HAEMATOLOGY - HODGKIN LYMPHOMA

Question 1

Why is it called Hodgkin lymphoma?

Answer 1

Tumour derived from lymphocytes, specifically B cells, that form hodgkin cells (reed-sternberg cells)
Thomas Hodgkin first described these tumours

Question 2

Outline the pathophysiology of Hodgkin lymphoma?

Answer 2

B cells in germinal centre undergo transforming events (of which pathogenesis is not clear but may be EBV) to form Reed-Sternberg cells. These cells lack their normal apoptosis function and undergo clonal proliferation
The exact mechanism is unclear

Question 3

What are the main differences between Hodgkin lymphoma and non-hodgkin lymphoma?

Answer 3

Hodgkin lymphomas tend to spread nearby and NH lymphoma doesnt
H lymphoma is rarely extranodal whilst NH lymphoma often spreads to skin, GIT, Brain
H lymphoma has reed-sternberg cells whilst NH does not

Question 4

Why are neoplastic cells considered ‘crippled’/

Answer 4

As they don’t produce antibodies as they usually would

Question 5

What are neoplastic cells usually surrounded by? What can they activate?

Answer 5

Surrounded by inflammatory cells such as T cells which are attracted to chemo kidneys
Activate fibroblasts which make collagen and activate eosinophils

Question 6

What ar the 2 major subgroups?

Answer 6

Classical Hodgkin lymphoma
Nodular lymphocyte predominant Hodgkin lymphoma

Question 7

What are the characteristics of classical Hodgkin lymphoma?

Answer 7

90-95% of all hodgkin lymphomas
Affect male:female 15:1
4 subtypes
Have reed sternberg cells
Express CD30 and CD15

Question 8

What are the 4 subtypes of classical hodgkin lymphoma?

Answer 8

Nodular sclerosis hodgkin lymphoma
Mixed cellularity hodgkin lymphoma
Lymphocyte rich hodgkin lymphoma
Lymphocyte depleted hodgkin lymphoma

Question 9

Whats the most common subtype of classical hodgkin lymphoma?

Answer 9

Nodular sclerosis (70% of cases)

Question 10

Which of the classical hodgkin lymphomas has the best prognosis?

Answer 10

Lymphocyte rich hodgkin lymphoma

Question 11

Which of the classical hodgkin lymphomas are the least common?

Answer 11

Lymphocyte depleted hodgkin lymphoma

Question 12

What are the characteristics of nodular sclerosis hodgkin lymphoma?

Answer 12

Neoplastic and inflammatory cells get surrounded by collagen from fibroblasts which forms nodules
A unique Reed-Sternberg cell called a lacunae cell may be seen histologically - nucleus looks like its sitting in the middle of a lake

Question 13

What are the characteristics of mixed cellularity hodgkin lymphoma?

Answer 13

Named after the mixed inflammatory background composed of variable numbers of eosinophils, neutrophils, lymphocytes, plasma cells and histiocytes that surround the reed-sternberg cells

Question 14

What are the characteristics of lymphocyte rich hodgkin lymphoma?

Answer 14

Named for having mostly lymphocytes surround the reed sternberg cells

Question 15

What are the characteristics of lymphocyte depleted hodgkin lymphoma?

Answer 15

Named for the lack of lymphocytes in the abundance of hodgkin and Reed-Sternberg cells

Question 16

What are the characteristics of nodular lymphocyte predominant hodgkin lymphoma?

Answer 16

More common in men
B cells express CD20 and CD45 on their surface and don’t have CD15 or CD30
Have a variant of Reed-Sternberg cells called lymphocyte predominant cells (cells with a lobulated nucleus that looks a little like popcorn). Large groups of lymphocytes form nodules around these popcorn cells

Question 17

What are the symptoms of hodgkin lymphoma?

Answer 17

Painless cervical lymphadenopathy
Fever
Drenching night sweats
Weight loss

Rarer:
Cough due to mediastinal lymphadenopathy
Hepatosplenomegaly
Pruritis
Alcohol-related pain at the site of lymphadenopathy - related to vasodilation within a lymph node pressing on a nerve

Question 18

How does nodular lymphocyte predominant hodgkin lymphoma present differently to classical hodgkin lymphoma?

Answer 18

Usually doesn’t have any symptoms other than a couple of cervical lymph node swellings

Question 19

How is hodgkin lymphoma diagnosed?

Answer 19

Imaging studies to establish the stage
Lymph node biopsy - diagnostic

Question 20

What does treatment depend on for hodgkin lymphoma?

Answer 20

Stage, age and general health

Question 21

How do we treat classic hodgkin lymphoma?

Answer 21

Chemotherapy - ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine)
Radiation therapy
Autologous transplant for some relapsed cases

Question 22

How do you treat nodular lymphocyte predominant non hodgkin lymphoma?

Answer 22

Rituximab as B cells have CD20 so it can cause compliment mediated lysis

Question 23

What has a better prognosis, hodgkin or non-hodgkin lymphoma?

Answer 23

Hodgkin lymphoma

Question 24

Which viruses is hodgkin lymphoma associated with?

Answer 24

EBV - 50% of cases have tumour cells EBV-positive
HIV - higher incidence but note that HL is not considered an AIDS-defining neoplasm

Question 25

How is hodgkin lymphoma staged?

Answer 25

Using Ann Arbor staging

Question 26

Outline Ann Arbor staging of hodgkin lymphoma?

Answer 26

Stage 1 - involvement of single lymph node region or strtuction or a single extralymphatic site Stage 2 - involvement of 2+ lymph nod regions on the same side of the diaphragm Stage 3 - involvement of lymph node regions on both side of diagohgram Stage 4 - diffuse or disseminated involvement of extranodal organs (involvement of liver or bone marrow is considered this stage) A- denotes no symptoms B - B symptoms present (denotes fever, drenching night sweats, unexplained weight loss >10% body weight in past 6 months) X - bulky disease E - involvement of a single extranodal site that is proximal to the known nodal site

Question 27

What is bulky disease in hodgkin lymphoma?

Answer 27

A widening of the mediastinum by more than 1/3rd or the presence of a nodal mass with a maximal dimension >10cm

Question 28

When should you refer someone for suspected Hodgkin’s lymphoma?

Answer 28

2 WW - adults with unexplained lymphadenopathy

Question 29

What demographic does Hodgkin lymphoma mostly affect?

Answer 29

Male: female 1.5:1 Age range is bifocal with peaks at 20 and 75 years

Question 30

What clusters of differentiation would you see in A) classic Hodgkin lymphoma B) nodular lymphocyte-predominant Hodgkin lymphoma

Answer 30

A - CD30 and CD15 (in 30% of cases CD20) B - CD20 and CD45

Question 31

What is the 2 week wait guidelines for Hodgkin lymphoma?

Answer 31

Refer any adults with unexplained lymphadenopathy

Question 32

On average, what’s the prognosis of Hodgkin lymphoma for 5 years after diagnosis?

Answer 32

90% if stage 1 70% if stage 2