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Haematology Flashcards

1
Q

What is sickle cell

A

Haemaglobinopathy caused by inappropriate exchange of valine for glutamic acid on BETA globin chain
HbS forms if homozygous = aa,SS

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2
Q

What type of inheritance is sickle cell, alpha and beta thalassaemia

A

Autosomal recessive

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3
Q

Consequences of sickle cell

A
  1. Painful venoocclusion
  2. Haemolysis - anaemia, jaundice, pigmented gallstones
  3. Splenic atrophy, infection
  4. Priapism
  5. Acute sickle chest syndrome
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4
Q

How is sickle managed

A
  1. Folic acid supplementation
  2. Infection prophylaxis
  3. Blood transfusion
  4. Hydrozycarbamide or hydroxyurea to inc HbF in crises
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5
Q

What is sickle trait

A

HbAS so they make some HbA and HbS because one b chain normal
protect against malaria, asymptomatic

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6
Q

Risks assd with being sickle trait

A
  1. pregnancy
  2. can sickle under stress
  3. splenic infarction
  4. renal disease
  5. vte risk
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7
Q

What is alpha thalassaemia

A

Lack of alpha chain -> excess b and g production = abnormal hb produced
HbH = bbbb
HbBarts = GGGG

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8
Q

What is beta thalassaemia and types

A 
BTM = B0B0 absent alleles for beta
BTI = B0B+
one absent one mutated
BTT = BB0
one normal one absent

all of them -> relative alpha xs = unstable = extravascular haemolytic anaemia

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9
Q

Chelation drugs

A 
Deferiprone
= neutropenia risk
Deferasirox
= renal + liver tox
Desferrioxamine
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10
Q

Differences in MCV, chromicity, and epidemiology of sickle and thalassaemia

A 
Sickle is normocytic and normochromic
- Thalassaemia is microcytic and hypochromic
- 
Sickle = black and asian
Thalassaemia = mediteranean
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11
Q

Causes of microcytic anaemia MCV <80

A 
TAILS
Thalassaemia
AofCD
IDA
Lead poisoning
Sideroblastic
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12
Q

Causes of IDA

A
  1. Poor diet
  2. Poor absorption (coeliac, Crohns)
  3. Inc demand (preg)
  4. Iron loss
    - Bleeding due to cancer
    - Ulcers
    - IBD
    - Gastritis
    - Menorrhagia
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13
Q

Features of IDA on blood film

A

Microcytic, hypochromic cells
Pencil shaped
Target cells

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14
Q

Presentation of lead poisoning + tx

A

Abdo signs + neuropathy + anaemia + blue gum line

Tx - dimercaprol

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15
Q

Causes of normocytic anaemia

A

BABS

  1. blood loss
  2. A of cd
  3. BM failure - aplastic, chemo
  4. Sickle + other haemolytic anemias
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16
Q

Causes of macrocytic anaemia

A
  1. Megaloblastic (b12, folate)
  2. Alcohol, liver disease
  3. Drugs eg antiepileptics, hydroxycarbamide
  4. Myelodysplasia
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17
Q

Causes of folate deficiency

+ sign on blood smear

A
  1. Poor diet
  2. Malabsorption (coeliac, crohns, drugs eg cholestyramine)
  3. Inc requirement
    (pregnancy, haemolysis, inflammation, homocystinuria)
  4. Inc loss
    (dialysis, drugs eg methotrexate)

Hypersegmented nuclei of neutrophils > 5

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18
Q

Causes of B12 deficiency

A
  1. Poor intake
  2. Pernicious anaemia (AI destruction of gastric pariteal cells releasing IF)
  3. Malabsorption (crohns, terminal ileum, drugs eg metformin)
  4. Nitrous oxide
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19
Q

What is the significance of G6PD deficiency? What are the triggers and genetics

A

G6PD protects against oxidative stress on RBCs so deficiency assd with haemolytic anaemia
It is X linked autosomal dominant, women carriers may have malaria protection
TRIGGERS:
Fava(broad) beans
Infection
Drugs (aspirin, malaria meds)

