Differential diagnosis of mature B-cell leukemias/lymphomas by clinical and laboratory parameters
| . | B-PLL . | CLL/PL . | HCL variant . | SMZL . | MCL . |
|---|---|---|---|---|---|
| Median age, y | 69 | 70 | 71 | 62 | 60 |
| Male/female ratio | 1.6:1 | 2:1 | 1.6:1 | 1:1 | 2:1 |
| Median OS, y | 3 | 3-7 | 9 | 12 | 3-5 |
| Lymphocyte count | High, > 100 × 109/L | Variable | Modest, 20-40 × 109/L. No monocytopenia | Usually normal or low level increase | Usually < 50 × 109/L |
| PB morphology | Prominent single nucleolus; round nucleus, regular cytoplasmic outline (Figure 7A) | PLs twice the size of CLL cells and account for < 55% (Figure 7B) | Prominent nucleolus, occasional bilobed nuclei, “hairy” cytoplasmic projections (Figure 7C) | Short polar villi; basophilic cytoplasm (Figure 7D) | Heterogeneous; larger size; irregular nucleus clefting or indentation common |
| Immunophenotype | |||||
| CD22 | +(strong) | + (weak) | ± | ± | ± |
| CD79b | ± | + (weak) | ± | ± | ± |
| CD23 | − | ± | − | 30% + (weak) | − |
| CD5 | − (most) | ± | − | − (usually) | + (most) |
| SmIg | Strong (IgM) | Weak (IgM/IgD) | Strong (IgG) | Strong (IgM±IgD) | Strong (IgM/IgD) |
| Other antigens | CD10− | CD10− | CD11c +, CD103 +, CD25−, CD123−, TRAP−, annexin 1 (unlike typical HCL) | CD103−, CD123-annexin 1−, cyclin D1− | Cyclin D1 + SOX 11 +, BCL2+ |
| Cytogenetics | Del 11q, del, 13q, del17p, rarely C-MYC | Del 13q, 11q, and 17p; trisomy 12 | No consistent changes | Del 7q, trisomy 3, trisomy 18 | t (11;14) |
| BM histology | Nodular/interstitial; no proliferation centers | Nodular/interstitial proliferation centers | Intrasinusoidal; no increased reticulin | Nodular/intrasinusoidal | Cells with irregular nuclei |
| Spleen histology | White and red pulp infiltration by prolymphocytes | White and red pulp involvement; some proliferation centers | Expanded diffuse red pulp with blood lakes, atrophic white pulp | Infiltration of red and white pulp with expansion of white pulp; rarer red pulp lymphoma with atrophic white pulp | Mantle zone; blastoid and small cell variants |
| . | B-PLL . | CLL/PL . | HCL variant . | SMZL . | MCL . |
|---|---|---|---|---|---|
| Median age, y | 69 | 70 | 71 | 62 | 60 |
| Male/female ratio | 1.6:1 | 2:1 | 1.6:1 | 1:1 | 2:1 |
| Median OS, y | 3 | 3-7 | 9 | 12 | 3-5 |
| Lymphocyte count | High, > 100 × 109/L | Variable | Modest, 20-40 × 109/L. No monocytopenia | Usually normal or low level increase | Usually < 50 × 109/L |
| PB morphology | Prominent single nucleolus; round nucleus, regular cytoplasmic outline (Figure 7A) | PLs twice the size of CLL cells and account for < 55% (Figure 7B) | Prominent nucleolus, occasional bilobed nuclei, “hairy” cytoplasmic projections (Figure 7C) | Short polar villi; basophilic cytoplasm (Figure 7D) | Heterogeneous; larger size; irregular nucleus clefting or indentation common |
| Immunophenotype | |||||
| CD22 | +(strong) | + (weak) | ± | ± | ± |
| CD79b | ± | + (weak) | ± | ± | ± |
| CD23 | − | ± | − | 30% + (weak) | − |
| CD5 | − (most) | ± | − | − (usually) | + (most) |
| SmIg | Strong (IgM) | Weak (IgM/IgD) | Strong (IgG) | Strong (IgM±IgD) | Strong (IgM/IgD) |
| Other antigens | CD10− | CD10− | CD11c +, CD103 +, CD25−, CD123−, TRAP−, annexin 1 (unlike typical HCL) | CD103−, CD123-annexin 1−, cyclin D1− | Cyclin D1 + SOX 11 +, BCL2+ |
| Cytogenetics | Del 11q, del, 13q, del17p, rarely C-MYC | Del 13q, 11q, and 17p; trisomy 12 | No consistent changes | Del 7q, trisomy 3, trisomy 18 | t (11;14) |
| BM histology | Nodular/interstitial; no proliferation centers | Nodular/interstitial proliferation centers | Intrasinusoidal; no increased reticulin | Nodular/intrasinusoidal | Cells with irregular nuclei |
| Spleen histology | White and red pulp infiltration by prolymphocytes | White and red pulp involvement; some proliferation centers | Expanded diffuse red pulp with blood lakes, atrophic white pulp | Infiltration of red and white pulp with expansion of white pulp; rarer red pulp lymphoma with atrophic white pulp | Mantle zone; blastoid and small cell variants |
B-PLL indicates B-cell prolymphocytic leukemia; CLL/PL, mixed cellularity chronic lymphocytic leukemia with increased prolymphocytes; HCL, hairy cell leukemia; SMZL, splenic marginal zone lymphoma (including splenic diffuse red pulp lymphoma); and MCL, mantle cell lymphoma.