Abim Allergy And Immunology

Front 1

20 yo gentleman with h/o gingivitis, recurrent skin infections (staph infections), and at least three prior episodes of pneumonia presents with a liver abscess.

Which of the following tests would best determine the underlying cause for his recurrent infections?

Back 1

Neutrophil Function Testing

•Childhood disease (but diagnosis as late as 3rd decade)

•NADPHoxidasedeficiencyreducestheleukocyte
oxidative burst responsible for killing bacteria & fungi

•Recurrent infections (especially **S.aureus**,Serratia,
Burkholderia, Nocardia, Aspergillus)

•Noncaseating granulomas (especially of brain, lungs,
liver, spleen, GI tract, GU tract)

•Diagnosis – neutrophil function testing [e.g. nitroblue tetrazolium test, dihydrorhodamine 123 (DHR) test

•Treatment–antimicrobialprophylaxis (i.e. TMP/ SMX, itraconazole); +/- interferon-

Front 2

Dermatitis, recurrent bacterial sinopulmonary infection, scoliosis, retention of primary teeth, fractures with minor trauma, elevated serum IgE

Distinctive facial features (esp. broad nasal base & bridge, frontal bossing, deep set eyes)

Back 2

Hyper IgE Syndrome (Job Syndrome)

Autosomal Dominant - impaired
development of interleukin 17 producing T helper cells

Tx: Anti-staph antibiotics

Front 3

Tests for CVID

Back 3

-SPEP*
-Serum IgG levels/IgG subclasses

Front 4

26 yo woman with h/o recurrent sinusitis & diarrheal illness presents with her 3rd episode of pneumococcal pneumonia in 2 years. CT shows RLL pneumonia with no endobronchial or other mass lesions. WBC 13K; CD4/CD8 lymphocyte ratio normal; total hemolytic complement (CH50) normal; neutrophil function testing normal; sweat chloride test normal. SPEP shows gamma globulins in low-normal range.

Which treatments are most likely to provide protection against similar infections in the future?

Back 4

Monthly IVIG

Pt has CVID

- Bimodal distribution of age at onset (<5 yers and mid-to-late 20s
- Recurrent bacterial infections (esp resp with encapsulated organisms) and GI (Giardia)
-Dx: low levels of IgG or one of its subsets
- POOR response to vaccines; check pneumococcal and tetanus antibody titers
- Increased risk of autoimmune disorders, malignancy (esp. B-cell lymphoma,gastric cancer) and interstitial lung disease

TX = IVIG

Front 5

32 yo woman with steroid-refractory idiopathic thrombocytopenia purpura and migraine headaches complains of several hours of headache, nuchal rigidity, fever, and photophobia. Her medications include sumatriptan and IV Ig (last dose was earlier today). She has no respiratory or skin complaints. Her CSF shows pleocytosis with a neutrophil predominance, no RBCs, normal glucose, normal protein, and negative gram stain.

What is the most likely cause of her symptoms?

Back 5

IVIG

Front 6

23 yo woman presents with her second episode of bacterial meningitis. Her first episode was as a child. Her total hemolytic complement (CH50) is low and her individual complement component levels are pending.

The most likely cause of her recurrent meningitis is?

Back 6

Neisseria meningitidis

Front 7

True allergic transfusion reaction with anaphylaxis, what immune deficiency should you suspect?

Back 7

IgA Deficiency

Front 8

43 yo woman with IgA deficiency needs a blood transfusion acutely in the setting of a GI bleed. She has not had previous transfusions.

What would you recommend?

Back 8

Premedicate with diphenhydramine and have epinephrine on standby

• Anaphylaxis with transfusions (rare) – only patients with absent IgA get anaphylaxis
because they can have anti-IgA antibodies
– most patients have small amount of IgA and therefore no anti-IgA antibodies

• In patients who need transfusion – can test for IgA level before transfusion, but not
practical
– usually premedicate with diphenhydramine and stand-by with epinephrine in case of reaction

• In IgA-deficient patients with prior anaphylaxis during transfusion use:
– washed PRBC or frozen deglycerolized RBC – FFP from IgA-deficient donors

• Platelets are a problem because these need to be fresh and there are no IgA-deficient donors “on-call”

Front 9

Recurrent Giardia infections

Back 9

IgA deficiency

Front 10

27 yo woman presents with complaint of new crops of hives appearing and disappearing on her skin more days than not for the last 2 months. She also has a
history of intermittent lip and tongue swelling.

What do you recommend?

Back 10

H1-blocker (first line)

– if inadequate relief try adding:
• H2-blocker
• doxepin (H1, H2, serotonin receptor blocker)
• calcium channel blocker

Front 11

35 yo gentleman with father who died of “swelling of the throat” presents with recurrent abdominal pain for 9 months and episodes of face and scrotal swelling that do not respond to antihistamines.

Back 11

Hereditary Angioedema - what to check for:

Low C4 and Low C1 esterase inhibitor

C1-inhibitor concentrate or kallikrein inhibitor ecallantide

Front 12

41 yo gentleman presents to the ER with anaphylaxis after a bee sting. He is treated and recovers. Prior to discharge, you counsel him on bee avoidance and the use of an epinephrine autoinjector.

In addition, which of the following should you advise him to do to reduce his risk of anaphylaxis from bee stings in the future?

Back 12

Obtain a venom skin test and receive venom immunotherapy if the skin test is positive

Front 13

46 yo gentleman is diagnosed with neurosyphilis. He gives a history of allergy to penicillin as a young child. He has avoided penicillin and cephalosporins ever since.

What would you do?

Back 13

Perform a penicillin skin test and prescribe penicillin if the skin test is negative

OR

Desensitize to penicillin and then treat with penicillin

DO NOT DESENSITIZE IF NON-IGE MEDIATED (>72 HOURS)

Front 14

24 yo woman with spina bifida is undergoing a urologic surgery (her most recent of many) when she suddenly develops hypotension, bronchospasm, and edema of her skin intraoperatively.

What is the most likely cause?

Back 14

Latex Allergy

Front 15

Tx of Anaphylaxis

Back 15

– Epinephrine
– IVF
– H1-blocker (diphenhydramine)
– H2-blocker (ranitidine)
– Steroids
– Inhaled β-agonist
– Vasopressors
– Glucagon – if patient on β-blocker as these can prolong symptoms and complicate treatment

Front 16

Urticaria pigmentosa, elevated serum tryptase

Back 16

Systemic Mastocytosis

Front 17

On a TNF-alpha inhibitor and new lung findings

Back 17

Check for TB

Front 18

66 yo woman with erosive arthritis treated with prednisone, methotrexate, hydroxychloroquine, and infliximab presents to ED with 10-day history of sore throat, several days of fever to 39°C, and 1 day of diffuse vesicular rash and confusion. She has never had oral, lip or genital ulcers. She did have chicken pox as a child.

What type of isolation should she be in during her ED evaluation and subsequent admission?

Back 18

Airborne precautions --- disseminated zoster