Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Y3- TO LEARN > Haematology > Flashcards

Haematology Flashcards

1
Q

Standard dose of iron supplementation?

A

Ferrous Fumarate 210mg tds for 3 months

Take with food (reduce SE)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Standard dose for folate deficiency?

A

Folic acid 5mg od for 4 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Standard dose for B12 deficiency?

A

Loading doses- 3 injections weekly for 2 weeks, them 3 monthly (usually for life)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is microangiopathic haemolytic anaemia associated with?

A

Prosthetic heart valves (malfunctioning)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Smoking can cause an increase in?

A

Hb, RBC, WCC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What does chronic marrow hyperplasia cause?

A

Short stature

Increase energy demand in marrow= decreased energy for growth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the test for autoimmune haemolytic anaemia?

A

Direct Coomb’s test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is warm autoimmune haemolytic anaemia?

A

IgG mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is cold autoimmune haemolytic anaemia?

A

IgM mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Lifespan of a neutrophil?

A

7-8 hours in blood

Few days in tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are B symptoms?

A

Weight loss, fever, night sweats

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What does high PV indicate?

A

Increased protein in blood

  • immunoglobulins (very high increase- usually malignant),
  • acute phase reaction proteins (mild increase) etc.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How can high PV be further investigated?

A

Electrophoresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is rouleaux?

A

Stacks or aggregations of RBC

Occurs when plasma protein concentration is high

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the target INR?

A

2.5 (2-3)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is a leucoerythroblastic blood film?

A

Immature RBC and immature white cells in the blood stream

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What can interact with warfarin and increase efficacy? (high INR)

A

Antibiotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

In haemolysis, what happens to haptoglobin and LDH levels in blood?

A

Haptoglobin decreases- binder for free Hb in RBC

LDH increases - releases from RBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is MAHA?

A

Increased activation of coagulation forms fibrin mesh- RBC are physically damaged by these networks.
Small vessel damage to organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What 3 things does TTP (Thrombotic Thrombocytopenic Purpura) cause?

A

Low platelets
Low RBC
Kidney Failure

(similar to HUS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are CRAB criteria for myeloma?

A

Calcium elevation
Renal failure
Anaemia
Bone disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is Rituximab?

A

Monoclonal antibody than binds CD20 on lymphoma/leukaemia cells and induces lysis and apoptosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is Imatinib?

A

Tyrosine Kinase Inhibition

For cancers with Philadelphia chromosome (CML, ALL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What can gram negative bacteria cause in neutropenic patients?

A

Fulminant life-threatening sepsis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What should everyone with a diagnosis of Non-Hodgkin’s Lymphoma be tested for?

A

HIV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Important cause of generalised lymphadenopathy +/- lymphopenia/thrombocytopenia?

A

HIV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What is used to monitor intrinsic pathway?

A

APTT

play table tennis INside

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is used to monitor extrinsic pathway?

A

PT

PT= less extra letters

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Which pathway does heparin act on?

A

Intrinsic pathway

INtrinsic= heparIN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Which pathways does warfarin act on?

A

eXtrinsic

X-1= Warfarin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Red cell fragmentation + polychromasia?

A

MAHA- microangiopathic haemolytic anaemia

- can be due to HUS- ecoli (if + renal impairment)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What is the best test to use for sickle cell disease?

A

HPLC- haemoglobin high performance liquid chromatography (of whole blood lysate)
- confirm presence of HbS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Macroovalocytes and hypersegmented neutrophils?

A

B12/folate deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Excess of blasts with Auer rods?

A

Acute Myeloid Leukaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Where does erythropoiesis take place in embryo?

A

Spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Where does erythropoiesis take place at birth?

A

Bone marrow + liver/spleen if needed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Where does erythropoiesis take place in adults

A 
Bone marrow of:
Skull
Ribs
Sternum
Pelvis
Proximal head of femur etc.
(more in children, progressive loss of sites in ageing)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What feature do stem cells have that allow them to remain in good numbers?

A

Self-renewal

When divide, one stays as stem cell and one differentiates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What happens to erythrocytes as they differentiate? (3)

A

Increased Hb
Decreased nuclear size + RNA
Decreased cell size

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Describe platelet formation?

A

In megakaryocytes

Form in cytoplasm at periphery and bud off

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What can raised neutrophils?

A

Infection
Trauma
Infarction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What are monocytes?

A

Precursors to macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

How to test for specific precursor cells and how does it work?

A

Immunophenotyping

Studies antigen expression on lymphoid cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Where is bone marrow commonly aspirated from in adults?

A

Posterior iliac crests

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

RBC energy source?

A

Glycolysis (no mitochondria)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What is haemoglobin made up of?

A

1 haem group (porphyrin ring + Fe2+)
2 alpha, 2 beta globin chains
4O2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

How are cells destroyed in the spleen?

A

Actively phagocytosed by macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What do globin chains become after destruction?

A

Amino acids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What do haem groups become after destruction?

A

Bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

How do RBC prevent Fe2+ becoming Fe3+?

A

Reduced by NADH from glycolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

How do RBC prevent oxidative damage from free radicals?

A

Glutathione formed from NADPH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What is the rate limiting enzyme involved in the conversion of NADPH to glutathione?

A

G6PD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

How is CO2 transported in blood?

A

Mostly bicarbonate (also bound to Hb or dissolved)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

What is cooperative binding?

A

O2 binding changes Hb shape to make more binding easier

SIGMOID CURVE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

How do small molecules binding to Hb affect O2 affinity and tissue O2 delivery?

A

e.g. H+, CO2, 2,3-DPG

O2 affinity decreased
More released to tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Redistributive causes of low blood count?

