Nodular Lymphocytic Predominant Hodgkin Lymphoma (NLPHL) (bsh 2015)
CD45+, CD20+, CD79a+, PAX-5+, OCT-2+, BCL6+, BOB-1+, PU-1+, BTK+, EMA+
CD19 +/-
CD10-, CD30-, CD15-
Background
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.
Pathology
B cell neoplasm characterized by CD20+ lymphocyte predominant (LP) or popcorn cells.
Immunophenotyping comparison
cHL is CD30+, CD15+, MUM1+, CD45-, CD20-, CD79a-, CD19-
LN pathology
Nodular or nodular and diffuse lymphoid infiltrate with follicular dendritic cells
‘Popcorn cells’ have scant cytoplasm, polylobated nuclei and distinct nucleoli
Differentials
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.
Pre-Treatment Work-up
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
Follow-up imaging
No evidence for interim scan at present
CT or PET at end of treatment