Level 2 - Haematology COPY Flashcards
Most common brain tumours? How common?
- Mostly primary and 60% intratentorial (below cerebrum)
- Most common solid tumour in children
Name the different types of brain tumours?
Astrocytoma
Medulloblastoma
Ependymoma
Brainstem glioma
Craniopharyngioma
Most common brain tumour?
Astrocytoma
What is astrocytoma?
In cortex
Varies from benign to malignant (glioblastoma multiforme)
What is medulloblastoma?
Arises in midline of posterior fossa
May seed through CNS via CSF and often have spinal metastases
What is ependymoma?
Mostly in posterior fossa, behaves like medulloblastoma
What is craniopharyngioma?
Midline
Arises from squamous remnant of Rathke pouch
Locally invasive but not malignant
General symptoms of brain tumours?
- Related to raised ICP o Headaches (worse in the morning), vomiting, behaviour changes, visual disturbance, papilloedema o Separation of sutures, increased head circumference
Focal neurological signs in brain tumours?
o Supratentorial
Seizures, hemiplegia, focal signs
o Midline
Visual field loss, growth failure, diabetes insipidus, weight gain
o Cerebellar
Truncal ataxia, abnormal eye movements, coordination difficulties
o Brainstem
CN defects, pyramidal signs
Symptoms in spinal tumours?
o Back pain, peripheral weakness of arms and legs, bladder/bowel dysfunction depending on level of lesion
Investigations in brain tumours?
- MRI scan
- Magnetic Resonance spectroscopy
- NO LP IF RAISED ICP
Management of brain tumours?
- Urgent referral is unexplained headache and focal symptoms
- Surgery
o Aimed to treat hydrocephalus, provide tissue diagnosis and resection
- Radiotherapy and Chemotherapy depending on tumour type and size
Definition of haemophilia? Types?
- X-linked recessive blood disorder
- Haemophilia A = Factor VIII deficiency (most common)
- Haemophilia B = Factor IX deficiency
- Usually carrier females and affected males
Epidemiology of haemophilia?
- Most common inherited coagulation disorder
o Haemophilia A – 1 in 5000 live male births
o Haemophilia B - 1 in 30000 live male births
- Males affected more common
Presentation of haemophilia?
- Most present towards end of first year of life
- Can present in neonatal period with intracranial haemorrhage, bleeding post-circumcision, oozing heel prick
Grading of haemophilia?
- Graded:
o Severe: Spontaneous joint/muscle bleeds, arthropathy/arthritis, haematomas
o Moderate: Bleed after minor trauma
o Mild: Bleed after surgery
Investigations of haemophilia?
- Diagnose by increased APTT and decreased FVIII/IX assay
- Can perform CT head or USS to identify extent of bleeding
- Prenatal DNA analysis available
Management of haemophilia?
- Minor bleeds
o Pressure and elevation
o Desmopressin raises Factor VIII
- Major bleeds
o Recombinant Factor VIII/IX concentrate IVI
Home treatment can be taught
To 50% of normal (life-threatening bleeds need levels of 100%)
Best management approach and what to avoid?
- MDT approach , specialised physio to strengthen muscles and limit damage, psychosocial support and self-help groups
- Avoid IM injections, NSAIDs and aspirin
Complications of haemophilia treatment?
- Inhibitors (antibodies to Factor VIII/IX)
o Reduce or inhibit effect of treatment
- Transfusion transmitted infections
What is HSP?
Henoch Schönlein Purpura
- Acute IgA immune complex mediated vasculitic condition, causing medium sized arteries to become inflamed
- The IgA and IgG interact to produce complexes that activate complement and are deposited in affected organs leading to inflammatory response – vasculitis
Epidemiology of HSP?
- Occurs between 3-10 years
- Boys 2:1
- Peaks during winter
Aetiology of HSP?
- Unknown – potentially genetic disposition and antigen exposure
- Infections: for example, Group A streptococci, mycoplasma, Epstein-Barr virus
- Environmental exposures: for example, drug and food allergens, pesticides, cold exposure, insect bites
What precedes HSP in most cases?
- Preceding URTI in 50-90% cases
Symptoms of HSP?
Skin rash Arthralgia Colicky abdo pain Renal nephropathy Scrotal oedema
Features of skin rash in HSP?
Palpable purpura symmetrically over the lower limbs
Commonly extensor surfaces and buttocks
It may also involve arms, face and ears but usually spares the trunk
Features of arthralgia in HSP?
o Knees and ankles mainly o 2/3 of patients and usually resolves
Features of abdo pain in HSP?
o Occurs commonly
o Can cause haematemesis, and melaena
o Intussusception (2/3%)
Features of renal nephropathy in HSP?
o 80% of cases have micro/macro-scopic haematuria or mild proteinuria
o Complete recovery is normal but if proteinuria is severe, nephritic syndrome may occur - PROTEINURIA, HYPOALBUMINAEMIA, OEDEMA
DDx of HSP?
