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zero to finals Flashcards

1
Q

fibrinogen is a

A

clotting factor

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2
Q

when clotting factors are removed from the blood what is left

A

serum

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3
Q

reticulocytes originate from what stem cells

A

myeloid

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4
Q

how many months to red cell survive for

A

4

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5
Q

lifespan of platelets

A

around 10 days

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6
Q

target cells are mostly seen in

A

iron deficiency anaemia and post splenectomy

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7
Q

what refers to a variation in the size of the red blood cells

A

Anisocytosis

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8
Q

what are Heinz bodies

A

individuals blobs (inclusions) seen inside red blood cells

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9
Q

the inclusions inside Heinz bodies ar

A

denatured haemoglobin

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10
Q

Heinz bodies are mostly seen in

A

G6PD deficiency and alpha thalassaemia

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11
Q

Howell jolly bodies are blobs of what seen inside red blood cells

A

DNA material

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12
Q

why may see Howell jolly bodies

A

splenectomy, non functioning spleen (caused by sickle anaemia ) or in severe anaemia

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13
Q

Reticulocytes still have what material

A

RNA

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14
Q

RNA in reticulocytes have a what appearance

A

mesh like

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15
Q

normal to for about what % of red blood cells to be reticulocytes

A

1%

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16
Q

This percentage of reticulocytes goes up where there is a rapid turnover of red blood cells, such as with —, where the bone marrow is actively trying to replace lost cells.

A

haemolytic anaemia

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17
Q

what are fragments of red blood cells

A

Schistocytes

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18
Q

Schistocytes can also been seen in what replacements

A

metabolic heart valves

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19
Q

3 main causes of Microangiopathic haemolytic anaemia causing schistocytes

A

Haemolytic uraemia syndrome (HUS)
Disseminated intravascular coagulation (DIC)
Thrombotic thrombocytopenia purpura (TTP)

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20
Q

what are immature red blood cells with a nucleus surrounded by iron blobs

A

sideroblasts

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21
Q

sideroblastic anaemia occurs when

A

bone marrow cannot incorporate iron into the haemoglobin molecules

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22
Q

smudge cells are

A

ruptured white blood cells

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23
Q

smudge cells are particularly associated with what

A

CLL

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24
Q

what are spherocyets

A

sphere shaped red blood cells

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25
Q

spherocytes can indicate

A

autoimmune haemolytic anaemia or heredity spherocytosis

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26
Q

MCV in men and woman is

A

80-100 femtolitres

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27
Q

mnemonic for remembering the cause of microcytic anaemia is

A

TAILS
T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia

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28
Q

Treatment for anaemia of chronic disease

A

erythropoietin

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29
Q

what are the 3A’s and 2H’s for normocytic anaemia

A

A – Acute blood loss
A – Anaemia of chronic disease
A – Aplastic anaemia
H – Haemolytic anaemia
H – Hypothyroidism

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30
Q

macrocytic anaemia can be – or –

A

megaloblastic or normobalstic

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31
Q

megaloblastic macrocytic anaemia results from impaired

A

DNA synthesis

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32
Q

type of macrocytic megaloblastic anaemia

A

B12/ folate deficiency

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33
Q

Normoblastic macrocytic anaemia can be caused by

A

Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs, such as azathioprine

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34
Q

when might reticulocytosis occur

A

haemolytic anaemia or blood loss

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35
Q

symptoms specific too iron deficiency anaemia

A

Pica (dietary cravings for abnormal things, such as dirt or soil)
Hair loss

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36
Q

tachycardia or bradycardia is a sign of anaemia

A

tachycardia and raised red rate

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37
Q

what is atrophic glossitis

A

smooth tongue due to atrophy of the papillae

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38
Q

jaundice can indicate what kind of anaemia

A

haemolytic

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39
Q

bone deformities can indicate

A

thalassaemia

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40
Q

FBC measures

A

haemoglobin and MCV

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41
Q

LFTS are raised in

A

haemolysis

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42
Q

might look for what antibodies in pernicious anaemia

A

Intrinsic factor

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43
Q

what are indicated in unexplained iron defieicney anaemia to exclude GI cancer

A

colonoscopy and oesophagogastroduodenoscopy (OGD)

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44
Q

what is indicated for unexplained anaemia

A

bone marrow biopsy

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45
Q

iron deficiency causes what anaemia

A

microcytic hypochromic

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46
Q

other causes of iron deficiency anaemia

A

pregnancy due to increased requirements or peptic ulcer

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47
Q

most common cause of iron deficiency anaemia is

A

blood loss eg menorrhgia

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48
Q

what state of iron is soluble

A

Ferrous - Fe2+

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49
Q

what medications can interfere with iron absorption

A

PPIs eg omeprazole

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50
Q

what is also an acute phase protein released with inflammation, infection or cancer

