Lymphoma (Hodgkin's and non-Hodgkin's)

Question 1

What is lymphoma.

Answer 1

Malignancy of the lymph system.
These malignancies arise from the lymphoid tissue.
It involves clonal expansion of B and T white blood cells.

Question 2

How common is Hodgkin’s lymphoma. (2)

Answer 2

Rare.

4/100,000 new cases per year.

Question 3

At what ages is it most common to get Hodgkin’s lymphoma. (2)

Answer 3

Between 20-35 years.

Between 50-60 years.

Question 4

What are the clinical features of Hodgkin’s lymphoma. (9)

Answer 4

Painless rubbery lymphadenopathy - localized or generalized (usually in the neck or supraclavicular fossae).
Hepatosplenomegaly may be present.
Rarely large mediastinal masses may cause dry cough and breathlessness.
Cachexia.
There may be alcohol induced lymph node pain.
Constitutional or ‘B’ symptoms are experienced in up to 25% of patients:
Fever.
Weight loss (>10%).
Pruritus.
Sweats.

Question 5

What lymph nodes are most commonly affected in Hodgkin’s lymphoma.

Answer 5

The cervical chain is the commonest site.

Question 6

What is the staging system used for Hodgkin’s lymphoma.

Answer 6

ANN ARBOR.

Question 7

What is a stage 1 Hodgkin’s lymphoma.

Answer 7

Confined to a single lymph node group.

Question 8

What is a stage 2 Hodgkin’s lymphoma. (2)

Answer 8

Two or more lymph node groups.

Confined to one side of the diaphragm.

Question 9

What is a stage 3 Hodgkin’s lymphoma. (2)

Answer 9

Two or more lymph node groups.

Involves both sides of the diaphragm.

Question 10

What is a stage 4 Hodgkin’s lymphoma.

Answer 10

Involvement of the extralymphatic sites (eg bone marrow).

Question 11

What other feature is included when staging a Hodgkin’s lymphoma.

Answer 11

Includes the absence (A) or presence (B) of systemic symptoms.

Question 12

What is the 10 year survival rate for patients with Hodgkin’s lymphoma. (2)

Answer 12

Varies from 50% for stage 4 to 80% for stage 1A.

Between 50-70% with advanced disease can be cured.

Question 13

How common is non-Hodgkin’s lymphoma.

Answer 13

It is 10 times more common than Hodgkin’s lymphoma.

Question 14

What are the risk factors for developing Hodgkin’s lymphoma. (7)

Answer 14

Increases with age.
Slight male preponderance.
Geographical variation: Burkitt’s lymphoma is common in Africa.
Viruses are implicated, eg. EBV, HSV.
Immunosuppression is a risk factor (including HIV/AIDS).
SLE.
Obesity.

Question 15

What are the two main types of non-Hodgkin’s lymphoma. (2)

Answer 15

B cell lymphoma (80% of cases).

T cell lymphoma (20% of cases).

Question 16

How can non-Hodgkin’s lymphomas be classified. (3)

Answer 16

As low, intermediate, or high grade.

Question 17

What are the clinical signs of non-Hodgkin’s lymphoma. (3)

Answer 17

Lymphadenopathy (60-70%).
Hepatosplenomegaly.
B symptoms.

Question 18

How are non-Hodgkin’s lymphomas staged.

Answer 18

Using the ANN ARBOR staging system.

Question 19

What is the 10 year survival rate for patients with Non-Hodgkin’s lymphoma. (2)

Answer 19

Varies from 35% for stage 4 to 80% for stage 1 disease.

There is a median survival of 10 years.

Question 20

How are lymphomas classified. (2)

Answer 20

They are diagnosed from the pathological findings on biopsy as Hodgkin’s or non-Hodgkin’s lymphoma.
The majority are of B cell origin.

Question 21

How are non-Hodgkin’s lymphomas classified.

Answer 21

non-Hodgkin’s lymphomas are classified as low or high grade tumours on the basis of heir proliferation rate.

Question 22

What are high grade non-Hodgkin’s lymphomas. (3)

Answer 22

They are rapidly dividing.
They present for a matter of weeks before diagnosis.
They may be life-threatening.

Question 23

What are low grade non-Hodgkin’s lymphomas. (3)

Answer 23

They divide slowly.
They may be present for many months before diagnosis.
They typically present in an indolent fashion.

Question 24

What is the hallmark finding in Hodgkin’s lymphoma.

Answer 24

Reed-Sternberg cells.

Question 25

What are Reed-Sternberg cells.

Answer 25

These are large malignant lymphoid cells of B cell origin.

Question 26

How are Reed-Sternberg cells usually present in the bone marrow. (2)

Answer 26

They are present in small numbers. | They are surrounded by large numbers of reactive non-malignant T cells, plasma cells and eosinophils.

Question 27

What is the male:female ratio for Hodgkin's lymphoma.

Answer 27

1.5:1.

