Clinical characteristics of ATLL patients with HTLV-1–infected HRS-like cells
| Case . | Age, y/sex . | Affected organs . | Ann Arbor stage . | Background status . | sIL2R. U/mL . | LDH, IU/L . | Clinical course . | Status at the last follow-up . | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1* | 69/M | Generalized lymphadenopathy, splenomegaly, bone marrow | IV | Smoldering type ATLL (10 y)† | 6502 | 275 | Treated with ABVD chemotherapy; in partial remission for 6 months and relapsed in bone marrow; treated with brentuximab vedotin (anti-CD30 antibody) | Alive with disease (18 mo) | ||||
| 2 | 55/M | Axillary lymph node‡ | I‡ | HTLV-1 carrier | 6024 | 296 | Treated with VCAP-AMP-VECP | Alive with disease (4 mo) | ||||
| 3 | 79/F | Mediastinal mass, bone marrow | IV | HTLV-1 carrier | 3205 | 322 | Treated with 1 cycle of CHOP followed by ABVD chemotherapy | Alive in CR (7 mo) | ||||
| 4 | 55/F | Generalized lymphadenopathy, hepatosplenomegaly, bone marrow | IV | Chronic-type ATLL (7 mo)† | 65713 | 320 | Treated with VCAP-AMP-VECP and mogamulizumab (anti-CCR4 antibody), but disease status was progressive | Died of disease (3 mo) | ||||
| 5 | 80/F | Cervical and supraclavicular lymphadenopathy | II | HTLV-1 carrier | 635 | 307 | Treated with CHOP chemotherapy followed by oral etoposide therapy; stable disease for 16 mo followed by recurrence | Died of disease (18 mo) | ||||
| 6 | 74/M | Generalized lymphadenopathy§ | IIIB§ | HTLV-1 carrier | 5660 | 188 | Treated with palliative care; no chemotherapy because of accompanying liver cirrhosis | Died of disease (2 mo) | ||||
| 7 | 72/F | Inguinal lymphadenopathy | I | HTLV-1 carrier | 2493 | 212 | Treated with CHOP | Alive in CR (28 mo) | ||||
| 8 | 81/M | Generalized lymphadenopathy§ | IIIA§ | HTLV-1 carrier | 5732 | 486 | Treated with CHOP | Alive in CR (28 mo) | ||||
| Case . | Age, y/sex . | Affected organs . | Ann Arbor stage . | Background status . | sIL2R. U/mL . | LDH, IU/L . | Clinical course . | Status at the last follow-up . | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1* | 69/M | Generalized lymphadenopathy, splenomegaly, bone marrow | IV | Smoldering type ATLL (10 y)† | 6502 | 275 | Treated with ABVD chemotherapy; in partial remission for 6 months and relapsed in bone marrow; treated with brentuximab vedotin (anti-CD30 antibody) | Alive with disease (18 mo) | ||||
| 2 | 55/M | Axillary lymph node‡ | I‡ | HTLV-1 carrier | 6024 | 296 | Treated with VCAP-AMP-VECP | Alive with disease (4 mo) | ||||
| 3 | 79/F | Mediastinal mass, bone marrow | IV | HTLV-1 carrier | 3205 | 322 | Treated with 1 cycle of CHOP followed by ABVD chemotherapy | Alive in CR (7 mo) | ||||
| 4 | 55/F | Generalized lymphadenopathy, hepatosplenomegaly, bone marrow | IV | Chronic-type ATLL (7 mo)† | 65713 | 320 | Treated with VCAP-AMP-VECP and mogamulizumab (anti-CCR4 antibody), but disease status was progressive | Died of disease (3 mo) | ||||
| 5 | 80/F | Cervical and supraclavicular lymphadenopathy | II | HTLV-1 carrier | 635 | 307 | Treated with CHOP chemotherapy followed by oral etoposide therapy; stable disease for 16 mo followed by recurrence | Died of disease (18 mo) | ||||
| 6 | 74/M | Generalized lymphadenopathy§ | IIIB§ | HTLV-1 carrier | 5660 | 188 | Treated with palliative care; no chemotherapy because of accompanying liver cirrhosis | Died of disease (2 mo) | ||||
| 7 | 72/F | Inguinal lymphadenopathy | I | HTLV-1 carrier | 2493 | 212 | Treated with CHOP | Alive in CR (28 mo) | ||||
| 8 | 81/M | Generalized lymphadenopathy§ | IIIA§ | HTLV-1 carrier | 5732 | 486 | Treated with CHOP | Alive in CR (28 mo) | ||||
ABVD, Adriamycin, bleomycin, vinblastine, and dacarbazine; AMP, doxorubicin, ranimustine, and prednisone; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisone; F, female; LDH, lactate dehydrogenase; M, male; sIL2R, soluble interleukin 2 receptor; VCAP, vindesine, cyclophosphamide, doxorubicin, and prednisone; VECP, vindesine, etoposide, carboplatin, and prednisone.
Pathological features of this case have been reported previously.19
Duration from the diagnosis of indolent type ATLL to the development of ATLL with HTLV-1–infected HRS-like cells.
This patient showed increased lymphocytes in the cerebrospinal fluid. However, no HRS-like large cells were recognized.
Bone marrow involvement was not assessed in these patients.