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Pathology > Haematology > Flashcards

Haematology Flashcards

1
Q

Acquired risks of VTE? (13)

A
  1. Age
  2. Obesity
  3. Previous DVT/PE
  4. Immobilisation
  5. Major surgery
  6. Long distance travel
  7. Mlaignancy
  8. Pregnancy
  9. COCP
  10. HRT
  11. Anti-phospholipid syndrome
  12. Polycythaemia
  13. Thrombocythaemia
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2
Q

Factor V Leiden

  • who
  • what
A

caucasian

resistance to Protein C, therefore increased clotting

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3
Q

Inherited risk factors of VTE? (7)

A
  1. Antithrombin deficiency
  2. Protein C deficiency
  3. Protein S deficiency
  4. Factor V Leiden
  5. Prothrombin G20210A
  6. Lupus anticoagulant
  7. coag excess - VIII, II, fibrinogen
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4
Q

DVT Prophylaxis

A
  1. TED stockings

2. sc LMWH

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5
Q

Treatment of 1st VTE unknown cause?

A

Warfarin

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6
Q

Action of Heparin?

A

potentiates antithrombin II
inactivates thrombin
inactivates factors 9, 10, 11

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7
Q

Action of Warfarin?

A

inhibits reductase enzyme responsible for regenerating Vitamin K
Inhibits synthesis of factors 2, 7, 9, 10

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8
Q

Warfarin side effects?

A

teratogenicity

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9
Q

Action of Rivaroxiban?

A

direct inhibitor of factor Xa

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10
Q

Target INR in AF?

A

2-3

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11
Q

Target INR in recurrent VTE/PE?

A

2.5 - 3.5

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12
Q

Target INR in mechanical heart valve?

A

3.5

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13
Q

INR raised >5 no bleeding?

A

Stop Warfarin, restart when INR

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14
Q

INR raised 5 - 8 and bleeding?

A

Stop Warfarin, Vit K slowly

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15
Q

Anaemia in men?

A

Hb

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16
Q

Anaemia in women?

A

Hb

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17
Q

Symptoms of anaemia? (7)

A
  1. fatigue
  2. dyspnoea
  3. faintness
  4. palpitations
  5. headache
  6. tinnitus
  7. anorexia
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18
Q

Leukoerythroblastic anaemia

  • what
  • morphological features (3)
  • causes (4)
A
  • anaemia of white and red cell precursors in the peripheral blood
  • marrow infiltration
    1. nucleated RBCs
    2. myelocytes
    3. poikilocytes “tear drop”
    Causes
    1. haematological malignancies
    2. non-haematological malignancies
    3. severe infection e.g. miliary TB
    4. myelofibrosis
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19
Q

Lab findings of haemolysis (5)

A
  1. anaemia
  2. reticulocytosis
  3. raised bilirubin
  4. raised LDH
  5. reduced haptoglobins
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20
Q

Haemolytic anemia

  • what is it?
  • types (2)
A

breakdown of RBCs before their normal lifespan of 120 days

a) inherited
b) acquired 1) immune 2) non-immune

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21
Q

Lab findings auto-immune haemolytic anaemia? (2)

A
  1. spherocytes

2. DAT +

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22
Q

Types of non-immune acquire haemolytic anaemia? (2)

A
  1. Infection - malaria

2. Micro-angiopathic haemolytic anaemia

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23
Q

Causes of microcytic anaemia? (4)

A
  1. IDA
  2. Anaemia of chronic disease
  3. Sideroblastic anaemia
  4. Thalassaemia
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24
Q

Causes of normocytic anaemia? (7)

A
  1. acute blood loss
  2. anaemia of chronic disease
  3. bone marrow failure
  4. renal failure
  5. hypothyroidism
  6. haemolysis
  7. pregnancy
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25
Q

Causes of macrocytic anaemia? (7)

A
  1. pregnancy
  2. anti-folates
  3. hypothyroidism
  4. reticulocytosis
  5. B12 or folate deficiency
  6. cirrhosis
  7. myelodysplastic syndromes
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26
Q

Causes of neutrophilia (5)

A
  1. corticosteroids
  2. neoplasm
  3. inflammation
  4. myeloproliferative disorders
  5. bacterial infection
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27
Q

Causes of eosinophilia (4)

A
  1. parasitic infection
  2. allergic disease e.g. asthma, RA
  3. neoplasm
  4. drug reaction e.g. erythema multiforme
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28
Q

Signs of IDA (5)

A
  1. Koilonychia
  2. atrophic glossitis
  3. anugular cheilosis
  4. Plummer-Vinson syndrome
  5. brittle hair & nails
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29
Q

Features of blood film in IDA? (4)

A
  1. microcytic
  2. hypochromic
  3. anisocytosis
  4. poikilocytosis
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30
Q

Causes of IDA? (5)

A
  1. blood loss
  2. increased utilisation e.g. pregnancy
  3. decreased intake e.g. prematurity, dietary
  4. decreased absorption e.g. coeliac
  5. intravascualr haemolysis e.g. MAHA
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31
Q

