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CC2 > Haematology > Flashcards

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
Q

What is warm AI haemolytic anaemia and causes

A

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
Q

How is warm haemolytic anaemia dx and which leukaemia is it assd with

A

DAT (coombs) +

CLL

27
Q

What is cold haemolytic anaemia

A

igM that binds weakly to rbc at cooler temperatures

28
Q

What is MAHA

A

Microangiopathic haemolytic anaemia = pathological changes to blood in small vessels usually secondary to TTP

Schistocytes + anaemia

29
Q

Signs and symptoms of PCV

A

Fullness = headache, blurred vision, splenomegaly
+
aquagenic pruritis
plethora

30
Q

Tx of PCV

A

Venesection

cytoreduction + aspirin (like ET)

31
Q

How does ET present

A

asymptomatic but may
erythromelalgia
acroparasthesia
bleeding

32
Q

How is ET treated

A

depends on risk
cytoreduction + aspirin
cytoreduction = hyroxycarbamide, anagrelide 2nd

33
Q

What is APML

  • genetics
  • smear
  • tx
A

defect in promyelocyte -> myelocyte = no neutrophils
Genetic: 15,17
Smear: auer
Tx: tretinoin + arsenic trioxide based therapy

34
Q

Genetic basis of CML and which organs are enlarged

Tx

A

9,22 Ph
spleen only
imatinib

35
Q

Symptoms of MM and what do we look for in dx

A 
CRABI
C- hypercalcaemia
R- renal failure
A - anaemia
B - bone pain 
I - infections

M protein , bence jones protein i nurine

36
Q

Presentation of hodgkin lymphoma

A

Painless lymphadenopathy or pain precipitated by alcohol

B symptoms

37
Q

What disease causes bulky lymphadenopathy

A

Diffuse large B cell lymphoma

38
Q

What is the most common low grade NHL and genetics of it

A

follicular

14,18 translocation

39
Q

Describe clotting mechanism

A
  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
  7. intrinsic pathway (starts with 12) requires 8 to convert 9a -> 10
  8. extrinsic pathway (starts with 7 -> 7a which -> 10)
  9. Common final pathway
    10 -> 10a
    10a requries 5 to convert prothrombin to thrombin
    thrombin converts fibrinogen to fibrin
    fibrin + primary clot = stable
40
Q

Describe anticoagulation pathway

A
  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
Q

Which parts of clotting are vit k dependent

A

2,7,9,10

protein C - so warfarin can cause paradoxical thrombosis at first

42
Q

What is vw disease
genetics
common defect assd

A

abnormality in quantity (t1) or quality (t2) of vWF
Autosomal dominant
TTP = AI to ADAMST13 which cleaves xs vwF so xs vWF = microthrombi

43
Q

Name 4 types of platelet disease and what is prolonged

A
  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
Q

what is the most common inherited RF for VTE and the genetics

A

factor 5 leiden mutation
means resistance to c/s cleaving
= prothrombotic
autosomal dominant

45
Q

What is absent in haemophilia A

A

factor 8

ACE910 emicizumab

46
Q

Inheritance of haemophilias

A

X linked recessive

47
Q

What is absent in haemophilia B

A

factor 9

48
Q

Universal rbc donor

A

O rhd-

49
Q

Universal rbc recipient

A

AB rh +

50
Q

What antibodies are involved in transfusion reactions

A

igM

51
Q

What transfusion product presents biggest risk of infection

A

platelets (temp stored 22, shelf life 7 days)

52
Q

Indications for RBC transfusion

A

Hb <70g/L stable

Hb < 80g/L CVD

53
Q

Indications for platelet transfusion

A

significant bleed
platelet dysfunction
prior to invasive
prophylactic eg recurrent intracranial bleed

54
Q

Indications for FFP

A

Major haemorrhage
PT INR ration >1.5 + bleed or pre procedure
PT INR ratio > 2 + liver disease + pre procedure

55
Q

Indications for cryoprecipitate

A 
PURPOSE TO INC fibrinogen 
Major haemorrhage + <1.5g/l
<1 + pre-procedure
CLD
DIC
56
Q

3 most serious transfusion

A

ABO incompatibility
Anaphylaxis (esp pxs who have igA deficiency)
Bacterial

57
Q

3 most common transfusion reactions

A

TACO
NHFTR
Allergic

58
Q

Signs of ABO incompatible haemolytic reaction

A

fever, abdo pain, hypotensive

59
Q

Signs of TACO

A

pulmonary odema, hypertension

caused by high flow or HF existing

60
Q

What are B symptoms

A 
Fatigue
Weight Loss
Night sweats
Fever
Pruritis

CC2 (8 decks)

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