Haematology 5 Flashcards
What is Hodgkin lymphoma?
• Classically presents with painless lymphadenopathy- most frequently in the neck (may cause airway obstruction)
Systemic symptoms are uncommon
• Lymph node biopsy, radiological assessment of all nodal sites and bone marrow biopsy, used to stage disease and determine treatment
• Combination chemotherapy with or without radiotherapy- positron emission tomography (PET) scanning is used in the UK to monitor treatment response and guide further management
• Overall, about 80% of all patients can be cured, even with disseminated disease, about 60% can be cured
What is non-hodgkin lymphoma?
• T-cell malignancies may present as acute lymphoblastic leukaemia or non-Hodgkin lymphoma, with both being characterised by a mediastinal mass with varying degrees of bone marrow infiltration, the mediastinal mass may cause superior vena caval obstruction
• B-cell malignancies present more commonly as non-Hodgkin lymphoma- with localised lymph node disease usually in the head and neck or abdomen
Pain from intestinal obstruction, a palpable mass, intussusception
What are the investigations and management for non-hodgkin lymphoma?
biopsy, radiological assessment of all nodal sites (CT or MRI) and examination of the bone marrow and CSF
• Management: multi-agent chemotherapy- the majority of patients now do well and survival rates of over 80% are expected for both T- and B-cell disease
What are the differences between non-hodgkin’s and Hodgkin’s lymphoma?
- Hodgkin’s lymphoma has reed-sternberg cells
- Hodgkin’s lymphoma often starts in the upper body
- Hodgkin’s spreads very slowly and is very receptive to chemotherapy and radiotherapy
When is chemotherapy used?
o as primary curative treatment e.g. in acute lymphoblastic leukaemia
o to control primary or metastatic disease before definitive local treatment with surgery and/or radiotherapy- e.g. in sarcoma or neuroblastoma
o as adjuvant treatment to deal with residual disease and to eliminate presumed micrometastases-
e.g. after initial local treatment with surgery in Wilms tumour
What are the side effects of chemotherapy?
o Hair loss o Anaemia o Infection o Bruising o Sore mouth o Nausea o Vomiting o Mood changes o Irritability o Weight gain
What are the long term side effects of chemotherapy?
o Delayed puberty o Reduced fertility o Reduced growth o Neurotoxicity o Hepatotoxicity o Renal toxicity o Cardiotoxicity o Pulmonary toxicity o Secondary cancer o Psychological effects
When are bone marrow transplants used?
• Transplantation of bone marrow stem cells can be used as a strategy to intensify the treatment of patients with the administration of potentially lethal doses of chemotherapy and/or radiation, the source of the marrow stem cells may be
o Allogeneic- from a compatible donor
o Autologous- from the patient him/herself, harvested beforehand, while the marrow is uninvolved or in remission
• Allogeneic transplantation is principally used in the management of high-risk or relapsed leukaemia and autologous stem cell support is used most commonly in the treatment of children with solid tumours whose prognosis is poor using conventional chemotherapy e.g. advanced neuroblastoma.
What is a neuroblastoma?
• Neuroblastoma and related tumours arise from neural crest tissue in the adrenal medulla and sympathetic nervous system- it is a biologically unusual tumour in that spontaneous regression sometimes occurs in very young infants
spectrum of disease from the benign (ganglioneuroma) to the highly malignant (neuroblastoma)-
neuroblastoma is most common before the age of 5 years.
What are the clinical features of neuroblastoma?
Abdominal mass
primary tumour can lie anywhere along the sympathetic chain from the neck to the pelvis
tumour mass is often large and complex, crossing the midline and enveloping major blood vessels and lymph nodes
• Paravertebral tumours may invade through the adjacent intervertebral foramen- causes spinal cord compression
What are the symptoms of a neuroblastoma?
o Pallor o Weight loss o Abdominal mass o Hepatomegaly o Bone pain o Limp Less common o Paraplegia o Cervical lymphadenopathy o Proptosis o Periorbital bruising o Skin nodules
What are the investigations for a neuroblastoma?
- Characteristic clinical and radiological features with raised urinary catecholamine levels suggest neuroblastoma, confirmatory biopsy is usually obtained and evidence of metastatic disease detected with bone marrow sampling, MIBG (metaiodobenzyl-guanidine) scan with or without a bone scan
- Overexpression of the N-myc oncogene- evidence of deletion of material on chromosome 1 (del 1p) and gain of genetic material on chromosome 17q in tumour cells are all associated with a poorer prognosis
What is the management for a neuroblastoma?
- Localised primaries without metastatic disease can often be cured with surgery alone
- Metastatic disease is treated with chemotherapy- including high-dose therapy with autologous stem cell rescue, surgery and radiotherapy
- Risk of relapse is high and the prospect of cure for children with metastatic disease is still little better than 30%- immunotherapy and the use of long-term ‘maintenance’ treatment with differentiating agents (retinoic acid) are now establishing a role in those with high-risk disease
What are the clinical features of Wilms tumour?
Large abdominal mass o Abdominal pain o Anorexia o Anaemia- haemorrhage into mass o Haematuria o Hypertension
How is Wilms tumour diagnosis?
- Ultrasound and/or CT/MRI is usually characteristic- showing an intrinsic renal mass distorting the normal structure
- Staging is to assess for distant metastases (usually in the lung), initial tumour respectability and function of the contralateral kidney
