Nodular Lymphocytic Predominant Hodgkin Lymphoma (NLPHL) (2015)
CD45+, CD20+, CD79a+, PAX-5+, OCT-2+, BCL6+, BOB-1+, PU-1+, BTK+, EMA+
CD10-, CD30-, CD15-
- Rare disease. 5% of Hodgkins (HL) in adults. 10-20% of HL in children.
- 3:1 Male to Female ratio
- Usually presents as early stage, non-bulky disease with few adverse risk factors
- Favourable prognosis, indolent course
- 5% transformation to DLBCL (outcome is same as de novo DLBCL, ie OS 60% at 10 years)
- No prospective randomized controlled trials to compare treatments.
- B cell neoplasm characterized by CD20+ lymphocyte predominant (LP) or popcorn cells.
- Immunophenotyping comparison
o cHL is CD30+, CD15+, MUM1+, CD45-, CD20-, CD79a-, CD19-
- LN pathology
o Nodular or nodular and diffuse lymphoid infiltrate with follicular dendritic cells
o Popcorn cells have scant cytoplasm, polylobated nuclei and distinct nucleoli
- Lymphocyte rich classical HL (LRcHL) – need detailed immunophenotyping to separate
- Progressive transformation of germinal centres (PTGC) – benign condition
- T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) – ?spectrum of same disorder
- Reactive conditions.
- FBC, ESR, U&E, LFT, LDH, Bone, Immunoglobulins
- HIV, Hep B/C
- CT or FDG-PET staging (1/3 of patients will be upstaged with PET vs CT)
- BM biopsy not required in early disease. Not in advanced if PET performed.
- Fertility preservation
- Irradiated blood products flag
- No evidence for interim scan at present
- CT or PET at end of treatment