A

ferritin

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51
Q

does normal ferritin exclude iron deficiency anaemia

A

no

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52
Q

what is not a very useful measure as it varies throughout the day

A

serum iron

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53
Q

what increases with iron deficiency anaemia

A

Total iron binding capacity and transferrin

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54
Q

what indicates the total iron in the body

A

transferrin saturation

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55
Q

what should do before taking a transferrin saturation

A

fast as it can temporarily increase after a meal

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56
Q

other cause of iron overload

A

acute liver damage ( liver contains lots of iron)

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57
Q

oral iron can be

A

ferrous sulphate or ferrous fumarate

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58
Q

when should iron infusions be avoided

A

during infections as there is a potential for it to feed the bacteria

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59
Q

3 key causes of low B12

A

pernicious anaemia, insufficient dietary B12, medications

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60
Q

medication that can cause low B12

A

PPIs and metformin

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61
Q

B12 is absorbed in the

A

distal ileum

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62
Q

3 symptoms of pernicious anaemia

A

Peripheral neuropathy, with numbness or paraesthesia (pins and needles)
Loss of vibration sense
Loss of proprioception

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63
Q

first line investigation for pernicious anaemia

A

intrinsic factor antibodies

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64
Q

what is initially given to patients with B12 deficiency

A

Intramuscular hydroxocobalamin

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65
Q

what is given for maintenance of vitamin B12 defieicny if its pernicious anaemia

A

2-3 monthly injections for life

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66
Q

what is given for maintenance of vitamin B12 defieicny if its diet related

A

oral cyanocobalamin or twice-yearly injections

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67
Q

other symptom of destruction of red blood cells

A

Splenomegaly (the spleen becomes filled with destroyed red blood cells)

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68
Q

blood film shows schistocytes (fragments of red cells ) in

A

haemolytic anaemia

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69
Q

most common inherited haemolytic anaemia in northern Europeans

A

hereditary spherocytosis

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70
Q

hereditary spherocytosis inheritance pattern

A

autosomal dominant

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71
Q

what presents with anaemia, jaundice, gallstones and splenomegaly

A

hereditary spherocytosis

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72
Q

what is a notable feature of hereditary spherocytsosi

A

aplastic crisis in the presence of the parvovirus

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73
Q

what is raised in terms of bloods in hereditary spherocytosis

A

MCHC ( mean corpuscular haemoglobin concentration) and reticulocyte count

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74
Q

what is treated with folate supplementation

A

hereditary spherocytosis

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75
Q

what enzyme is responsible for protecting the cells from oxidative damage

A

glucose 6 phosphate dehydrogenase

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76
Q

G6PD deficiency has what inheritance pattern

A

X linked recessive

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77
Q

what condition results in acute episodes of haemolytic anaemia triggered by infections , drugs or fava beans

A

G6PD deficiency

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78
Q

key medications that can be triggers for G6PD deficiency are

A

ciprofloxacin, sulfonylureas (e.g., gliclazide) and sulfasalazine.

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79
Q

what can diagnose G6PD defieicny

A

G6PD enzyme assay

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80
Q

presents with jaundice (often in the neonatal period), gallstones, anaemia, splenomegaly and Heinz bodies on a blood film.

A

G6PD deficiency

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81
Q

drug class that can cause G6PD deficiency is also

A

antimalarials

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82
Q

what autoimmune haemolytic anaemia is more common

A

warm

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83
Q

cold AIHA can be secondary to

A

lymphoma, leukaemia, systemic lupus erythematosus and infections (e.g., mycoplasma, EBV, CMV and HIV).

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84
Q

warm autoimmune haemolytic anaemia is usually

A

idiopathic

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85
Q

alloimmune haemolytic anaemia 2 scenarios are

A

transfusion reactions and haemolytic disease of the newborn.