A

Portal hypertension- splenomegaly- blood trapped in spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Redistributive causes of high blood count?

A

Steroids redistribute neutrophils to centre of vessel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

What is haematocrit a measure of?

A

Volume of RBCs as % of total blood volume

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Do reticulocytes have a nucleus/RNA?

A

No nucleus

Still some RNA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Cause of microcytic RBCs?

A

Problem with haemoglobin production (cytoplasmic maturation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Cause of macrocytic RBCs?

A

Problems with nuclear maturation (cell division)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

In iron deficiency, what happens to transferrin levels?

A

Increase (apotransferrin- unbound)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Causes of iron deficiency?

A

Dietary
Blood loss
Pregnancy
Malabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

What increase in iron should be seen with iron replacement therapy?

A

7-10g/week

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

What is compensated haemolysis?

A

Increased RBC production so not anaemic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Ways of measuring haemolysis? (2)

A 
Reticulocytes
Breakdown products (increased)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What is extravascular haemolysis?

A

In spleen+ liver- organomegaly
Release porphyrin–>
JAUNDICE + GALLSTONES
(normal products in excess)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What is intravascular haemolysis and causes?

A

Destroyed within circulation:

  • ABO incompatibility
  • G6PD deficiency
  • Falciparum malaria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

What is released during intravascular haemolysis? (4)

A

Abnormal products:

  • Free Hb in serum
  • Hb + albumin complex
  • Pink urine- free Hb (black if oxidised)
  • Haemosideruria (iron protein in urine)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Warm autoimmune haemolysis?

A

IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Cold autoimmune haemolysis?

A

IgM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Diagnosis of autoimmune haemolysis?

A

Direct Coomb’s test- identify antibody bound to own RBCs- agglutination= +ve test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Other causes of autoimmune haemolysis? (2)

A

Haemolytic transfusion reaction

Haemolytic disease of newborn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Mechanical causes of haemolysis?

A 
DIC
HUS
TTP
Leaking heart valve- damage membrane
Infections e.g. malaria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Name 1 acquired cause of abnormal RBC membrane leading to haemolysis?

A

Vitamin E deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Pathology of hereditary spherocytosis?

A

Mutations in membrane proteins that maintain flexibility/integrity- form spherical shape and spleen destroys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Features of G6PD deficiency?

A

X linked- men only

Can’t generate ATP in RBCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

In haemolysis, what happens to serum haptoglobins?

A

Decreased (bind the excess free Hb released)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

What should all patients with a form of haemolytic anaemia be given and why?

A

Folic acid

Stimulate haematopoesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Causes of microcytic anaemia? (7)

A 
Iron deficiency
Thalassemia
Sideroblastic anaemia
Anaemia of chronic disease
Lead poisoning
Alcohol/drugs
Haemolysis (hereditary or acquired)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Causes of macrocytic anaemia? (5)

A 
Pernicious anaemia
Folate def.
Alcohol/drugs
Liver disease
Hypothyroidism
Marrow failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

What is HbA?

A

Normal Hb- 2 alpha 2 beta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

What is HbA2?

A

2 alpha + 2 delta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

What is HbF?

A

Foetal Hb- 2 alpha + 2 gamma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

How is expression of globin changes regulated throughout life?

A

Genes arranged in order of expression- get turned on/off in right-left pattern

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

What are thalassemias?

A

Decreased globin synthesis (therefore low haemoglobin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

2 types of thalassemias?

A

Alpha thalassemia= alpha chains affected

Beta thalassemia= beta chains affected

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

Unbalanced accumulation of globin chains in thalassemias lead to..?

A

Toxic- leads to ineffective erythropoesis + haemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

Where are alpha genes found and how many are there?

A

Chromosome 16

4 genes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

Where are beta genes found and how many are there?

A

Chromosome 11

2 genes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

Alpha thalassemia affects which types of haemoglobin?

A

All types (all have alpha chains)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

Symptoms of alpha thalassemia trait?

A

Asymptomatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

What is HbH disease?

A

One alpha gene left

Anaemia, Splenomegaly, Jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

What is Hb Barts Hydrops Fetalis?

A

No alpha genes- no HbA
Severe anaemia, cardiac failure, reduced growth, severe hepatosplenomegaly, skeletal abnormalities
most DIE IN UTERO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

What is beta thalassemia caused by?

A

Point mutation

Only affects HbA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

Symptoms of beta thalassemia major?

A

Pallor, failure to thrive, hepatosplenomegaly, skeletal changes- forehead/skull enlarged

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

Management of beta thalassemia major?

A

Regular transfusions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

How do sickling disorders occur?

A

Point mutation in beta gene- makes HbS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

How do sickling disorders cause anaemia?

A

HbS polymerises in low O2- damage RBC membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

When might people with sickle trait suffer?

A

In severe hypoxia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

Features of HbSS (sickle cell anaemia)?

A

2 abnormal genes- AR

Haemolysis + hyposplenism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

What is sickle crisis?

A

Causes tissue infarction de to vascular occlusion

Pain- CNS, lung, marrow, hands, feet etc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

Precipitants of sickle crisis?

A 
Hypoxia
Dehydration
Infection
Cold
Stress
Fatigue
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

Management of sickle cell anaemia?

A

Prophylactic penicillin
Folic acid
Regular transfusion
Hydroxycarbamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

Diagnosis of sickle cell anaemia?

A

Blood film

Gel ELECTROPHORESIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

What does megaloblastic mean?

A

Abnormally large nucleated red cells with immature nuclei

failure to become smaller

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

Cause of megaloblastic anaemia?

A

Defects in DNA synthesis/nuclear maturation + more prone to apoptosis:
B12/folate deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

Sources of folate?

