Leukaemia, Brain tumours, Lymphoma Flashcards
What are the 4 main subtypes of leukaemia?
1) Acute lymphoblastic leukaemia (ALL) - 80% (most common)
2) Acute myeloid leukaemia (AML)
3) Chronic myeloid leukaemia (CML)
4) Chronic lymphocytic leukaemia (CLL)
What is leukaemia?
- Leukaemia is the presence of rapidly proliferating immature blast blood cells (RBC, WBC or platelets) in the bone marrow - non functional.
- 2 problems: leukaemia cells rapidly dividing providing no function - wasting energy for useful cell production, and due to rapid replication these cells take up space in bone marrow meaning there is little space within bone marrow for other cells to grow.
- Bone marrow produces few functioning blood cells - symptoms of leukaemia.
- When there is no space in bone marrow - leukaemia cells present in blood too.
- ANY AGE, Acute lymphoblastic mainly seen in childhood, CLL seen in elderly.
Acute lymphoblastic anaemia (ALL) Ex, RF, PPx?
Ex - 80% of leukaemia, rest tend to have AML. Peak incidence - 2-5yrs.
RF - genetics, ionising radiation (x-rays during pregnancy)< Down’s syndrome.
PPx - Malignancy of immature lymphoid cells (gives rise to T and B cells)
Clinical presentation of ALL? Ddx?
- Presents insidiously over several weeks, more acute presentation and progression in some.
- Malaise and anorexia
1) Bone marrow infiltration gives rise to pancytopenia:
thrombocytopenia - bruising, petechiae, nose bleeds, leukopenia - infection, anaemia - pallor and lethargy, and bone pain.
2) Hepatomegaly and Splenomegaly
Other organ infiltration during relapse: CNS - headaches, vomiting and nerve palsies, testes - ENLARGEMENT (common site of relapse).
Ddx: aplastic anaemia, ITP
Diagnosis of acute lymphoblastic leukaemia?
1) FBC - Abnormal - Low Hb, thrombocytopenia, circulating leukaemia blast cells
2) Bone marrow aspirate - DIAGNOSTIC (assess immunological phenotype 75% B-cell and 15% T-cell).
3) CXR - Mediastinal mass characteristic of T-cell disease
3 steps before chemotherapy for ALL?
1) Blood and platelet transfusion to correct anaemia before starting therapy
2) Prophylactic antivirals, antifungals and antibacterial due to neutropenia (Co-trimoxazole)
3) Allopurinol - protect renal function against effects of rapid cell lysis
5-phase chemotherapy?
1) Induction - 4-week 3 drug chemo: combination of Vincristine, Dexamethasone, L-Asparginase.
2) Consolidation and CNS protection - Methotrexate, Cytarabine, Hydrocortisone (intra-thecal therapy)
3) Interim maintenance - therapy of modest intensity is continued up to 3 years.
4) Delayed intensification
5) Maintenance
Relapse - high dose chemotherapy, total body irradiation, bone marrow transplant
Brain tumours in children?
- Almost always primary (compared to adults) - mostly infratentorial
- Astrocytoma (most common) - glioblastoma multiform most malignant, medulloblastima, ependyma, brainstem glioma.
Clinical presentation of brain tumours in infants?
- Raised ICP Infants: 1) Vomiting 2) Separation of cranial sutures/tense fontanelle 3) Increased head circumference 4) Head tilt/posturing 5) Developmental delay/regression
Clinical presentation of brain tumours in children/adolescents?
- Raised ICP
1) Headache worse in the morning
2) Vomiting - especially on waking and in the morning
3) Behavioural change/personality change
4) Ataxia
5) Visual disturbances
6) Papilloedema
Spinal tumours - back pain, peripheral weakness of arms/legs/bladder/bowel dysfunction
Diagnosis of brain tumours in children?
1) MRI
2) Magnetic resonance spectroscopy - to examine tumour biological activity
3) AVOID LP - unless with neurosurgical advice due to increased risk of coning due to raised ICP
Treatment of brain tumours in children?
1) Surgery to achieve maximum resection - dependant on anatomical position of tumour (CANNOT REMOVE BRAINSTEM TUMOUR)
2) Radiotherapy - for older children - high energy X-rays, cannot be used in younger children as it causes damage to developing nervous system. (often used following surgery)
3) Chemotherapy increasingly being used but many agents unable to penetrate blood brain barrier.
What is lymphoma?
- Malignancies of cells of the immune system resulting in proliferations of lymphocytes, these lymphocytes accumulate in lymph nodes resulting in lymphadenopathy.
- Non-Hodgkins more common in childhood, Hodgkins more common in adolescents.
Hodgkins lymphoma Ex, RF?
- More common in adolescents 13-19yrs, ALL have Reed-Sternberg (popcorn) cells.
RF: EBV, SLE
Clinical presentation of Hodgkins lymphoma?
1) Painless lymphadenopathy (most frequently in neck) – larger, firmer, ‘rubbery’ than benign lymphadenopathy seen in children.
2) Airway obstruction - breathlessness and cough (lymphadenopathy in mediastinum)
3) B-SYMPTOMS - weight loss, fever, and sweating (uncommon)
4) Clinical history over several months
Ddx: Benign lymphadenopathy due to bacterial/viral infection
Diagnosis of Hodgkins lymphoma?
1) Lymph node biopsy - particularly if unusual site (supraclavicular e.g.) - REED-STERNBERG CELLS (popcorn cells - large B-cells that have multiple nuclei that have nucleoli inside them - owl face).
2) CT/MRI of chest, abdomen, and pelvis for ANN ARBOR STAGING
3) Bone marrow biopsy
Treatment of Hodgkins lymphoma?
1) Combination chemotherapy with or without radiotherapy
2) Positive emission tomography (PET) scanning is used to monitor treatment response.
Non-Hodgkin’s lymphoma Ex and RF?
- Around 80% B-cell in origin and 20% T-cell
- Includes ALL lymphomas without Reed-Sternberg cells
RF: EBV, Burkitts lymphoma (presents with jaw lymphadenopathy), FH
Clinical presentation of Non-Hodgkin’s?
- B-cell malignancy: Localised lymph node disease usually in head, neck or abdomen
- T-cell malignancy: Mediastinal mass (breathless and cough), varying degrees of bone marrow infiltration
- Abdominal disease presents with pain from intestinal obstruction and a palpable mass or even intussusception in cases with ileum involvement. (PAIN AND PALPABLE MASS)
- Fever, night sweats, weight loss
Ddx: Hodgkin’s, ALL
Diagnosis of Non-Hodgkins?
1) Lymph node biopsy - WILL NOT SEE Reed-Sternberg cells
2) Bone marrow biopsy - classification
3) CT/MRI of chest, abdomen and pelvis for Ann Arbor staging
Treatment for Non-Hodgkins?
Milti-agent chemotherapy
Ann Arbor staging for both Hodgkins and Non-hodgkins?
Stage 1 - Confined to one region of lymph nodes
Stage 2 - In more than one region but 1 side of the diaphragm (above or below)
Stage 3 - Affects lymph nodes above and below diaphragm
Stage 4 - Widespread involvement including non-lymphatic organs such as lungs or liver.
