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Year 3 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the origin of platelets? What type of storage granules do they contain?

A

Megakaryocytes- come from the common myeloid precursor in haematopoesis.

3 types of storage granules:

  • alpha: contain clotting factors
  • dense: contain mediators of platelet activation eg. serotonin
  • glycogen- provide energy
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2
Q

Which factors are involved specifically in the extrinsic pathway of secondary haemostasis?

A

Tissue Factor

Factor 7

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3
Q

Which factors are involved specifically in the intrinsic pathway of secondary hemostasis?

A

Factor 12
Factor 11
Factor 8
Factor 9

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4
Q

Which clotting factor is deficient in:
A- Hamophilia A
B- Hamophilia B
C- Hamophilia C

A

A- Factor 8
B- Factor 9
C- Factor 11

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5
Q

Which of the clotting factors are vitamin k dependant?

A

2 (prothrombin), 7 (extrinsic), 9 (intrinsic) and 10

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6
Q

How does Warfarin work?

A

Prevents intrinsic and extrinsic clotting cascade by inhibiting the synthesis of Vitamin K dependent clotting factors (Prothrombin, 7, 9 and 10)

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7
Q

How does Apixaban work?

A

Prevents intrinsic and extrinsic clotting cascades by inhibiting factor 10a.

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8
Q

What is fibrinolysis?

A

The process by which the fibrin clot is degraded.

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9
Q

How does the body prevent unwanted clots?

A
  1. Protein C in the venous circulation (activated by thrombin) degrades 5a and 8a.
  2. Natural heparins on the endothelial cell surfaces enhance the release of prostacyclin, and also the activity of anti-thrombin.
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10
Q

What are the 2 main blood tests for clotting?

A
  1. Prothrombin time- tests the extrinsic pathway

2. Activated partial prothrombin time- tests the intrinsic pathway.

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11
Q

What normally prevents platelets from adhering to endothelial cells?

A
  1. Nitrous oxide
  2. Prostacyclin

PREVENT platelets from adhering to endothelial cells.

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12
Q

What are acanthocytes?

A

RBCs with spikes on surface

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13
Q

In which conditions may acanthocytes be present in the blood?

A

Splenectomy
Alcoholic liver disease
Spherocytosis

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14
Q

What are blasts?

A

Nucleated precursors

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15
Q

In which conditions may blasts be present in the blood?

A

Leukaemia

Myelofibrosis

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16
Q

What are Burr cells and In which conditions may they be present in the blood?

A

RBCs with surface projections, present in:

Renal failure
Liver failure

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17
Q

What is a Cabot ring and in which conditions may they be present in the blood?

A

Thin red/ purple ring in a RBC, present in:

Pernicious anaemia
Lead poisoning
Bad infections

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18
Q

Which condition may have cells with Auer rods present?

A

AML

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19
Q

Which conditions have hypochromic RBCs?

A

Iron deficiency anaemia

Thalassaemia

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20
Q

What are the blood film features in iron deficiency anaemia?

A
  1. Pencil cells
  2. Hypochromic RBCs
  3. Target cells
  4. Microcytic RBCs
  5. Decreased ferritin
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21
Q

What are the blood film features in haemolytic anaemia?

A
  1. Spherocytes
  2. Reticulocytes
  3. Tear shaped RBCs
  4. Normocytic/ macrocytic
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22
Q

What are the blood film features in B12/ folate deficiency?

A
  1. Macrocytic RBCs

2. Hypersegmented neutrophils (6-8 lobes)

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23
Q

When are tear drop shape RBCs found?

A

Extra-medullary haematopoeisis eg. Myelofibrosis, haemolysis

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24
Q

When are target cells seen?

A
  1. Iron deficiency anaemia
  2. Thalassaemia
  3. Liver disease
  4. Hyposplenism
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25
Q

What are spherocytes and in which conditions may they be present?

A

Spherical hyperchromic RBCs:

  1. Hereditary spherocytosis
  2. AIHA
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26
Q

What are schistocytes and in which conditions may they be present?

A

Fragmented RBCs which have been sliced by fibrin
DIC
Thrombotic thrombocytopenic purpura

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27
Q

What are reticulocytes and in which conditions may they be present?

A

Large young RBCs

Haemolytic anaemia

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28
Q

How are blood tests affected in DIC?

