Introduction
Materials and methods
Patients
Investigations and treatment
Statistical analysis
Results
Patients
Clinicopathological features | Number (%) |
---|---|
Gender | |
Male | 38 (47) |
Female | 43 (53) |
Median age (range), years | 57 (15–86) |
Diagnosis | |
Non-gastric MALT lymphoma | 66 (81) |
Splenic marginal zone B cell lymphoma | 11 (14) |
Nodal marginal zone lymphoma | 4 (5) |
Ann Arbor stage | |
I | 41 (51) |
II | 13 (16) |
III | 5 (6) |
IV | 22 (27) |
Presence of B symptoms | 6 (7) |
Performance status | |
0 | 46 (57) |
1 | 21 (26) |
2 | 11 (13) |
3 | 3 (4) |
4 | 0 (0) |
International prognostic index | |
Low (0 or 1) | 35 (43) |
Low/intermediate (2) | 23 (28) |
Intermediate/high (3) | 16 (20) |
High (4 or 5) | 7 (9) |
Lactate dehydrogenase | |
Increased | 65 (80) |
Normal | 14 (17) |
Not available | 2 (3) |
Paraprotein | |
Immunoglobulin G | 4 (5) |
Immunoglobulin M | 6 (7) |
Immunoglobulin A | 2 (2) |
Two monoclonal antibodies | 3 (4) |
Not present | 60 (74) |
Not available | 6 (7) |
Hepatitis B virus infection | |
Positive | 14 (17) |
Negative | 64 (79) |
Not available | 3 (4) |
Concomitant autoimmune disorders | |
Present | 18 (22) |
Absent | 63 (79) |
Non-gastric MALT lymphoma
Clinicopathological features | Number (%) |
---|---|
Gender | |
Male | 27 (41) |
Female | 39 (59) |
Median age (range), years | 57 (15–86) |
Primary site(s) of presentation | |
Ocular and ocular adnexae | 19 (29) |
Salivary gland | 13 (20) |
Buccal mucosa | 2 (3) |
Nasopharynx | 1 (2) |
Cricoid | 1 (2) |
Tonsils | 1 (2) |
Thyroid gland | 4 (6) |
Thymus | 1 (2) |
Lungs | 6 (9) |
Intestine | 5 (8) |
Omentum | 1 (2) |
Testes | 1 (2) |
Cutaneous | 3 (5) |
Two or more anatomical sites | 8 (12) |
Ann Arbor stage | |
I | 41 (62) |
II | 12 (18) |
III | 4 (6) |
IV | 9 (14) |
International Prognostic Index | |
Low (0 or 1) | 55 (83) |
Low/intermediate (2) | 9 (14) |
Intermediate/high (3) | 2 (3) |
High (4 or 5) | 0 (0) |
Paraprotein | |
Immunoglobulin G | 3 (5) |
Immunoglobulin M | 4 (6) |
Immunoglobulin A | 2 (3) |
Two monoclonal antibodies | 3 (5) |
Not present | 49 (74) |
Not available | 5 (8) |
Associated autoimmune diseases | |
Sjögren syndrome | 9 (14) |
Parotid MALT lymphoma | 4 (6) |
Submandibular MALT lymphoma | 1 (2) |
Minor salivary gland MALT lymphoma | 2 (3) |
Thymic MALT lymphoma | 1 (2) |
Multifocal MALT lymphoma involving also the thymus | 1 (2) |
Autoimmune thyroiditis with hypothyroidism | 5 (8) |
Thyroid MALT lymphoma | 4 (6) |
Cutaneous MALT lymphoma | 1 (2) |
Mixed connective tissue disease | 1 (2) |
Submandibular MALT lymphoma | 1 (2) |
Graves’ disease | 1 (2) |
Conjunctival MALT lymphoma | 1 (2) |
Splenic MZL
Parameters | Number (%) |
---|---|
Gender | |
Male | 9 (82) |
Female | 2 (18) |
Median age (range), years | 50 (35–80) |
B symptoms | 3 (27) |
Splenomegaly | 11 (100) |
