Haem Flashcards
ALL is what?
malignancy of bone marrow. prolifetion of lymphoblasts
pathology in ALL
lymphoblasts, arrested in early stage of development, undergo malignant transformation andp proliferation, with subsequent replacement of normal marrow elemets»_space; marrow failure, infiltration into other tissues.
Assocaitions and risk factors for ALL
environmental (radiation, viruses)
Genetic: Down;s syndrome, NF1, Fanconi;s anaemia, ataxia telangiectasia
epidemiology of ALLL
most common malignancy of childhood, peak is between 2 and 5 y/o UK incidnce 1 in 70 000
History of ALL
Sx of marrow failure: Anaemia (fatigue, dyspnoea), bleeding (dbruising, bleeding gums), infections
Sx of organ infiltration: tender bones, ly,phadenopathy, mediastinal compression, abdo discomfort (hepatosplenomegaly)
Blood tests in ALL
Normochromic normocytic anaemia, low paltelets variable WCC high URIC ACID and LDH blood film: lymphoblasts Bone marrow aspirate: hypercellular with >30%lymphoblasts
cure rates in ALL
> 70.% in children, adults only a third
poor prognostic factors in ALL:
ages outside 2-10 male gender high WCC T-cell phenotype translocations (4,11) or (9,22) CNS involvement
good prognosis in ALL
translocation (12,21)
definition of neutropenic sepsis
Neutrophil count of < 0.5 x 109/l
Or < 1.0 x 109/l with a predicted decrease to 0.5 x 109/l
WITH
a temperature >38ºC on two occasions, at least 1 hour apart within a 12 hour period,
Or
a single temperature of > 38.5ºC
causes of neutropenia
Following chemotherapy
New haematological malignancy i.e. Acute leukaemia
Following drugs i.e. Azathioprine, 6MP, MTX,
Autoimmune
Sepsis
Inherited
Racial variant
presentation of neutropenia
Fever / cough / cold Sepsis Shock; sys BP 50mmHG in hypertensive patient Acute respiratory syndrome Disseminated intra-vascular coagulation Bilateral diffuse infiltrates on CXR Multiple organ failure Gram negative sepsis can kill within hours
ICU care indications in neutropenics
lactate ≥4,
base excess ≥ -10,
Scv02 85%
neturopenic diet
NO: salads unpeeled fruit uncooked veg soft cheeses live yogurts
Myeloma – diagnostic criteria
Bone marrow plasma cells >10%
•And/or serum paraprotein >30 g/l
•Incurable disease so only treat if symptomatic
features that suggest symptomatic myelo
Calcium Elevation in Blood (>2.75 mmol/l)
Renal Insufficiency related to myeloma (creatinine >173 mmol/l)
Anaemia related to myeloma (Hb 2 in previous 12 months)
myeloma investigaitons
FBC, ESR, plasma viscosity U&Es, Ca2+, albumin •Immunoglobulins •Serum protein Electrophoresis •Serum free light chains •Urinary Bence Jones Protein •Urinary protein (24 hour) •Bone marrow biopsy •Skeletal survey
typical myeloma pathological features re diagnosis and survival
- Diagnosed on BM and serum / urine monoclonal protein (paraprotein)
- Ig G > IgA > Ig M
- Median age ~70
- Median survival with conventional treatment ~5 years
lymphoma staging
Needs CT whole body & bone marrow for staging
•Staging (CT or PET)
–Stage 1 – single nodal site
–Stage 1E – single extra nodal site
–Stage 2 – 2 or more sites on one side of diaphragm
–Stage 3 – both sides of diaphragm
–Stage 4 – extra nodal sites (BM, liver etc.)
–A or B – if B symptoms
polycythaemia vera
- Headaches, shortness of breath, blurred vision, itch
- Plethoric appearance
- Enlarged spleen
- Hemorrhage or thrombosis
- Gout
the most common cause of inherited haemolytic anaemia in N Europeans
hereditary spherocytosis, nhertied Autosomal DOminant. Defect in RBC membrane - deficiency of protein spectrin. RBCs become rigind, elss deformable, destroyed prematurely in spleen
how hereditary spherocytosis may present?
with jaundice (pigment gallstoens0, or incidentally detected w/ anaemia, splenomegaly and leg ulcers can have apastic crisis due to parvovirus B19