- Connective tissue diseases
- eg, systemic lupus erythematosus (SLE).
- Other causes of purpuric rash
- eg, thrombocytopenia, leukaemia, ITP
- Other causes of glomerulonephritis
Investigations of HSP?
- Bedside Tests
o Mid-stream urine dipstick – proteinuria, +/- haematuria
o BP - Blood Tests o FBC (ESR, WCC), U&E, IgA
- Imaging
o Renal US and Biopsy if renal complications
o Abdominal US if needed
Management of HSP? Complications?
- Most resolve in <2 months
- Usually self-limiting, regular paracetamol and NSAIDs may help joint pain but caution in renal insufficiency
- If severe, prednisolone may help resolve abdominal pain
- May require admission to hospital for monitoring of abdominal and renal complications
- Chronic renal failure occurs in 5%
What is leukaemia?
- Cancer of the white blood cells
- Arises from malignant proliferation of myeloid, lymphoid, pre-B or T-cell lymphoid precursors
What are the types of leukaemia?
o Acute lymphoblastic leukaemia (ALL), chronic lymphocytic leukaemia (CLL)
o Acute myeloid leukaemia (AML), chronic myeloid leukaemia (CML)
Characteristics of acute leukaemia?
o Acute characterised by rapid increase in number of immature blood cells
Characteristics of chronic leukaemia?
o Chronic characterised by build-up of relatively mature but still abnormal white blood cells
What are lymphoblastic cells?
o Lymphoblastic/cytic are of lymphocytes, mainly B-cells
What are myeloid cells?
o Myeloid are marrow cells that go on to form red blood cells, platelets
Define acute lymphblastic leukaemia?
- Acute lymphoblastic leukaemia (ALL) defined as rapidly progressing, aggressive which requires immediate treatment
What is Burkitt leukaemia?
- Burkitt leukaemia is mature B-cell ALL
Epidemiology of leukaemia? Peak age?
- ALL accounts for 80% of leukaemias
- 25% of all malignancies
- Peak age 2-6 years
- Incidence highest in white children
Aetiology of leukaemia?
o Unknown mostly
o Genetic components
Down’s syndrome increased risk
Philidelphia chromosome occurs in 15-30%
- t(9:22) and is associated with poor prognosis
o Environmental Factors
Prenatal exposure to x-rays
In utero infection
Symptoms of leukaemia?
- Duration usually 2-4 weeks
- Symptoms
o Pancytopenia (pallor, infection, bleeding)
o Fatigue
o Anorexia
o Fever
o Bone Pain
o Headaches, vomiting
Signs of leukaemia?
o Painless lumps in neck, axilla, groin
o Anaemia
o Neutropenia
o Thrombocytopenia
o Hepatosplenomegaly
o Orchidomegaly
Investigations of leukaemia?
- Bloods
o FBC (increased WCC, normochromic normocytic anaemia, low platelets)
o Increased urate and LDH
- Bone Marrow biopsy
o Nucleated cells will be blasts
- Chest X-ray
- Cytogenic analysis
Risk Classification in leukaemia?
- Risk Classification
o Low, standard, high risk groups
o Depends on clinical signs, biologic features of lymphoblasts and response to induction chemotherapy
Management of leukaemia?
Induction phase
Consolidation phase
Maintenance phase
Management in induction phase?
3-drug induction over 4 weeks
Intrathecal methotrexate, cytarabine and hydrocortisone
Remission in >95%
High-risk patients may need daunorubicin
Management in consolidation phase?
Further chemotherapy
Cranial irradiation if CNS signs
Management in maintenance phase?
For 2.5 years daily mercaptopurine, weekly methotrexate + vincristine
Complications in leukaemia?
- Neutropenic Sepsis
- Hyperuricaemia
- Poor growth
- Cancer elsewhere
- Relapses
Definiton of lymphoma?
- Malignancy of lymphocytes which can be divided into Hodgkin and non-Hodgkin lymphoma
What is Hodgkin’s lymphoma?
- Hodgkin’s lymphoma is a malignant tumour of the lymphatic system that is characterised histologically by the presence of multinucleated giant cells (Reed-Sternberg cells)
What is non-Hodgkin lymphoma?
- Non-Hodgkin’s lymphomas (NHLs) are an heterogeneous group of lymphoproliferative malignancies. More likely to disseminate to extranodal sites than in Hodgkin’s lymphoma
When is HL and NHL more common?
- NHL more common in childhood
- Hodgkin lymphoma seen more in adolescents
Types of HL?
Classical HL (95%) – nodular sclerosis, mixed cellularity, lymphocyte rich/depleted
Nodular lymphocyte-predominant Hodgkin’s lymphoma (NLPHL)
Risk factors for HL?
EBV, HIV, immunosuppression, cigarette smoking
Types of NHL?
