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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
Porphyria cutanea tarda (PCT)
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
26
too much warfarin
INR < 5 WH > 5 add vitamin K bleeding - prothrombinex, FFP or novovseven
27
thrombophilia screen
AT3, protein C, protein S will be altered post clot / with treatment - cannot reliably assess levels
28
chronic liver disease clotting
factor 8, VWF go up all else down ? due to lack of thrombin mediated down regulation of factor 8
29
Burkitts Lymphoma
often an abdominal presentation with massive ascites | or face / jaw involvement
30
MDS treatment
azacitadine
31
alpha thal
``` 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
PNH
``` 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
APML translocation
t 15:17 | Rx: ATRA
34
paroxysmal cold haemoglobinuria
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
idiopathic cold agglutinin disease
immune haemolytic anaemia | IgM v RBC
36
warm antibody haemolytic anaemia
spherocytic haemolytic anaemia | DAT positive
37
CLL
poor prognosis if 17p deletion (= loss of p53)
38
unconjugated bilirubin
does not usually reach gut - if it does - is reabsorbed to liver exacerbating hyperbilirubinaemia
39
UGT1
conjugates bilirubin | congenital abnormality in Crigler Najjar Syndrome
40
Crigler Najjar
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
Gilbert
unconjugated bili < 70 uGTA levels 33% lower than normal irinotecan toxicity
42
iron studies
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
haemachromatosis inheritance
autosomal recessive
44
beta thal
minor - 1 gene affected intermedia - 2 genes affected; no normal beta chains major - no beta chain production
45
target cells
liver disease, thalassaemia, post splenectomy
46
tear drop cells
myelofibrosis, thalassaemia
47
splenectomy
howell jolly bodies target cells spherocytes
48
rouleaux
most commonly in multiple myeloma
49
dilute russell viper venom time
prothrombotic venom activates factor 10; blocked by antiphospholipid antibodies therefore: prolonged with antiphospholipid Abs
50
ristocetin
plt aggregation in setting of vWF plts will not clump in absence of vWF therefore: reduce activity in VWD esp type 3
51
acquired vWD
typically a delayed inhibitor | ie: mixing corrects then reverts to being prolonged
52
reptilase test
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
NOACs bleeding
tranexamic aicd 15-30mg/kg QID (anti fibrinolytic) HDx for dabigatran prothrombinex if severe novoseven
54
Fe overload
avoid Vit C as leads to excess free radicals
55
liver disease blood film
spur cells / acanthocytes
56
fanconis anaemia
auto rec | pancytopaenia, short, cafe au laid spots
57
myelodysplasia
Pelger Huet anomaly - bilobed neutrophil without granules, large plts, macrocytic anaemia del 5q - lenalidomide good else azacitadine
58
mantle cell lymphoma
t(11;14) cyclin D1
59
CLL
CD5+ CD19+ CD23+ | 13q del is good prognosis
60
B12
methylmalonate MMA and homocysteine levels raised
61
CML
50% have ongoing response off imatinib | dose depends on OCT1 activity (ability of imatinib to enter cells)
62
essential thrombocytosis
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
MM
ESRF from tubular damage - calcium, amyloid, light chains RTA2 beta2 microglobulin most potent predictor of survival

FCRAP (19 decks)

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