Clinical characteristics at the initial MZL diagnosis, relapse, and transformation
Median age at MZL diagnosis (range) | 65 (25-75) |
Male sex, n (%) | 2 (20) |
Ethnicity, n (%) | |
Hispanic White | 1 (10) |
Non-Hispanic White | 7 (70) |
Non-Hispanic Black | 2 (20) |
Ann Arbor stage MZL, n (%) | |
I-II | 2 (20) |
III-IV | 8 (80) |
Type of MZL at presentation, n (%) | |
EMZL | 3 (30) |
SMZL | 6 (60) |
NMZL | 1 (10) |
Location of EMZL of subtype, n (%) | |
Eye | 2 (66%) |
Lung | 1 (33%) |
MZL-directed therapies received before HT, n (%) | |
Splenectomy | 3 (30) |
Rituximab | 3 (30) |
Rituximab + splenectomy | 1 (10) |
XRT | 2 (20) |
XRT + tositumomab | 1 (10) |
Response to initial MZL treatment, n (%) | |
CR | 6 (60) |
PR | 2 (20) |
SD | 1 (10) |
PD | 1 (10) |
Type of MZL in patients with CR, n (%) | |
EMZL | 1 (16.7) |
SMZL | 4 (66.6) |
NMZL | 1 (16.7) |
Relapse of MZL, n (%) | 6 (60) |
Stage at initial MZL diagnoses of relapsed patients, n (%) | |
IE | 1 (16.7) |
IV | 5 (83.3) |
Treatment of relapsed patients, n (%) | |
No therapy | 2 (33.3) |
Rituximab | 2 (33.3) |
R-CVP | 1 (16.7) |
XRT + interferon | 1 (16.7) |
Response to treatment of relapsed patients, n (%) | |
CR | 2 (33.3) |
PR | 1 (16.7) |
Unknown | 3 (50) |
Median time to transformation, mo (range) | 93 (8-146) |
Median time to transformation by MZL subtype, mo (range) | |
EMZL | 92 (8-122) |
SMZL | 94.5 (8-146) |
NMZL | 32 (n = 1) |
Median age at time of transformation, y (range) | 70 (28-80) |
Pathology at transformation, n (%) | |
Classical HL | 10 (100%) |
Ann arbor stage HL, n (%) | |
I-II | 2 (20%) |
III-IV | 8 (80%) |
Treatment of HL, n (%) | |
ABVD or BV-AVD | 5 (50) |
RICE + BEAM and auto-HCT | 1 (10) |
R-AVD + Pembro-GVD | 1 (10) |
BV-CHOP | 2 (20) |
Rituximab | 1 (10) |
Lost to follow-up, n (%) | 2 (20) |
CR after treatment, n (%) | 3 (37.5) |
Deaths, n (%)∗ | 3 (37.5) |
Median age at MZL diagnosis (range) | 65 (25-75) |
Male sex, n (%) | 2 (20) |
Ethnicity, n (%) | |
Hispanic White | 1 (10) |
Non-Hispanic White | 7 (70) |
Non-Hispanic Black | 2 (20) |
Ann Arbor stage MZL, n (%) | |
I-II | 2 (20) |
III-IV | 8 (80) |
Type of MZL at presentation, n (%) | |
EMZL | 3 (30) |
SMZL | 6 (60) |
NMZL | 1 (10) |
Location of EMZL of subtype, n (%) | |
Eye | 2 (66%) |
Lung | 1 (33%) |
MZL-directed therapies received before HT, n (%) | |
Splenectomy | 3 (30) |
Rituximab | 3 (30) |
Rituximab + splenectomy | 1 (10) |
XRT | 2 (20) |
XRT + tositumomab | 1 (10) |
Response to initial MZL treatment, n (%) | |
CR | 6 (60) |
PR | 2 (20) |
SD | 1 (10) |
PD | 1 (10) |
Type of MZL in patients with CR, n (%) | |
EMZL | 1 (16.7) |
SMZL | 4 (66.6) |
NMZL | 1 (16.7) |
Relapse of MZL, n (%) | 6 (60) |
Stage at initial MZL diagnoses of relapsed patients, n (%) | |
IE | 1 (16.7) |
IV | 5 (83.3) |
Treatment of relapsed patients, n (%) | |
No therapy | 2 (33.3) |
Rituximab | 2 (33.3) |
R-CVP | 1 (16.7) |
XRT + interferon | 1 (16.7) |
Response to treatment of relapsed patients, n (%) | |
CR | 2 (33.3) |
PR | 1 (16.7) |
Unknown | 3 (50) |
Median time to transformation, mo (range) | 93 (8-146) |
Median time to transformation by MZL subtype, mo (range) | |
EMZL | 92 (8-122) |
SMZL | 94.5 (8-146) |
NMZL | 32 (n = 1) |
Median age at time of transformation, y (range) | 70 (28-80) |
Pathology at transformation, n (%) | |
Classical HL | 10 (100%) |
Ann arbor stage HL, n (%) | |
I-II | 2 (20%) |
III-IV | 8 (80%) |
Treatment of HL, n (%) | |
ABVD or BV-AVD | 5 (50) |
RICE + BEAM and auto-HCT | 1 (10) |
R-AVD + Pembro-GVD | 1 (10) |
BV-CHOP | 2 (20) |
Rituximab | 1 (10) |
Lost to follow-up, n (%) | 2 (20) |
CR after treatment, n (%) | 3 (37.5) |
Deaths, n (%)∗ | 3 (37.5) |
ABVD, doxorubicin, bleomycin, vinblastine, dacarbazine; Auto-HCT, autologous hematopoietic cell transplantation; BEAM, carmustine, etoposide, cytarabine, melphalan; BV-AVD, brentuximab vedotin, doxorubicin, vinblastine, dacarbazine; BV-CHOP, brentuximab vedotin, cyclophosphamide, doxorubicin, prednisolone; CR, complete response; IE, stage I extranodal; Pembro-GVD, pembrolizumab + gemcitabine, vinorelbine, and doxorubicin; PD, progressive disease; PR, partial response; R-AVD, rituximab, doxorubicin, vinblastine, dacarbazine; R-CVP, rituximab, cyclophosphamide, vincristine, prednisone; RICE, rituximab, ifosfamide, carboplatin, etoposide; SD, stable disease; XRT, radiation.
All deaths were due to lymphoma.