Paeds: Lymphoma Flashcards
NHL Incidence
10 per million
NHL Classification
- Lymphoblastic (30% of NHL)
- Mature B cell (30% of NHL)
Large cell Lymphomas (15-20% of NHL)
Lymphoblastic Types
90% T-cell
10% pre-B
Lymphoblastic Presentation
Most present with an anterior mediastinal mass
Disease may be present in bone, bone marrow, skin, CNS, liver, kidneys and spleen.
Lymphoblastic Cytogenetics
T (1;14) or t (11;14)
Lymphoblastic Note
Cases with >25% blasts in bone marrow are regarded as leukaemia
Mature B cell Definition
Burkitt or Burkitt like
Mature B cell Presentation
Occur in the abdomen, head and neck, bone marrow and CNS
May grow rapidly
Mature B cell Association
Endemic or African Burkitt’s associated with early EBV infection and frequently affects the jaw.
Mature B cell Cytogenetics
Expresses surface immunoglobulin and characteristic translocations t(8;14), t (8;22), or t(2;8)
Large cell Lymphomas: Subtypes
Diffuse large B cell (BLCL)
Large cell lymphoma (ALCL)
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
Large cell Lymphomas: ALCL cytogenetics
Characterised by CD30 expression and t(2;5)
NHL staging
(St jude system)
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
Investigations:
NHL
- Tissue: bone marrow aspirate; lumbar puncture; pleural and abdominal (peritoneal) fluid aspirate; exclusion biopsy.
- Imaging: CT and positron emission tomography (PET) scans.
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
Prognosis of NHL
Survival is >70% (>90% in those with localized disease).
Hodgkin’s Lymphoma:
Incidence
Very low before the age of 5yes and rises with age
Common in patients with pervious EBV
Hodgkin’s Lymphoma:
Histology
Reed-sternberg cells in an apparently reactive lymph node infiltrate
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
Hodgkin’s Lymphoma: Symptoms
B symptoms:
• Fever
• Night sweats
Weight loss (>10%)
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
Hodgkin’s Lymphoma:
Nodular lymphocyte predominant HL
Characterised by its distinctive histology and favourable prognosis
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
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.
Hodgkins Prognosis:
5-yr survival >90% (stage Iv, 70%; stage I, 97%)
Hodgkins relapse
Cure is still possible with second line therapy, including autologous stem cell transplant.