Pathophysiology of anaemia of chronic disease

- lab findings

A
  • cytokine driven inhibition of RBC production
  • cytokines IFNs, TNF, IL1 reduced EPO receptor, and therefore EPO synthesis
  • ferritin is HIGH as iron is sequestered in macrophage to deprive invading bacteria of iron
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32
Q

Causes of anaemia of chronic disease (4)

A
  1. chronic infection
  2. vasculitis
  3. RA
  4. Malignancy
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33
Q

Sideroblastic anaemia

  • what
  • causes (4)
  • diagnosis
A
  • inability to incorporate iron in to Hb resulting in iron loading
    1. myelodysplastic disorders
    2. chemotherapy
    3. irradiation
    4. alcohol excess
  • ring sideroblasts
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34
Q

Acanthocytes (3)

A

Abetalipoproteinaemia
liver disease
hyposplenism

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35
Q

Basophilic RBC stippling (3)

A

accelerated erythropoiesis

  1. lead poisoning
  2. liver disease
  3. haemoglobinopathy
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36
Q

Burr cells (3)

A
  1. uraemia
  2. GI bleeding
  3. stomach carcinoma
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37
Q

Heinz bodies

A

Glucose-6-phosphate dehydrogenase deficiency

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38
Q

Howell-Jolly bodies (4)

A
  1. post-splenectomy
  2. hyposplenism
  3. megaloblastic anaemia
  4. hereditary spherocytosis
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39
Q

Polychromasia

A

premature or inappropriate release from bone marrow

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40
Q

High no of reticulocytes

A

haemolytic anaemia

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41
Q

Low no of reticulocytes

A

aplastic anaemia

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42
Q

Right shift of white cells (3)

A

hypermature white cells

megaloblastic anaemia
uraemia
liver disease

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43
Q

Rouleaux formation (3)

A

paraproteinaemia
myeloma
chronic inflammation

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44
Q

Schistocytes (3)

A

microangiopathic anaemia
DIC
HUS

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45
Q

Spherocytes (2)

A

hereditary spherocytosis

autoimmune haemolytic anaemia

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46
Q

Stomacytes (3)

A

hereditary stomacytosis
high alcohol intake
liver disease

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47
Q

Target cells (4)

A

IDA
liver disease
hyposplenism
thalassemia

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48
Q

Megaloblastic anaemia

A

results from inhibition of DNA synthesis during RBC production

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49
Q

Vitamin B12 Deficiency

  • causes (2)
  • sources
  • symptoms (8)
A

1) dietary
2) malabsorption

  • meat, dairy products
  1. glossitis
  2. angular chelitis
  3. irritability
  4. depression
  5. psychosis
  6. dementia
  7. peripheral neuropathy
  8. paraesthiasiae
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50
Q

Pernicious anaemia

  • what
  • test
A

autoimmune atrophic gastritis with lack of intrinsic factor

Schilling test

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51
Q

Folate deficiency

  • causes (4)
  • sources
A
  1. poor diet
  2. pregnancy
  3. malabsorption
  4. alcohol

green vegetables, nuts

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52
Q

Herditary spherocytosis

  • inheritance pattern
  • deficiency
  • susceptibilities (2)
  • Blood film
  • Diagnostic test
  • Sign
A
  • autosomal dominant
  • spectrin deficiency
    1) parvovirus B19
    2) gallstones
  • spherocytes
  • DAT -ve
  • splenomegaly
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53
Q

Hereditary elliptocytosis

  • inheritance pattern
  • deficiency
  • outcome
A
  • autosomal dominant
  • spectrin
    asymptomatic to hydrops fetalis
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54
Q

G6PD Deficiency

  • who
  • inheritance pattern
  • blood film
  • precipitants (3)
  • attack
A
  • Mediterranean
  • X-linked
  • Heinz bodies, bite cells
    1) broad beans
    2) drugs - anti-malarials, sulphonamides, aspirin
    3) acute infection
  • rapid anaemia and jaundice
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55
Q

Pyruvate kinase deficiency

  • inheritance pattern
  • clinical features (3)
A
  • autosomal recessive
    1. neonatal jaundice
    2. splenomegaly
    3. haemolytic anaemia
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56
Q

single base mutation in sickle cell anaemia?

A

GAG - GTG

Glutamine to valine

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57
Q

age of onset of sickle cell anaemia?

A

3-6 months, coincides with decreasing fetal Hb

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58
Q

features of sickle cell anaemia?

A 
SICKLED  MP
S - stroke
I - infection, Parvovirus B19 
C - crises 
K - kidney necrosis, nephrotic syndrome 
L - liver, gallstones 
E - eyes, retinopathy 
D - dactilitis, impaired growth 
M - mesenteric ischaemia
P - priapism
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59
Q

Clinical features of B thalassemia? (3)

A
  1. frontal bossing
  2. maxillary hypertrophy
  3. hairs on end skull x-ray
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60
Q

Inherited haemolytic anaemia DAT/Coombs test?

A

DAT -ve

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61
Q

Acquired haemolytic anaemia DAT/Coombs test?