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86
Q

paroxysmal nocturnal haemoglobinuria is caused by mutation in what

A

haemoatpoieic stem cells - mutations occur during the patients lifetime- mutation means activation of complement cascade

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87
Q

management for paroxysmal nocturnal haemoglobinuria

A

Eculizumab or bone marrow transplantation

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88
Q

what is monclonal antibody that targets C5

A

Eculizumab

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89
Q

the red urine in the morning in paroxysmal nocturnal haemoglobinuria contains

A

haemoglobin and haemosiderin

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90
Q

Microangiopathic haemolytic anaemia is usually secondary to something and what is a key finding seen on blood film

A

Schistocytes

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91
Q

what supplementation is given for prosthetic valve haemolyssi

A

iron and folic acid

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92
Q

the thalasaemias has what inheritance pattern

A

autosomal recessive

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93
Q

what is used to diagnose global abnormalities

A

haemoglobin electrophoresis

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94
Q

what may occur in thalassaemias

A

iron overload

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95
Q

what chromosome are the genes that code for alpha and beta thalassaemias on

A

alpha- 16
beta - 11

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96
Q

thalassaemia intermedia has how many abnormal copies of the beta global gene

A

2

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97
Q

how many functioning beta global genes do beta thalassaemia major have

A

none

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98
Q

what are features of thalassaemia major

A

prominent forehead, prominent cheekbones, flat nose, protruding upper teeth

99
Q

sickle red blood cells are what shape

A

crescent

100
Q

at birth how much haemoglobin is fetal and how much is adult

A

1/2 and 1/2

101
Q

sickle cell anaemia has what inheritance pattern

A

autosomal recessive

102
Q

sickle cell anaemia affects beta global on chromosome

A

11

103
Q

difference between sickle cell trait and sickle cell disease

A

triat - one abnormal copy of the gene - usually asymptomatic
disease- two abnormal copies

104
Q

those with sickle cell trait has a selective advantage against

A

malaria

105
Q

when is sickle cell screened for in babies

A

day 5 - newborn blood spot

106
Q

what can trigger sickle cell crises

A

dehydration, infection, stress, cold weather

107
Q

what is the treatment for sickle cell crisis

A

supportive

108
Q

what is the most common type of sickle cell crisis

A

vaso occlusive crisis

109
Q

vaso oclusive crisis can cause what

A

distal ischaemia with pain and swelling in the hands or feet but can affect chest or back etc. it can be associated with fever and can cause priapism in men (painful and persistent erection)

110
Q

how to treat priapism

A

urological emergency - aspirate blood from the penis

111
Q

what sickle cell crisis causes an acutely enlarged and painful spleen

A

Splenic sequestration crisis

112
Q

splenic sequestration crisis causes hyper or hypo splenism

A

HYPO

113
Q

what describes the temporary absence of the creation of new red blood cells

A

aplastic crisis

114
Q

aplastic crisis is usually triggered by infection with

A

parvovirus B19

115
Q

cxr in acute chest syndrome will show

A

pulmonary infiltrates

116
Q

antibiotic prophylaxis in sickle is with

A

penicillin V (phenoxymethylpenicillin)

117
Q

what stimulates the production of Fetal haemoglobin

A

Hydroxycarbamide

118
Q

monoclonal antibody that ca n be used for sickle

A

Crizanlizumab

119
Q

what leukemias is associated with down syndrome

A

ALL

120
Q

what leukemias is associated with warm haemolytic anaemia, Richters transformation and smudge cells

A

CLL

121
Q

what leukemias has three phases, including a long chronic phase, and is associated with the Philidelphia chromosome

A

CML

122
Q

what leukemias results in a transformation from a myeloproliferative disorder and is associated with auger rods

A

AML

123
Q

a key presenting feature of leukemias is bleeding under the skin due to

A

thrombocytopenia

124
Q

suspected leukemia what should happen

A

FBC within 48hrs

125
Q

children or young people with what symptoms should be sent for immediate specialist assessment

A

petechiae or hepatosplenomegaly

126
Q

what establishes a definitive diagnosis of leukaemia

A

bone marrow biopsy

127
Q

what bone marrow biopsy is better for seeing the cells

A

trephine

128
Q

the Philadelphia chromosome is more associated with CML but can be seen in

A

ALL

129
Q

ALL usually affects what lymphocytes

A

B

130
Q

what other thing may be seen in ALL

A

pancytopenia

131
Q

CLL usually affects what lymphocyte ( the same as ALL)

A

B

132
Q

what may cause warm autoimmune haemolytic anaemia

A

CLL

133
Q

what is righters transformation

A

CLL into high grade B cell lymphoma

134
Q

what cells are particularly associated with CLL

A

smear or smudge cells

135
Q

smear or smudge cells are what kind of cells

A

ruptured white blood cells

136
Q

what phase of CML is often asymptotic and diagnosed after an incidental finding of a raised white cell count

A

chronic

137
Q

what phase of CML the patient develops anaemia, thrombocytopenia and immunodeficiency

A

accelerated

138
Q

what phase of CML the patient develops pancytopenia

A

blast

139
Q

what has occurred on the Philadelphia chromosome in CML

A

reciprocal translocation

140
Q

translocation of the Philadelphia chromosome creates a gene sequence called — which codes for a abnormal tyrosine kinase enzyme

