Table 1

Clinical features of 171 SMZL patients with available survival data

Featuren (%)
Median age, y (range) 67 (32-88) 
Sex, male/female 81/90 
ECOG ≥ 2 5 (7.1) 
Splenomegaly 112/138 (81.2) 
Bone marrow involvement 76/81 (93.8) 
Lymphocytosis ≥ 5 × 109/L 62/137 (45.3) 
Villous lymphocytes 34/52 (65.4) 
LDH ≥ 450 mg/L 28/91 (30.8) 
β2-Microglobulin ≥ 3 mg/L 48/81 (59.3) 
Albumin ≤ 35 g/L 17/74 (23) 
Hb < 12 g/dL 75/143 (52.4) 
Platelet count ≤ 100 × 109/L 31/143 (21.7) 
M component 16/41 (39) 
HCV infection 4/48 (8.3) 
Autoimmune phenomena 5/36 (13.8) 
CD5 expression* 26/115 (22.6) 
IG gene rearrangements  
    Unmutated 41/100 (41) 
    Mutated 59/100 (59) 
    IGHV1–2 32/100 (32) 
Follow-up data  
    Deaths 69/171 (40.3) 
    Median overall survival, y (range) 10.6 (0.4-18.8) 
    Median follow-up, y (range) 5 (2 mo-25.3) 
IIL score  
    Low risk 23/65 (35.4) 
    Intermediate risk 20/65 (30.8) 
    High risk 22/65 (33.8) 
Treatment  
    No therapy 28/159 (17.6) 
    Splenectomy 85/159 (53.5) 
    Chemotherapy 23/159 (14.5) 
    Splenectomy + chemotherapy 23/159 (14.5) 
    CR after therapy 22/134 (16.4) 
    PR after therapy 35/134 (26.1) 
    Stable disease 11/134 (8.2) 
    Progression after initial therapy 60/134 (44.8) 
    Nonresponse 6/134 (4.5) 
Featuren (%)
Median age, y (range) 67 (32-88) 
Sex, male/female 81/90 
ECOG ≥ 2 5 (7.1) 
Splenomegaly 112/138 (81.2) 
Bone marrow involvement 76/81 (93.8) 
Lymphocytosis ≥ 5 × 109/L 62/137 (45.3) 
Villous lymphocytes 34/52 (65.4) 
LDH ≥ 450 mg/L 28/91 (30.8) 
β2-Microglobulin ≥ 3 mg/L 48/81 (59.3) 
Albumin ≤ 35 g/L 17/74 (23) 
Hb < 12 g/dL 75/143 (52.4) 
Platelet count ≤ 100 × 109/L 31/143 (21.7) 
M component 16/41 (39) 
HCV infection 4/48 (8.3) 
Autoimmune phenomena 5/36 (13.8) 
CD5 expression* 26/115 (22.6) 
IG gene rearrangements  
    Unmutated 41/100 (41) 
    Mutated 59/100 (59) 
    IGHV1–2 32/100 (32) 
Follow-up data  
    Deaths 69/171 (40.3) 
    Median overall survival, y (range) 10.6 (0.4-18.8) 
    Median follow-up, y (range) 5 (2 mo-25.3) 
IIL score  
    Low risk 23/65 (35.4) 
    Intermediate risk 20/65 (30.8) 
    High risk 22/65 (33.8) 
Treatment  
    No therapy 28/159 (17.6) 
    Splenectomy 85/159 (53.5) 
    Chemotherapy 23/159 (14.5) 
    Splenectomy + chemotherapy 23/159 (14.5) 
    CR after therapy 22/134 (16.4) 
    PR after therapy 35/134 (26.1) 
    Stable disease 11/134 (8.2) 
    Progression after initial therapy 60/134 (44.8) 
    Nonresponse 6/134 (4.5) 

ECOG indicates Eastern Cooperative Oncology Group; HCV, hepatitis C virus; and IIL, Intergruppo Itallano Linfomi.

*

CD5 expression in PB lymphocytes by flow cytometry.

After the 98% cut-off value for identity to germline.

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