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Test ID: SZMON Sezary Monitoring Flow Cytometry, Blood

Useful For

Monitoring response to therapy in patients with previously diagnosed Sezary syndrome or mycosis fungoides

Additional Tests

Test ID Reporting Name Available Separately Always Performed
FIRST Flow Cytometry, Cell Surface, First No Yes
ADD1 Flow Cytometry, Cell Surface, Addl No Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
FCIMS Flow Cytometry Interp, 9-15 Markers No No
FCINS Flow Cytometry Interp,16 or greater No No

Testing Algorithm

This Sezary panel is ordered for patients with previously diagnosed Sezary syndrome or cutaneous T-cell lymphoma (CTCL) with peripheral blood involvement.

 

For patients with a clinical suspicion of Sezary syndrome, but no previously confirmed diagnosis or immunophenotyping performed in our laboratory, order SZDIA / Sezary Diagnostic Flow Cytometry, Blood. A triage panel will also be performed to evaluate for and exclude monotypic B cells or increased blasts.

 

The panel is charged based on number of markers tested (FIRST for first marker, ADD1 for each additional marker).

Method Name

Immunophenotyping 

Reporting Name

Sezary Monitoring Flow Cytometry, B

Specimen Type

Whole blood


Ordering Guidance


This test is for monitoring response to therapy in patients who have been diagnosed with Sezary syndrome or mycosis fungoides. For patients with a clinical suspicion, but no diagnosis, of Sezary syndrome, order SZDIA / Sezary Diagnostic Flow Cytometry, Blood.



Specimen Required


Container/Tube:

Preferred: Yellow top (ACD solution A or B)

Acceptable: Lavender top (EDTA), green top (sodium heparin)

Specimen Volume: 6 mL

Collection Instructions:

1. Send whole blood specimen in original tube. Do not aliquot.

2. Label specimen as blood.


Specimen Minimum Volume

1 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Whole blood Ambient (preferred) 4 days
  Refrigerated  4 days

Clinical Information

Sezary syndrome (SS) and mycosis fungoides (MF) are two distinct but intimately related T-cell lymphoproliferative disorders involving the skin and are commonly referred to as cutaneous T-cell lymphomas (CTCLs). SS is defined by the triad of erythroderma, generalized lymphadenopathy, and the presence of circulating cells with irregular nuclear features (Sezary cells). MF typically presents with slowly progressing patch and plaque lesions. Detection of neoplastic CD4-positive T-cells in peripheral blood (>1000 cells/microliter) is essential to establish a diagnosis of SS. Disease staging and assessment of therapy response in CTCL require a quantitative assessment of peripheral blood involvement in absolute number of neoplastic cells (Sezary cells) per microliter. Flow cytometry is now considered the method of choice to estimate the number of Sezary cells in peripheral blood, largely replacing the less reproducible and time-consuming morphologic quantitation of atypical lymphocytes on a peripheral blood smear, proposed by the International Society for Cutaneous Lymphomas, and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer. Typically, Sezary cells are immunophenotypically distinct, and they are clonal.

Reference Values

An interpretive report will be provided. This test will be processed as a laboratory consultation. An interpretation of the immunophenotypic findings and, if available, morphologic features will be provided by a board-certified hematopathologist for every case.

Interpretation

An immunophenotypically distinct T-cell population is suggestive of clonality when the subset exhibits a restricted T-cell receptor beta-chain (TRBC) staining pattern defined as either 1) >85% of TRBC1-positive events, 2) <15% TRBC1-positive events, or 3) homogenous TRBC1-dim expression. The immunophenotype of the distinct T-cell population, its percentage of total lymphocytes, and its percentage of total analyzed events will be reported. The test will be resulted as "No phenotypically aberrant T-cell population detected" if there is no specific immunophenotype that allows the detection of TRBC-restricted T cells.

Clinical Reference

1. Horna P, Deaver DM, Qin D, et al. Quantitative flow cytometric identification of aberrant T cell clusters in erythrodermic cutaneous T cell lymphoma. Implications for staging and prognosis. J Clin Pathol. 2014;67(5):431-436

2. Berg H, Otteson GE, Corley H, et al. Flow cytometric evaluation of TRBC1 expression in tissue specimens and body fluids is a novel and specific method for assessment of T-cell clonality and diagnosis of T-cell neoplasms. Cytometry B Clin Cytom. 2021;100(3):361-369

3. Horna P, Shi M, Olteanu H, Johansson U. Emerging role of T-cell receptor constant beta chain-1 (TRBC1) expression in the flow cytometric diagnosis of T-cell malignancies. Int J Mol Sci. 2021;22(4):1817

4. Wilcox RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol. 2016;91(1):152-165. doi:10.1002/ajh.24233

5. Horna P, Olteanu H, Jevremovic D, et al. Single-antibody evaluation of T-cell receptor beta constant chain monotypia by flow cytometry facilitates the diagnosis of T-cell large granular lymphocytic leukemia. Am J Clin Pathol. 2021;156(1):139-148

6. Horna P, Shi M, Jevremovic D, Craig FE, Comfere NI, Olteanu H. Utility of TRBC1 expression in the diagnosis of peripheral blood involvement by cutaneous T-cell lymphoma. J Invest Dermatol. 2021;141(4):821-829

7. Scarisbrick JJ, Hodak E, Bagot M, et al. Blood classification and blood response criteria in mycosis fungoides and Sezary syndrome using flow cytometry: recommendations from the EORTC cutaneous lymphoma task force. Eur J Cancer. 2018;93:47-56

8. Illingworth A, Johansson U, Huang S, et al. International guidelines for the flow cytometric evaluation of peripheral blood for suspected Sezary syndrome or mycosis fungoides: Assay development/optimization, validation, and ongoing quality monitors. Cytometry B Clin Cytom. 2021;100(2):156-182

Day(s) Performed

Monday through Saturday

Report Available

1 to 3 days

Test Classification

This test was developed using an analyte specific reagent. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

88184-Flow cytometry; first cell surface, cytoplasmic or nuclear marker x 1

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

88188-Flow Cytometry Interpretation, 9 to15 markers (if appropriate)

88189-Flow Cytometry Interpretation, 16 or more markers (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
SZMON Sezary Monitoring Flow Cytometry, B 101117-0

 

Result ID Test Result Name Result LOINC Value
CK130 Sezary Monitoring No LOINC Needed
CK131 Final Diagnosis 50398-7
CK132 Special Studies 30954-2
CK133 Microscopic Description 22635-7

Forms

If not ordering electronically, complete, print, and send a Hematopathology/Cytogenetics Test Request (T726) with the specimen.

Mayo Clinic Laboratories | Hematology Catalog Additional Information:

mml-lymphoma