A

BCR- ABL1

141
Q

what myeloproliferative disorder might AML transform from

A

polycythaemia ruby vera or myelofibrosis

142
Q

Auer rods

A

AML

143
Q

bone marrow biopsy will show a high proportion of blasts in

A

AML

144
Q

what is a tyrosine kinase inhibitor

A

ibrutinib

145
Q

what is a monoclonal antibody that targets B cells

A

rituximab

146
Q

what is low in tumour lysis syndrome

A

calcium

147
Q

what reduces risk of tumour lysis syndrome

A

very good hydration and urine output before chemo

148
Q

what may be used to suppress the uric acid levels in tumour lysis syndrome

A

allopurinol or rasburicase

149
Q

what is the most common specific type of lymphoma

A

hodgkins

150
Q

what has a bimodal age distribution with peaks around early 20s then at 80

A

hodgkins

151
Q

4 rf for hodgkins

A

HIV, EBV, Autoimmune conditions and FH

152
Q

NHL lymphomas include

A

diffuse large B cell , Burkitt, Malt

153
Q

what typically presents as a rapidly growing painless mass in older patients

A

diffuse large B cell

154
Q

what NHL is particularly associated with EBV and HIV

A

Burkitt

155
Q

what lymphoma particularly affect the mucosa associated lymphoid tissue usually around the stomach

A

MALT

156
Q

h. pylori is associated with what lymphoma

A

MALT

157
Q

pesticides exposure increases your risk of

A

NHL

158
Q

patients with what lymphoma may experience lymph node pain after drinking alcohol

A

hodgkins

159
Q

what is a critical diagnostic investigation in lymphoma

A

lymph node biopsy

160
Q

what are large cancerous B lymphocytes with two nuclei and prominent nucleoli, giving them a cartoonish appearance of an owl face with large eyes.

A

Reed sternerg cells - hodgkins lymphoma

161
Q

what classification has replaced the Ann Arbor for Lymphoma

A

Lugano

162
Q

critical treatment for hodgkins lymphoma is

A

chemo and radiotherapy

163
Q

rituximab may be used for what lymphoma

A

NHL

164
Q

plasma cells are what lymphocytes

A

B

165
Q

Cancer in a specific type of plasma cell results in the production of large quantities of a specific

A

paraprotein (or M protein)

166
Q

what is a paraprotein

A

abnormal antibody or part of an antibody

167
Q

— involves the production of a specific paraprotein without other features of myeloma or cancer.

A

Monoclonal gammopathy of undetermined significance (MGUS)

168
Q

what is the % risk of MGUS progressing to myeloma

A

1%

169
Q

Smouldering myeloma involves abnormal plasma cells and paraproteins but no organ damage or symptoms. It has a greater risk of progression to myeloma (about — % per year).

A

10

170
Q

paraprotein is often the

A

light chain

171
Q

bence jones protein refers to

A

free light chains in the urine

172
Q

there is paraproteinaemia ( abnormally high level of this paraprotein) in

A

myeloma

173
Q

calcium in myeloma

A

elevated

174
Q

most common complications of myeloma

A

anaemia

175
Q

anaemia in myeloma is

A

normocytic and normochromic

176
Q

hyperviscocity syndrome may occur in

A

myeloma

177
Q

what is raised in myeloma

A

calcium , ESR and plasma viscocity

178
Q

what detects paraproteinaemia

A

serum protein electrophoresis

179
Q

what detects Bence Jones protein

A

urine protein electrophoresis

180
Q

what is required to confirm the diagnosis in myeloma

A

bone marrow biopsy

181
Q

what imaging is first lien for bone lesion in myeloma

A

whole body MRI

182
Q

lytic lesions in myeloma may be described as looking

A

punched out

183
Q

what refers to multiple lytic lesion seen in the skull on X-ray in myeloma

A

Raindrop skull, sometimes called pepper pot skull

184
Q

treatment of myeloma usually involves a combination of chemo which may include

A

Bortezomib (a proteasome inhibitor)
Thalidomide
Dexamethasone

185
Q

what is meant by a autologous stem cell transplant

A

using the persons own stem cells

186
Q

what is meant by allogeneic transplant

A

using stem cells from a healthy donor

187
Q

drug for myeloma bone disease

A

biphosphonates

188
Q

Myeloproliferative disorders involves the uncontrolled proliferation of

A

single type of stem cell

189
Q

—are considered a form of cancer occurring in the bone marrow, although they tend to develop and progress slowly