A

Veg, cereal, kidney beans, yeast

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
109
Q

B12 absorption? (5)

A
  1. R-binder protein in mouth + stomach
  2. Intrinsic factor from parietal cells
  3. Pancreatic proteases in duodenum increase pH
  4. Breaks down R-binder, B12 binds to intrinsic factor
  5. Through to ileum- endocytosed
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
110
Q

Body reserves of B12?

A

2-4 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
111
Q

Causes of B12 deficiency?

A 
Veganism
Gastritis
Bypass surgery
Chronic pancreatitis
Coeliac/Crohn's
PERNICIOUS ANAEMIA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
112
Q

What is pernicious anaemia?

A

Autoimmune destruction of parietal cells- intrinsic factor deficiency- low B12 absorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
113
Q

What is pernicious anaemia associated with?

A

Other autoimmune- hypothyroid, vitiligo, Addison’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
114
Q

Where is folate absorbed?

A

Jejunum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
115
Q

Causes of folate deficiency?

A 
Dietary
Haemolysis
Malignancy
Coeliac/Crohn's
Pregnancy
Anti-convulsants
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
116
Q

Body reserves of folate?

A

4 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
117
Q

Clinical features of folate deficiency?

A 
Anaemia
Weight loss
Diarrhoea
Jaundice
Developmental problems
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
118
Q

Clinical features of B12 deficiency?

A

Sore (beefy) tongue

NEUROLOGICAL- neuropathy, dementia, column abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
119
Q

Autoantibodies in pernicious anaemia?

A

IF- anti-intrinsic factor

GPC- anti-gastric parietal cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
120
Q

What is non-megaloblastic anaemia?

A

Due to red cell membrane changes (mature but large cells)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
121
Q

Causes of non-megaloblastic anaemia?

A

Alcohol
Liver disease
Hypothyroidism
Marrow failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
122
Q

When might you get a false macrocytosis (false high MCV)? (2)

A
  1. Reticulocytosis- analysed as RBC but larger so give false large MCV
  2. Cold agglutinations- in lymphoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
123
Q

What can cause normocytic normochromatic anaemia? (3)

A
  1. Acute blood loss
  2. Early iron def.
  3. Hypoproliferative- chronic disease, renal failure, hypothyroid, marrow failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
124
Q

What is renal anaemia?

A

Lack of erythropoietin in renal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
125
Q

How does anaemia of chronic disease occur?

A

Inflammation causes upregulation of HEPCIDIN

  • inhibits Fe absorption + release
  • low erythropoiesis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
126
Q

Where does iron get absorbed?

A

Duodenum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
127
Q

What enhances iron absorption? (3)

A
  1. Haem iron (red meat)- easy to digest
  2. Ascorbic acid (vit C)
  3. Alcohol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
128
Q

What inhibits iron absorption? (3)

A
  1. Tannins (tea)
  2. Phytates (cereals, nuts, seeds)
  3. Calcium
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
129
Q

In the duodenum, what converts Fe3 to Fe2?

A

Duodenal cytochrome B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
130
Q

What transports Fe2 into duodenal enterocyte?

A

DMT 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
131
Q

How is iron exported from the duodenal enterocyte to bind to transferrin in the blood?

A

Ferroportin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
132
Q

What does hepcidin do?

A

Inhibit iron uptake- trap iron in duodenal cells

due to inflammation, iron overload etc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
133
Q

How much iron is needed per day?

A

25mg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
134
Q

How much iron is in plasma?

A

4mg (rest in macrophages/hepatocytes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
135
Q

Skin and nail signs of iron deficiency?

A

Koilonychia

Angular stomatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
136
Q

In iron overload, where is iron kept?

A

In parenchymal tissue e.g. liver, heart etc.

Oxidative stress causes organ damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
137
Q

Mutation in hereditary heaemochromatosis?

A

HFE gene

Low hepcidin- high iron absorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
138
Q

Bloods in hereditary heaemochromatosis?

A

Iron >5g
Transferrin sats >50%
Ferritin >300 micrograms/L

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
139
Q

Presentation of hereditary heaemochromatosis?

A

In 40s-50s

Fatigue, joint pain, arthritis, cirrhosis, diabetes, cardiomyopathy, impotence etc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
140
Q

Treatment of hereditary heaemochromatosis?

A 
Weekly venesection (450-500ml)
Keep ferritin <50micrograms/L
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
141
Q

Secondary cause of iron overload?

A

Repeated transfusions

Thalassemias etc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
142
Q

What does FFP contain?

A

Clotting factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
143
Q

What does cryoprecipitate contain?

A

Fibrinogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
144
Q

The genes responsible for ABO are on which chromosome?

A

9

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
145
Q

Which plasma antibodies do blood group A- have?

A

anti-B antibody

anti-D antibody

146
Q

Which plasma antibodies do blood group O have?

A

anti-A and anti-B antibodies

147
Q

O blood can be given to?

148
Q

A blood can be given to?

149
Q

B blood can be given to?

150
Q

AB blood can be given to?

151
Q

Group AB can receive blood from?

152
Q

Which reagent is used to identify RBC antigens?

153
Q

Which reagent is used to identify plasma antibodies?

A

Reagent red cells

154
Q

In tests to confirm a persons blood type, what is a positive test?

A

When agglutination occurs

155
Q

In tests to confirm a COMPATIBILITY of blood transfusion (crossmatching), what is a good outcome?