Median size (range), cm below left costal margin | 10 (5 25) |
Hepatomegaly | 2 (18) |
Hematological parameters | |
Anemia (Hb <11 g/dL) | 7 (64) |
Lymphocytosis (>4 × 109/L) | 5 (45) |
Neutropenia (<2 × 109/L) | 2 (18) |
Thrombocytopenia (<100 × 109/L) | 5 (45) |
Leukemic phase | 5 (55) |
Marrow infiltration | 11 (100) |
Paraproteinemia | |
IgG | 1 (9) |
IgM | 1 (9) |
International prognostic index | |
Low (0 or 1) | 1 (9) |
Low/intermediate (2) | 3 (27) |
Intermediate/high (3) | 5 (45) |
High (4 or 5) | 2 (18) |
Treatment and outcome | |
Splenectomy + chemotherapya
| 3 (27) |
CR | 2/3 (67) |
PR | 1/3 (33) |
Splenectomy + rituximab + chemotherapya
| 2 (18) |
CR | 2/2 (100) |
Chemotherapya
| 5 (45) |
CR | 1/5 (20) |
NR | 4/5 (80) |
Chemotherapya + rituximab | 1 (9) |
PR | 1/1 (100) |
Nodal MZL
Treatment outcome of non-gastric MZL
Treatment and outcome | Number (%) |
---|---|
Refused treatment or defaulted | 2 (3) |
Local resection only | 11 (17) |
CR | 8/11 (73) |
PR | 0/11 (0) |
NR | 3/11 (27) |
Radiotherapy alone | 3 (5) |
CR | 3/3 (100) |
PR | 0/3 (0) |
NR | 0/3 (0) |
Local resection + radiotherapy | 3 (5) |
CR | 3/3 (100) |
PR | 0/3 (0) |
NR | 0/3 (0) |
Rituximab ± local treatment | 8 (12) |
CR | 7/8 (88) |
PR | 1/8 (12) |
NR | 0/8 (0) |
Chemotherapy ± local treatment | 27 (41) |
CR | 20/27 (74) |
PR | 5/27 (19) |
NR | 2/27 (7) |
Rituximab + chemotherapy ± local treatment | 12 (18) |
CR | 10/12 (83) |
PR | 2/12 (17) |
NR | 0/12 (0) |
Splenic MZL
Nodal MZL
Relapse characteristics and transformation
Clinicopathological features and outcome | Number (%) |
---|---|
Non-gastric MALT lymphoma | 15/66 (23) |
Site of relapse | |
Same | 8 (53) |
Different | 7 (47) |
Pattern of relapse | |
Locoregional | 8 (53) |
Advanced/disseminated | 7 (47) |
Transformation | |
None | 63/66 (95) |
Diffuse large B cell lymphoma | 2/66 (3) |
High-grade follicular lymphoma | 1/66 (2) |
Treatment and outcome | |
Radiotherapy alone | 1 |
CR | 1/1 (100) |
Radiotherapy + FND | 1 |
CR | 1/1 (100) |
Excision + radiotherapy + rituximab | 1 |
CR | 1/1 (100) |
Excision + R-COPP | 1 |
CR | 1/1 (100) |
FND | 2 |
CR | 2/2 (100) |
CEOP | 1 |
CR | 1/1 (100) |
NOPP | 1 |
NR | 1/1 (100) |
CEOP followed by autologous PBSCT consolidation | 1 |
CR | 1/1 (100) |
DHAP followed by autologous PBSCT consolidation | 1 |
CR | 1/1 (100) |
Splenic marginal zone lymphoma | 3/11 (27) |
Site of relapse | |
Same | 3 (100) |
Pattern of relapse | |
Advanced/disseminated | 3 (100) |
Transformation | |
Diffuse large B cell lymphoma | 1/11 (9) |
T cell-rich B cell lymphoma | 1/11 (9) |
Treatment and outcome | |
FND | 1 |
PR | 1/1 (100) |
CEOP | 1 |
NR | 1/1 (100) |
DHAP followed by matched sibling allogeneic BMT | 1 |
CR | 1/1 (100) |
Nodal marginal zone lymphoma | 1/4 (25) |
Site of relapse | |
Same | 1 (100) |
Pattern of relapse | |
Advanced/disseminated | 1 (100) |
