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Haematology and Vascular Flashcards

1
Q

What is Buerger’s Disease?

A

AKA Thromboangiitis Obliterans

A small + medium vessel vasculitis STRONGLY associated with smoking.

Px: Peripheral ischaemia (claudication + ulcers), superficial thrombophlebitis, + Raynaud’s phenomenon)

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2
Q

What is the pentad presentation if TTP (Thrombocytic Thrombocytopenic Purpura)

A

1) Fever
2) Neurological signs
3) Thrombocytopenia
4) Renal failure
5) Haemolytic anaemia

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3
Q

What is the Px triad for Aortic dissection?

A

Acute pain
Pulse deficit
Widened mediastinum

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4
Q

What is the most common childhood haematological malignancy?

A

Acute Lymphoblastic Leukaemia (ALL)

Children with Downs syndrome have x4 risk

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5
Q

ALL Px?

A 
Lymphadenopathy
Organomegaly
Bone pain
Petechiae 
Unexplained bruising/ bleeding
Fatigue + pallor
Recurrent infections
CNS manifestations
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6
Q

What malignancy has Auer rods seen on blood film?

A

AML

*NOTE: Auer rods are crystal aggregates of MPO

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7
Q

Which haematological malignancy is classically associated with DIC (disseminated Intravascular coagulopathy)?

A

AML (+APML)

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8
Q

How do you treat CML?

A

Tyrosine kinase inhibitor Eg Imatinib

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9
Q

Which leukaemia commonly transforms into AML

A

CLL (chronic lymphocytic leukaemia)

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10
Q

In which leukaemia do you see smear/smudge cells (ruptured B cells) on blood film?

A

CLL

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11
Q

CML Px

A

Insidious onset
Mild anaemic symptoms
Splenomegaly

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12
Q

Multiple Myeloma Px

A 
CRAB-I
C - Calcium high
R - renal injury ( high Cr and urea)
A - anaemia
B - bones (fractures) and bleeding
I - infection
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13
Q

Which haematological malignancy has Bence-Jones proteins in the urine?

A

Multiple Myeloma

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14
Q

What is the pre-malignant condition for multiple myeloma?

A

MGUS (Monoclonal gammopathy of undetermined significance)

When body makes abnormal antibody-like paraprotein (usually IgG or IgA)

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15
Q

In which haematological malignancy do you see a raised Ca and low PTH?

A

Multiple myeloma

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16
Q

What does raindrop/ salt and pepper skill on head XR indicate?

A

Multiple myeloma

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17
Q

In which myeloproliferative disorder do you see tear-drop poikilocytes?

A

Myelofibrosis

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18
Q

What is myelofibrosis?

A

Body makes megakaryocytes that stimulate fibroblasts to secrete too much collagen in bone marrow

Causes extra-medullary haematopoiesis —> hepato-splenomegaly

*haematopoiesis = production of all blood cells + plasma

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19
Q

What is pancytopenia suggestive of?

A

Myelofibrosis

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20
Q

What myeloproliferative disorder presents with high Hb and HCT

A

Polycythaemia Rubra Vera

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21
Q

What is essential thrombocytosis?

A

Megakaryocyte proliferation = overproduction of platelets

*:. Risk of thrombotic events!!

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22
Q

Lymphoma Px

A

Non-tender rubbery lymphadenopathy
Fever
Night sweats - drenched!
Weight loss (>10% of normal body weight over 6 months or less)

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23
Q

What does alcohol-induced pain in lymph nodes indicate?

A

Hodgkin’s lymphoma

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24
Q

What do Reed-Sternberg cells (Owl’s eyes) on blood film indicate?

