T Cell Lymphomas Flashcards
ALK+ Anaplastic large cell lymphoma has what prognosis?
Good
Anaplastic large cell lymphoma in general has a ____ prognosis compared to PTCL?
Much better
GATA-3 denotes what prognosis in PTCL?
Poor
Initial amnagement of PTCL
CHO(E)P
Initial management of PTCL that is CD30+
BV-CHP
Who should not get etoposide added to inital management in PTCL?
Age >60
When should we take PTCL to autoSCT?
First CR
Treatment for localized nasal NK/T cell lymphoma in an unfit patient?
RT alone
Treatment for localized nasal NK/T cell lymphoma in a fit patient?
Sequential or concurrent chemo then RT
SMILE then RT
LVP then RT
VIC+RT
Cis+RT then VIPD
Treatment for disseminated NK/T-cell lymphoma? (2)
SMILE +/- RT
DDGP (Cisplatin, Dex, Gem, Asp)
Treatment for breast implant assocaited anaplastic LCL?
Remove the implant
MoA of Mogamulizumab?
Anti-CCR4 mAB
1L Management of early stage CTCL? (4)
Steroids
Bexarotene
PUVA
Localized RT
2L treatment of early stage CTCL? (3)
Total skin electron
Interferon-alpha
MTX
1L treatment for advanced CTCL?
Steroids, PUVA, RT
MTX
ECP
Gem
Doxil
Preferred front line regimen for ALK+ ALCL?
BV-CHP
Treatment of unicentric Castleman’s diseae?
Surgical resection of the involved LN
What is the definition of unicentric vs multicentric Castleman’s disease?
Unicentric only involves a single enlarged LN.
Multicentric involves multiple, usually has severe symptoms and HHV-8 infection
What infection is associated with multicentric Castleman’s disease?
HHV-8
Treatment of active Multicentric Castleman’s disease with organ failure?
R-CHOP
R monotherapy
Treatment for multicentric Castleman’s disease without organ failure in patients who are HIV negative and HHV8 negative?
Siltuximab
Treatment for multicentric Castleman’s disease without organ failure in patients who are HIV+ and/or HHV-8+?
Rituximab
3 criteria for active Castlemans disease?
Fever
Increased CRP >20
At least 3 MCD symptoms: LAD, splenomegaly, edema, effusion, ascites, cough, nasal obstruction, xerostomia, rash, neuro sx, jaundice, AIHA
Immunophenotype of Adult T-cell leukemia/lymphoma?
CD2+, CD3+. CD4+, CD5+,
CD7-, CD8-
CD25++, CD29+
Negative for CD10, 19, 20)
What is Erdheim Chester disease?
Multisystem histiocyte disorder that leads to infiltration of organ systems. Usually has osteosclerotic lesions in long bones.
Treatment of Erdheim Chester disease with BRAF V600E mutation?
vemurafenib
Patient presents with a pleural effusion, ascites, generalized lymphadenopathy, fever, and hepatosplenomegaly. LN biopsy shows a CD3+, CD4+, CD7+, CD8+ lymphoma. Diagnosis?
Aitl
Preferred treatment for AITL (4)
Bv-CHP (for CD30+)
CHOEP
CHOP
DA-EPOCH
Treatment for primary cutaneous ALCL that is localized
Surgery +/- ISRT or ISRT alone
Treatment for primary cutenaous ALCL taht is multifocal but no visceral disease
Bv
Treatment of ERdheim chester disease with a MAPK mutation?
Cobimetinib
Treatment of Erdheim Chester disease without any special mutation?
Cladribine