A

NO agglutination means blood in compatible

156
Q

Indications for RBC transfusion? (2)

A

Symptomatic anaemia

Major haemorrhage

157
Q

Indications for platelets transfusion? (3)

A

Prophylaxis for surgery in thrombocytopenia
Bleeding in thrombocytopenia
Prophylaxis in bone marrow failure

158
Q

Indications for FFP transfusion? (3)

A

Bleeding in coagulopathy
Prophylaxis for surgery in coagulopathy
Massive haemorrhage

159
Q

Symptoms of acute reaction in transfusion?

A 
Chills
Rigors
Rash/itch
Flushing
Fever
Increased HR
Decreased BP
Collapse
PAIN
Sense of impending doom
160
Q

Cause of acute haemolytic reaction in transfusion and type of hypersensitivity reaction?

A

ABO incompatibility- type 2 hypersensitivity
IgM + free Hb released
Cause renal failure!

161
Q

Other cause of severe reactions in transfusion?

A

Bacterial contamination

162
Q

What is TACO and symptoms?

A

Transfusion Associated Circulatory Overload

resp. distress, high BP, high JVP, +ve fluid balance

163
Q

Risk factors for TACO?

A 
Elderly
HR failure
Low albumin
Renal impairment
Fluid overload
164
Q

Management of TACO?

A

O2
Furosemide
Slow transfusion rate

165
Q

Steps in massive haemorrhage protocol? (4)

A
  1. Urgent blood samples
  2. ABCDE
  3. Transfuse blood component
  4. Thromboprophylaxis once controlled
166
Q

What is shock?

A

Tissue hypoperfusion due to circulatory failure

167
Q

What can shock lead to?

A

Anaerobic metabolism- metabolic acidosis + lactic acid

Eventually cellular NECROSIS

168
Q

Recognition of shock? (5)

A
  1. BP, HR, RR etc.
  2. Mottling
  3. GCS
  4. Urine output <0.5ml/kg/hr
  5. Lactate levels
169
Q

Which types of shock have cold, clammy peripheries?

A

Cardiogenic
Obstructive
Hypovolaemic

170
Q

Which types of shock have warm, red peripheries?

A

Distributive (due to vasodilation)

171
Q

Lifespan of platelets?

172
Q

What is primary haemostasis?

A

Formation of platelet plug

173
Q

Steps of primary haemostasis? (3)

A
  1. Endothelial damage= exposed collagen
  2. Release von Willebrand Factor (vWF) which attracts platelets- ADHESION
  3. Platelets secretes chemical causing AGGREGATION
174
Q

Typical bleeding in primary haemostasis failure?

A 
Spontaneous bruising
Purpura (lower limbs)
Mucosal bleeding- GI, nose, eye, menorrhagia
Intracranial haemorrhage
Retinal haemorrhage
175
Q

What is secondary haemostasis?

A

Formation of fibrin clot- stabilise

176
Q

Steps of secondary haemostasis? (6)

A
  1. Platelet plug secretes calcium to make -ve surface positive
  2. Attracts -ve clotting factors- activate each other
  3. Endothelium releases tissue factor- activates factor VII
  4. Activates factors V + Xa
  5. Activates prothrombin (factor IIa) to thrombin (activates factors VIII + IXa to AMPLIFY)
  6. Thrombin causes fibrinogen- FIBRIN
177
Q

Typical bleeding in secondary haemostasis failure?

A

Into joints (ankles, knees etc.) and muscles

178
Q

Prolonged PT=

A

factor VII deficiency

179
Q

Prolonged APTT=

A

Haemophilia A/B

von Willebrand deficiency

180
Q

Vascular causes of primary haemostasis failure? (4)

A

Age (low collagen)
Connective tissue disorders
Scurvy (vit C needed for collagen)
Vasculitis

181
Q

Platelet function causes of primary haemostasis failure? (2)

A 
Drugs- aspirin/anti-platelets, NSAIDs
Renal failure (uraemia interrupts function)
182
Q

Platelet number causes of primary haemostasis failure + examples? (3)

A

Low production- Marrow failure
High destruction- DIC, hypersplenism, immune thrombocytopenic purpura (ITP)
Consumption- bleeding

183
Q

What is von Willebrand’s disease and what does it affect?

A

AD inherited deficiency of vWF

Affects platelet adhesion (primary haemostasis)

184
Q

Why do men often have undiagnosed von Willebrand’s disease?

A

Asymptomatic (women will have menorrhagia)

185
Q

Which other clotting factor is affected in von Willebrand’s disease?

A

Factor VIII low

vWF normally carries and protects it

186
Q

Causes of multiple clotting factor deficiency? (4)

A

Liver failure (no synthesis)
Vitamin K deficiency (no carboxylation of factors)
Warfarin
DIC

187
Q

What is DIC?

A

Excessive and inappropriate activation of haemostasis

Causes microvascular thrombus in organs- end organ failure + clotting factor consumption (bleeding)

188
Q

Why is vitamin K important?

A

Carboxylation of glutamic acid to make factors II, V, IX, X + protein C + S

189
Q

Causes of vitamin K deficiency?

A

Poor diet
Malabsorption
Obstructive jaundice
Haemorrhagic disease of newborn

190
Q

When can DIC occur?

A 
Trauma
Sepsis
Burns
Meningitis
Cancer
Hyovolaemic shock
191
Q

What happens to PT and APTT in multiple clotting factor deficiency?

A

Both prolonged

192
Q

What else is raised in multiple clotting factor deficiency?

A

D-dimers (fibrin degradation products)

high in DIC) (low in liver disease

193
Q

What is haemophilia A?

A

X linked disorder causing prolonged bleeding due to factor VIII deficiency
(x5 as common as B)

194
Q

What is haemophilia B?

A

X linked disorder causing prolonged bleeding due to factor IX deficiency

195
Q

Why does haemophilia cause prolonged bleeding?

