Initial clinical, morphologic, and biologic characteristics of the 37 patients included in this analysis at the time of diagnosis in comparison with SMZL (control group of 51 patients from our hospital) and published data for HCL-V36
| Clinical data . | Present series . | SMZL1 . | HCV-V . | ||
|---|---|---|---|---|---|
| Total patient . | Data missing . | Total patient . | Data missing . | Total patient . | |
| Frequency (% of chronic lymphoid malignancies) | 0.5* | 25* | 0.4* | ||
| N | 37 | — | 51 | — | 52 |
| Male/female ratio | 1.64 | 0 | 0.48 | — | 1.6 |
| Median age, y | 77 | — | 62 | — | 71 |
| Age > 60 y | 36 (97%) | — | 30 (59%) | — | ND |
| IPI | — | 16 | — | 1 | ND |
| Low risk | 4 (21%) | — | 13 (26%) | — | — |
| Low intermediate risk | 13 (62%) | — | 18 (36%) | — | — |
| High intermediate risk | 4 (21%) | — | 14 (28%) | — | — |
| High risk | 0 (0%) | — | 5 (10%) | — | — |
| Poor performance status ≥ 2 | 3 (13%) | 14 | 10 (21%) | 4 | — |
| Stage IV | 37 (100%) | 0 | 48 (96%) | 1 | 52 (100%) |
| Splenomegaly | 32 (94%) | 3 | 50 (98%) | 0 | 44 (85%) |
| Peripheral lymph node | 3 (8%) | — | 2 (4%) | 2 | 2 (4%) |
| Anemia (hemoglobin < 10 g/dL) | 3 (8%) | 0 | 15 (33%) | 6 | 15 (29%) |
| Thrombocytopenia (platelet < 100 × 109/L) | 8 (22%) | 0 | 14 (30%) | 5 | 22 (43%) |
| Lymphocytosis | 28 (76%) | 0 | 22 (48%) | 5 | ND |
| >4 × 109/L | 28 | — | 15 (33%) | — | — |
| >10 × 109/L | 18 | — | 7 (15%) | — | — |
| Neutropenia (neutrophils < 1.5 × 109/L) | 1 (3%) | 0 | 5 (11%) | 6 | No |
| Monocytopenia | 0 | 0 | 7 (15%) | 6 | No |
| LDH higher than normal values | 6 (25%) | 13 | 18 (39%) | 5 | ND |
| β2-microglobulin > 3 mg/L | 10 (55%) | 19 | 30 (77%) | 12 | ND |
| Treatment | — | 7 | — | 0 | — |
| Watchful waiting | 17 | — | 12 | — | 7 |
| Other | — | — | — | 42 | |
| Splenectomy | 8 | — | 22 | — | 19 |
| Splenic irradiation | 0 | — | 0 | — | 4 |
| Alkalating agents (chlorambucil or cyclophosphamide) | 3** | — | 5 | — | 12 |
| CHOP or high-dose CHOP (+/- rituximab) | 1** | — | 11 | — | 2 |
| Interferon | 0 | — | 0 | — | 14 |
| Pentostatin | 1 | — | 0 | — | 15 |
| Cladribine | 0 | — | 0 | — | 8 |
| Fludarabine | 0 | — | 1 | — | 3 |
| Clinical data . | Present series . | SMZL1 . | HCV-V . | ||
|---|---|---|---|---|---|
| Total patient . | Data missing . | Total patient . | Data missing . | Total patient . | |
| Frequency (% of chronic lymphoid malignancies) | 0.5* | 25* | 0.4* | ||
| N | 37 | — | 51 | — | 52 |
| Male/female ratio | 1.64 | 0 | 0.48 | — | 1.6 |
| Median age, y | 77 | — | 62 | — | 71 |
| Age > 60 y | 36 (97%) | — | 30 (59%) | — | ND |
| IPI | — | 16 | — | 1 | ND |
| Low risk | 4 (21%) | — | 13 (26%) | — | — |
| Low intermediate risk | 13 (62%) | — | 18 (36%) | — | — |
| High intermediate risk | 4 (21%) | — | 14 (28%) | — | — |
| High risk | 0 (0%) | — | 5 (10%) | — | — |
| Poor performance status ≥ 2 | 3 (13%) | 14 | 10 (21%) | 4 | — |
| Stage IV | 37 (100%) | 0 | 48 (96%) | 1 | 52 (100%) |
| Splenomegaly | 32 (94%) | 3 | 50 (98%) | 0 | 44 (85%) |
| Peripheral lymph node | 3 (8%) | — | 2 (4%) | 2 | 2 (4%) |
| Anemia (hemoglobin < 10 g/dL) | 3 (8%) | 0 | 15 (33%) | 6 | 15 (29%) |
| Thrombocytopenia (platelet < 100 × 109/L) | 8 (22%) | 0 | 14 (30%) | 5 | 22 (43%) |
| Lymphocytosis | 28 (76%) | 0 | 22 (48%) | 5 | ND |
| >4 × 109/L | 28 | — | 15 (33%) | — | — |
| >10 × 109/L | 18 | — | 7 (15%) | — | — |
| Neutropenia (neutrophils < 1.5 × 109/L) | 1 (3%) | 0 | 5 (11%) | 6 | No |
| Monocytopenia | 0 | 0 | 7 (15%) | 6 | No |
| LDH higher than normal values | 6 (25%) | 13 | 18 (39%) | 5 | ND |
| β2-microglobulin > 3 mg/L | 10 (55%) | 19 | 30 (77%) | 12 | ND |
| Treatment | — | 7 | — | 0 | — |
| Watchful waiting | 17 | — | 12 | — | 7 |
| Other | — | — | — | 42 | |
| Splenectomy | 8 | — | 22 | — | 19 |
| Splenic irradiation | 0 | — | 0 | — | 4 |
| Alkalating agents (chlorambucil or cyclophosphamide) | 3** | — | 5 | — | 12 |
| CHOP or high-dose CHOP (+/- rituximab) | 1** | — | 11 | — | 2 |
| Interferon | 0 | — | 0 | — | 14 |
| Pentostatin | 1 | — | 0 | — | 15 |
| Cladribine | 0 | — | 0 | — | 8 |
| Fludarabine | 0 | — | 1 | — | 3 |
ND indicates not done; IPI, International Prognostic Index; LDH, lactate dehydrogenase; and CHOP, cyclophosphamide, doxorubicin, vincristin and prednisone; and —, not applicable.
Frequency reported in the literature for HCL-V37 and calculated for the present series and classic SMZL from PB infiltrate examined in our laboratory between 1986 and 2007.
Treatment corresponding to cases with progression or transformation.