Question # 1

A 31-year-old farmer is hospitalized with a severe reaction to a bee (Hymenoptera) sting.

He developed respiratory distress requiring endotracheal intubation in the field. He was
treated with epinephrine and corticosteroids with rapid recovery. The patient has no prior
similar incidents and no allergies. He is given an epinephrine autoinjector and instructed
on its use.

Which of the following is the most important part of discharge planning in this patient?

A. Antihistamine therapy

B. Prolonged course of corticosteroids

C. Referral for immunotherapy

D. Serum total immunoglobulin E levels

E. Serum tryptase levels

 Explanation

Answer : 3
Hymenoptera stings can cause life-threatening anaphylaxis. One study found that up
to 50% of patients with anaphylaxis had no previous reaction or known allergy. Sixty
percent of adult subjects with a history of anaphylaxis from a previous sting will have
anaphylaxis with a repeat sting. Venom immunotherapy (VIT) reduces this risk to less
than 5% by producing venom-specific immunoglobulin (Ig) G, changing T cell responses,
and decreasing venom-specific IgE. VIT is indicated in patients with a history of systemic
allergic reaction to an insect sting and evidence of venom-specific IgE, either by skin or in
vitro test. Discharge care should also include education on avoidance and an epinephrine
autoinjector.

(Choices A and B) Acute medical treatment for anaphylaxis includes

intramuscular epinephrine, H-1 receptor antagonists, corticosteroids, and supportive
therapy for shock. However, these agents are not as beneficial after resolution of the
acute episode.

(Choice D) Serum IgE levels rise during the acute anaphylaxis stage and during the first
few months of immunotherapy before decreasing over the next 3-5 years of VIT. However,
these levels do not reliably predict protection from future anaphylaxis reactions.

(Choice E) Tryptase is a mediator released from mast cells and basophils. Serum
tryptase levels may be elevated in anaphylaxis but also in other mast cell disorders (eg,
systemic mastocytosis) and myelodysplastic syndromes. In addition, tryptase decreases
after the anaphylaxis reaction and would not be as useful to monitor as immunotherapy in
this patient.

Educational objective:
Venom immunotherapy reduces the risk of anaphylaxis recurrence from 60% to 5%. It is
indicated for patients with history of systemic reaction to an insect sting and venom-specific
immunoglobulin E.

References
• Chapter 4: Stinging insect allergy and venom immunotherapy.
• A controlled trial of immunotherapy in insect hypersensitivity
Question # 2
A 60-year-old man is hospitalized for acute pancreatitis. He reports swelling and
tenderness in his jaw that appeared 6 months ago. He has no medical problems, takes no
medications, and does not use tobacco, alcohol, or illicit drugs. There is no history of
trauma to the abdomen. He does not have dry eyes or dry mouth. On admission,
ultrasound of the abdomen shows no gallstones. CT of the abdomen reveals a 3x4-cm
solid mass in the mid-portion of his pancreas with some surrounding edema. Chest CT
reveals bilateral hilar adenopathy. He is started on intravenous fluids, opioids for pain
control, and nothing by mouth, and his abdominal pain resolves.

On examination, he is well-nourished and appears comfortable. His blood pressure is 133/

78 mm Hg, pulse is 90/min, and oxygen saturation is 98% on room air. There is bilateral
enlargement of his parotid glands and some enlargement of his lacrimal glands. The
remainder of his examination is within normal limits.

Laboratory results are as follows:

Complete blood count

Hematocrit 38%

Mean corpuscular volume 88 fL

Leukocytes 8,000/L

Neutrophils 60%

Eosinophils 8%

Lymphocytes 30%

Monocytes 2%

Serum chemistry and fasting lipid panels, liver function tests, urinalysis, and erythrocyte
sedimentation rate are all within normal limits. HIV testing is negative.

Which of the following conditions is the most likely cause of this patient’s presentation?

A. IgG4-related disorder

B. Large B cell lymphoma

C. Occult alcoholism

D. Pancreatic carcinoma
E. Sjögren’s syndrome
 Explanation

Answer : 1

This patient’s presentation of acute idiopathic pancreatitis, bilateral salivary and lacrimal
gland enlargement, and lymphadenopathy suggests immunoglobulin IgG4-related disease
(IgG4-RD), a fibro-inflammatory disease causing fibrosis and tumor-like swelling of affected
organs. IgG4-RD is an emerging disease of unknown etiology. It most commonly affects
middle-aged men and should be suspected in patients presenting with any of the following:

• Idiopathic pancreatitis
• Sclerosing cholangitis
• Bilateral salivary and/or lacrimal gland enlargement

Up to 40% of patients may have peripheral eosinophilia. Diagnosis is confirmed with tissue
biopsy showing IgG4-positive plasma cells and lymphocytes. Elevated serum IgG4 can be
 helpful as well although serum levels can be normal. Patients typically respond to
glucocorticoids.

