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FCRAP > Haematology > Flashcards

Haematology Flashcards

1
Q

Causes of microcytic anaemia

A 
Iron deficiency
Thalassaemia
Lead poisoning
Sideroblastic anaemia (congenital)
Anaemia of chronic disease
Hyperthyroidism
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2
Q

What are HbH and where do you see them?

A

Excess beta chains

Sees in alpha thalassaemia

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3
Q

Philadelphia Chromosome

A

t(9;22) in CML; imatinib; adverse risk ALL

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4
Q

Splenectomy vaccinations

A
  • if elective, should be done 2 weeks prior to operation
  • Hib, meningitis A & C
  • annual influenza vaccination
  • pneumococcal vaccine every 5 years
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5
Q

Haemochromatosis: Iron aims

A

Aim Ferritin <35%

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6
Q

Causes macrocytosis

A 
Liver disease
Alcohol
B12/folate
Methotrexate 
Hydroxyurea
Reticulocytosis
Hypothyroid
Myelodysplasia
Cold agglutinins
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7
Q

Pernicious anaemia

A

20% of low b12
Antibodies against gastric parietal cells
Low intrinsic factor

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8
Q

Major features of neutrophil engraftment syndrome

A

Fever, non-cardiogenic pulmonary oedema, erythrodermatous rash
Minor: Renal, liver, increased weight, encephalopathy

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9
Q

basophilic stippling

A

indicates defective Hb synthesis eg: thalassemias, B12 deficiency

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10
Q

Heinz bodies

A 
classic G6PD
(fava) beans means Heinz!
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11
Q

Target cells

A

liver disease, thalassemia major, sickle cell anaemia, hyposplenism

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12
Q

cabots rings; howell jolly bodies

A

post splenectomy, hyposplenism (coeliac), megaloblastic anaemia

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13
Q

CLL

A 
fragile; smear / smudge cells
CD19, CD20, CD5, CD23
CD38 a/w high risk CLL
high risk shingles
a/w cold agglutinin disease
expect hypogammaglobulinaemia
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14
Q

polycythaemia rubra vera

A

JAK2
Rx: hydroxyurea, phlebotomy
aim Hct - <0.45 to avoid thromboses and CVS disease

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15
Q

TTP

A 
pentad: 
1 fever
2 renal failure
3 neuro symptoms
4 MAHA
5 thrombocytopaenia
def of vWF cleaving enzyme ADAMTS13 --> long polymers of vWF --> thrombosis
Rx: plasma exchange / plasmapheresis
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16
Q

MDS

A

ineffective haematopoiesis, peripheral cytopaenia
hypercellular BM with a dysplastic lineage
a/w abnormalities on chromosome 3, 5, 7, 8, 17
low rate of conversion to AML
5q- subtype:high plts; low neuts; responds to lenalidomide
classic MDS: azacitidine

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17
Q

Fe defiicency

A 
transferrin high (moves free Fe)
ferritin low (low stores)
TIBC high (trying to maximise available Fe)
Iron low (Fe def)

Fe level is least specific (low in chronic disease too)
Ferritin is unreliable unless low as it is an acute phase reactant
Transferrin - most reliable indicator

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18
Q

HITS

A

Low platelet count 50% decrease from baseline.

Increased tendency for thrombosis (generally at areas of vascular injury

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19
Q

Mechanism of HITS

A

Formation of platelet activiting Factor-4 antibodies (form complex with heparin) leading to aggregation and activation

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20
Q

Management of HITS

A

Ceased Heparin/LMWH

Direct thrombin inhibitors: Argatroban, Lepuridin, Bivalirudin

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21
Q

Severity classification of haemophilia

A

Factor deficiency
<1% Severe
1-5% Moderate
5-30% Mild

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22
Q

pseudohyperkalaemia

A

beware if high plts or WCC

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23
Q

pt bleeding low fibrinogen

A

give cryoprecipitate

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24
Q

Acute intermittent porphyria (AIP)

A

autosomal dominant
defect in porphobilinogen deaminase
female and 20-40 year olds more likely to be affected
typically present with abdominal symptoms, neuropsychiatric symptoms
hypertension and tachycardia common
urine turns deep red on standing

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25
Q

Porphyria cutanea tarda (PCT)

A

most common hepatic porphyria
defect in uroporphyrinogen decarboxylase
may be caused by hepatocyte damage e.g. alcohol, oestrogens
classically photosensitive rash with bullae, skin fragility on face and dorsal aspect of hands
urine: elevated uroporphyrinogen and pink fluorescence of urine under Wood’s lamp
manage with chloroquine

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26
Q

too much warfarin

A

INR < 5 WH
> 5 add vitamin K
bleeding - prothrombinex, FFP or novovseven

27
Q

thrombophilia screen

A

AT3, protein C, protein S will be altered post clot / with treatment - cannot reliably assess levels

