Initial assessment:
- Symptoms: Fever, wt loss, night sweats, skin rash, itching
- Exam: rash, adenopathy, cachexia, HSM
Investigations:
1. Excisional nodal bx/ skn bx/GI bx
2. BMBX: look for hemophagocytosis
3. LP is suspected CNS involvement
4. Echo
5. PET
Disease specific
- Immunophenotyping
- Cytogenetics /FISH
- Targeted genome sequencing
- T cell clonality
Ann Arbor staging
ALK+ ALCL--> BV CHP
low or low intermediate IPI PTCL--> CHOEP
MEITL and Hepatosplenic--> ICE
EATL= CHOEP followed by ASCT or Newcastle regimen
Adult T cell leukemia- rx of HTLV with antivirals and also VCAP-AMP-VECP
NK cell--> SMILE regimen
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