A 
APTT increased
PT (INR) increased
TT increased
D-dimer increased
Platelet count decreased
Presence of schistocytes
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29
Q

How are blood tests affected in Haemophilia?

A

APTT increased

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30
Q

How are blood tests affected in VWD?

A

APTT increased

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31
Q

How are blood tests affected in Vitamin K deficiency?

A

PT and APTT increased

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32
Q

How are blood tests affected in liver disease?

A

PT increased
APTT increased
Platelet count may decrease

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33
Q

What are the characteristics of Haemophilia?

A

Bleeding into joints

Bleeding into muscles

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34
Q

What are the characteristics of Von Willebrand disease?

A

Bleeding
Epistaxis
Usually less severe than Haemophilia

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35
Q

What is desmopressin used for?

A

Managing haemophilia and Von Willebrands disease

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36
Q

Which triad may be present in Haemochromatosis?

A
  1. Tanned skin
  2. Cirrhosis
  3. Diabetes
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37
Q

What is haemochromatosis?

A

Excessive intestinal absorption of iron

Iron overload and accumulation in tissues (liver, adrenals, heart, skin, pancreas)

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38
Q

Which gene is usually mutated in hereditary haemochromatosis and how is this inherited?

A

Autosomal recessive inheritance- mutations in HFE gene

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39
Q

Which factors promote fibrinolysis?

A
  1. Thrombin induces the release of TPA which converts plasminogen to plasmin. Plasmin helps to break the fibrin mesh
  2. Thrombin also stimulates the production of anti-thrombin: which inhibits the production of thrombin and factor 10a, 9a and 11a.
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40
Q

Which myeloproliferative neoplasm affects WBCs?

A

CML

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41
Q

Which myeloproliferative neoplasm affects RBCs?

A

Polycythaemia vera

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42
Q

Which myeloproliferative neoplasm affects platelets?

A

Essential thrombocythaemia

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43
Q

Which myeloproliferative neoplasm affects fibroblasts?

A

Myelofibrosis

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44
Q

Most myeloproliferative neoplasms have an abnormality in which gene?

A

JAK2

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45
Q

Which myeloproliferative neoplasm may present with headaches, facial plethora, burning sensation in fingers and toes, and tinnitus?

A

Polycythaemia vera

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46
Q

In polycythaemia vera, there is excess production of ______

A

RBCs
WBCs
Platelets

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47
Q

In polycythaemia vera there is increased risk of __________

A 
Hyperviscosity
Venous thrombi (DVT, PE), arterial thrombi (MI)
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48
Q

Other than polycythaemia vera, what are the other causes of polycythaemia (increased RBC production)?

A
  1. Hypoxia

2. Excess EPO hormone

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49
Q

How can polycythaemia vera be treated?

A
  1. Venesection
  2. Aspirin
  3. Hydroxycarbamide for thrombocytosis
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50
Q

What are the 3 main consequences of essential thrombocythaemia?

A
  1. Bleeding
  2. Thrombi (Arterial or venous)
  3. Microvascular occlusion
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51
Q

Microvascular occlusion is a consequence of thrombocythaemia and can cause symptoms such as…..

A 
Headache
Blurred vision
Chest pain
Digital ischemia
Burning in fingers/ toes (erythromelalgia)
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52
Q

What are the differentials for essential thrombocythaemia?

A

Cancer
Chronic inflammation
Past surgery

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53
Q

The BCSH criteria are used for diagnosing…..

A

Essential thrombocythaemia

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54
Q

Essential thrombocythaemia can be treated using ……….

A
  1. Aspirin

2. Hydroxycarbamide

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55
Q

In __________ there is clonal proliferation of megakaryocytes causing platelet counts of >1000 with abnormal functioning platelets.

A

Essential thrombocythaemia

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56
Q

What is myelofibrosis?

A

Clonal proliferation of fibroblasts
Bone marrow fibrosis
Leads to extramedullary haematopoiesis (spleen and liver)

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57
Q

What are the consequences of myelofibrosis?

A
  1. Anaemia
  2. Infection
  3. Extramedullary haematopoeisis
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58
Q

If a patient presented with the following symptoms what may the diagnosis be?
B symptoms (fever, night sweats, weight loss)
Anaemia
Infections
Enlarged spleen
Gout

A

Myelofibrosis

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59
Q

How does myelofibrosis appear on blood films?