A

DAT +ve

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62
Q

Paroxysmal Cold Haemoglobinuria

  • what
  • cause
  • antibodies
A

autoimmune haemolysis where haemoglobin is present in the urine
virus e.g. measles, syphilis, VZV
Donath-Landsteiner antibodies

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63
Q

Paroxysmal Nocturnal Haemoglobinuria

  • what
  • pathophysiology
  • signs (2)
  • test
A 
acquired haemolysis 
loss of GPI markers of RBCs
1. morning haemoglobinuria
2. thrombosis 
- Ham's test
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64
Q

Microangiopathic Haemolytic Anaemia

  • what
  • blood film
  • causes (4)
A 
mechanical destruction of RBCs
schistocytes 
1. HUS
2. TTP
3. DIC
4. pre-eclampsia
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65
Q

Thrombotic Thrombocytopenic Purpura

  • what
  • pentad of symptoms
A
  • autoimmune platelet activation
    1. MAHA
    2. fever
    3. renal impairment
    4. neuro abnormalities
    5. thrombocytopenia
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66
Q

Haemolytic Uraemic Syndrome

  • cause
  • pathophysiology
  • who
  • symptoms (2)
A
  • E.Coli
  • toxin damages endothelial cells
  • children & elderly
    1. renal impairment
    2. diarrhoea
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67
Q

Normal platelet count

A

150 - 400

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68
Q

Intrinsic pathway of coagulation cascade?

A 
Factor TwelvE
EleveN
NinE
EighT
Ten
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69
Q

Extrinsic pathway of coagulation cascade?

A

Enodthelial damage
Tissue Factor activation
Factor Seven
Ten

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70
Q

Common pathway of coagulation cascade?

A

Ten

Two

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71
Q

Prothrombin is?

A

Factor II

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72
Q

Thrombin is?

A

Factor IIa

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73
Q

Antithrombin

A

inhibits clotting factors

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74
Q

Phases of clotting cascade? (4)

A
  1. initiation
  2. activation
  3. propagation and thrombin burst
  4. stable clot
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75
Q

Inhibitors of clotting cascade? (4)

A
  1. Tissue factor pathway inhibitor
  2. Protein C
  3. Protein S
  4. Antithrombin III
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76
Q

Monitoring of intrinsic pathway?

- which drug?

A

Activated Partial Thromboplastin Time (APTT)

- Heparin

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77
Q

Monitoring of extrinsic pathway?

- which drug?

A 
Prothrombin Time (PT)
- Warfarin
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78
Q

Monitoring of common pathway?

A

Thrombin Time (TT)

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79
Q

Fibrinolysis

  • initiated by?
  • outcome
A
  • fibrin clot formation

- plasmin lyses fibrin clot in to degradation products

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80
Q

what is TPA?

A

tissue plasminogen activator

“clot-buster”

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81
Q

Types of bleeding disorders (4)

A
  1. vascular defects
  2. platelet defects
  3. coagulation disorders
  4. mixed
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82
Q

Symptoms of vascular and platelet disorders (3)

A
  1. superficial bleeding into skin
  2. bleeding of mucosal membranes
  3. immediate bleeding after injury
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83
Q

symptoms of coagulation disorders (3)

A
  1. prolonged bleeding
  2. bleeding into deep tissues, muscles, joints
  3. delayed, severe bleeding after injury
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84
Q

Osler-Weber- Rendu Syndrome

  • inheritance pattern
  • signs (3)
A

Hereditary Haemorrhagic Telangiectasia

  • autosomal dominant
    1. telangiectasia of mucous membranes
    2. epistaxis
    3. arteriovenous malformations
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85
Q

Ehlers Danlos

  • what
  • inheritance pattern
  • signs
A
  • connective tissue disorder
  • autosomal dominant
    1. hyper mobility
    2. fragile skin
    3. arteriovenous malformations
    4. valvular heart disease
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86
Q

Acquired vascular defects (4)

A
  1. senile purpura
  2. infection - measles, meningococcal
  3. steroids
  4. scurvy - perifollicular haemorrhages
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87
Q

Causes of thrombocytopenia?

A
  1. decreased production - Bone marrow failure

2. increased destruction - AITP, drugs

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88
Q

Auto Immune Thrombocytopenia Purpura

  • what
  • sign and symptoms (2)
A

antibodies against platelet antigens so platelets are destroyed by splenic macrophages

  1. petichiae
  2. menorrhagia
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89
Q

von Willebrand Disease

  • what
  • inheritance pattern
  • symptoms (5)
  • lab findings (3)
A

hereditary coagulation disorder - low platelet function

autosomal dominant

  1. easy bruising
  2. muscosal membrane bleeding
  3. epistaxis
  4. menorrhagia
  5. prolonged bleeding
  6. increased APTT
  7. increased bleeding time
  8. low factor VIII
90
Q

Haemophilia A

  • what
  • which factor?
  • inheritance pattern
  • symptoms
  • who
A 
congenital condition of failure to generate fibrin to stabilise a platelet plug 
Factor VIII deficiency 
X-linked recessive 
prolonged bleeding after surgery/trauma
M>F
91
Q

Haemophilia B

  • what
  • which factor?
  • inheritance pattern
A

congenital condition of failure to generate fibrin to stabilise a platelet plug
Christmas Disease
Factor IX deficiency
X-linked recessive

