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Flashcards in NLPHD Deck (11):

What is the neoplastic cell in NLPHD?

Neoplastic cell = Lymphohistocytic or L&H Reed-Sternberg cell variant

This is a germinal center B cell
With productive immunoglobulin gene rearrangements with evidence of ongoing somatic hypermutation of their immunoglobulin genes


What is T cell/histiocyte-rich B Cell Lymphoma (T/HRBCL)?

A variant of DLBCL
Similar morphology and identical immunophenotype to NLPHL

NLPHL may transform to tumors closely resembling T/HRBCL
- an event that is a/w the loss of b/g dendritic cells


Describe the L&H cell

An atypical variant of the RS cell

Vesicular, polylobulated nuclei
Distinct but small, usually peripheral nucleoli
Without perinucleolar halos

= popcorn cells


What is the b/g cells of cHL like?

Scattered S cells in a polymorphous b/g of acute and chronic inflammatory cells


What is the b/g cells of NLPHL like?

L&H cells in a b/g of:
- small B-lymphocytes,
- follicular dendritic cells
- follicular CD57+ T-lymphocytes (often form rosettes around L&H cells)


What is the immunophenotype of L&H cells in NLPHL?

BCL6 +
CD19+, CD20+, CD79a+
CD30 usually -

B/g cells consisting of:
- small B cells
- CD3+/CD4+/CD57+ T cells
- CD21+, CD23+ Follicular dendritic cells


What is the immunophenotype of cHL?

CD45- (Leucocyte common antigen)


What are the differential diagnoses for NLPHL?

1) Lymphadenopathy
- infectious
- autoimmune
- benign
- malignant
2) Reactive hyperplasia or PTGC
- PTGC = progressive transformation of germinal centers
(Not considered a malignant condition)
3) Tcell/HRBCL
4) Lymphocyte-rich cHL


What is the main modality of treatment in early stage favorable NLPHL?

Limited treatment with RT only

Retrospective studies from GHSG, MD Anderson Cancer centre, European Task Force on Lymphoma Study:
Did not show that addition of chemo Improved Relapse-free survival nor OS


What are the options for Stage III/IV NLPHL disease?

Combination chemotherapy
* ABVD may not be as effective as other alklyator-based therapy
- Rituximab can be used as monotherapy for those who are not candidates for cytotoxic chemo or for those with low volume of recurrent disease
- Localized palliative RT `


Do you know of any risk stratification scores?

From The GHSG study of 400 patients with NLPHL treated within 9 prospective studies:

Multivariate analysis identified that:
- Albumin <40 (1 point)
- Atypical histological variant (1 point)
- Male (2 points)

3 scores:
- low risk 0-1 point
- intermediate 2 points
- high 3-4 points

PFS: 95%, 90%, 70% respectively
OS: 99%, 95%, 90% respectively