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20
Q

Blood smear findings of G6PD deficiency

A

Heinz bodies

Bite cells

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21
Q

What is the genetic inheritance of PK

A

Autosomal recessive

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22
Q

What is the most common cause of inherited haemolytic anaemia in west

A

Hereditary Spherocytosis

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23
Q

What is the inheritance of HS

A

Autosomal dominant

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24
Q

What is hereditary spherocytosis

A

beta spectrin or alpha ankyrin deficiency = spherical rbc = haemolytic anaemia

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25
What is warm AI haemolytic anaemia and causes
igG against rbc = extravascular destruction CAUSES: 1. idiopathic 2. secondary to conditions eg SLE, HIV,, CLL + B lymphoma etc 3. Secondary to drugs eg anti malarial, penicillin, dopa
26
How is warm haemolytic anaemia dx and which leukaemia is it assd with
DAT (coombs) + | CLL
27
What is cold haemolytic anaemia
igM that binds weakly to rbc at cooler temperatures
28
What is MAHA
Microangiopathic haemolytic anaemia = pathological changes to blood in small vessels usually secondary to TTP Schistocytes + anaemia
29
Signs and symptoms of PCV
Fullness = headache, blurred vision, splenomegaly + aquagenic pruritis plethora
30
Tx of PCV
Venesection | cytoreduction + aspirin (like ET)
31
How does ET present
asymptomatic but may erythromelalgia acroparasthesia bleeding
32
How is ET treated
depends on risk cytoreduction + aspirin cytoreduction = hyroxycarbamide, anagrelide 2nd
33
What is APML - genetics - smear - tx
defect in promyelocyte -> myelocyte = no neutrophils Genetic: 15,17 Smear: auer Tx: tretinoin + arsenic trioxide based therapy
34
Genetic basis of CML and which organs are enlarged | Tx
9,22 Ph spleen only imatinib
35
Symptoms of MM and what do we look for in dx
``` CRABI C- hypercalcaemia R- renal failure A - anaemia B - bone pain I - infections ``` M protein , bence jones protein i nurine
36
Presentation of hodgkin lymphoma
Painless lymphadenopathy or pain precipitated by alcohol | B symptoms
37
What disease causes bulky lymphadenopathy
Diffuse large B cell lymphoma
38
What is the most common low grade NHL and genetics of it
follicular | 14,18 translocation
39
Describe clotting mechanism
1. BV damaged 2. Collagen exposed to blood so vwF binds 3. vwF binds to platelets via gp1b 4. platelets accumulate via gp2b/3a 5. Bv wall releases tissue factor 6. Bv constricts 5+6 stimulate clotting cascade 1. intrinsic pathway (starts with 12) requires 8 to convert 9a -> 10 2. extrinsic pathway (starts with 7 -> 7a which -> 10) 3. Common final pathway 10 -> 10a 10a requries 5 to convert prothrombin to thrombin thrombin converts fibrinogen to fibrin fibrin + primary clot = stable
40
Describe anticoagulation pathway
1. thrombin goes to distal site and binds to thrombomodulin 2. this complex activates protein c 3. c binds to s 4. c/s complex cleaves factors 5 and 8 5. plasminogen converts to plasmin which breaks down clot
41
Which parts of clotting are vit k dependent
2,7,9,10 | protein C - so warfarin can cause paradoxical thrombosis at first
42
What is vw disease genetics common defect assd
abnormality in quantity (t1) or quality (t2) of vWF Autosomal dominant TTP = AI to ADAMST13 which cleaves xs vwF so xs vWF = microthrombi
43
Name 4 types of platelet disease and what is prolonged
1. Gray platelet syndrome = no alpha granules 2. Storage pool disease = no beta granules 3. Bernard Soullier = absent gp1b 4. Glanzmanns = absent gp2b/3a bleeding time prolonged if platelet dysfunction
44
what is the most common inherited RF for VTE and the genetics
factor 5 leiden mutation means resistance to c/s cleaving = prothrombotic autosomal dominant
45
What is absent in haemophilia A
factor 8 | ACE910 emicizumab
46
Inheritance of haemophilias
X linked recessive
47
What is absent in haemophilia B
factor 9
48
Universal rbc donor
O rhd-
49
Universal rbc recipient
AB rh +
50
What antibodies are involved in transfusion reactions
igM
51
What transfusion product presents biggest risk of infection
platelets (temp stored 22, shelf life 7 days)
52
Indications for RBC transfusion
Hb <70g/L stable | Hb < 80g/L CVD
53
Indications for platelet transfusion
significant bleed platelet dysfunction prior to invasive prophylactic eg recurrent intracranial bleed
54
Indications for FFP
Major haemorrhage PT INR ration >1.5 + bleed or pre procedure PT INR ratio > 2 + liver disease + pre procedure
55
Indications for cryoprecipitate
``` PURPOSE TO INC fibrinogen Major haemorrhage + <1.5g/l <1 + pre-procedure CLD DIC ```
56
3 most serious transfusion
ABO incompatibility Anaphylaxis (esp pxs who have igA deficiency) Bacterial
57
3 most common transfusion reactions
TACO NHFTR Allergic
58
Signs of ABO incompatible haemolytic reaction
fever, abdo pain, hypotensive
59
Signs of TACO
pulmonary odema, hypertension | caused by high flow or HF existing
60
What are B symptoms
``` Fatigue Weight Loss Night sweats Fever Pruritis ```

CC2 (8 decks)

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