Peripheral Blood Involvement
This input is used for staging
Description
Peripheral blood involvement, summarized in “B category”, refers to the percentage of peripheral blood lymphocytes that are atypical (Sezary) cells and whether they are “Clone negative” or “Clone positive.” Mycosis fungoides is the most common type of primary cutaneous T-cell lymphoma. Sezary syndrome is a more aggressive type of primary cutaneous T-cell lymphoma in which a specific type of malignant T lymphocytes (Sezary cells) are present in the circulating blood. Staging of mycosis fungoides includes analysis of the circulating blood for Sezary cells. This analysis can be done by microscopy or flow cytometry. Results of microscopy are reported as counts of Sezary cells per cubic millimeter or the percentage of Sezary cells as a proportion of total lymphocytes. Flow cytometry looks for specific cell surface markers such as CD26. Information about peripheral blood involvement and T-cell clonality identified by polymerase chain reaction (PCR) or Southern blot analysis is combined in a “B” category unique to mycosis fungoides staging in the TNM system.Rationale
Peripheral blood involvement is a prognostic factor required in AJCC 8th edition, Chapter 81 *Primary Cutaneous Lymphomas*, for staging of Mycosis Fungoides and Sezary Syndrome. It was previously collected as Mycosis Fungoides, CS SSF #1.Additional Info
**Source documents:** pathology report, clinical laboratory reports of blood analysis (tissue and blood samples) **Other names include** circulating Sezary cells, T-cell clonality: T-cell receptor (TCR) gene rearrangement, Monoclonal: clone +, clone positive, Polyclonal: clone –, clone negativeNotes
**Note 1:** **Physician statement** * Physician statement of Peripheral Blood Involvement and clonality can be used to code this data item when no other information is available * If the physician does not provide a B rating but counts or percentages of neoplastic cells, flow cytometry test results, and/or clonality test results are performed, use the appropriate code for the amount of blood involvement with “clone unknown **Note 2:** **Categories for Peripheral Blood Involvement** * B0 (no significant blood involvement) (codes 0-2) * Categorized by clonality * B1 (low blood tumor burden) (code 3-5) * Categorized by clonality * B2 (high blood tumor burden) (code 6) * Any mention of B2 puts the case into Stage IVDefault
9NAACCR Item
NAACCR #3910Metadata
SSDI| Code | Description | B Map |
|---|---|---|
| 0 | Absence of significant blood involvement 5% or less of peripheral blood lymphocytes are atypical (Sezary) cells Clone unknown Stated as B0 | B0 |
| 1 | Absence of significant blood involvement 5% or less of peripheral blood lymphocytes are atypical (Sezary) cells Clone negative Stated as B0a | B0a |
| 2 | Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary) cells Clone positive Stated as B0b | B0b |
| 3 | Low blood tumor burden More than 5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 Clone unknown Stated as B1 | B1 |
| 4 | Low blood tumor burden More than 5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 Clone negative Stated as B1a | B1a |
| 5 | Low blood tumor burden More than 5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 Clone positive Stated as B1b | B1b |
| 6 | High blood tumor burden Greater than or equal to 1000 Sezary cells per microliter (uL) Clone positive Stated as B2 | B2 |
| 7 | Test ordered, results not in chart | BX |
| 9 | Not documented in medical record Peripheral Blood Involvement not assessed or unknown if assessed | BX |
| <BLANK> | Death Certificate Only, no value provided | BX |