92
Q

Acquired causes of defects in haemostasis (3)

A
  1. liver failure
  2. DIC
  3. Vitamin K deficiency
93
Q

Causes of DIC (5)

A
  1. malignancy
  2. sepsis
  3. toxins
  4. obstetric complications
  5. trauma
94
Q

Pathophysiology of DIC

A

TF is released in the circulation causing binding with coagulation factors and intitation of the extrinsic pathway triggering small clots to form within blood vessels throughout the body -> multiorgan failure

consumption and depletion of coagulation factors and platelets

95
Q

Clinical picture of bone marrow failure (3)

A
  1. anaemia
  2. thrombocytopenia
  3. neutropenia
96
Q

Signs of bone marrow failure (7)

A 
hepatomegaly 
splenomegaly 
lymphadenopathy 
bone pain 
CNS involvement 
easy bruising 
gum hypertrophy
97
Q

Acute Myeloid Leukaemia

  • what
  • who
  • common gene mutation
A

neoplastic process of bone marrow and blood cells causing a block in maturation of white blood cells therefore an increase in BLAST cells

adulthood

RUNX1

98
Q

Acute Promyelocytic Leukaemia

- association

A

subtype of acute leukaemia

DIC

99
Q

Blood film finding associated with AML?

A

Auer Rods

100
Q

Cytochemistry staisn associated with AML? (2)

A
  1. myeloperoxidase

2. Sudan black stain

101
Q

Monocytic AML

- associations (3)

A
  1. petichiae
  2. gum infiltration
  3. hypokalaemia
102
Q

Cause of retinal haemorrhage in AML?

A

if WBC is very high causes hyperviscosity

103
Q

Acute Lymphoblastic Leukaemia

  • who
  • types
  • prognosis
A 
male, caucasian CHILDREN 
commonest cancer in children 
T-cell lineage 15%
B-cell lineage 85%
85% of children cured
104
Q

Clinical Features of ALL? (9)

A

ALO BOFFIN

A-anaemia
L-lymphadenopathy (thymic enlargement)
O - organomegaly (splenomegaly, hepatomegaly

B-bleeding (easy bruising, petichiae)
-bony pain
-brain (CNS involvement)
O-orchidomegaly 
F-fever
F-fatigue
I-infection
N-neutropenia
105
Q

Investigations in ALL?

A

Male Caucasian Children Love Big Rocket Blasts

L-LP look for blasts
B-biopsy look for blasts
-FBC lymphocytosis
R-X-ray, CT - mediastinal & abdominal lymph nodes
B-blast cells
106
Q

Subtype of ALL with good prognosis?

A

hyperdipoloidy

107
Q

Miantenance of chemotherapy in

a) boys?
b) girls?

A

boys - 3 years

girls - 2 years

108
Q

Increased risk of AML/ALL?

A

Down’s syndrome

109
Q

Neonates in comparison to adults…

  1. A higher Hb
  2. A lower WBC
  3. Smaller red blood cells
  4. The same percentage of haemoglobin F
A

have a higher Hb

110
Q

Complications of Sickle cell anaemia? (5)

A 
Hand-foot syndrome
Hyposplenism
Red cell aplasia
Splenic sequestration
Stroke
111
Q

Complications of sickle cell anaemia more common in adults than children?

A

Hyposlenism due to previous splenic sequestration or splenectomy

112
Q 
Siblings with sickle cell anaemia present simultaneously with anaemia and a low reticulocyte count?
Splenic sequestration
2. Parvovirus B19 infection
3. Folic acid deficiency
4. Haemolytic crisis
5. Vitamin B12 deficiency
A

Parvovirus B19 infection

113
Q

A 6-year-old Afro-Caribbean boy presents with chest and abdominal pain; Hb is 63 g/l, MCV 85 fl and blood film shows sickle cells—likely diagnosis?

  1. Sickle cell trait
  2. Sickle cell anaemia
  3. Sickle cell/beta thalassaemia
A

Sickle Cell Anaemia

114
Q

What additional therapy to blood transfusion will a child with B thalassemia require?

A

Iron chelation - desferrioxamine to stop iron overload

115
Q

Clinical signs of haemolysis? (4)

A
  1. jaundice
  2. splenomegaly
  3. increased unconjugated bilirubin
  4. increased reticulocytes
116
Q

Chronic Myeloid Leukaemia

  • what
  • who
  • presentation
A

uncontrolled proliferation of myeloid cells
middle aged 40-60years M>F
picked up on routine bloods

117
Q

CML

Signs & symptoms (9)

A 
fatigue
weight loss
fever
sweats
gout
bruising
hepatomegaly 
splenomegaly 
anaemia
118
Q

Chromosomal translocation present in CML

A

Philadelphia chromosome translocation 9;22 creating BCR-ABL fusion gene with tyrosine kinase activity

119
Q

Treatment of CML in chronic phase?

