Version May 2024
| Entity | Histology | Immunophenotype | Cytogenetic/Molecular features |
| T cell prolymphocytic leukemia (T-PLL) | Typically, tumor cells are "prolymphocytes" of medium size with moderately condensed chromatin and a visible nucleolus. Round or oval nucleus with moderately abundant agranular cytoplasm. Cytoplasmic protrusions (blebs) are common. Small cell and cerebriform variants can be seen. | Express CD52 (strongly) and pan-T cell markers (CD2, CD3, and CD7). TdT is negative. Expression of CD4 and CD8 is variable. Immunohistochemistry and/or flow cytometry can detect TCL1 or MTCP in most cases. | Abnormalities in 14q32 or Xq28 are characteristic. Can also see abnormalities in chromosomes 5, 8, 11, 12, 13, and 22, as well as a complex karyotype. TCR genes are clonally rearranged. |
| B cell prolymphocytic leukemia | >55 percent (and usually >90 percent) of circulating white cells are "prolymphocytes". The bone marrow is infiltrated in an interstitial or nodular pattern by similar cells. | Express bright surface IgM +/- IgD and bright CD20 as well as other B-cell antigens (CD19, CD22, CD79a, FMC7). | t(11;14) must be excluded. No associated paraproteinemia. TCR genes are not clonally rearranged. |
| Chronic lymphocytic leukemia/Small lymphocytic lymphoma (CLL/SLL) | "Typical" CLL cells are small mature appearing lymphocytes with a dense nucleus, partially aggregated chromatin, no discernible nucleoli, and a narrow border of cytoplasm. A minority of cells may have a prolymphocytic morphology. | Typically express B-cell antigens (CD19, CD22, CD79a, FMC7); CD5; and CD23. Expression of CD20 and surface immunoglobulin is dim. | Trisomy 12, deletions of 6q, 11q, 13q, and 17p TCR genes are not clonally rearranged. |
| Mycosis fungoides (MF)/Sézary syndrome | Small to medium-sized atypical mononuclear cells with cerebriform nuclei (Sézary cells). Variant of T-PLL has cerebriform morphology similar to that of MF/Sézary syndrome. | Typically lack one or more of the following: CD2, CD3, CD5, and CD7. | TCR genes are clonally rearranged. Diagnosis uses an algorithm incorporating clinical data. |
| Adult T cell lymphoma/leukemia | Tumor morphology is variable, but often demonstrates medium-sized lymphocytes with condensed chromatin and bizarre hyperlobated nuclei ("clover leaf" or "flower" cells). | Express pan-T cell antigens (CD2, CD4, CD5), typically lack CD7, and may have dim expression of CD3. Typically express CD25 at high levels. | Positive serology or PCR for the human T-lymphotropic virus, type I (HTLV-I). TCR genes are clonally rearranged. |
| T cell large granular lymphocyte leukemia | Large lymphoid cells with abundant cytoplasm containing typical azurophilic granules with a reniform or round nucleus. | Express pan-T cell antigens (CD2, CD3, CD5, CD7). Express CD8 and CD57. CD16 and CD56 expression is variable. | TCR genes are clonally rearranged. |
| Hairy cell leukemia variant | Variant has circulating tumor cells with morphology intermediate between hairy cells and prolymphocytes. | Strongly express pan-B cell antigens (CD19, CD20, CD22), CD11c, CD103, and surface immunoglobulin, and usually lack expression of CD5, CD10, CD21, and CD23. | TCR genes are not clonally rearranged. |
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