Screening for mast cell activation disorders including systemic mastocytosis using random 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, or 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.
Specimen Type
Urine
Container
Urine Cup
Collection Instructions
Container/Tube: Urine Container Specimen: 5 mL Random Urine (4 mL min) Transport Temperature: Refrigerated Stability: 8 Hours Room Temperature 14 Days Refrigerated 30 Days Frozen
Transport Instructions
Refrigerated
Specimen Stability
8 Hours Room Temperature 14 Days Refrigerated 30 Days Frozen
Methodology
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS) Creatinine: Enzymatic Colorimetric Assay