A

Imantinib - tyrosine kinase inhibitor
Hydroxycarbamide
a-interferon

120
Q

Chronic Lymphblastic Leukaemia

  • what
  • who
  • where
A

lymphoproliferative disease
elderly population M>F
bone marrow

121
Q

Small lymphocytic leukaemia (SLL)

  • what
  • where
A

lymphoproliferative disease

lymph nodes

122
Q

Clinical features of CLL (4)

Association with CLL (2)

A
  1. painless lymphadenopathy
  2. anaemia
  3. thrombocytopenia
  4. weight loss, low grade fever, night sweats

Autoimmunity AIHA, ITP

123
Q

Evan’s Syndrome

A

autoimmunity associated with CLL

124
Q

Good prognostic feature in CLL?

A

hypermutated Ig gene

125
Q

Bad prognostic feature in CLL?

A

raised LDH

126
Q

Staging of CLL?

A

Binet Staging
A - High WBC, 3 LNs
C - anaemia, thrombocytopenia

127
Q

Hodgkin’s Lymphoma

  • percentage of lymphoma
  • who
  • association
  • clinical presentation
  • B constitutional symptoms (5)
A
  • 20%
  • bimodal age incidence 1. 20-29years 2. >60years
  • EBV
  • symmetrical painless lymphadenopathy
  • pain in LNs after alcohol
    1. weight loss
    2. night sweats
    3. pruritis
    4. fever
    5. fatigue
128
Q

Blood film finding in Hodgkins Lymphoma

A

Reed-Sternberg binucleate cells

129
Q

Staging of Hodgkins lymphoma

A

Stage 1 - one LN region
Stage 2 - >2 LN regions SAME sides of diaphragm
Stage 3 - >2 LN regions OPPOSITE sides of diaphragm
Stage 4 - extranodal sites

A - no constitutional symptoms
B - constitutional symptoms

130
Q

Chemotherapy in Hodgkins lymphoma

A 
AVBD
Adriamycin
Bleomycin 
Vinblastine 
Dacarbazine
131
Q

Non-Hodgkins lymphoma

  • what
  • percentage of lymphomas
A
  • all lymphomas other than Hodgkins

- 80%

132
Q

Burkitts Lymphoma

  • cell type
  • 3 types
  • mutation
  • high or low grade?
  • histology
  • chemotherapy
A

B cell

  1. Endemic
  2. Sporadic
  3. Immuno-deficiency

c-myc oncogene overexpression

high grade

starry-sky appearance

rituximab

133
Q

Endemic Burkitts Lymphoma

  • who
  • where
  • association
  • presentation
A

children/teenagers
equatorial Africa
EBV
jaw involvement

134
Q

Sporadic Burkitts Lymphoma

  • where
  • association
  • presentation
A

outside of Africa
EBV
no jaw involvement

135
Q

Immunodeficiency Burkitts Lymphoma

- who (2)

A
  1. HIV patients

2. post-transplant patients

136
Q

Follicular Lymphoma

  • cell type
  • histology
  • characteristics
A
  • B cell
  • nodular appearance
  • indolent and incurable
137
Q

MALT/Marginal Zone Lymphoma

  • who
  • associations (2)
A

middle-aged

  1. H.pylori
  2. Sjogren’s syndrome
138
Q

Richter’s syndrome

A

CLL transforms into a large B cell lymphoma with poor prognosis

139
Q

Diffuse Large B Cell Lymphoma

  • who
  • histology
A

middle-aged and elderly

large sheets of lymphoid cells

140
Q

Mantle Cell lymphoma

  • who
  • nature of cancer
  • histology
A

middle-aged men
aggressive
angular nuclei

141
Q

Anaplastic large cell lymphoma

  • cell type
  • who
  • histology
A

T cell
children and young adults
large epithelioid lymphocytes

142
Q

Coeliac disease is associated with which lymphoma?

A

Enteropathy-associated T cell lymphoma

143
Q

Multiple Myeloma

  • what
  • who
  • production of?
A

neoplasia of plasma cells
middle aged - elderly
high incidence in Afro-Caribbean
paraproteins IgG

144
Q

Signs and symptoms in Multiple Myeloma? (CRAB)

A

C - hyperCalcemia
R - Renal impairment - amyloidosis, nephrotic syndrome
A - Anaemia
B - Bones - osteolytic lesions, fractures

145
Q

Clinical findings in Multiple Myeloma? (5)

A
  1. dense, narrow single monoclonal band of serum electrophoresis
  2. rouleaux on blood film
  3. Bence Jones proteins in urine
  4. High ESR
  5. > 10% plasma cells in bone marrow
146
Q

MGUS Monoclonal Gammaglobinopathy of Unknown Significance

A
147
Q

Smoldering Myeloma (2)

A

plasma cells >10% but no CRAB symptoms

148
Q

Waldenstrom’s Macroglobinaemia

  • what
  • who
  • which antibody?
  • symptoms (3)
A

lymphoproliferative low grade non-hodgkins lymphoma
elderly men
IgM
1. weight loss
2. fatigue
3. hyperviscosity syndrome (spontaneous bleeding, retinopathy, neuropathy)

149
Q

Systemic Amyloidosis

  • which antibody?
  • investigation
  • presentation (5)
A

Ig light chain paraprotein deposition in tissues
Congo Red stain to apple green birefringence
1. macroglossia
2. carpal tunnel syndrome
3. peripheral neuropathy
4. HF
5. renal failure

150
Q

What are myelodysplastic syndromes?