28
Q

chronic liver disease clotting

A

factor 8, VWF go up
all else down
? due to lack of thrombin mediated down regulation of factor 8

29
Q

Burkitts Lymphoma

A

often an abdominal presentation with massive ascites

or face / jaw involvement

30
Q

MDS treatment

A

azacitadine

31
Q

alpha thal

A 
4 chains - normal
3 chains - silent carrier Hb 90
2 chains - trait - sin / trans Hb 80
1 chain - HbH disease Hb 70 
0 chains - Barts hydrops fetalis
32
Q

PNH

A 
defect in GPI anchor due to abnormal PIG-A gene
RBCs vulnerable to haemolysis
MAHA, clotty, bicytopaenia
--> MDS, AML
mean age 33yo
Rx: eciluzimab anti C5
33
Q

APML translocation

A

t 15:17

Rx: ATRA

34
Q

paroxysmal cold haemoglobinuria

A

haemoglobinuria and anaemia with cold
complement intravascular haemolysis
lack of GPI due to PIGA gene defect x linked
flow cytometry for diagnosis - discrete CD59, 55 population
rx ecluzimab

35
Q

idiopathic cold agglutinin disease

A

immune haemolytic anaemia

IgM v RBC

36
Q

warm antibody haemolytic anaemia

A

spherocytic haemolytic anaemia

DAT positive

37
Q

CLL

A

poor prognosis if 17p deletion (= loss of p53)

38
Q

unconjugated bilirubin

A

does not usually reach gut - if it does - is reabsorbed to liver exacerbating hyperbilirubinaemia

39
Q

UGT1

A

conjugates bilirubin

congenital abnormality in Crigler Najjar Syndrome

40
Q

Crigler Najjar

A

autosomal recessive; abnormal UGT1 for bilirubin conjugation
type 1: more severe; unconjugated bilirubin > 345; no response to phenobarbital; UGT1 absent, kernicterus
type 2: uncon bili < 345; phenobarbital decreases bili by > 25%; UGT1 low levels; kernicterus rare

41
Q

Gilbert

A

unconjugated bili < 70
uGTA levels 33% lower than normal
irinotecan toxicity

42
Q

iron studies

A

Ferritin < 15 indicates absent body stores; high could be anything
Transferrin saturation < 20% is Fe deficiency; > 50% indicates Fe being delivered to nonerythroid tissues (aim < 35% in HH)

43
Q

haemachromatosis inheritance

A

autosomal recessive

44
Q

beta thal

A

minor - 1 gene affected
intermedia - 2 genes affected; no normal beta chains
major - no beta chain production

45
Q

target cells

A

liver disease, thalassaemia, post splenectomy

46
Q

tear drop cells

A

myelofibrosis, thalassaemia

47
Q

splenectomy

A

howell jolly bodies
target cells
spherocytes

48
Q

rouleaux

A

most commonly in multiple myeloma

49
Q

dilute russell viper venom time

A

prothrombotic venom
activates factor 10; blocked by antiphospholipid antibodies
therefore: prolonged with antiphospholipid Abs

50
Q

ristocetin

A

plt aggregation in setting of vWF
plts will not clump in absence of vWF
therefore: reduce activity in VWD esp type 3

51
Q

acquired vWD

A

typically a delayed inhibitor

ie: mixing corrects then reverts to being prolonged

52
Q

reptilase test

A

snake venom which activates fibrinogen
prolonged TCT = prolonged reptilase
except if heparin the cause - normal reptilase
therefore: normal in heparin; tells you if heparin is the issue

53
Q

NOACs bleeding

A

tranexamic aicd 15-30mg/kg QID (anti fibrinolytic)
HDx for dabigatran
prothrombinex if severe
novoseven

54
Q

Fe overload

A

avoid Vit C as leads to excess free radicals

55
Q

liver disease blood film

A

spur cells / acanthocytes

56
Q

fanconis anaemia

A

auto rec

pancytopaenia, short, cafe au laid spots

57
Q

myelodysplasia

A

Pelger Huet anomaly - bilobed neutrophil without granules, large plts, macrocytic anaemia
del 5q - lenalidomide good
else azacitadine

58
Q

mantle cell lymphoma

A

t(11;14) cyclin D1

59
Q

CLL

A

CD5+ CD19+ CD23+

13q del is good prognosis

60
Q

B12

A

methylmalonate MMA and homocysteine levels raised

61
Q

CML

A

50% have ongoing response off imatinib

dose depends on OCT1 activity (ability of imatinib to enter cells)

62
Q

essential thrombocytosis

A

associated primarily ith haemorrhage due to acquired vWD
plts > 1000; vWD removed from circulation and destroyed by enlarged platelet mass
hydroxyurea if plus > 600

63
Q

MM

A

ESRF from tubular damage - calcium, amyloid, light chains
RTA2
beta2 microglobulin most potent predictor of survival

FCRAP (19 decks)

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