A
  1. Tear drop shape RBCs

2. Nucleated RBCs (leukoerythroblastic cells)

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60
Q

Ruxolitinib is an oral JAK2 inhibitor, used for managing ___________. It causes decreased nightsweats and reduction in splenomegaly.

A

Myelofibrosis

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61
Q

The BCR:ABL translocation is most commonly present in which condition?

A

CML

62
Q

How can AML and CML be distinguished on blood film?

A

AML:

  1. Auer rods
  2. Myeloblasts
  3. Monoblasts

CML:

  1. High cell count (hypercellular marrow)
  2. Raised eosinophils
  3. Raised basophils
63
Q

Which type of leukaemia is most common in children?

A

ALL

64
Q

What are the 3 phases of CML?

A
  1. Chronic phase
  2. Accelerated phase
  3. Blast transformation
65
Q

What are the myelodysplastic syndromes?

A

Group of disorders which present as bone marrow failure (anaemia, neutropenia and thrombocytopenia)

66
Q

What is the pathogenesis of myelodysplastic syndromes?

A

MDS arises in haematopoietic stem cells
Abnormal cells produced due to a partial block in differentiation
Blasts may accumulate

67
Q

How can myelodysplasia be diagnosed on blood film?

A

Pancytopenia
Increased marrow cellularity
Ring sideroblasts

68
Q

How may myelodysplasia be treated?

A
  1. Transfusion of RBCs or platelets
  2. EPO
  3. G-CSF
  4. Allogenic stem cell transplant
69
Q

If a patient presented with the following 3 symptoms what may be the diagnosis?

  1. Jaundice
  2. Hepatosplenomegaly
  3. Pigment gallstones
A

Haemolytic anaemia

70
Q

What is the direct Coombs test used for?

A

Autoimmune haemolytic anaemia

detects antibodies for RBCs. Agglutination = positive test result

71
Q

When is the indirect Coombs test used?

A

Prenatal testing
Before blood transfusion

Detects anti RBC antibodies free in serum

72
Q

Warm AIHA is mediated by ____

A

IgG

73
Q

Cold AIHA is mediated by ____

A

IgM

74
Q

How is warm autoimmune haemolytic anaemia treated?

A
  1. Steroids
  2. Rituximab
  3. Potential splenectomy
75
Q

How is cold autoimmune haemolytic anaemia treated?

A
  1. Keep warm
  2. Blood transfusions
  3. Chlorambucil
76
Q

What are the ACQUIRED types of haemolytic anaemia?

A
  1. Autoimmmune (warm or cold)
  2. Drug induced
  3. Infection
  4. Microangiopathic haemolytic anaemia
77
Q

What are the HEREDITARY types of haemolytic anaemia?

A
  1. Enzyme defects eg. GP6D deficiency, pyruvate kinase deficiency
  2. Membrane defects eg. Hereditary spherocytosis
  3. Haemogobinopathys eg. Thalassaemia, Sickle cell
78
Q

Which type of haemolytic anaemia will be Coombs test POSITIVE?

A

Autoimmune haemolytic anaemia (acquired)

79
Q

What happens to iron, ferritin and total binding capacity in IDA?

A

Iron decreases
Ferritin decreases
TIBC increases

80
Q

What happens to iron, ferritin and total binding capacity in anaemia of chronic disease?

A

Iron decreases
Ferritin increases
TIBC decreases

81
Q

What happens to iron, ferritin and total binding capacity in haemolytic anaemia?

A

Iron increases
Ferritin increases
TIBC decreases

82
Q

_______ inhibits iron absorption by binding ferroportin, causing it to become internalised.

A

Hepcidin

83
Q

In inflammation/ infection, Il-6 is released, causing Hepcidin to be switched ___

A

ON

84
Q

Sickle cell anaemia is caused by a genetic polymorphism in the _______ gene, causing Glu -> Val translocation, leading to HbA -> HbS.

A

Beta globin

85
Q

What happens to HbS when it is deoxygenated?

A

It polymerises, causing RBCs to deform and block small vessels, leading to ischemic pain.

86
Q

What are the potential complications of sickle cell anaemia?

A
  1. Vaso-occlusive crises
  2. Infections
    3, Hyposplenism- pneumococcus, meningococcus
  3. Acute chest syndrome
  4. End organ damage
87
Q

Which type of Thalassaemia is more common?