A

Can’t amplify clotting system

196
Q

Symptoms of haemophilia?

A

Recurrent haemarthroses
Soft tissue bleeds
++ bleeding in surgery and dental extractions

197
Q

How do recurrent haemarthroses occur?

A

One bleed increases the risk of another due to neovasculisation af the synivium

198
Q

In haemophilia, which blood test will be abnormal?

A

APTT will be prolonged

199
Q

In venous system, what kind of clots are formed?

A

FIBRIN rich clots

NO platelet activation

200
Q

Virchows triad applies to Venous system. What are the components?

A

vessel Wall (valves cause sluggish flow)
Hypercoaguability
Stasis

(WH Smith)

201
Q

Signs of PE? (4)

A

SOB
Pleuritic chest pain
Rub
Hypoxia

202
Q

Main differential of DVT?

A

Cellulitis

203
Q

Risk factors for DVT/PE? (13)

A 
Age
Marked obesity
Pregnancy (high clotting factors)
Puerperium
Smoking 
Oestrogen therapy
Previous DVT/PE
Surgery/Trauma
Malignancy
Paralysis 
Infection
IV drug use/PIC lines
Thrombophilia
204
Q

Diagnosis of DVT? (4)

A

Peripheral pulses
Doppler USS
D dimer (raised in other things- -ve in low risk= exclude DVT)
Scoring systems

205
Q

Treatment of DVT?

A 
TED stockings
Early mobilisation
Physiotherapy
Heparin
Rivaroxaban
Warfarin
206
Q

What is thrombophilia?

A

Deficiency in naturally occuring anticoagulants causing increased TENDENCY TO THROMBOSE (high coagulation activity)

207
Q

What are the naturally occuring anticoagulants?

A

Antithrombin- acts on thrombin and Xa, IX, XI, XII

Protein C+S- acts on V and VIII

208
Q

Causes of hereditary thrombophilia? (3)

A

Factor V Leiden
Anti-thrombin deficiency
Protein C/S deficiency

209
Q

What is Factor V Leiden?

A

Mutation of factor V stopping protein C binding and having its inhibitory effect–> overdrive leads to hypercoaguability

210
Q

When should hereditary thrombophilia be considered? (4)

A

VTE <45 (esp men)
Recurrent VTE
Unusual VTE e.g. arm
FH of VTE or thrombophilia

211
Q

Name a cause of acquired thrombophilia?

A

Anti-phospholipid syndrome (higher risk than inherited causes)

212
Q

4 features of anti-phospholipid syndrome?

A

Activation of primary + secondary coagulation
Recurrent thrombosis
Recurrent pregnancy loss
Mild thrombocytopenia (ITP)

213
Q

What is anti-phospholipid syndrome associated with? (4)

A

Autoimmune disorders
Lymphoproliferative disorders
Viral infections
Drugs

214
Q

Treatment of thrombosis in anti-phospholipid syndrome?

A

Aspirin + warfarin

215
Q

In general, what do anti-coagulants do?

A

Stop FURTHER clotting from occuring/breaking off

Target fibrin clot formation

216
Q

Indications for anti-coagulants?

A

Venous thrombosis

AF

217
Q

How does heparin work?

A

Increases action of anti-thrombin to prevent amplification of coagulation (with immediate effect)

218
Q

2 forms of heparin?

A

LMWH- maintain antithrombin + Xa complex to keep switched on

Unfractionated- needs lots of monitoring

219
Q

How is heparin monitored?

A

APTT (unfractionated)
or
Anti Xa assay (LMWH)

220
Q

Complications of heparin? (3)

A

Bleeding
Heparin induced thrombocytopenia
Osteoporosis after long-term

221
Q

How is heparin reversed?

A

Stop- out of system in 12-24 hrs

Protamine sulphate

222
Q

How does warfarin work?

A

Inhibition of vitamin K

223
Q

Steps in starting warfarin therapy? (4)

A
  1. Initiation (Slow for AF, liver failure, elderly. Fast for acute thrombosis)
  2. Stabilisation
  3. Maintenance (same time each day)
  4. Monitoring- PT to calculate INR
224
Q

What is a normal INR and the target for warfarin therapt?

A

0.9-1.2

Target= 2-3

225
Q

Major adverse effects of warfarin therapy?

A

Haemorrhage:
mild- brusing, epitaxis, haematuria
severe- GI, intracerebral, low Hb

226
Q

Name some drugs that increase warfarin effect?

A 
Antibiotics
Alcohol
Amiodarone
NSAIDS (GI bleeds)
\+ pomegranate
227
Q

In patients with metallic heart valves, which is the only anticoagulant that can be used?

228
Q

How long should warfarin be stopped for before a surgery?

229
Q

If INR is too high or there is major bleeding, how can the effect of warfarin be reversed? (3)

A 
Omit dose(s)
Oral vitamin K
Clotting factors (factor concentrates)
230
Q

How long does oral vitamin K take to work?

231
Q

What are DOACs?

A

Direct oral anticoagulants

232
Q

DOACs: How do rivaroxaban, apixaban etc. work?

A

Activated factor X inhibitors (Xa-ban)

233
Q

DOACs: How does dabigatran work and when can’t it be used?

A

Thrombin inhibitor

Avoid in renal failure

234
Q

What is fragmin?

A

Dalteparin- another anticoagulant

235
Q

In arterial system, what kind of clots are formed?

A

Platelet rich clots

236
Q

Cause of arterial thrombosis?

A

Usually atherosclerosis, recruit foamy macrophages

237
Q

Stable plaques are calcified. What can happen to unstable plaques?

A

Rupture + recruit platelets –> acute thrombosis

238
Q

Prevention of arterial thrombosis + examples?

