Haem

Question 1

ALL is what?

Answer 1

malignancy of bone marrow. prolifetion of lymphoblasts

Question 2

pathology in ALL

Answer 2

lymphoblasts, arrested in early stage of development, undergo malignant transformation andp proliferation, with subsequent replacement of normal marrow elemets&raquo_space; marrow failure, infiltration into other tissues.

Question 3

Assocaitions and risk factors for ALL

Answer 3

environmental (radiation, viruses)

Genetic: Down;s syndrome, NF1, Fanconi;s anaemia, ataxia telangiectasia

Question 4

epidemiology of ALLL

Answer 4

most common malignancy of childhood, peak is between 2 and 5 y/o UK incidnce 1 in 70 000

Question 5

History of ALL

Answer 5

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)

Question 6

Blood tests in ALL

Answer 6

Normochromic normocytic anaemia,
low paltelets
variable WCC
high URIC ACID and LDH
blood film: lymphoblasts
Bone marrow aspirate: hypercellular with >30%lymphoblasts

Question 7

cure rates in ALL

Answer 7

> 70.% in children, adults only a third

Question 8

poor prognostic factors in ALL:

Answer 8

ages outside 2-10
male gender
high WCC
T-cell phenotype
translocations (4,11) or (9,22)
CNS involvement

Question 9

good prognosis in ALL

Answer 9

translocation (12,21)

Question 10

definition of neutropenic sepsis

Answer 10

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

Question 11

causes of neutropenia

Answer 11

Following chemotherapy
New haematological malignancy i.e. Acute leukaemia
Following drugs i.e. Azathioprine, 6MP, MTX,
Autoimmune
Sepsis
Inherited
Racial variant

Question 12

presentation of neutropenia

Answer 12

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

Question 13

ICU care indications in neutropenics

Answer 13

lactate ≥4,
base excess ≥ -10,
Scv02 85%

Question 14

neturopenic diet

Answer 14

NO: salads
unpeeled fruit
uncooked veg
soft cheeses
live yogurts

Question 15

Myeloma – diagnostic criteria

Answer 15

Bone marrow plasma cells >10%
•And/or serum paraprotein >30 g/l
•Incurable disease so only treat if symptomatic

Question 16

features that suggest symptomatic myelo

Answer 16

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)

Question 17

myeloma investigaitons

Answer 17

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

Question 18

typical myeloma pathological features re diagnosis and survival

Answer 18

• Diagnosed on BM and serum / urine monoclonal protein (paraprotein)
• Ig G > IgA > Ig M
• Median age ~70
• Median survival with conventional treatment ~5 years

Question 19

lymphoma staging

Answer 19

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

Question 20

polycythaemia vera

Answer 20

• Headaches, shortness of breath, blurred vision, itch
• Plethoric appearance
• Enlarged spleen
• Hemorrhage or thrombosis
• Gout

Question 21

the most common cause of inherited haemolytic anaemia in N Europeans

Answer 21

hereditary spherocytosis, nhertied Autosomal DOminant. Defect in RBC membrane - deficiency of protein spectrin. RBCs become rigind, elss deformable, destroyed prematurely in spleen

Question 22

how hereditary spherocytosis may present?

Answer 22

with jaundice (pigment gallstoens0, or incidentally detected w/ anaemia, splenomegaly and leg ulcers can have apastic crisis due to parvovirus B19