Protocol Number: 08-I-0184

Title:

Studies in the Pathogenesis of Idiopathic Anaphylaxis

Number:

08-I-0184

Summary:

This study will explore the possible cause of unexplained, or idiopathic, anaphylaxis. Anaphylaxis is a rapid, life-threatening, severe reaction that occurs suddenly after contact with an allergy-causing substance, usually a particular food, drug or stinging insect. The allergen triggers mast cells to release several substances, including histamine. Histamine is responsible for many of the symptoms that may occur, such as flushing, hives, swelling of the palms and soles or tongue and vocal cords, nasal congestion, itching and tearing of the eyes, shortness of breath and wheezing, stomach pain, vomiting, low blood pressure, loss of consciousness, shock, and, rarely, death. Severe episodes of anaphylaxis are treated with epinephrine (adrenaline), followed by oral antihistamines and steroids. In more than half of cases of anaphylaxis, a clear cause is not identified. These cases are called idiopathic anaphylaxis. There is no cure or long-term preventive therapy for patients with recurrent episodes of idiopathic anaphylaxis.

People between 18 and 55 years of age who have idiopathic anaphylaxis episodes at least 6 times a year (with at least one episode every 3 months) may be eligible for this study.

Participants are evaluated at the NIH Clinical Center with the following tests and procedures:

-Medical history, physical examination and blood tests.

-Bone marrow biopsy. For this test, the skin over the hipbone and the outer surface of the hipbone itself are numbed with local anesthesia. Then, a needle is inserted into the hipbone and a small amount of bone marrow is drawn into a syringe. The needle also cuts a small core of bone marrow, which is removed for analysis.

-Other tests that may be needed for evaluation of the patient's condition.

Sponsoring Institute:

National Institute of Allergy and Infectious Diseases (NIAID)

Recruitment Detail

Type: Participants currently recruited/enrolled

Gender: Male & Female

Referral Letter Required: Yes

Population Exclusion(s): Children

Eligibility Criteria:

INCLUSION CRITERIA:

Subject must be at least 18 years of age and no older than 55 years of age.

Diagnosis of IA is a diagnosis of exclusion that is made only after other causes of anaphylaxis and other differential diagnoses have been considered and rejected.

Documented history of anaphylaxis that occurs at least 6 times per year with at least 1 event per quarter and with 1 of the following documented on at least 1 occasion (with in the last three years):

- Elevated serum tryptase above subject's baseline measurement within 2 hours of the event.

- Emergency room visit or hospitalization with documented anaphylaxis without etiology established; with involvement of the skin, mucosal tissue, or both (e.g., generalized hives, pruritus, or flushing, swollen lips-tongue-uvula) and at least one of the following:

-- Respiratory compromise (e.g., dyspnea, wheeze-bronchospasm, stridor, reduced peak expiratory flow, hypoxemia).

-- Reduced blood pressure or associated symptoms of end-organ dysfunction (e.g. hypotonia [collapse], syncope, incontinence).

Letter of referral, with copies of pertinent medical history and laboratory studies, from prospective study participant's referring physician.

Ability to give informed consent.

Willing to donate blood for sample storage to be used for future research.

EXCLUSION CRITERIA:

Presence of conditions which in the judgment of the investigator or the referring physician may put the subject at undue risk for travel (including frequent episodes of IA not preventable by pre-medication, acute infection, severe thrombocytopenia [minimum platelet count of 30,000], or significant cardiovascular disease)

Any condition that in the view of the principal investigator would make the subject unsuitable for enrollment in this study

Use of beta blockers

Inability to provide informed consent

Inability or refusal to undergo a bone marrow biopsy and aspirate

History of HIV positive or other known immunodeficiency

History or evidence of chronic Hepatitis B and/or C infection

Known cause for anaphylaxis

Special Instructions:

Currently Not Provided

Keywords:

Mast Cell

Tryptase

Bone Marrow

Hives

Adult

Recruitment Keyword(s):

Anaphylaxis

Condition(s):

Anaphylaxis

Investigational Drug(s):

None

Investigational Device(s):

None

Intervention(s):

None

Supporting Site:

National Institute of Allergy and Infectious Diseases

Contact(s):

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citation(s):

Sampson HA, Mu–oz-Furlong A, Campbell RL, Adkinson NF Jr, Bock SA, Branum A, Brown SG, Camargo CA Jr, Cydulka R, Galli SJ, Gidudu J, Gruchalla RS, Harlor AD Jr, Hepner DL, Lewis LM, Lieberman PL, Metcalfe DD, O'Connor R, Muraro A, Rudman A, Schmitt C, Scherrer D, Simons FE, Thomas S, Wood JP, Decker WW. Second symposium on the definition and management of anaphylaxis: summary report--second National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network symposium. Ann Emerg Med. 2006 Apr;47(4):373-80.

Webb LM, Lieberman P. Anaphylaxis: a review of 601 cases. Ann Allergy Asthma Immunol. 2006 Jul;97(1):39-43.

Patterson R, Hogan MB, Yarnold PR, Harris KE. Idiopathic anaphylaxis. An attempt to estimate the incidence in the United States. Arch Intern Med. 1995 Apr 24;155(8):869-71.

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