Hodgkin Lymphoma Flashcards
Two types of HL
**I. CHL **(95% of HL, 2 peaks in age, EBV associated, IMPT: CD15+, CD30+, CD45-)
* Nodular Sclerosing (70%)
* Mixed cellularity
* Lymphocyte rich
* Lymphocyte depleted
II. Non-CHL (5% of HL, 30-50 yo males, LP cells, IMPT: CD15-, CD30- and CD45+)
* Nodular lymphocyte predoninant
Treatment of HL
PDL-1 Inhibitors:
- Pembroluzumab
- Nivolumab
What is true of all the four subtypes of
CHL?
- derived from B cells
- mononuclear Hodgkin cells and multinucleated RS cells present in all
- reduced expression of B cell antigens
- CD20, CD79a and PAX5
- positive for: CD15 and CD30
- reduced expression of B cell antigens
- variable EBV association (subtype specific)
What are the 4 subtypes of
CHL?
- Nodular Sclerosis
- Lymphocyte rich
- Mixed cellularity
- Lymphocyte depleted
What is the Syncytial variant of
Nodular Sclerosing CHL?
- lacunar cells form cohesive nests in the centres of the nodules
- necrosis may or may not be present
- if prominent should consider this variant
- may think this is Anaplastic large cell lymphoma
- PAX5 positive
Features of NS CHL
- Nodules
- Mediastinum and bulky disease
What two subtypes of CHL are often
associated with EBV infection?
- Mixed cellularity CHL
- Lymphocyte depleted CHL
Note: NLPHL is only rarely associated with EBV infection (<5%)
Features of MC CHL
- Diffuse
- Peripheral LNs and spleen
Features of LR CHL
- Nodular
- Peripheral LNs
- B-Sx = rare
Features of LD CHL
- > HRS : Lymphocytes
- Intra-abdominal LNs and Extranodal disease
DDx for HRS cells
6
- HL
- EBV+ DLBCL
- 1’ DLBCL of CNS
- 1’ mediastinal LBCL
- 1’ Effusion lymphoma
- TCHR LBCL
DDx for CD30+ lymphomas
HL, BCL(5), TCL(5)
I. HL
II. BCL
* Anaplastic variant of DLBCL
* Lymphomatoid granulomatosis
* PBL
* ALK+ LBCL
* 1’ effusion lymphoma
III. TCL
* ATCL
* Extranodal NK/TCL
* Enteropathy ass. TCL
* PTCL, NOS
* ALCL
What is the morphology of Classic
Reed-Sternberg Cells?
- large with abundant and slightly basophilic cytoplasm
- have at least 2 nuclear lobes and 2 nuclei
- can have more than this
- nuclei are large, rounded contours
- prominent, irregular nuclear membrane
- pale chromatin, eosinophlic nucleolus
- perinuclear halo
- 2 prominent nucleoli in 2 separate nuclei in one cell
What is the EBV expression of HS cells
of CHL ?
- EBV infected cells express LMP1 and EBNA1
- no expression of EBNA2
- this is characteristic of type II EBV infection
Hodgkin lymphomas generally affect which
organs and what is a common feature of
most of them?
- usually affect Lymph Nodes
- variable mixture of associated, non-neoplastic inflammatory cells
- sometimes associated with variable fibrosis (thick band like)
What are the 2 types of
Hodgkin’s Lymphomas?
- Nodular lymphocyte predominant (NLPHL)
- Classic Hodgkin Lymphoma
Which HL is the most common type and
what age groups is it often seen in?
- Classic Hodgkin Lymphoma
- 90% of all HL
- Two peaks
- 15-35 years old
- late life
- Has different subtypes that fall into these categories
How does HL (both types, general) present
clinically?
- peripheral lymphadenopathy
- usually localized to 1-2 lymph nodes
- generally cervical
- NLPHL prefers peripheral lymph nodes
- B symptoms
- fever, drenching night sweats, weight loss
- NLPHL does not present with B symptoms, in fact it is usually asymptomatic
At what stage of B cell maturation are
Hodgkin/R-S cells derived from?
- > 98% of cases are neoplastic B cells
- derived from mature B cells of the germinal centre stage
- clonal IG genes
What are the 4 subtypes of
Classical Hodgkin’s Lymphoma?
- Nodular Sclerosis Hodgkin Lymphoma
- Lymphocyte rich CHL
- Mixed Cellularity CHL
- Lymphocyte depleted CHL
What age group is preferentially affected
by Nodular Lymphocyte Predominant HL?
- generally 4th-5th decade
- also common in children
- M > F
CHL subtypes tend to have a male predominance, with the exception of which subtype?
- Nodular sclerosing HL
- slight female predominance
What is the morphology of the Hodgkin cell
and the Mummified Hodgkin cell?
- smudgy large nuclei (may be slightly eosinophilic)
- can be anaplastic and confused with ALCL
What is the immunophenotype of the HS
of CHL?
- CD15 and CD30- membranous pattern with accentuation in the Golgi region
- PAX5
- weaker than reactive B cells (IMP)
- IRF4/MUM1 - strongly positive
- CD20
- positive in 30% of cases
- CD79a
- not often expressed
- CD45 and CD68 - negative
