Clinical and laboratory characteristics of T- and B-cell PLLs
| Characteristic findings . | T-PLL . | B-PLL . |
|---|---|---|
| Clinical features | Median age 61 y | Median age 69 y |
| Male:female 2:1 | Male:female, 1.6:1 | |
| Splenomegaly, lymphadenopathy, skin rash, edema and pleuroperitoneal effusions | B-symptoms, splenomegaly, minimal lymphadenopathy, high WBC | |
| Very high WBC | ||
| Morphology | Basophilic prolymphocytes with cytoplasmic blebs | > 55% prolymphocytes (usually > 90%) |
| Small cell (20%) and SS (5%) variants | Prolymphocyte is 2 times the size of CLL lymphocyte | |
| Immunophenotype | CD2+, CD3+, CD5+, | SmIG strong, CD19+, |
| CD7++ | CD20+, CD22+, | |
| CD4/CD8 variable | CD79a+,CD23− CD5± | |
| CD1a−, TdT−, CD25± | FMC7+ (CLL score 0-1) | |
| Cytogenetics | t(14;14); inversion 14; t(X;14); iso8q; complex | 13q del, 11q del, 17p del, 6qdel |
| No t(11;14) | ||
| Oncogenes | TCL-1, MTCP-1, ATM | TP53, C-MYC |
| Differential diagnosis | B-PLL, T-LGL leukemia, ATLL, SS | CLL/PL,* T-PLL, MCL (leukemic phase), SMZL, HCL-V |
| Prognosis | Median survival 7 mo with conventional therapy; 20 mo with alemtuzumab; 48 mo with alemtuzumab + HSCT | Median survival 3 y |
| Characteristic findings . | T-PLL . | B-PLL . |
|---|---|---|
| Clinical features | Median age 61 y | Median age 69 y |
| Male:female 2:1 | Male:female, 1.6:1 | |
| Splenomegaly, lymphadenopathy, skin rash, edema and pleuroperitoneal effusions | B-symptoms, splenomegaly, minimal lymphadenopathy, high WBC | |
| Very high WBC | ||
| Morphology | Basophilic prolymphocytes with cytoplasmic blebs | > 55% prolymphocytes (usually > 90%) |
| Small cell (20%) and SS (5%) variants | Prolymphocyte is 2 times the size of CLL lymphocyte | |
| Immunophenotype | CD2+, CD3+, CD5+, | SmIG strong, CD19+, |
| CD7++ | CD20+, CD22+, | |
| CD4/CD8 variable | CD79a+,CD23− CD5± | |
| CD1a−, TdT−, CD25± | FMC7+ (CLL score 0-1) | |
| Cytogenetics | t(14;14); inversion 14; t(X;14); iso8q; complex | 13q del, 11q del, 17p del, 6qdel |
| No t(11;14) | ||
| Oncogenes | TCL-1, MTCP-1, ATM | TP53, C-MYC |
| Differential diagnosis | B-PLL, T-LGL leukemia, ATLL, SS | CLL/PL,* T-PLL, MCL (leukemic phase), SMZL, HCL-V |
| Prognosis | Median survival 7 mo with conventional therapy; 20 mo with alemtuzumab; 48 mo with alemtuzumab + HSCT | Median survival 3 y |
T-PLL indicates T-cell prolymphocytic leukemia; T-LGL, T large granular lymphocytic leukemia; SS, Sezary syndrome; ATLL, adult T cell leukemia lymphoma; B-PLL, B-cell prolymphocytic leukemia; CLL/PL, chronic lymphocytic leukemia with increased prolymphocytes (< 55%); HCL-V, hairy cell leukemia variant; MCL, mantle cell lymphoma; SMZL, splenic marginal zone lymphoma; WBC, white blood cell count; and HSCT, hematopoietic stem cell transplant.
CLL/PL < 55% prolymphocytes.