A

Heterogenous group of progressive disorders of
1) ineffective proliferation
2) ineffective differentiation
of abnormally maturing myeloid stem cells

151
Q

Myelodysplastic syndrome

  • who
  • risk
  • how are they diagnosed?
  • blasts
A
  • elderly
  • transformation into AML
  • incidental finding of routine bloods
152
Q

Clinical features of myelodysplastic disorder (4)

A
  1. BM failure - infection, fatigue, easy bleeding
  2. Pseudo-Pelger-huet anomaly -hyposegmented neutrophils
  3. sideroblasts
  4. micromegakarycytes
153
Q

Pseudo-Pelger-huet anomaly is associated with?

A

Myelodysplastic syndrome

154
Q

Classification of Myelodysplastic Syndrome (5)

A
  1. Refractory Anaemia with/without sideroblasts
  2. Refractory Cytopenia with multilineage dysplasia
  3. Refractory Anaemia with Excess Blasts I (5-10%) II (11-20%)
  4. MDS with 5q deletion
  5. Unclassified MDS - with fibrosis, or childhoos MDS
155
Q

Death from myelodysplastic syndrome?

A

1/3 bleeding
1/3 infection
1/3 leukaemia

156
Q

Bone Marrow Failure

A

damage to or suppression of haematopoietic pluripotent stem cells

157
Q

Primary Bone Marrow Failure (2)

A

a) inherited

b) idiopathic

158
Q

Secondary Bone Marrow Failure (5)

A
  1. malignancies
  2. radiation
  3. drugs - chemo, Abx, thiazides
  4. viruses
  5. auto-immune - SLE
159
Q

Inherited causes of Aplastic Anaemia (4)

A
  1. Fanconi Anaemia
  2. Dyskeratosis Congenita
  3. Schwachman-Diamond Syndrome
  4. Diamond-Blackfan Syndrome
160
Q

Fanconi Anaemia

  • who
  • inheritance pattern
  • cytopenia?
  • clinical features (5)
A
  • children aged 5-10yrs
  • autosomal recessive
  • PANCYTOPENIA
    1. short stature
    2. skin pigmentation - cafe-au-lait spots
    3. microophthalmia
    4. abnormal thumbs
    5. renal malformations
161
Q

Dyskeratosis Congenita

  • inheritance pattern
  • characterised by (3)
  • genetic mutation
A 
X-linked
1. Bone Marrow Failure
2. Cancer predisposition 
3. Somatic abnormalities 
telomere shortening
162
Q

Triad of Dyskeratosis Congenita

A
  1. nail dystrophy
  2. leukoplakia
  3. skin pigmentation
163
Q

Diamond-Blackfan Syndrome

  • who
  • cytopenia?
A

neonates - 1 year

pure red cell aplasia

164
Q

Schwachman-Diamond Syndrome

  • inheritance pattern
  • cytopenia
  • risk association
  • clinical features (5)
A 
autosomal recessive 
neutropenia 
AML
1. short stature
2. pancreatic dysfuncion 
3. hepatic impairment 
4. endocrine dysfunction 
5. skeletal abnormalities
165
Q

Myeloproliferative disorder

A

clonal prolieration of one or more haematopoeitic line of cells i.e increased production of mature cells

166
Q

Mutation associated with Philadelphia chromosome negative disorders?

A

JAK2

167
Q

Polycythaemia lab results? (4)

A
  1. raised red cell mass
  2. raised Hb
  3. raised red cell count
  4. raised packed cell volume
168
Q

Causes of pseudo-polycythaemia (4)

A
  1. Burns
  2. Dehydration
  3. D&V
  4. smoking
169
Q

Causes of secondary polycythaemia? (3)

A

RAISED EPO

  1. renal Ca
  2. high altitude
  3. chronic hypoxia
170
Q

Polycythaemia Rubra Vera

  • mutation
  • predominate precursor cell
  • how is it diagnosed?
  • who?
A

JAK2
erythroid precursors
routine bloods
elderly

171
Q

Clinical Features of Polycythaemi arubra Vera (10)

- why?

A

HYPERviscosity HYPERvolaemia HYPERmetabolism

  1. blurred vision
  2. headache
  3. plethoric red nose
  4. gout - high RBC turnover high uric acid
  5. stroke
  6. retinal vein engorgement
  7. splenomegaly
  8. erythromelagia
  9. aquagenic pruritis - itching on water contact
  10. peptic ulcers
172
Q

Lab results in Polycythaemia Rubra Vera?

A

Hb high
HCT high
Plts high
WCC high

EPO low

173
Q

Essential Thrombocythaemia

  • dominant cell in BM
  • mutation
  • who?
A

megakaryocytes (-> plts)
JAK2
~30 years F=M
~55 year F>M

174
Q

Clinical features of Essential Thrombocythaemia? (9)

A 
DVT/PE
Stroke
MI
Gangrene
Haemorrhage
Erythromelagia
Splenomegaly
Dizziness
Headaches
175
Q

Management of essential thrombocythaemia?

