrome stand for?
Drug Rash with Eosinophilia and Systemic Symptoms
Associated with anti-seizure medications (phenobarbital, carbamazepine, phenytoin)
Up to 2-8 weeks after drug exposure.
Fevers, facial edema, dusky erythematous papules that can progress to vesicles and bullous lesions
SPARES THE MUCOSAL surfaces
How can you distinguish DRESS from SJS/TEN?
DRESS spares mucosal surfaces
SJS/TEN- usually has hemorrhagic mucositis
Treatment of DRESS syndrome?
2mg/kg/day steroids, tapered over weeks
Stop offending agent
Mode of inheritance and characteristics of Wiskott-Aldrich Syndrome, including Igs trends?
X-linked disorder-
Think of a “wise ostrich” wearing a TIE- Thrombocytopenia (or also small platelets), recurrent Infections, and Eczema
Low IgM, high IgA (upside M is a W–opposite so low IgM, A in Aldrich same–high IgA)
Name the disorder and preferred treatment:
Can be seen several weeks after exposure to a drug, rash that erupts in the form of a ring of tense blisters (looks somewhat target like)
Preference for lower abdomen/genital area, string of pearl blisters on trunk
Linear IgA deposition at the dermal-epidermal junction.
Linear IgA bullous dermatosis; treatment is DAPSONE (FDA approved).
Darier sign
Formation of urticaria or a wheal when you rub a lesion on the skin (lesion contains mast cells that degranulate)
True or False. It is contraindicated to give the influenza vaccine to a patient with a history of significant egg allergy (anaphylaxis).
FALSE. No contraindication per 2018-2019 Influenza guidelines to give influenza vaccine for anyone with any degree of severity of allergic reaction to eggs.
What factor poses a child at high risk for asthma?
Parent with asthma.
True or False. Radio-contrast reactions are associated with shellfish or iodine allergies.
False.
Serum sickness reaction can commonly occur in response to which drugs?
Cefaclor, Amoxicillin, Snake Anti-venom
Serum sickness reaction findings
Fever, lymphadenopathy, arthralgia, arthritis, nephritis, rash
Name the disorder: cold and deep abscesses without surrounding erythema due to impaired neutrophil chemotaxis (cannot adhere to the endothelium and go out into the tissues), significant eczema; delayed shedding of primary teeth
Hyper IgE Syndrome, significant eczema due to extrusion of eosinophils into tissues and over production of IgEs
How is SCID screened for on the newborn screen?
T-cell Receptor Excision Circles– surrogate measure for naive T-cells; junk DNA that is excised in a circular fashion. Low TRECs, low number of lymphocytes. Confirm by flow cytometry.
Those with selective IgA deficiency are at increased risk of gastroenteritis related to which organism?
Giardia
DRESS is thought to be associated with the reactivation of which virus?
HHV-6
Diagnostic criteria for selective IgA deficiency?
IgA <7 with otherwise normal serum IgG and IgM in children >4 years old
Which live-attenuated vaccine has increased risk of transmission to immunocompromised household members and should therefore be avoided?
Oral polio vaccine (not very common in the USA, but still seen around the world)
Disorder with recurrent sinopulmonary infections, bronchiectasis, pneumatoceles, empyemas, and cold/deep abscesses.
Hyper IgE syndrome AKA Job syndrome
Name the disorder:
- Light skin, silver hair, prominent scarring over previous infections, photophobia, oculocutaneous albinism
- Large lysosomal granules in neutrophils
Chediak-Higashi syndrome; problems with lysosomal degranulation and so infective bactericidal activity
Chediak-Higashi is associated with which hematologic issue?
Prolonged bleeding time due to faulty platelet aggregation
Treatment for Chediak-Higashi
Bone Marrow Transplant
CHARGE syndrome has a strong association with which immunologic disorder?
Complete DiGeorge phenotype (thymic aplasia)– up to 1/3 with thymic aplasia can have CHARGE syndrome
What does CHARGE syndrome stand for?
Coloboma Heart defects Atresia of choanae Retarded growth or mental abilities Genital hypoplasia in males Ear abnormalities
Delayed umbilical cord falling, appears black but not purulent–what disorder is this associated with? What is a classic finding on CBC?
Leukocyte Adhesion Deficiency– leukocytes are not able to adhere to the endothelium and move out to the endothelium; may see significant leukocytosis
Urticaria that forms over flea or mosquito bites, biopsy shows eosinophilic infiltration?
Papular urticaria
What is the pathophysiology of Common Variable Immunodeficiency?
B-cells recognize pathogens and multiply but fail to differentiate into plasma cells–> panhypogammaglobulinemia
Common Variable Immunodeficiency vs Bruton’s agammaglobulinemia differences?
CVID- cannot form plasma cells, but otherwise phenotypically normal B-cells detected on flow cytometry; sporadic; also see bronchiectasis and nodular lymphoid hyperplasia
XLA (Bruton’s)- no mature B-cells, so absent B-cells on flow cytometry, no lymphoid tissue, X-linked inheritance, usually susceptible to encapsulated organism infection
Chronic Granulomatous Disease diagnostic test?
Nitroblue Tetrazolium or Dihydrorhodamine
Chronic Granulomatous Disease inheritance?
X-linked recessive
Which two immunodeficiencies are associated with PCP PNA?
SCID and Hyper IgM Syndrome