A

Beta thalassaemia

88
Q

When does Beta Thalassaemia usually present?

A

Aged under 1 year

Newborn has HbF but this declines in levels
Present with severe anaemia and failure to thrive

89
Q

Alpha thalassaemia major/ Hb Barts usually results in _________

A

Death in utero

90
Q

What are some inherited causes of thrombophilia?

A
  1. Protein C & S deficiency
  2. Factor V Leiden
  3. Anti thrombin deficiency
91
Q

What are some acquired causes of thrombophilia?

A
  1. Oral contraceptive pill

2. Antiphospholipid syndrome

92
Q

What is myeloma?

A

Monoclonal proliferation of plasma cells in bone marrow

Leads to IgG/ IgA secretion (paraprotein) which can’t function properly

93
Q

What are the clinical features of myeloma? (CRABI)

A 
C- Hypercalcaemia
R- Renal impairment
A- Anaemia, neutropenia and thrombocytopenia
B- Bone pain and destruction
I- Infection
94
Q

How can myeloma be diagnosed?

A
  1. Monoclonal protein band on electrophoresis of blood or urine
  2. Plasma cells >10% in marrow
  3. Organ damage: anaemia, hyperkalaemia, renal failure
  4. Xrays: bone lesions
95
Q

What are the complications of myeloma?

A
  1. Hypercalcaemia: renal stones, brittle bones
  2. Anaemia
  3. Spinal cord compression
  4. Hyperviscosity
  5. AKI
96
Q

Which bones does myeloma often affect?

A

Pelvis
Spine
Ribs
Shoulders

97
Q

How can myeloma be treated?

A
  1. Analgesia
  2. Bisphosphonates (Zoledronate) slow down osteoporosis
  3. Blood transfusions
  4. Iv immunoglobulins
  5. Chemotherapy (Lenalidomide, Dexamethosone, Velcade)
  6. Autologous stem cell transplant
98
Q

Give examples of microcytic anaemia.

A

Iron deficiency anaemia
Haematinic deficiencies- sickle cell, thalassaemia
Lead poisoning
Anaemia of chronic disease

99
Q

Give examples of normocytic anaemia

A 
Anaemia of chronic disease
Bone marrow failure
Acute blood loss
Renal failure
Aplastic anaemia
100
Q

Give examples of macrocytic anaemia.

A 
Megaloblastic: B12 or folate deficiency
Alcohol
Hypothyroidism
Haemolytic anaemia
Liver disease
Myelodysplasia
Serum paraprotein
101
Q

Monoclonal gammopathy of unknown significance significance usually preceeds _________.

A

Myeloma

102
Q

What is the lower limit of the Hb reference range for women?

A

120g/L

103
Q

What is the lower limit of the Hb reference range for men?

A

130g/L

104
Q

Which protein effluxes iron into the blood from enterocytes?

A

Ferroportin

105
Q

What are the causes of iron deficiency anaemia?

A

Poor dietary intake
Poor absorption: Crohn’s, coeliac
Iron loss: GI bleeding, menorrhagia
Increased requirement: pregnancy

106
Q

Which drugs may cause folate deficiency?

A

Methotrexate

Phenytoin

107
Q

The majority of B12 is absorbed by binding ________ and being absorbed in the terminal ileum.

A

Intrinsic factor

108
Q

What is pernicious anaemia?

A

Autoimmune gastritis meaning a lack of intrinsic factor secreted from parietal cells
Poor absorption of Vitamin B12

109
Q

Which blood group is the universal donor of RBCs?

A

Group O neg

110
Q

Which blood group is the universal recipient of RBCs?

A

Group AB positive

111
Q

What is the most common blood group in Caucasians?

A

Group O

112
Q

Which Ig antibodies against RBCs cause extravascular haemolysis slowly?

A

IgG

113
Q

Which Ig antibodies against RBCs cause acute and dramatic intravascular haemolysis?

A

IgM

114
Q

How does forward blood typing work?

A

Patients RBCs added to antibodies
If agglutination appears = patients RBCs has those antigens

ANTIGEN SCREENING

115
Q

How does reverse blood typing work?

A

Patients plasma is added to RBCs
Used to detect which antibodies are in the patients serum

ANTIBODY SCREENING

116
Q

Which type of blood product is stored for <35days at 4degrees?

A

RBCs

117
Q

Which type of blood product is stored for <7days at 22degrees?