- side effects (2)

A

Anagrelide

  1. palpitations
  2. flushing
176
Q

Myelofibrosis

A

myeloproliferative disease whereby myeloproliferation causes fibrosis of the bone marrow and ultimatley leads to replacement of bone marrow with collagenous tissue

177
Q

Myelofibrosis

  • who
  • cytopenia
  • prognosis
A
  • elderly
  • pancytopenia
  • 2-5 years
178
Q

Clinical Features of Myelofibrosis (7)

A
  1. hepatomegaly
  2. splenomegaly
  3. fatigue
  4. weight loss
  5. dyspnoea
  6. Budd-Chiari syndrome
  7. night sweats
179
Q

Myelofibrosis
Blood film? (2)
Bone Marrow? (2)

A

Blood film - poikilocytes, leukoerythroblasts

BM - “dry tap” collagen fibrosis

180
Q

Antibodies present in blood type A?

Antigens present in blood type A?

A

Anti-B

A antigen

181
Q

Antibodies present in blood type B?

Antigens present in blood type B?

A

Anti-A

B antigen

182
Q

Antibodies present in blood type AB?

Antigens present in blood type AB?

A

No antibodies

A and B antigen

183
Q

Antibodies present in blood type O?

Antigens present in blood type O?

A

Anti-A, Anti-B

No antigens

184
Q

Most common blood groups in the UK?

A

A & O

185
Q

Haemolytic Disease of the newborn

  • how?
  • common antibody
  • other antibodies (4)
A

If a Rh negative patient is transfued with Rh positive blood they can form Anti-D antibodies
No acute problem
However, if later during pregnancy a Rh negative woman encounters fetal cells either during pregnancy or delivery, and the Anti-D is high… it can destroy the fetal red cells with the corresponding antigen.
Resulting in
i) anaemia
ii) jaundice
iii) hydrops fetalis

Anti-D

Anti-c, anti-K IgG ABO

186
Q

Indications for platelets transplant? (3)

A
  1. Massive transfusion aim plts >75
  2. Prevent bleeding post-chemo
  3. Prevent bleeding post-surgery
187
Q

Indications for FFP transplant? (3)

- what is it?

A
  1. Massive transfusion blood loss >150L/min
  2. DIC with bleeding
  3. Liver disease + risk

contaisn clotting factors

188
Q

Febrile Non-Haemolytic Transfusion Reaction

  • symptoms (3)
  • when
  • management
  • cause
A
  • Rise of temp 1*C during or soon after transfusion, rigors, chills
  • Acute
189
Q

Allergic Transfusion Reactions

  • symptoms (2)
  • when
  • management
  • cause
  • type of transfusion
  • who
A
  • mild urticaria, wheeze

- acute

190
Q

Wrong ABO Blood

  • symptoms (7)
  • when
  • cause
  • management (4)
A
  • Restless, chest/ loin pain, fever, vomiting, flushing, collapse, haemoglobinuria
  • Acute
191
Q

Delayed Haemolytic Transfusion Reaction

  • symptoms (4)
  • when
  • cause
  • management (3)
A
  • high bilirubin, low Hb, high retics haemoglobinuria
  • over next few days > 24hrs
  • patients who are transfused can develop an ‘immune’ antibody to one of the ‘foreign’ RBC antigens they were exposed to, but not tested for … if the patient has another transfusion with RBCs expressing the same ‘foreign’ antigen the ‘immune’ antibodies cause RBC destruction
    = EXTRAVASCULAR HAEMOLYSIS
  • repeat x-match: detect new Ab
    May need further transfusion
    Treat renal failure
192
Q

Anaphylaxis following transfusion

  • what
  • when
  • symptoms (4)
  • causes (3)
A
  • Severe, life-threatening reaction soon after start of transfusion
  • acute
193
Q

Transfusion associated Cardiac Overload (TACO)

  • what
  • when
  • symptoms (5)
  • who
  • causes
A
  • pulmonary oedema due to fluid overload

- acute

194
Q

Transfusion Related Acute Lung Injury (TRALI)

  • what & symptoms (3)
  • when
  • who
  • cause (4 steps)
  • prevention (2)
A
  • Acute dyspnoea with hypoxia and bilateral pulmonary infiltrates during or within 6 hours of transfusion, not due to circulatory overload or other likely causes
  • acute TRAIL … leave a trail of anti-leucocyte antibodies”
    1. Donor anti-leucocyte antibodies (HLA or anti-granulocyte Abs)
    2. Interact with patient’s leucocyte antigens
    3. Aggregates of white blood cells get stuck in the pulmonary small capillaries
    4. Release neutrophil proteolytic enzymes and toxic oxygen metabolites causes lung damage
  • don’t give FFP from female donors, stop unnecessary use of FFP
195
Q

Delayed Non-Immune adverse reactions to transfusions? (3)

A
  1. Bacterial infection
  2. Viral infection
  3. Iron overload
196
Q

Presentation of delayed bacterial transmission?