A

Hodgkin’s lymphoma

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25
What virus is Hodgkin’s lymphoma associated with?
Epstein Barr Virus (EBV)
26
Lymphadenopathy in Hodgkin’s vs Non-Hodgkin’s
Hodgkin’s = asymmetrical + continuous amongst continuous LN groups Eg cervical -> axillary Non-Hodgkin’s = widespread symmetrical LN involvement
27
What haematological emergencies are common in chemotherapy pts?
Neutropenic sepsis + Tumour Lysis syndrome
28
How do you treat neutropenic sepsis?
Take FBC Start broad-spectrum abx IV immediately Can give prophylactic Ciprofloxacin to high risk pts
29
Tumour Lysis Syndrome Px
High serum K, phosphate + uric acid Low Ca Can cause cardiac arrhythmias, AKI, seizures
30
Tumour lysis syndrome prophylaxis?
Pre-hydration | Allopurinol or Rasburicase
31
SVC Obstruction Px
``` Dyspnoea Raised JVP Fullness in head Syncope Visual changes Facial oedema Pemberton’s sign (bilateral arms up causes red face) ```
32
SVC obstruction causes
Usually mediastinal lymphoma or lung tumour
33
SVC Obstruction treatment
High dose oral dexamethasone +/- stenting and radiotherapy
34
Causes of microcytic anaemia
Iron deficiency Thalassaemia minor Anaemia of chronic disease
35
Causes of normocytic anaemia
Haemolysis Blood loss Marrow hypoplasia (underproduction of bone marrow + :. Less RBCs) Leukaemia infiltration (end stage leukaemia) Shear mechanical destruction by metallic heart valves DIC Hypersplenism Sickle cell anaemia
36
Causes of macrocytic anaemia
Megaloblastic: Folate deficiency + B12 deficiency In these, there is DNA impairment Will be >120fL Focus on these Non-Megaloblastic: alcohol, drugs (azathioprine, methotrexate, trimethoprim, phenytoin), pregnancy, haemolysis, liver disease, hypothyroidism, myelodysplasia
37
What iron study results are seen in iron deficiency anaemia?
Low iron Low ferritin High total iron binding capacity (TIBC)
38
What is a common side effect of ferrous fumarate?
Black tarry stools, altered bowel habit and dyspepsia (heartburn)
39
What is haemolytic anaemia?
RBC destruction prior to it’s expected lifespan (normally 90-120d)
40
What are the features of haemolytic anaemia?
Hyperbilirubinaemia +/- jaundice Pigment gallstones Splenomegaly Dark urine (haemoglobinuria)
41
What type of anaemia is haemolytic anaemia?
Normocytic However, MCV may be high due to compensatory increase in reticulocytes
42
In which inherited haemolytic anaemia would Heinz bodies and Bite cells be present?
G6PD deficiency
43
In which inherited haemolytic anaemia are spherocytes found?
Hereditary spherocytosis
44
What is the inheritance pattern of G6PD deficiency?
X-Linked recessive
45
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant
46
What is the inheritance pattern of sickle cell anaemia?
Autosomal recessive
47
What is a common Px of sequestration crisis in sickle cell anaemia?
Hepatosplenomegaly - pooling of blood in spleen + liver Hypovolaemia
48
Px of vaso-occlusive crisis in sickle cell?
``` Painful dactylitis Mesenteric ischaemia CNS infarction Avascular necrosis Priapism (persistent erection) Leg ulcers ``` *NOTE: All due to microvascular occlusion
49
What is the inheritance pattern of thalassemia?
Autosomal recessive
50
Where is thalassemia most commonly found in the world?
Mediterranean and far east
51
What is thalassemia?
Unbalanced Hb synthesis (underproduction or absent alpha/beta globin chain) Abnormal RBC prone to haemolysis
52
What is beta-thalassemia?
A microcytic anaemia (microcytosis disproportionate to level of anaemia) Variable Px depending on phenotype - minor = asymptomatic -intermedia -Major = severe Sx, hepatosplenomegaly, chronic transfusion dependency
53
What is Idiopathic Thrombocytic Purpura (ITP)?
Autoimmune destruction of platelets. In kids, often precipitated by viral infection.
54
What is Von-Willebrand Disease (VWD)?
VWD is the most common inherited (autosomal dominant) bleeding disorder. -Deficiency of Von-Willebrand factor + :. clotting factor 8.
55
Px of Von-Willebrand Disease?
- Prolonged bleeding time +APTT | - Normal platelet count
56
Management of Von-Willebrand?
Desmopressin
57
What is TTP (Thrombotic Thrombocytopenic Purpura)?
Protease deficiency --> Accumulation of hyperadhesive multimers of VWF --> Microthrombi + microangiopathic haemolytic anaemia
58
What is Haemolytic Uraemic Syndrome (HUS)?
Shiga toxin inhibits protease, which causes thrombocytopenia + microangiopathic haemolytic anaemia + AKI
