Nodular Lymphocytic Predominant Hodgkin Lymphoma (NLPHL) (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

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

 

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

 

Management

nlphl.png