Mature (peripheral) B-cell neoplasms
- Diffuse large B-cell lymphoma (30-60%)
- Mediastinal large B cell
- Burkitt’s lymphoma
- Follicular lymphoma (20-25%)
- MALT lymphoma
Precursor T-cell neoplasms
Mature (peripheral) T-cell neoplasms
- Enteropathy-type T-cell lymphoma
- Peripheral T-cell lymphoma
Risk factors of NHL?
Chromosomal translocations
EBV, HepC, Kaposi’s sarcoma
Pesticides, herbicides, hair dye, chemo
Hashimoto’s thyroiditis
H.pylori
Symptoms of HL? What are the B symptoms?
o Painless, large, firm lymphadenopathy
o May cause airway obstruction, SVC obstruction
o Several months
o Systemic B-symptoms
Sweating, pruritus, night sweats, weight loss, fever
Symptoms of NHL? B symptoms?
o Mediastinal mass with bone marrow infiltration
o Superior vena cava obstruction
o Lymphadenopathy
o Abdominal pain
o Systemic B-symptoms
Sweating, pruritus, night sweats, weight loss, fever
Investigations in lymphoma?
- Lymph node biopsy
- Bloods
o FBC, film, ESR, LFT, LDH, urate, Ca
- Imaging
o CXR, CT
Staging in HL?
o Ann Arbor system
Stage 1: single site
Stage 2: >1 site and on 1 side
Stage 3: on both sides of diaphragm
Stage 4: disseminated disease
Staging in NHL?
o St. Jude System
Stage 1: single site or nodal area (not abdomen or mediastinum)
Stage 2: regional nodes, abdominal
Stage 3: disease on both sides of diaphragm
Stage 4: bone marrow or CNS disease
Management of HL?
o Chemotherapy (ABVD) with radiotherapy (if high risk)
o PET scans monitor disease progression
Management of NHL?
o Chemotherapy (R-CHOP regimen) with radiotherapy
Prognosis of lymphomas?
- 5-year survival rate >90% (NHL 70%)
Definition of sickle cell anaemia?
- Inherited (autosomal recessive) blood disorder in which red blood cells develop abnormally
Pathology of sickle cell disease?
- Abnormal beta-globin chain that causes it to polymerize when deoxygenated, which distorts the erythrocyte into a sickle shape
- Deformed erythrocytes form clusters which block blood vessels
What benefit does heterozygous form of sickle cell disease have?
- Heterozygous form protects against malaria; is most common in people of black tropical descent
Epidemiology of sickle cell disease? Where is highest prevalence?
- Most common haemoglobinopathies
- 1 in every 2000 live births in England, and it is now the most common genetic condition at birth
- Highest prevalence of sickle cell disease is among Black African and Black Caribbean people
- Mortality rate 3% in childhood
Aetiology of sickle cell disease?
- Genetic, autosomal recessive – can be carrier or homogenous
Symptoms of sickle cell disease?
Moderate anaemia usually with jaundice due to chronic haemolysis Increased susceptibility to encapsulated organisms (pneumococci, haemophilus influenzae, OSTEOMYELITIS due to Salmonella ) Vaso-occlusive crises Acute anaemic crises Priapism
- Needs prompt transfusion Splenomegaly
Features of vaso-occlusive crises?
- Precipitated by cold, dehydration, excessive exercise/stress, hypoxia or infection
- Pain affecting many organs
- Dactylitis with swelling in hands/feet
- Commonest in limbs and spine
- Acute chest syndrome
o Severe hypoxia, need ventilation and transfusion
- Avascular necrosis
Investigations for sickle cell disease? What about in a acute crisis?
Newborns screened – Guthrie test
Blood Tests - FBC, reticulocyte count
Blood film Hb electrophoresis (HPLC)= definitive test
Acute crisis investigations: Hb decreased, reticulocytes increased, blood culture, U&E, creatinine, LFT, CRP, CXR
Prophylaxis treatment in sickle cell disease?
- Up to date with all immunisations
- Oral penicillin given to prevent infection
- Oral folic acid
- Hydroxycarbamide in patients with severe disease
Acute sickle cell crisis treatment? General measures? Drug treatment? When to transfuse?
- Avoid other triggers such as cold weather and excessive physical activity.
- Use distraction techniques, such as games, computers, and television
- Increase fluid intake (150% IV or oral)
- Prescribe paracetamol and/or ibuprofen (avoid ibuprofen if the person has renal impairment or significant proteinuria). Add codeine phosphate if these are not effective
- Abx given if there is infection
- Oxygen to improve saturations
- Transfusion for acute chest syndrome, stroke, priapism
Management of sickle cell trait?
- Very rarely have symptoms
- Avoid high altitudes, such as travelling in an unpressurized aircraft.
- Inform anaesthetist of sickle cell carriers
Complications of sickle cell disease?
- Short stature and delayed puberty
- Stroke
- Adenotonsillar hypertrophy
- Cardiac enlargement and heart failure
- Renal dysfunction
- Pigment Gallstones
- Psychosocial problems