59
Who typically develops haemolytic uraemic syndrome (HUS)?
Children, following E.Coli gastroenteritis
60
What is the inheritance pattern of Haemophilia A + B?
Haemophilia is X-Linked recessive ``` A = Factor 8 deficiency B = Factor 9 deficiency ```
61
Haemophilia Px?
Deep tissue bleeding (joints, muscles etc), large subcut + soft tissue haematomas. Prolonged APTT, normal PT (both A + B)
62
What is DIC (disseminated intravascular coagulopathy)?
DIC = inappropriate activation of the clotting cascade --> microthrombi from +platelets and clotting factors are used up --> organ dysfunction + bleeding tendancy (as no more platelets or clotting factors left)
63
When do we see DIC?
Infection + sepsis, trauma, obstetric complications, malignancy
64
What blood results are expected in DIC?
- Prolonged bleeding time + APTT + PT + thrombin time - Low fibrinogen - High D-dimer - Thrombocytopenia
65
What is the target INR for a pt on warfarin who has PMH of AF, VTE, cardioversion, or bio-prosthetic mitral valve replacement?
2.5
66
What is the target INR for a pt on warfarin with recurrent VTE despite anti-coagulation?
3.5
67
What is the target INR for a pt on warfarin with a metallic valve replacement or AF?
3-3.5
68
Which anti-coagulant drugs are contra-indicated in pregnancy?
Warfarin and DOACs
69
Which drugs interact with warfarin?
Hepatic CYP450 inducing and inhibiting drugs
70
What is the reversal agent for Dabigatran (DOAC)?
Idarucizumab
71
What is the safest DOAC to prescribe in renal impairment?
Apixaban
72
How do you monitor unfractioned heparin use/effect?
APTT
73
How do you monitor low molecular weight heparin effect?
Factor 10a | Monitor in renal impairment and preganacy
74
What is unfractioned heparin's MOA?
Potentiates anti-thrombin 3 which inhibits factors 10 and 2
75
What is the reversal agent for unfractioned heparin?
Protamine sulphate | *Rapid!
76
When is FFP use indicated?
When a pt has MULTIPLE clotting factor deficiencies (abnormal PT + APTT) and is BLEEDING.
77
What is HIT (Heparin induced thrombocytopenia)?
A pro-thrombotic disorder caused by antibodies to complexes of platelet factor 4 + heparin. Causes platelet activation + :. generation of thrombin, monocyte activation and other inflamm cells. This = endothelial injury, which produces the characteristic venous and arterial thromboses of HIT.
78
When is the typical onset of HIT (heparin-induced thrombocytopenia)?
5-14 days after the start of heparin therapy.
79
What is the warfarin reversal strategy in major bleeding?
IV prothrombin complex concentrate (PCC, Beriplex) IV Vit K
80
What is the warfarin reversal strategy in minor bleeding?
IV Vit K
81
How do you reverse the effects of warfarin if there is no bleeding?
INR > 5 = withhold 1-2 doses of warfarin and reduce maintenace dose. INR > 8 = Oral Vit k
82
What is Non-haemolytic Febrile Reaction?
Non-haemolytic febrile reaction is caused by abs reacting to WBC fragments in the blood product + cytokines that have leaked from the blood during storage.
83
Non-haemolytic febrile reaction Px?
Fever + chills. | Occurs in 10-30% of platelet transfusions.
84
What is the management of non-haemolytic febrile reaction?
Slow/stop transfusion. Paracetamol. Monitor.
85
What causes a minor allergic reaction in blood transfusions + what is its Px?
Foreign plasma proteins in the blood product. | Px = Pruritus + urticaria
86
How do you manage a minor allergic reaction to a blood transfusion?
Temporarily stop the transfusion. Paracetamol. Monitor.
87
What causes anaphylaxis in a blood transfusion?
Anaphylaxis can be caused by pts with an IgA deficiency who have anti-IgA abs.
88
Px of anaphylaxis during blood transfusion?
Hypotension, dyspnoea, wheezing, angioedema
89
Rx of anaphylaxis during blood transfusion?
Stop transfusion IM adrenaline 1;1,000 Oxygen Fluid resuscitation
90
What is an acute haemolytic reaction?
ABO-incompatible blood Eg secondary to human error.
91
Px of acute haemolytic reaction?
Fever Abdo pain Hypotension
92
Rx of acute haemolytic reaction?
``` Stop transfusion. Send blood for Coombe's test. Repeat cross-matching + typing. Supportive care. Fluid resuscitation. ```
93
What causes transfusion-associated circulatory overload (TACO)?
Caused by excessive rate of transfusion, pre-existing heart failure or frailty.
94
What is the Px of Transfusion-associated circulatory overload (TACO)?
Pulmonary oedema | Hypertension
95
