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

 

Management

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