A

Anti-platelets e.g. aspirin, clopidogrel, dipyridamole, abciximab

239
Q

How does aspirin work?

A

Inhibits cyclo-oxygenase so can’t produce thromboxane A2

240
Q

How does clopidogrel work?

A

ADP receptor antagonist (reduce platelet aggregation)

241
Q

How can the effects of anti-platelets be reversed in the case of severe bleeding?

A

Platelet infusion

242
Q

What are myelocytes?

A

Precursor of neutrophils

243
Q

Which cell layer are stem cells derived from?

244
Q

Where is bone marrow commonly aspirated from in children?

245
Q

How do cells exit the bone marrow?

A

Through fenestrations in the membranes- actively migrate down gradient into sinusoid

246
Q

What is red marrow?

A

Haemopoietically active

247
Q

What is yellow marrow?

A

Fatty, less cellular, inactive

More with age

248
Q

What is the myeloid:erythroid ratio and what is the normal range?

A

neutrophil precursors:nucleated RC precursors

1.5:1 - 3.3:1

249
Q

When might the myeloid:erythroid ratio reverse?

A

When increased erythroid production e.g. haemolysis, blood etc.

250
Q

What is a stem cell niche?

A

The microenvironment in which stem cells are found, which interacts with stem cells to regulate stem cell fate (expansion, differentiation, dormancy)

251
Q

Secondary lymphoid tissue?

A

Lymph nodes
Spleen
(tonsils)

252
Q

In lymph nodes, where are B cells found?

A

Follicles (also where antigens are presented)

253
Q

In lymph nodes, where are T cells found?

A

Trabeculae

254
Q

Causes of lymphadenopathy? (6)

A 
Infection (local- e.g. TB or generalised- e.g. virus)
Vaccination
Autoimmune
Connective tissue disorder
Malignancy
Sarcoidosis
255
Q

Which artery supplies the spleen?

A

Splenic artery from the coeliac axis

256
Q

Which vein drains the spleen?

A

Splenic vein to the portal vein

257
Q

The spleen is made up of red and white pulp. What is the function of each?

A

Red- sinusoids filters blood with marcophages

White- antigen presenting cells

258
Q

3 components of hypersplenism?

A

Splenomegaly
Reduced cellular component in blood
Correction of bloods with splenectomy

259
Q

Causes of hypersplenism? (6)

A
  1. Infection- EBV, malaria, TB
  2. Congestion- portal hypertension
  3. Haematological- ITP, haemolytic anaemia, leukaemia, lymphoma
  4. Inflammatory- SLE, RA
  5. Storage diseases
  6. Others- amyloid, tumour, cyst
260
Q

Causes of hyposplenism? (4)

A

Splenectomy
Coeliac disease
Sickle cell disease
Sarcoidosis

261
Q

When might Howell-Jolly bodies be seen and what are they?

A

Nuclear remnant in RBC

Seen in hyposplenism (splenectomy, sickle cell disease etc.)

262
Q

Characteristics of malignant haematopoiesis? (4)

A 
MONOCLONAL
High numbers of abnormal/dysfunctional cells
- increased proliferation
- decreased differentiation
- decreased maturation
- decreased apoptosis
263
Q

Why is malignant haematopoiesis monoclonal?

A

Due to mutated regulatory genes (driver mutations) being selected in evolution- one lineage chosen

264
Q

Feature of acute haematological malignancy?

A

Primitive cells/precursors

265
Q

Feature of chronic haematological malignancy?

A

Differentiated/mature cells

266
Q

What can chronic lymphocytic leukaemia affect?

A

Lymph and blood

267
Q

Features of acute leukaemias?

A

AGGRESSIVE- usually younger people

Large cells + nuclei with MATURATION defects- blasts

268
Q

What is the most common childhood cancer and how does it present?

A

Acute Lymphoblastic Leukaemia (ALL)- malignant lymphoblasts!

Marrow failure, bone pain (rapid marrow expansion)

269
Q

Which type of acute leukaemia is more common in elderly people (>60) and how does it present??

A

Acute Myeloid Leukaemia (AML)
May be de novo or following another haematological disorder
Marrow failure

270
Q

How to differentiate between AML + ALL

A

Immunophenotyping

271
Q

Treatment of AML + ALL?

A

Chemotherapy (most will go into remission but many will relapse again)
Stem cell transplant

272
Q

Which pathology of lymph nodes will not be hard, but soft/rubbery?

273
Q

Which pathology of lymph nodes will not be smooth, but irregular?

A

Metastatic cancers

274
Q

Which pathology of lymph nodes will cause overlying skin inflammation?

A

Bacterial infection

275
Q

Which pathology of lymph nodes will cause the node to be tethered?

A

Metastatic cancers, sometimes bacterial infections

276
Q

Ways to biopsy lymph nodes?

A

Core biopsy or FNA
or
CT guided biopsy of whole node

277
Q

What tests are done to a lymph node biopsy?

A 
Histology
Immunohistochemistry- for pattern of proteins
Immunophenotyping
Cytogenetic analysis
Molecular analysis
278
Q

What is lymphoma?

A

Neoplasm of mature lymphocytes (more commonly B cells) in lymphoid tissue

279
Q

What is Hodgkin’s lymphoma?

A

More common in younger patients
Good prognosis
Had REED-STERNBERG (RS) CELLS

280
Q

Treatment of Hodgkin’s lymphoma?

A

Early- radiotherapy

Late- chemotherapy

281
Q

What is Non-Hodgkin’s lymphoma?

A

No reed-sternberg cells
In elderly
Low or high grade

282
Q

Prognosis of Non-Hodgkin’s lymphoma?

