Haem Flashcards

1
Q

ALL is what?

A

malignancy of bone marrow. prolifetion of lymphoblasts

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2
Q

pathology in ALL

A

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.

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3
Q

Assocaitions and risk factors for ALL

A

environmental (radiation, viruses)

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

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4
Q

epidemiology of ALLL

A

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

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5
Q

History of ALL

A

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)

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6
Q

Blood tests in ALL

A
Normochromic normocytic anaemia,
low paltelets
variable WCC
high URIC ACID and LDH
blood film: lymphoblasts
Bone marrow aspirate: hypercellular with >30%lymphoblasts
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7
Q

cure rates in ALL

A

> 70.% in children, adults only a third

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8
Q

poor prognostic factors in ALL:

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

good prognosis in ALL

A

translocation (12,21)

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10
Q

definition of neutropenic sepsis

A

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

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11
Q

causes of neutropenia

A

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

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12
Q

presentation of neutropenia

A
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
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13
Q

ICU care indications in neutropenics

A

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

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14
Q

neturopenic diet

A
NO: salads
unpeeled fruit
uncooked veg
soft cheeses
live yogurts
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15
Q

Myeloma – diagnostic criteria

A

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

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16
Q

features that suggest symptomatic myelo

A

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)

17
Q

myeloma investigaitons

A
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
18
Q

typical myeloma pathological features re diagnosis and survival

A
  • Diagnosed on BM and serum / urine monoclonal protein (paraprotein)
  • Ig G > IgA > Ig M
  • Median age ~70
  • Median survival with conventional treatment ~5 years
19
Q

lymphoma staging

A

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

20
Q

polycythaemia vera

A
  • Headaches, shortness of breath, blurred vision, itch
  • Plethoric appearance
  • Enlarged spleen
  • Hemorrhage or thrombosis
  • Gout
21
Q

the most common cause of inherited haemolytic anaemia in N Europeans

A

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

22
Q

how hereditary spherocytosis may present?

A

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