A
  1. Haemolysis

2. Anaphylaxis

197
Q

Transfusion Associated Graft-Versus-Host Disease

  • who (2)
  • cause
  • prevention
A
  1. immunocompramised patients
  2. donor is HLA-matched or HLA-similar
    - donor’s blood contains some viable lymphocytes, they should be recognised as “foreign” by recipient, if recipient is unable to do this…lymphocytes are not recognised and destroyed
    - lymphocytes recognise same antigen tissue as “foreign” - > Causes severe diarrhoea, liver failure, skin desquamation, bone marrow failure  death weeks to months post transfusion
    - irradiate donors blood before transfusion to ensure no lymphocytes are capable of dividing
198
Q

Post-transfusion Purpura

  • what
  • when
  • cause
  • management
A

development of purpura within 7-10 days of transfusion
delayed > 24 hrs
HPA-1a negative patients previously immunised by pregnancy or transfusion, causes autoimmune destruction of platelets
IVIG

199
Q

Prevention of Anti-D sensitisation? (2)

A
  1. Must always transfuse RhD negative females of child bearing potential with RhD negative blood
  2. Can give intra-muscular injection of anti-D immunoglobulin, at times when mother is at risk of a fetomaternal bleed e.g. at delivery
    - must be within 72 hrs
200
Q

Evenst when FetoMaternal Haemorrhage is likely to occur (5)

A
  1. spontaneous miscarriages if surgical evacuation needed and therapeutic abortions
  2. amniocentesis and chorionic villous sampling
  3. abdominal trauma (falls and car accidents)
  4. external cephalic version (turning the fetus)
  5. stillbirth or intrauterine death
201
Q

Test for fetomaternal haemorrhage?

A

Kleihauer Test

202
Q

Changes in maternal FBC during pregnancy? (4)

A
  1. mild anaemia
  2. macrocytosis
  3. neutrophilia
  4. thrombocytosis
203
Q

Why mild anaemia during pregnancy?

A
  • dilutionary effct
  • red cell mass increases
  • plasma volume increases
  • overall increased total iron but decreased concentration
  • due to increased iron requirement during pregnancy
204
Q

How much does plasma volume increase during pregnancy?

A

150%

205
Q

How much does red cell mass increase during pregnancy?

A

120-130%

206
Q

Consequence of iron deficiency during pregnancy?

A

IUGR

207
Q

Consequence of folate deficiency during pregnancy?

A

neural tube defects

208
Q

Recommended iron supplement dose during pregnancy?

A

60 mg RDA

209
Q

Recommended folic acid supplement dose during pregnancy?

A

400mcg RDA

210
Q

Level of expected thrombocytopenia during pregnancy?

A

175-199 x 10*9/L

211
Q

Causes of thrombocytopenia during pregnancy? (5)

A
  1. Gestational/Physiological
  2. Pre-eclampsia
  3. ITP
  4. Microangiopathic syndromes e.g. APLS, HUS, SLE
  5. Other e.g. haematological malignancies, DIC
212
Q

Why is pregnancy a hypercoagulable state? (3)

A
  1. increased thrombin generation
  2. increased fibrin cleavage
  3. reduced fibrinolysis
213
Q

Highest risk of PE at what gestation?

A

40 - 46 weeks

214
Q

Post Partum Haemorrhage definition

A

> 500mL blood loss

215
Q

Amniotic Fluid Embolism

  • what
  • why
  • presentation (4)
A
  • fetal debris e.g. amniotic fluid, hair etc enters maternal circulation and triggers an allergic-type repsonse
  • coagulation changes during pregnancy predispose to DIC
    1. sudden onset shivers
    2. vomiting
    3. shock
    4. DIC
216
Q

Newborns in contrast to adults have

a) A higher Hb
b) A lower WBC
c) Smaller red blood cells
d) The same percentage of haemoglobin F

A

a) a higher Hb

217
Q

Haemoglobin A

  • globin chains
  • when is it present (4)
A

a2b2

  1. late fetus
  2. infant
  3. child
  4. adult
218
Q

Haemoglobin A2

  • globin chains
  • when is it present? (3)
A

a2theta2

  1. infant
  2. child
  3. adult
219
Q

Haemoglobin F

  • globin chains
  • when is it present? (2)
A

a2gamma2

  1. fetus
  2. infant
220
Q

Why does sickle cell anaemia not manifest at birth?

A

Clinical features become manifest as gamma chain production and haemoglobin F synthesis decrease and betaS and haemoglobin S production increase

221
Q

Complications of Sickle Cell Anaemia that are more common in adults than in children?

a. Hand-foot syndrome
b. Hyposplenism
c. Red cell aplasia
d. Splenic sequestration
e. Stroke

A

b. hyposplenism because recurrent infarction has left the spleen small and fibrotic

222
Q

Siblings with sickle cell anaemia present simultaneously with severe anaemia and a low reticulocyte count—likely diagnosis?

a. Splenic sequestration
b. Parvovirus B19 infection
c. Folic acid deficiency
d. Haemolytic crisise
e. Vitamin B12 deficiency

A

b. Parvovirus B19 Infants and children with sickle cell disease initially have no immunity to parvovirus B19—their first exposure leads to pure red cell aplasia

Pathology (7 decks)

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