Transformation | 1/4 (25) |
Diffuse large B cell lymphoma | 1/4 (25) |
Treatment and outcome | |
R-CEOP followed by 90Y-irbitumomab tiuxetan consolidation | 1 |
CR | 1/1 (100) |
Toxicity of treatment
Toxicity | Number (%) |
---|---|
Anemia | |
Grade 1 | 10 (12) |
2 | 6 (7) |
3 | 3 (4) |
4 | 2 (3) |
Neutropenia | |
Grade 1 | 5 (6) |
2 | 4 (5) |
3 | 10 (12) |
4 | 14 (17) |
Thrombocytopenia | |
Grade 1 | 1 (1) |
2 | 3 (4) |
3 | 4 (5) |
4 | 8 (10) |
Intracranial hemorrhage | 1 (1) |
Febrile neutropenia | 13 (16) |
Bacteraemia | 9 (11) |
Invasive fungal infections | 1 (1) |
Reactivation of tuberculosis | 1 (1) |
Localized herpes zoster | 1 (1) |
Disseminated herpes zoster | 1 (1) |
Reactivation of hepatitis B virus infection | 1 (1) |
Therapy-related acute myeloid leukemia | 1 (1) |
Miscellaneousa
| 4 (5) |
Therapy-related mortality | 2 (3) |
Prognostic factors
Treatment | Number | OR | CR | PR | SD | PD | Relapse |
---|---|---|---|---|---|---|---|
Local treatment alone | 17 | 14/17 (82%) | 14/17 (82%) | 0/17 (0%) | 2/17 (12%) | 1/17 (6%) | 5/14 (36%) |
Systemic chemotherapy | |||||||
Fludarabine containing | 28 | 25/28 (89%) | 23/28 (82%) | 2/28 (7%) | 1/28 (4%) | 2/28 (7%) | 3/23 (13%) |
Non-fludarabine containing | 34 | 28/34 (82%) | 22/34 (65%) | 6/34 (18%) | 3/34 (9%) | 3/34 (9%) | 9/22 (41%) |
Rituximab | |||||||
Rituximab ± chemotherapy | 25 | 23/25 (92%) | 21/25 (84%) | 2/25 (8%) | 1/25 (4%) | 1/25 (4%) | 2/21 (10%) |
Non-rituximab-containing chemotherapy | 37 | 30/37 (81%) | 24/37 (65%) | 6/37 (16%) | 3/37 (8%) | 4/37 (11%) | 10/24 (42%) |
Fludarabine and rituximab | |||||||
Fludarabine + rituximab containing | 11 | 10/11 (91%) | 10/11 (91%) | 0/11 (0%) | 0/11 (0%) | 1/11 (9%) | 0/10 (0%) |
Fludarabine, non-rituximab containing | 17 | 15/17 (88%) | 13/17 (76%) | 2/17 (12%) | 1/17 (6%) | 1/17 (6%) | 3/13 (23%) |
Rituximab, non-fludarabine containing | 14 | 13/14 (93%) | 11/14 (79%) | 2/14 (14%) | 1/14 (7%) | 0/14 (0%) | 2/11 (18%) |
Non-fludarabine, non-rituximab | 20 | 15/20 (75%) | 11/20 (55%) | 4/20 (20%) | 2/20 (10%) | 3/20 (15%) | 7/11 (64%) |
Pretreatment characteristics | Number | OR | CR | PR | SD | PD | Relapse |
---|---|---|---|---|---|---|---|
Disease stage | |||||||
Stage I/II disease | 52 | 50/52 (96%) | 44/52 (85%) | 6/52 (12%) | 1/52 (2%) | 1/52 (2%) | 13/44 (30%) |
Stage III/IV disease | 27 | 19/27 (70%) | 14/27 (52%) | 5/27 (19%) | 4/27 (15%) | 4/27 (15%) | 6/14 (43%) |
Performance status | |||||||
≤1 | 65 | 60/65 (92%) | 53/65 (82%) | 7/65 (11%) | 2/65 (3%) | 3/65 (5%) | 13/53 (25%) |
>1 | 14 | 9/14 (64%) | 5/14 (36%) | 4/14 (29%) | 3/14 (21%) | 0/14 (0%) | 1/5 (20%) |
IPI score | |||||||
<3 | 56 | 53/56 (95%) | 46/56 (82%) | 7/56 (13%) | 2/56 (4%) | 1/56 (2%) | 15/46 (33%) |
≥3 | 23 | 16/23 (70%) | 12/23 (52%) | 4/23 (17%) | 3/23 (13%) | 4/23 (2%) | 4/12 (33%) |