A

Platelets

118
Q

Which type of blood product is stored frozen?

A

Plasma

119
Q

What is the volume of the average bag of donor blood?

A

274ml

120
Q

What are the 4 types of blood product that can be transfused?

A
  1. RBCs
  2. Platelets
  3. Plasma (FFP)
  4. Cryoprecipitate (frozen fibrinogen replacement)
121
Q

How long should a bag of RBCs be transfused over?

A

2-3 hours

122
Q

How long should a bag of platelets be transfused over?

A

30 mins

123
Q

What is the definition of major haemorrhage?

A
  1. Loss of >50% total blood in <3 hours
  2. Loss of whole blood volume over 24 hours
  3. Loss of >150ml/minute
124
Q

What are the 3 most SERIOUS risks of blood transfusion?

A
  1. ABO incompatibility
  2. Bacterial contamination
  3. Anaphylaxis
125
Q

What are the 3 most COMMON risks of blood transfusion?

A
  1. Transfusion associated circulatory overload (TACO)
  2. Allergy
  3. Febrile non haemolytic transfusion reaction (delayed)
126
Q

How many pooled donations are used in a bag of platelets?

A

4

or 1 apheresis

127
Q

How many pooled donations are used in a bag of cryoprecipitate?

A

2

128
Q

Which haematological cancer presents with B symptoms, cervical/axillary lymphadenopathy and alcohol related lymph node pain?

A

Hodgkin’s lymphoma

129
Q

The Ann Arbor system is used to stage which haematological cancer?

A

Hodgkin’s lymphoma

130
Q

Which chemotherapys are used for Hodgkin’s lymphoma?

A

A- Adriamycin
B- Bleomycin
V- Vinblastine
D- Dacarbazine

131
Q

Which drugs make up the RCHOP regimen for high grade NHL lymphoma treatment?

A 
R- Rituximab
C- Cyclophosphamide
H- Hydroxydaunomycin
O- Oncovin (Vincristine)
P- Prednisolone
132
Q

Rituximbab and Chlorambucil are used to treat low grade ________

A

Non Hodgkin’s Lymphoma

133
Q

Thrombopoeitin usually stimulates __________

A

Platelet production

134
Q

Which clotting factor does von willebrand bind?

A

Factor 8

135
Q

What is aplastic anaemia?

A

Bone marrow failure

Bone marrow stem cells disappear leaving fat spaces

136
Q

Serum LDH is raised in which type of anaemia?

A

Haemolytic anaemia

137
Q

Which antibody is secreted in breast milk?

A

IgA

138
Q

IgA deficiency leads to an increased susceptibility to which type of bacteria?

A

Giardia lamblia

139
Q

Bernard-Soulier syndrome is an autosomal recessive bleeding disorder caused by a deficiency in the platelet glycoprotein complex Ib-IX-V, which would normally bind to __________________________ to allow platelet adherence.

A

von Willebrand factor (vWF)

140
Q

Thrombocytopenia is ______

A

Abnormally low numbers of platelets

141
Q

DiGeorge syndrome involves failure of the thymus and can be rememberd by the mnemonic CATCH:

A 
C-Cardiac abnormalities
A- Abnormal facial expression
T- Thymus failure
C- Cleft palate
H- Hypoparathyroidism
142
Q

Ring Sideroblasts are a feature of which condition?

A

Myelodysplastic syndromes

143
Q

What is the chance of contracting HIV from a needle stick injury?

A

0.3%

144
Q

What is the chance of contracting HepC from a needle stick injury?

A

3%

145
Q

What condition presents with:

  1. Neurological symptoms (headache, confusion)
  2. AKI
  3. Fever
  4. Low platelets
  5. Microangiopathic haemolytic anaemia
A

Thrombotic thrombocytopaenic purpura

146
Q

Smear cells on blood film are characteristic of which haematological cancer?

A

CLL

147
Q

CD19, CD10 and CD5 are markers of which type of cell?

A

Lymphocytes

148
Q

Pale proliferation centres and an increase in lymphocytes is associated with which haematological cancer?

A

CLL

149
Q

Lenalidomide, Dexamethosone and Velcade are used for which haematological disorder?

A

Myeloma

150
Q

ADAMTS13 is attacked, preventing its maintenance breakage of VWF in which condition?

A

TTP

Year 3 (22 decks)

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