Table 1.

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.

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