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Test ID: PLAFL Platelet Surface Glycoprotein by Flow Cytometry, Blood

Useful For

Identification of markedly decreased CD41 (GPIIb) and CD61 (GPIIIa) expression levels, which are diagnostic for Glanzmann thrombasthenia

 

Identification of markedly decreased CD42a (GPIX) and CD42b (GPIb-alpha) expression levels, which are diagnostic for Bernard-Soulier syndrome

 

Identification of decreased GPVI expression, which suggests collagen receptor deficiency

 

Identification of decreased CD49b (GPIa), which suggests collagen receptor deficiency

Method Name

Immunophenotyping

Reporting Name

Platelet Glycoprotein Flow, B

Specimen Type

Whole Blood ACD


Shipping Instructions


Specimen must be shipped ambient and arrive within 96 hours of draw.

 

Ship specimen overnight in an Ambient Shipping Box-Critical Specimens Only (T668) following the instructions in the mailer.



Specimen Required


Supplies: Ambient Shipping Box-Critical Specimens Only (T668)

Collection Container/Tube: ACD solution (A or B)

Specimen Volume: 6 mL

Pediatric Volume: 1 mL

Collection Instructions: Do not transfer blood to other containers.


Specimen Minimum Volume

Adult: 1 mL
Pediatric 200 mcL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Whole Blood ACD Ambient 4 days

Clinical Information

Platelets have essential roles in primary hemostasis. Exposed collagen at a vascular damage site can activate platelets via collagen receptor GPVI and GPIa and bind shear-stretched multimeric VWF proteins, which subsequently interact with the platelet surface receptor, GPIb-V-IX. Upon full activation, platelets can aggregate by binding to fibrinogen through activated GPIIb-GPIIIa receptors. Deficiency of platelet surface glycoproteins can cause bleeding diathesis.

 

Platelet flow cytometric analysis is the preferred method to assess hereditary platelet disorders due to quantitative surface glycoprotein (GP) deficiencies. GP expression levels can be measured by using fluorescent-conjugated GP-specific antibodies and their fluorescent intensities can be compared to normal ranges of various glycoproteins.

 

CD Number

Glycoprotein Name

Integrin Name

CD41

GPIIb

Alpha 2b

CD42a

GPIX

NA

CD42b

GPIb-alpha

NA

CD49b

GPIa

Alpha 2

CD61

GPIIIa

Beta 3

NA

GPVI

NA

Reference Values

GPIIb CD41: ≥70.0% (Normal Range-Median)

GPIIIa CD61: ≥70.0% (Normal Range-Median)

GPIX CD42a: ≥70.0% (Normal Range-Median)

GPIb-alpha CD42b: ≥70.0% (Normal Range-Median)

GPIa CD49b: ≥60.0% (Normal Range-Median)

Interpretation

CD Markers

% Reference Range Median

Comments

CD41 and CD61

50%-69%

(Marginally)

Marginally decreased platelet surface receptors CD41 (GPIIb) and CD61 (GPIIIa) are of uncertain clinical significance. This finding could be a laboratory artifact due to suboptimal sample condition, benign polymorphisms, or a heterozygous state of Glanzmann thrombasthenia. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

30%-50%: (Moderately)

 

<30%: (Markedly)

Platelet surface expression of CD41 (GPIIb) and CD61 (GPIIIa) are moderately or markedly decreased. This finding is suggestive for a variant of Glanzmann thrombasthenia. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

CD42a and CD42b

 

 

50%-69%

(Marginally)

Marginally decreased platelet surface receptors CD42a (GPIX) and CD42b (GPIb-alpha) are of uncertain clinical significance. This finding could be a laboratory artifact due to suboptimal sample condition, benign polymorphisms, or a heterozygous state of Bernard-Soulier syndrome. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

30%-50%: (Moderately)

 

<30%: (Markedly)

Platelet surface expression of CD42a (GPIX) and CD42b (GPIb-alpha) are moderately or markedly decreased. This finding is suggestive for a variant of Bernard-Soulier syndrome. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

CD49b

30%-59%

(Marginally)

Marginally decreased platelet surface receptor CD49b (GPIa) is of uncertain clinical significance. This finding could be a laboratory artifact due to suboptimal sample condition, a benign polymorphism, or a variant of platelet collagen receptor glycoprotein Ia/IIa deficiency. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

10%-30% (moderately)

 

<10%

(Markedly)

Platelet surface expression of CD49b (GPIa) is moderately or markedly decreased. This finding is suggestive for a variant of a variant of platelet collagen receptor glycoprotein Ia/IIa deficiency. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

GPVI

50%-69%

(Marginally)

Marginally decreased platelet surface receptor glycoprotein VI (GPVI) is of uncertain clinical significance. This finding could be a laboratory artifact due to suboptimal sample condition, a benign polymorphism or a variant of platelet collagen receptor GPVI deficiency. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

30%-50% (moderately)

 

<30%

(Markedly)

Platelet surface expression of glycoprotein VI (GPVI) is moderately or markedly decreased. This finding is suggestive for a variant of a variant of platelet collagen receptor GPVI deficiency. Recommend correlation with patient's clinical findings and results of platelet functional studies, and consider repeating platelet glycoprotein profile studies by flow cytometry to verify the present finding if clinically indicated.

Clinical Reference

1. Miller, JL: Glycoprotein analysis for the diagnostic evaluation of platelet disorders. Semin Thromb Hemost. 2009;35(2):224-232

2. Kannan M, Ahmad F, Yadav BK, et al: Carrier detection in Glanzmann thrombasthenia: comparison of flow cytometry and Western blot with respect to DNA mutation. Am J Clin Pathol. 2008;130(1):93-98

3. Savoia A, Pastore A, De Rocco D, et al: Clinical and genetic aspects of Bernard-Soulier syndrome: searching for genotype/phenotype correlations. Haematologica. 2011;96(3):417-423

4. Nurden AT, Freson K, Selifsohn U: Inherited platelet disorders. Haemophilia. 2012;18(4):154-160

5. Dumont B, Lasne D, Rothschild C, et al: Absence of collagen-induced platelet activation caused by compound heterozygous GPVI mutations. Blood. 2009;114(9):1900-1903

Day(s) and Time(s) Performed

Specimens are processed Monday through Sunday.

Results reported Monday through Friday.

Analytic Time

1 day

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

CPT Code Information

88184-Flow cytometry; first cell surface, cytoplasmic or nuclear marker

88185-Flow cytometry; additional cell surface, cytoplasmic or nuclear marker (each) X5

88187-Flow cytometry interpretation, 2 to 8 markers

LOINC Code Information

Test ID Test Order Name Order LOINC Value
PLAFL Platelet Glycoprotein Flow, B 93320-0

 

Result ID Test Result Name Result LOINC Value
CK111 GPIIb CD41 93319-2
CK112 GPIIIa CD61 93318-4
CK113 GPIX CD42a 93317-6
CK114 GPIb-alpha CD42b 93316-8
CK115 GPIa CD49b 93315-0
CK116 GPVI 93314-3
CK117 Final Diagnosis 93313-5

Forms

1. Platelet Esoteric Testing Patient Information in Special Instructions

2. If not ordering electronically, complete, print, and send a Coagulation Test Request (T753) with the specimen.

Mayo Clinic Laboratories | Hematology Catalog Additional Information:

mml-coagulation-disorders