(Choice B) Patients with lymphoma can present with a pancreatic mass and
lymphadenopathy, but they often present with B symptoms (eg, fever, weight loss, night
sweats) as well. This patient’s presentation of acute pancreatitis and bilateral parotid and
lacrimal gland swelling is not typical for lymphoma.

(Choice C) Alcoholism is sometimes associated with parotid gland enlargement in

malnourished patients. Alcoholism sufficient to cause acute pancreatitis is often associated
with increased mean corpuscular volume and elevated transaminases. This patient has
none of these features and denies alcohol use.

(Choice D) Autoimmune pancreatitis is a prototypical form of IgG4-RD that can mimic

pancreatic cancer with a pancreatic mass and painless jaundice. However, there is usually
no history of weight loss.

(Choice E) Patients with Sjögren’s syndrome usually complain of dry mouth and eyes,
and/or arthralgia. They also have antibodies to SSA and SSB proteins and do not have
recurrent episodes of pancreatitis, as is common in IgG4-RD. Patients with IgG4-related
salivary and lacrimal gland disease do not have severe sicca symptoms despite glandular
enlargement.

Educational objective:
IgG4-related disorders can present with autoimmune pancreatitis, lymphadenopathy, and
bilateral salivary and/or lacrimal gland enlargement. Diagnosis is confirmed by peripheral
eosinophilia, elevated serum IgG4 levels, and tissue biopsy showing IgG4-positive plasma
cells. Treatment involves glucocorticoids and possibly azathioprine, mycophenolate mofetil,
or rituximab for refractory disease.

References
• Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011.
• Outcome of patients with type 1 or 2 autoimmune pancreatitis.
Question # 3
A 26-year-old primigravid woman at 36 weeks gestation is admitted to the hospital because
of vaginal bleeding and lower abdominal pain. Her other medical problems include
selective IgA deficiency, allergic rhinitis, and recurrent sinusitis. Her anti-IgA antibody
screening is positive.

Evaluation reveals placental abruption. She is scheduled to undergo urgent cesarean

section and requires blood transfusion.

Which of the following should be administered to this patient?

A. Blood product desensitization therapy

B. Irradiated red cells

C. Leukoreduced red cells

D. Normal packed red cells

E. Washed red cells

 Explanation

Answer : 5

When red cells and plasma are separated from whole blood, small amounts of residual
plasma remain in the red cell concentrate. This plasma contains proteins, including IgA.
IgA deficient patients develop antibodies against IgA, which can react with IgA-containing
donor products and induce an anaphylactic reaction. To reduce this risk, red cells should
be washed to remove as much of the plasma as possible.

(Choices B & C) Packed red cells can also be contaminated with leukocytes or leukocyte
debris during the separation process. This can induce febrile transfusion reactions and HLA
alloimmunization. Cytomegalovirus (CMV) also resides in the leukocytes and can be
transmitted through transfusion. Thus, leukoreduction with filters to remove WBCs reduces
the risk of HLA alloimmunization, febrile nonhemolytic transfusion reactions, and
transmission of CMV.

However, leukoreduction alone cannot remove all lymphocyte components. But, gamma
radiation can essentially kill all the living lymphocytic components (T-lymphocytes). This is
 particularly useful in patients at risk for transfusion-associated graft-versus-host disease, a
rare, but fatal complication caused by the engraftment and proliferation of donor T
lymphocytes in the transfused recipient.

(Choice A) Desensitization to HLA and ABO antigens is indicated for certain patients who
are difficult to transfuse secondary to their broad immunogenicity.

Educational objective:
Patients with IgA deficiency should receive washed RBCs to remove as much plasma
(containing IgA) as possible. Patients at risk for transfusion-associated graft-versus-host
disease (e.g., bone marrow transplant) require irradiated RBCs. To reduce CMV
transmission (e.g., patients waiting for transplant or those with AIDS), patients should
receive leukoreduced RBCs.

References
• Red blood cell transfusion in clinical practice.