Question 28

What is the age of diagnosis of Hodgkin's lymphoma. (2)

Answer 28

Median age: 31. | First peak 20-35 years, second at 50-70 years.

Question 29

What are poor prognostic factors of Hodgkin's lymphoma on blood test (4)

Answer 29

Normochromic. Normocytic anaemia. Lymphopenia. Raised LDH.

Question 30

What may be seen on the blood tests of a patient with Hodgkin's lymphoma. (5)

Answer 30

FBC may be normal, normochromic, normocytic anaemic or lymphopenic. There may be eosinophillia or neutrophillia. ESR may be raised. Renal function tests are required before starting treatment. LFTs may be abnormal (it may reflect hepatic infiltration). LDH may be raised.

Question 31

What is the Hasenclever prognostic index for advanced Hodgkin's lymphoma. (7)

Answer 31

``` A score of 1 is given to each of the following: Age>45. Male. Serum albumin 15. Lymphopenia ```

Question 32

What is the incidence of non-Hodgkin's lymphoma.

Answer 32

Incidence has increased since 1970 to 1:10,000.

Question 33

Who is at greater risk of developing non-Hodgkin's lymphoma.

Answer 33

Slight male excess.

Question 34

What is the average age of onset of non-Hodgkin's lymphoma.

Answer 34

65-71.

Question 35

What are the risk factors for developing non-Hodgkin's lymphoma. (7)

Answer 35

No single causative abnormality has been found. Lymphoma is a late manifestation of HIV infection. Certain viruses are associated with lymphoma types (EBV, HHV8, HTLV). Gastric lymphoma can be associated with H.pylori infection (with MALT tissue). Congenital. Some lymphomas are associated with specific chromosomal translocations: t(11;14). Lymphoma may occur in congenital immunodeficiency states and in immunosuppressed patients after organ transplantation.

Question 36

Does Hodgkin's lymphoma tend to spread beyond the lymph nodes.

Answer 36

It does not tend to spread beyond the lymphatic system.

Question 37

Does non-Hodgkin's lymphoma tend to spread beyond the lymph nodes.

Answer 37

It is often widely disseminated at presentation.

Question 38

What are the symptoms of non-Hodgkin's lymphoma. (10)

Answer 38

``` Patients present with lymph node enlargement. May be associated with systemic upset: Weight loss (less common than in Hodgkin's lymphoma and associated with disseminated disease). Sweats. Fever. Itching. Hepatosplenomegaly may be present. Compression syndromes may occur: Gut obstruction. Ascites. Superior vena cava obstruction. Spinal cord compression. ```

Question 39

What are the typical extranodal sites that are involved in non-Hodgkin's lymphoma. (9)

Answer 39

``` Bone marrow. Gut. Small bowel (rare). Thyroid. Lung. Skin. Testis. Brain. Bone (rare). ```

Question 40

What investigations should be performed on non-Hodgkin's lymphoma. (7)

Answer 40

``` As for Hodgkin's lymphoma. In addition: Bone marrow aspiration and trephine. Immunophenotyping of surface antigents to distinguish T from B cell tumours. Cytogenetic analysis. Immunoglobulin determination. Measurement of uric acid. HIV testing. ```

Question 41

What are the main subtypes of Hodgkin's lymphoma. (2)

Answer 41

Classical Hodgkin's lymphoma. | Nodular lymphocyte predominant Hodgkin's lymphoma.

Question 42

What other tests should be done in Hodgkin's lymphoma. (2)

Answer 42

CT chest. | Chest X ray (to see if there is a mediastinal mass).

Question 43

What is Pel-Ebstein fever. (3)

Answer 43

It is associated with Hodgkin's lymphoma. It implies a cyclical fever with long periods (15-28days) of normal to low temperatures. It is very rare.

Question 44

What is the cellular difference between Hodgkin's and non-Hodgkin's lymphomas. (2)

Answer 44

Hodgkin's lymphomas have Reed-Sternberg cells on a blood film. Non-Hodgkin's lymphomas include all the lymphomas without Reed-Sternberg cells.

Question 45

What are the two subsets of presentation of non-Hodgkin's lymphoma. (2)

Answer 45

``` Nodal disease (75%). Extranodal disease (25%). ```

Question 46

What are the symptoms caused by bone marrow invovlement in non-Hodgkin's lymphoma. (3)

Answer 46

Pancytopenia from marrow involvement: Infection. Anaemia. Bleeding (due to low platelets).

Question 47

How is Hodgkin's lymphoma usually staged. (2)

Answer 47

Via CT/PET of the thorax. | Lymph node excision biopsy if possible.

Question 48

How is non-Hodgkin's lymphoma usually staged. (2)

Answer 48

Bone marrow and lymph node biopsy.

Question 49

What reduced the chances of a good prognosis in non-Hodgkin's lymphoma. (5)

Answer 49

``` If at presentation: Age>60. Systemic symptoms. Bulky disease (abdominal mass >10cm). Increased LDH. Disseminated disease. ```