Pocket Oncology (Pocket Notebook Series), 1st Ed.


Andrew M. Intlekofer and Steven M. Horwitz


• Heterogeneous group of often aggressive neoplasms arising from mature T lymphocytes & NK cells w/1° involvement of sites other than skin (eg, LN, blood, & visceral organs)

• Precursor T-lymphoblastic lymphoma is considered a separate entity that is treated like T-cell ALL (T-ALL)


• Comprise approximately 10% of all NHL, male > female

Clinical Presentation

• Sx/signs: Prominent B sx (wt loss >10%, fevers, sweats), pruritus, generalized LAN, HSM, rash

• Common extranodal involvement (often subtype specific): Bone marrow, blood, skin, liver, GI tract, nasopharynx, testes, CNS

Diagnostic Evaluation

• Bx: Excisional or incisional LN bx, skin or GI tract bx if involved

• Histology: Often difficult to diagnose, heterogeneous immune cell infiltrates

• Immunophenotype: Variable expression of T-cell markers (in general T-cell CD markers <10), CD2, CD3, CD4, CD5, CD7, CD8, CD25, CD30, CD56, granzyme, perforin, TCR expression α/β >> γ/δ, EBER ISH

• Cytogenetics/FISH: t(2:5) (ALK:NPM); other less common cytogenetic abnl

• Molecular diagnostics for clonal TCR β & γ rearrangement

• Lab evaluation: CBC w/diff (often ≠ Eos), CMP, LDH, quant Ig, Coombs, HBV/HCV serologies, HIV, HTLV-1 serology (ATLL), serum EBV PCR (NK/T)

• BM aspirate & core bx: Hemophagocytosis may be seen

• LP: If CNS involvement suspected clinically or by imaging

• Imaging: CT C/A/P ± FDG PET, echo prior to anthracyclines

• Fertility preservation prior to tx if possible

Staging and Risk Stratification

• Ann Arbor staging: As detailed in prior lymphoma sections; most PTCL pts present w/adv stage (III or IV)

• IPI: “A-P-L-E-S” acronym as detailed in prior lymphoma sections; most PTCL pts have high–int or high IPI; 5-y OS after combination chemo (eg, CHOP), 74% (low), 49% (low-int), 21% (high-int), 6% (high), respectively (Br J Haem 2005;129:366)

Principles of Treatment

• No standard of care: Most current treatments based on B-cell lymphoma regimens but lower RR & less durable remissions in PTCL, thus clinical trial preferred for all pts

• Induction chemo: Age <60 CHOEP(Blood 2010;116:3418)

Age >60 CHOP

Other options: da-EPOCH, CHOP/ICE, hyperCVAD

• HDT/ASCR: Consolidation w/HDT & autologous stem cell rescue in 1st remission for fit pts (see “special tx scenarios” for exceptions)

• RT: Curative intent as consolidation after induction chemo for localized disease; essential in localized NK/T cell; o/w palliative intent

• Relapsed/refractory disease: Combination vs. single-agent chemo (see chart)

• Allogeneic SCT: If possible for relapsed/refractory disease

• Special tx scenarios/Subtype-specific regimens:

→ ALK+ ALCL = favorable outcome w/CHO(E)P alone (Blood 2008;111:5496)

→ Localized (stage I/II) PTCL w/low/low–intermediate IPI = favorable outcome w/CHO(E)P (Br J Haem 2005;129:366)

→ Aggressive ATLL = zidovudine & IFNα ± combination chemotherapy; consider alloSCT in 1st remission (NEJM 1995;332:1745, 1749; JCO 2011;29:4696)

→ Hepatosplenic TCL = non-CHOP regimens; consider alloSCT in 1st remission

→ Localized extranodal NK/T cell lymphoma, nasal type (stage IE/IIE) = sequential vs. concurrent chemoradiation (JCO, 2009; 27:5594; 27:6027)

→ adv stage extranodal NK/T-cell lymphoma (stage IV) = SMILE regimen (JCO 2011;29:4410)

→ EATL = Newcastle regimen IVE/MTX (ifosfamide, etoposide, epirubicin/ MTX) followed by HDT/ASCR (Blood 2010;115:3664)

Supportive Care

• CNS Ppx: Consider intrathecal MTX/cytarabine for high-risk disease involving paranasal sinuses, testes, epidural

• Tumor lysis Ppx: IVF + allopurinol + phos binder +/- rasburicase

• Infectious Ppx: For VZV & PJP/PCP; skin care when involved

• Growth factor support: w/G-CSF to treat/prevent neutropenic fever