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M.L. Nijland et al.
malignant transformation of a stem cell with the capacity to differentiate in either a B- or a T-NHL or a shared genet- ic predisposition for the two lymphomas.44,45 An example of this is the ten-eleven translocation 2 (TET2) mutation.
TET2 is a dioxygenase that plays an important role in hematopoietic stem cells and progenitor cells by catalyz- ing multiple steps of 5-methylcytosine oxidation. TET2 mutations are commonly found in myeloid cancers, in
Table 5. Treatment and outcome.
Histological Subtype
PTCL, NOS
PTCL, NOS PTCL, NOS
PTCL, NOS PTCL, NOS PTCL, NOS ALCL ALK- ALCL ALK- ALCL ALK-
ALCL ALK-
ALCL ALK+
AITL
AITL
AITL MF
MF
Prim. C-CD30+ T-LPD
Prim. C-CD30+ T-LPD Precursor T-LBL
Precursor T-LBL
ENKL, nasal type HSTCL
T-PLL Liquor/CNS T-NHL, unclass.
First-line therapy
None, confirmation diagnosis after death
None, palliative treatment None, diagnosis at autopsy
Cytarabine, idarubicine
CHOEP, autologous SCT and HD-MTX CR
CHOP, alemtuzumab CR
None, palliative treatment
CHOP and intrathecal MTX CR, relapse DHAP
CHOP PD DHAP; third line:
OC S (m)
Death 0
<1 0
Death 4 Alive
Alive 111 <1
Alive 102 CR Alive
Death 1
Death 41
Death 3
Death 9
Death 4 Death 61
Alive 65 Alive 74
Alive 69
Alive 76 Alive 8
Alive 49 Death 3 Death 4 Death 1 Death 1
R Second-line therapy
R
Death Death
PD
Death CR
Cause of death
Sepsis (mycobacterial
infection)
Rapid progression
MOF and ARDS, high lymphoma load
Refractory disease 19
Rapid progression 21
Sepsis caused by
bowel perforation
due to lymphoma
NR, besides
lymphoma also
gallbladder cancer and HCC
Chemotherapy complications NR
NR NR
PR DHAP-VIM-DHAP
Reversed CHOP
Cyclofosfamide, CR, DHAP; third line: doxorubicine, prednisolone relapse VIM
CHOP PR
CHOP SD DHAP-VIM-DHAP + alemtuzumab
SD
SD
brentuximab; fourth line: Cyclophosphamide,
fludarabine, TBI, followed by allo-CB SCT
Prednisolone NR
PUVA, topical steroids, PD RT PR MTX, prednisolone
PUVA, topical steroids CR
No treatment, CR
self-limiting
RT CR, relapse RT
Multiple chemotherapeutic
agents (HOVON-70-protocol) CR
Prednisolone, vincristin, CR
daunorubicin, PEG-asparaginase
and intrathecal MTX
CHOP CR
NR, treatment in other center NR
Prednisolone NR
RT
None, palliative treatment
CR
NR
NR
Rapid progression Rapid progression
494
AITL: angioimmunoblastic T-cell lymphoma; ALCL: T-cell anaplastic large cell lymphoma; ALK: anaplastic lymphoma kinase; ARDS: acute respiratory distress syndrome; CB SCT: cord blood stem cell transplantation; CHOEP: cyclophosphamide, doxorubicin, vincristine, etoposide and prednisolone; CHOP: cyclophosphamide, doxorubicin, vincristine and prednisolone; CR: complete remission; DHAP: dexamethasone, high dose cytarabine and cisplatinum; ENKL: extranodal NK/T-cell lymphoma; HSTCL: hepatosplenic T-cell lym- phoma; LTF: lost to follow-up; MF: mycosis fungoides; MOF: multi-organ failure; MTX: methotrexate; NR: not recorded or not known; OC: outcome; PD: progressive disease; PR partial remission; Prim. C-CD30+ T-LPD: primary cutaneous CD30+ T-cell lymphoproliferative disease; PTCL, NOS: peripheral T-cell lymphoma, not otherwise specified; PUVA: pso- ralen plus ultraviolet A; R: response; RT: radiotherapy; S: survival; SD: stable disease; T-LBL: T-cell lymphoblastic lymphoma; T-PLL: T-cell prolymphocytic leukemia; TBI: total body irradiation;VIM: ifosfamide, mitoxantrone and etoposide.
haematologica | 2018; 103(3)