NLPHD

Question 1

What is the neoplastic cell in NLPHD?

Answer 1

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

Question 2

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

Answer 2

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

Question 3

Describe the L&H cell

Answer 3

An atypical variant of the RS cell

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

= popcorn cells

Question 4

What is the b/g cells of cHL like?

Answer 4

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

Question 5

What is the b/g cells of NLPHL like?

Answer 5

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)

Question 6

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

Answer 6

CD45+ 
BCL6 +
CD19+, CD20+, CD79a+
CD15- 
CD30 usually -

B/g cells consisting of:

• small B cells
• CD3+/CD4+/CD57+ T cells
• CD21+, CD23+ Follicular dendritic cells

Question 7

What is the immunophenotype of cHL?

Answer 7

CD45- (Leucocyte common antigen)
CD30+
CD20-
CD15+

Question 8

What are the differential diagnoses for NLPHL?

Answer 8

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

Question 9

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

Answer 9

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

Question 10

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

Answer 10

Combination chemotherapy

• RCHOP
• ABVD
• 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 `

Question 11

Do you know of any risk stratification scores?

Answer 11

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