A

Low grade- incurable but indolent (7-10 years)

High grade- 30-40% survival

283
Q

Treatment of Non-Hodgkin’s lymphoma?

A

Low grade- palliative + supportive chemo

High grade- combined chemo

284
Q

Presentation of lymphoma?

A 
PAINLESS lymphadenopathy
Fever, weight loss, drenching night sweats
Itch (no rash)- hot water
Alcohol induced pain
Hepatosplenomegaly
Bone marrow failure
285
Q

How is the variable Ig segment on antibodies generated?

A

VDJ segment of gene (many combinations)

286
Q

Fate of B cells after lymph node?

A

Circulate as memory B cells
or
Return to marrow as plasma cell

287
Q

Immunoglobulins contain?

A

2 heavy + 2 light chains

288
Q

Which immunoglobulin is a pentamer?

289
Q

Which immunoglobulin is a dimer?

290
Q

What is the marker of underlying clonal B cell disorder, and how is it detected?

A

Paraprotein (MONOclonal immunoglobulin in urine/blood)

by ELECTROPHORESIS

291
Q

What is Bence Jones protein?

A

Immunoglobulin light chain in urine if made in excess (leaks into urine)

292
Q

Causes of paraproteinaemia? (5)

A 
MGUS
Myeloma
Amyloidosis
Lymphoma
Waldenstrom's macroglobulinaemia
293
Q

What is myeloma and how is it classified?

A

Monoclonal proliferation of plasma cells (activated B cells)

Classified by type of antibody produced

294
Q

Why does myeloma affect bones and what does it cause?

A

Causes dysregulated OSTEOCLAST activity

  • LYTIC bone lesions
  • bone pain
  • pathological fracture
  • hypercalcaemia
295
Q

Symptoms of myeloma due to paraproteins?

A

Renal failure (CAST NEPHROPATHY- clumping of light chains)
Amyloidosis
Hyperviscosity
Immune suppression

296
Q

Investigations of myeloma?

A 
FBC, PV, U+E
Blood film
Serum Ca
Marrow aspirate
DEXA scan/Xray
Urine- BJP
297
Q

What would a blood film show in myeloma?

298
Q

Treatment of myeloma?

A 
Not cureable
Chemotherapy
Dexamethasone/pred
Stem cell transplant
New- thalidomide, bortezomib, lenalidomide
BISPHOSPHONATES
299
Q

How is response to treatment monitored in myeloma?

A

Paraprotein level

300
Q

What is monoclonal gammopathy of undetermined significance (MGUS)?

A

Paraprotein <30g/L (less than myeloma)
NO evidence of myeloma/organ damage
BENIGN (small chance of myeloma progression)

301
Q

What is AL amyloidosis?

A

(Amyloid light-chain amyloidosis)

Abnormal plasma cell- mutation in light chain- form insoluble beta sheet

302
Q

Which stain is used to confirm amyloidosis?

A

Congo Red Stain (appear apple green)

303
Q

Treatment of amyloidosis?

A

Chemotherapy (switch off light chain supply)

304
Q

What is Waldenstrom’s Macroglobulinaemia and what paraprotein is produced?

A

Clonal disorder of immediate cell between lymphocytes + plasma cells

IgM paraprotein

305
Q

Presentation of Waldenstrom’s Macroglobulinaemia?

A 
Lymphadenopathy
Splenomegaly
Marrow failure
Neuropathy
Night sweats, weight loss
HYPERVISCOSITY- bleeding, fatigue, cardiac failure
306
Q

Treatment of Waldenstrom’s Macroglobulinaemia?

A

Chemo

PLASMAPHERESIS- remove own and replace with donor plasma

307
Q

What is pancytopenia?

A 
Deficiency in all blood cell lineages
Low RBC
Low platelets
Low white cells
(generally excludes lymphocytes)
308
Q

Cause of pancytopenia due to reduced production?

A

Marrow failure

309
Q

Inherited causes of marrow failure?

A

Rare

Fanconi’s anaemia- imparied haemopoiesis, congenial abnormalities, cancer pre-disposition

310
Q

Acquired causes of marrow failure?

A

Primary- idiopathic aplastic anaemia, myelodysplastic syndromes (MDS), acute leukaemia
Secondary- drugs, B12/folate def., infiltrative, viral

311
Q

How does idiopathic aplastic anaemia affect marrow?

A

Reduced red marrow

312
Q

What can myelodysplastic syndromes (MDS) lead to?

313
Q

Cause of pancytopenia due to increased destruction?

A

Hypersplenism- blood trapped + increased phagocytic activity

314
Q

Causes of hypersplenism?

A

Portal hypertension
Rheumatoid arthritis
Splenic lymphoma

315
Q

Presentation of pancytopenia?

A

Anaemia
Infections
Bleeding

316
Q

Investigations of pancytopenia?

A 
FBC + retic count
Blood film
LFT
B12 + folate
Autoantibodies
Aspirate
317
Q

When is marrow hypocellular?

A

Aplastic anaemia

318
Q

When is marrow hypercellular?

A

B12/folate deficiency
Hypersplenism
Myelodysplastic syndromes

319
Q

Treatment of pancytopenia?

A

Red cell + platelet transfusion
Antibiotic prophylaxis
Marrow transplant
Consider splenectomy

320
Q

What are myeloproliferative disorders (MPD)?

A 
Clonal proliferations (increased production) or one/more lineages
MATURATION preserved
321
Q

Name some myeloproliferative disorders (MPD)?

A

Red cells- polycythaemia rubra vera
Platelets- essential thrombocythaemia
White cells- CML
Reactive fibroblasts- myelofibrosis

322
Q

Which myeloproliferative disorders are BCR-ABL1 positive?

