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-

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

LN pathology

Nodular or nodular and diffuse lymphoid infiltrate with follicular dendritic cells
‘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

One point for each:

Score correlates with 5yr PFS and 5yr OS

No evidence for interim scan at present

CT or PET at end of treatment