Rx of Transfusion-associated Circulatory Overload (TACO)?
Slow/stop transfusion. | IV loop diuretics (eg furosemide) and oxygen
96
What is Transfusion-assciated Acute Lung Injury (TRALI)?
Non-cardiogenic pulmonary oedema secondary to increased vascular permeability caused by neutrophils activated by substances in donated blood.
97
What is the Px of transfusion-associated acute lung injury (TRALI)?
Hypoxia Pulmonary infiltrates on CXR Fever Hypotension
98
What is the Rx of transfusion-associated acute lung injury (TRALI)?
Stop transfusion | Oxygen and supportive care.
99
What age groups does Hodgkin's lymphoma effect?
Bimodal - 30s and 70s
100
What age group does non-hodgkin's lymphoma effcet?
People over 50
101
Where are clotting factors made?
Liver
102
In a haemorrhage, what do you give if the pt is v low in fibrinogen?
Cryoprecipitate *Contains conc fibrinogen and factor 8
103
What do we give to inhibit fibrinolysis?
Tranexamic acid It stabilises any clot that is formed
104
What is prothrombin complex concentrate?
Concentrated factors 2,7,9,10 *NOTE: These are the clotting factors that fall when on Warfarin So better to use this if on warfarin
105
What is the platelet transfusion trigger level in acute bleeding?
75 x 10^9/L This is to ensure the platelet count does not fall below 50 x 10^9/L, which is the critical threshold of haemostatic failure
106
How do you manage ALL that has infiltrated the CNS?
Intrathecal chemotherapy *NOTE: Give prophylactic chemo anyway, as doing the lumbar puncture increases the risk of spread to CNS anyway, so just give the chemo while the needle is in there
107
In AML, what do the myeloblasts stain positive for?
Myeloperoxidase (MPO)
108
Which haematological malignancy is associated with Down’s syndrome?
AML
109
What is APML?
Acute promyelocytic anaemia Affects slightly younger pts than AML Also has Auer rods 90% curable
110
What is the treatment for APML?
ATRA and arsenic
111
What is a Richter transformation?
When CLL transforms into a diffuse large cell B lymphoma
112
Which haematological malignancy is characterised by the Philadelphia chromosome?
CML
113
Which haematological malignancy has a leukoerythroblastic blood film?
CML NOTE: differentials for leukoerythroblastic blood film include GCSF stimulation or acute sepsis
114
Which lymphoma is autoimmune?
Non-Hodgkins
115
What is the Pel Ebstein fever?
Cyclical fever over a couple of weeks Only in Hodgkin’s lymphoma
116
What is Burkitt’s lymphoma?
A non-hodgkins characterised by a starry sky appearance High grade tumour Can double in days - very fast replication V responsive to treatment
117
What is multiple myeloma?
A lymphoid disorder | Malignant explansion of memory B cells in bone marrow so it produces paraproteins and makes the blood hyper-viscous
118
What is sideroblastic anaemia?
Increased iron, ferritin, saturation. Low TIBC. Defective synthesis of the precursor of haem, so cant make RBCs properly and are not using up your iron. The iron gets stuck in the mitochondria and form a ring around the nucleus Can be caused by alcohol
119
Why do you fix the B12 deficiency before you check the folate deficiency?
Risk of precipitation of Subacute degeneration of the spinal cord
120
What is Polycythemia?
A high concentrationof haemoglobin in the blood. Polycythaemia gives patients a plethoric complexion. A high concentration of red blood cells and haemoglobin make the blood more viscous, making patients more prone to developing blood clots.
121
What would the ferritin, TIBC, Iron, and transferrin sats look like in a pt with Haemochromatosis?
Ferritin - high TIBC - low Iron - high Transferrin saturation - high
122
What is the Rx for Haemochromatosis?
Venesection is 1st line Desferrioxamine is 2nd line
123
What do Joint XRs show in Haemochromatosis?
Chondrocalcinosis
124
What are the 3 ANCA-associated conditions?
1. Granulomatosis with polyangiitis (Wegener's granulomatosis) 2. Microscopic Polyangiitis 3. Eosinophilic Granulomatosis with polyangiitis (Churg-Strauss syndrome)
125
What are the features of Eosinophilic Granulomatosis with polyangiitis (Churg-Strauss syndrome)?
- Asthma - Blood eosinophilia - Paranasal sinusitis - Mononeuritis multiplex - pANCA +ve
126
What is Burkitt's lymphoma?
A high-grade B-cell neoplasm. 2 major forms: -endemic (involves maxilla or mandible) - sporadic (abdominal tumours common in HIV pts)

Medicine (16 decks)

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