323
Q

What bloods would causes suspicion of myeloproliferative disorders?

A 
High granulocytes
High RBC/Hb
High platetetls
Eosinophilia/basophilia
May have splenomegaly
324
Q

What does chronic myeloid leukaemia cause?

A

Proliferation of myeloid (granulocytes + platelets)

325
Q

Presentation of chronic myeloid leukaemia?

A 
Peak 40-50 years
Often asymptomatic
Splenomegaly
B symptoms
Gout
Small vessel problems (e.g. eyes)
326
Q

3 phases of chronic myeloid leukaemia?

A
  1. Chronic phase
  2. Accelerated phase
  3. Blast phase (crisis)- like acute leukaemia
327
Q

Hallmark of CML?

A

PHILADELPHIA CHROMOSOME- produces BCR-ABL 1 gene

which is a tyrosine kinase

328
Q

Investigation of CML and findings?

A

FBC- normal/low Hb, leucocytosis, neutrophilia, myelocytes, thrombocytosis,

329
Q

Treatment of CML?

A

Imatinib- tyrosine kinase inhibitor

330
Q

Common features of BCR-ABL1 negative MPD? (6)

A 
Asymptomatic or
B symptoms
Gout
Splenomegaly
Marrow failure
Thrombosis (A+V)
331
Q

What polycythaemia rubra vera?

A

High Hb/haematocrit + ERYTHROCYTOSIS

may also cause increased WCC/platelets

332
Q

Causes of secondary polycythaemia?

A

Smoking
Chronic hypoxia
EPO-secreting tumours

333
Q

What is pseudopolycythaemia?

A

Apparent increase in RBCs due to reduction in plasma volume

e.g. dehydration, diuretic, obesity

334
Q

Clinical features of polycythaemia rubra vera?

A

55-60 yrs
Headache, fatigue, dizziness, sweating, plethora, ITCH AFTER WARM WATER
Splenomegaly
Thrombosis + bleeding

335
Q

What is polycythaemia rubra vera associated with?

A

JAK2 mutations in 95%

Kinase- loss of auto-inhibition of erythropoiesis (increased)

336
Q

Treatment of polycythaemia rubra vera?

A

Aspirin 75mg/day
Venesection
Hydroxycarbamide (oral chemo)

337
Q

What is essential thrombocythaemia?

A

Uncontrolled production of abnormal platelets

Leading to thrombosis

338
Q

Presentation of essential thrombocythaemia?

A

Peak 50-70yrs
Asymptomatic
Burning in hands/soles, cold peripheries, headache, dizziness, digital ischaemia, gangrene
Epitaxis, menorrhagia

339
Q

What is essential thrombocythaemia sometimes associated with?

A

acquired vWF deficiency due to excess platelets

Leads to bleeding

340
Q

Investigation of essential thrombocythaemia?

A

Exclude reactive causes
Exclude CML
JAK2 mutation (50%)
CALR in those without JAK2

341
Q

Treatment of essential thrombocythaemia?

A

Aspirin

Hydroxycarbamide (oral chemo)

342
Q

What is myelofibrosis?

A

Progressive scarring (fibrosis) in bone marrow

343
Q

2 causes of myelofibrosis?

A

Idiopathic
or
Secondary (transformed) for PRV or ET

344
Q

Presentation of myelofibrosis?

A

> 50s (60-70)
Extramedullary haemopoiesis (liver + spleen
Marrow failure
Fatigue, weight loss, itch

345
Q

Investigations of myelofibrosis + findings? (4)

A

Blood film (leukoerythroblastic film + TEARDROP RBCs)
Dry aspirate on attempt
Trephine biopsy (stain for fibrosis)
JAK2/CALR mutation

346
Q

How do teardrop RBCs occur?

A

Damaged when squeezing through sclerotic bone marrow

347
Q

Treatment of myelofibrosis?

A 
Blood/platelet transfusion
Antibiotics
Stem cell transplant
Consider splenectomy
JAK2 inhibitor- Ruxolitinib
348
Q

Name a JAK2 inhibitor?

A

Ruxolitinib

349
Q

How does cytotoxic chemotherapy work?

A

Damage DNA which promotes apoptosis

350
Q

Name some cell cycle specific (tumour specific) drugs? (3)

A

Methotrexate
Hydroxyurea
Mitotic spindle inhibitors

351
Q

Name some non-cell cycle specific drugs? (3)

A

Alkylating agents
Platinum derivatives
Cytotoxic antibiotics

352
Q

Nucleated red cells in blood of a newborn baby may suggest?

353
Q

What are Heinz bodies and some causes? (4)

A

Formed by damage to Hb (usually oxidative)

  1. G6PD def
  2. NADPH def
  3. Chronic liver disease
  4. Alpha thalassemia
354
Q

Name some anticoagulants and what are they used for?

A

Warfarin, Heparin, DOACs

Venous (red) thrombosis

355
Q

Name some antiplatelets and what are they used for?

A

Aspirin, Clopidogrel

Arterial (white) thrombosis

356
Q

Giving folate in B12 deficiency can lead to what?

A

Demyelination!!

make sure to find out cause of anaemia!

357
Q

How does methotrexate affect heamatopoiesis?

A

Inhibits folate (monitor levels/give folic acid)

358
Q

Smudge cells?

359
Q

t(8;14)?

A

Burkitt’s Lymphoma

360
Q

What are schistocytes seen in?

361
Q

Which clotting factors are used up first in DIC?

A

Consume factors V + VIII (and platelets) first

362
Q

Deficiencies of which clotting factors will cause prolonged PT and APTT?

A

II, V, X (common pathway factors)

Y3- TO LEARN (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions