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Flashcards in Paeds: Lymphoma Deck (28):
1

NHL Incidence

10 per million

2

NHL Classification

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

3

Lymphoblastic Types

90% T-cell
10% pre-B

4

Lymphoblastic Presentation

Most present with an anterior mediastinal mass
Disease may be present in bone, bone marrow, skin, CNS, liver, kidneys and spleen.

5

Lymphoblastic Cytogenetics

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

6

Lymphoblastic Note

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

7

Mature B cell Definition

Burkitt or Burkitt like

8

Mature B cell Presentation

Occur in the abdomen, head and neck, bone marrow and CNS
May grow rapidly

9

Mature B cell Association

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

10

Mature B cell Cytogenetics

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

11

Large cell Lymphomas: Subtypes

Diffuse large B cell (BLCL)
Large cell lymphoma (ALCL)

12

Large cell Lymphomas: Presentation

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

13

Large cell Lymphomas: ALCL cytogenetics

Characterised by CD30 expression and t(2;5)

14

NHL staging

(St jude system)

15

St jude system

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

16

Investigations:
NHL

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

17

Treatment of NHL

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

18

Prognosis of NHL

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

19

Hodgkin’s Lymphoma:
Incidence

Very low before the age of 5yes and rises with age
Common in patients with pervious EBV

20

Hodgkin’s Lymphoma:
Histology

Reed-sternberg cells in an apparently reactive lymph node infiltrate

21

Hodgkin’s Lymphoma: Presentation

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

22

Hodgkin’s Lymphoma: Symptoms

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

23

Hodgkin’s Lymphoma: Subtypes

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

24

Hodgkin’s Lymphoma:
Nodular lymphocyte predominant HL

Characterised by its distinctive histology and favourable prognosis

25

Hodgkin’s Lymphoma:

Investigations:

- 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

26

Hodgkin’s Lymphoma: Treatment:



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.

27

Hodgkins Prognosis:

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

28

Hodgkins relapse

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

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