Screening for mast cell activation disorders including systemic mastocytosis using 24-hour urine specimens
2,3-Dinor-11beta-prostaglandin F2 alpha (2,3 BPG) is elevated in the urine of patients with systemic mastocytosis (SM).
This test should be used as a screening test for SM.
When 2,3 BPG is used in combination with urinary leukotriene E4 and N-methyl histamine, the sensitivity for SM detection increases to 90%.
Test Id | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
T23BP | 2,3-dinor 11B-Prostaglandin F2a | No | Yes |
CRT24 | Creatinine, 24 HR, U | No | Yes |
T23BP: Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)
CRT24: Enzymatic Colorimetric Assay
11 Beta-Prostaglandin F2 Alpha
11BPG
2,3 11 Beta-Prostaglandin F2 Alpha
23BPG
BPG2
Mastocytosis
Prostaglandin
23BPT
23
BPT23
Urine
A 24-hour urine collection is the preferred specimen type, but a random specimen is also acceptable for 2,3-dinor 11beta-prostaglandin F2 alpha; order 23BPR / 2,3-Dinor 11 Beta-Prostaglandin F2 Alpha, Random, Urine.
Question ID | Description | Answers |
---|---|---|
TM10 | Collection Duration | |
VL8 | Urine Volume |
Patient Preparation: Patients taking aspirin or nonsteroidal anti-inflammatory drugs (NSAID) may have decreased concentrations of prostaglandin F2 alpha. If possible, discontinue for 2 weeks or 72 hours, respectively, prior to collecting a specimen.
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Container/Tube: Plastic, 5-mL tube
Specimen Volume: 5 mL
Collection Instructions:
1. Collect urine for 24 hours.
2. No preservative preferred.
Additional Information: See Urine Preservatives-Collection and Transportation for 24-Hour Urine Specimens for multiple collections.
Note: The addition of preservative or application of temperature controls must occur within 4 hours of completion of the collection.
Ambient | No |
Refrigerate | Preferred |
Frozen | OK |
50% Acetic Acid | OK |
Boric Acid | OK |
Diazolidinyl Urea | No |
6M Hydrochloric Acid | No |
6M Nitric Acid | No |
Sodium Carbonate | OK |
Thymol | No |
Toluene | No |
2.7 mL
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Urine | Refrigerated (preferred) | 14 days | |
Frozen | 30 days | ||
Ambient | 8 hours |
Screening for mast cell activation disorders including systemic mastocytosis using 24-hour urine specimens
2,3-Dinor-11beta-prostaglandin F2 alpha (2,3 BPG) is the most abundant metabolic product of prostaglandins released by activated mast cells. Systemic mastocytosis (SM) is a disease in which clonally derived mast cells accumulate in peripheral tissues. Degranulation of these mast cells releases large amounts of histamines, prostaglandins, leukotrienes, and tryptase.
World Health Organization diagnostic criteria for SM require the presence of elevated mast cell counts on a bone marrow biopsy and 1 of the following minor criteria:
-Abnormal mast cell morphology
-KIT Asp816Val variant
-CD25-positive mast cells
-Serum tryptase greater than 20 ng/mL
Alternatively, SM diagnosis can be made with the presence of 3 minor criteria in the absence of abnormal bone marrow studies.
Measurement of mast cell mediators in blood or urine is less invasive and is advised for the initial evaluation of suspected cases. Elevated levels of serum tryptase, urinary N-methylhistamine, 2,3 BPG, or leukotriene E4 are consistent with the diagnosis of systemic mast cell disease.
<1802 pg/mg creatinine
Elevated urine 2,3-dinor-11beta-prostaglandin F2 alpha is consistent with systemic mastocytosis.
Elevated levels of 2,3-dinor-11beta-prostaglandin F2 alpha (2,3 BPG) in urine are not specific for systemic mast cell disease and may be found in patients with angioedema, diffuse urticaria, or myeloproliferative diseases in the absence of diffuse mast cell proliferation.
Systemic mast cell disease is a heterogeneous disease, and some patients may not have elevated 2,3 BPG in urine.
1. Gotlib J, Pardanani A, Akin C, et al: International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) and European Competence Network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis. Blood. 2013 Mar 28;121(13):2393-2401. doi: 10.1182/blood-2012-09-458521
2. Butterfield JH: Increased leukotriene E4 excretion in systemic mastocytosis. Prostaglandins Other Lipid Mediat. 2010 Jun;92(1-4):73-76. doi: 10.1016/j.prostaglandins.2010.03.003
3. Roberts LJ 2nd, Sweetman BJ, Lewis RA, Austen KF, Oates JA: Increased production of prostaglandin D2 in patients with systemic mastocytosis. N Engl J Med. 1980 Dec 11;303(24):1400-1404. doi: 10.1056/NEJM198012113032405
4. Metcalfe DD: Mastocytosis syndromes. In: Middleton E Jr, Reed CE, Ellis EF, et al. eds. Allergy Principles and Practice. Vol II. 4th ed. Mosby Yearbook Inc; 1993:1537-1551
5. Butterfield J, Weiler CR: The utility of measuring urinary metabolites of mast cell mediators in systemic mastocytosis and mast cell activation syndrome. J Allergy Clin Immunol Pract. 2020 Sept;8(8):2533-2541
2,3-Dinor-11beta-prostaglandin F2 alpha (2,3 BPG) is quantified in urine by liquid chromatography tandem mass spectrometry.(Unpublished Mayo method)
Creatinine:
All 2,3 BPG concentrations are normalized to urine creatinine levels measured using a Roche cobas enzymatic method. The enzymatic method is based on the determination of sarcosine from creatinine with the aid of creatininase, creatinase, and sarcosine oxidase. The liberated hydrogen peroxide is measured via a modified Trinder reaction using a colorimetric indicator. Optimization of the buffer system and the colorimetric indicator enables the creatinine concentration to be quantified both precisely and specifically.(Package insert: Creatinine plus ver 2. Roche Diagnostics; V15.0, 03/2019)
Tuesday, Thursday
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.
84150
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
23BPT | 2,3-dinor 11B-Prostaglandin F2a, U | 94381-1 |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
TM10 | Collection Duration | 13362-9 |
VL8 | Urine Volume | 3167-4 |
CR_A | Creatinine, 24 HR, U | 2162-6 |
CR_24 | Creatinine Concentration, 24 HR, U | 20624-3 |
603460 | 2,3-dinor 11B-Prostaglandin F2a | 94381-1 |