Paeds: Lymphoma

Question 1

NHL Incidence

Answer 1

10 per million

Question 2

NHL Classification

Answer 2

1. Lymphoblastic (30% of NHL)
2. Mature B cell (30% of NHL)
   Large cell Lymphomas (15-20% of NHL)

Question 3

Lymphoblastic Types

Answer 3

90% T-cell

10% pre-B

Question 4

Lymphoblastic Presentation

Answer 4

Most present with an anterior mediastinal mass

Disease may be present in bone, bone marrow, skin, CNS, liver, kidneys and spleen.

Question 5

Lymphoblastic Cytogenetics

Answer 5

T (1;14) or t (11;14)

Question 6

Lymphoblastic Note

Answer 6

Cases with >25% blasts in bone marrow are regarded as leukaemia

Question 7

Mature B cell Definition

Answer 7

Burkitt or Burkitt like

Question 8

Mature B cell Presentation

Answer 8

Occur in the abdomen, head and neck, bone marrow and CNS

May grow rapidly

Question 9

Mature B cell Association

Answer 9

Endemic or African Burkitt’s associated with early EBV infection and frequently affects the jaw.

Question 10

Mature B cell Cytogenetics

Answer 10

Expresses surface immunoglobulin and characteristic translocations t(8;14), t (8;22), or t(2;8)

Question 11

Large cell Lymphomas: Subtypes

Answer 11

Diffuse large B cell (BLCL)

Large cell lymphoma (ALCL)

Question 12

Large cell Lymphomas: Presentation

Answer 12

Diffuse large B cell (BLCL) presents like Burkitt’s
Large cell lymphoma (ALCL) involves extranodal sites (skin and bone)
Lymphadenopathy often peripheral and painful
CNS or bone marrow disease is rare

Question 13

Large cell Lymphomas: ALCL cytogenetics

Answer 13

Characterised by CD30 expression and t(2;5)

Question 14

NHL staging

Answer 14

(St jude system)

Question 15

St jude system

Answer 15

Stage 1
Single site or nodal area (not abdomen or mediastinum)
Stage 2
Regional nodes, abdominal disease
Stage 3
Disease on both sides of the diaphragm
Stage 4
Bone marrow or CNS disease

Question 16

Investigations:

NHL

Answer 16

1. Tissue: bone marrow aspirate; lumbar puncture; pleural and abdominal (peritoneal) fluid aspirate; exclusion biopsy.
2. Imaging: CT and positron emission tomography (PET) scans.

Question 17

Treatment of NHL

Answer 17

Lymphoblastic (T cell, pre-B cell) lymphoma is treated like ALL. Mature B cell disease is treated with short series of dose-intensive courses of chemotherapy. Risk of tumour lysis is high

Question 18

Prognosis of NHL

Answer 18

Survival is >70% (>90% in those with localized disease).

Question 19

Hodgkin’s Lymphoma:

Incidence

Answer 19

Very low before the age of 5yes and rises with age

Common in patients with pervious EBV

Question 20

Hodgkin’s Lymphoma:

Histology

Answer 20

Reed-sternberg cells in an apparently reactive lymph node infiltrate

Question 21

Hodgkin’s Lymphoma: Presentation

Answer 21

Progressive painless lymph node enlargement, the most common sites being cervical (80%) and mediastinal (60%)
Dissemination to extranodal sites is less common, lungs and bone marrow being most frequently involved

Question 22

Hodgkin’s Lymphoma: Symptoms

Answer 22

B symptoms:
• Fever
• Night sweats
Weight loss (>10%)

Question 23

Hodgkin’s Lymphoma: Subtypes

Answer 23

Two subtypes which are further classified by histology:
Subtypes:
1.	Classical HL
-	Nodular sclerosing (most common)
-	Mixed cellularity
-	Lymphocyte depleting
2.	Nodular lymphocyte-predominant HL

Question 24

Hodgkin’s Lymphoma:

Nodular lymphocyte predominant HL

Answer 24

Characterised by its distinctive histology and favourable prognosis

Question 25

Hodgkin’s Lymphoma:

Investigations:

Answer 25

• CT of neck, chest, abdomen and pelvis
• FDG PET scan
• Bone marrow aspiration and trephine
• EBV serology and ESR
• Isotope bone scan (generally done with stage Iv disease, evidence of bone pain or B symptoms

Question 26

Hodgkin’s Lymphoma: Treatment:

Answer 26

Low stage disease: radiotherapy alone
Chemotherapy usually includes alkylating agents, vinca alkaloids, anthracyclines and steroids
Radiotherapy is essential at least for bulky mediastinal or stage IV disease.

Question 27

Hodgkins Prognosis:

Answer 27

5-yr survival >90% (stage Iv, 70%; stage I, 97%)

Question 28

Hodgkins relapse

Answer 28

Cure is still possible with second line therapy, including autologous stem cell transplant.