A

myeloproliferative disorders

190
Q

myeloproliferative disorders has the potential to transform into

A

AML

191
Q

Myeloproliferative disorders to remember are

A

Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia

192
Q

polycthaemia vera has what proliferating cell line

A

erythroid cells

193
Q

PV has what blood finding

A

high haemoglobin

194
Q

essential thrombocythaemi has what blood finding

A

high platelet count

195
Q

primary myelofibrosis has what blood finding

A

low haemoglobin

196
Q

JAK2 associated with the

A

myeloproliferative disorders

197
Q

ruxolitinib is a

A

JAK2 inhibitor

198
Q

what cytokine can cause bone marrow fibrosis

A

fibroblast growth factor

199
Q

what will a blood film in myelofibrosis show

A

Teardrop-shaped red blood cells
Anisocytosis (varying sizes of red blood cells)
Blasts (immature red and white cells)

200
Q

raised haemoglobin can present as

A

itching headaches and a red face

201
Q

what is a complication of polycythaemia

A

gout

202
Q

signs of polycythamia

A

Ruddy complexion (red face)
Conjunctival plethora (the opposite of conjunctival pallor)

203
Q

what is done to confirm the diagnosis of myeloproliferative disorders

A

bone marrow biopsy

204
Q

what may the bone marrow aspiration be with myelofibrosis

A

dry

205
Q

other mutation in what genes in myeloprolferative disorders except form JAK2

A

MPL, CALR

206
Q

polycythamei vera mx may involves

A

venesection and aspirin

207
Q

mx of essential thrombocythaemia

A

aspirin

208
Q

hydroxycarbamide is a type of

A

chemo

209
Q

example of a platelet lowering agent

A

Anagrelide

210
Q

rf for myelodysplastic syndrome

A

chemo or radiotherapy

211
Q

who may present with anaemia, neutropenia and thrombocytopenia

A

myelodysplastic syndrome

212
Q

myelodysplastic syndrome has the potential to transform into

A

AML

213
Q

medications that can increase platelet destruction causing thrombocytopenia

A

sodium valproate and methotrexate

214
Q

mx for immune thrrombocytpenic purpura

A

prednisolone

215
Q

rituximab targets what proteins on B cells

A

CD20

216
Q

—- is a condition where tiny thrombi develop throughout the small vessels, using up platelets.

A

Thrombotic thrombocytopenia purpura

217
Q

problem with what protein in thrombotic thrombocytopenic purpura

A

ADAMTS13

218
Q

heparin induced thrombocytopenia presents how long after starting treatment with heparin

A

5-10 days

219
Q

what alternative anticoagulant can be given in heparin induced thrombocytopenia

A

fondaparinux or argatoban

220
Q

what is the most common inherited cause of abnormal and prolonged bleeding

A

von willebrands disease

221
Q

most von willebrands disease has what inheritance pattern

A

autosomal dominant

222
Q

diagnosis of von will brands disease is through what

A

history

223
Q

mx for von willebrands

A

only given in response to significant bleeding or trauma or in preparation for operations - desmopressin, tranexamic acid

224
Q

both haemophilia have what inheritance

A

X linked recessive

225
Q

All X chromosomes need to have the abnormal gene to have –

A

haemophilia

226
Q

bleeding into the muscles I haemophilia can cause

A

compartment syndrome

227
Q

complication of clotting factor infusions in haemophilia is

A

foramtion of antibodies against the treatment

228
Q

when a thrombus develops it can travel from the deep veins through which side of the heart and into the lungs

A

right

229
Q

how is antiphospholipid syndrome diagnosed

A

blood test for antiphospholipid antibodies

230
Q

prophylaxis for VTE usually involves LMWH such as

A

enoxaparin

231
Q

contraindications to LMWH for VTW prophylaxis if

A

active bleeding or existing anticoagulation with warfarin or a DOAC

232
Q

what is the main contraindication for compression stockings

A

peripheral arterial disease

233
Q

Ultrasound of the leg is required to diagnose deep vein thrombosis. NICE recommends repeating negative ultrasound scans after — days if the patient has a positive D-dimer and the Wells score suggests a DVT is likely.

A

6-8

234
Q

what is the usual first line imaging investigation for a pulmonary embolism

A

CT Pulmonary angiogram

235
Q

initial anticoagulant for DVT /pE

A

apixaban/ rivaroxaban - can be started before scan if there is a delay in getting scan

236
Q

The NICE guidelines (2020) recommend considering — in patients with a symptomatic iliofemoral DVT and symptoms lasting less than 14 days.

A

catheter-directed thrombolysis

237
Q

The options for long-term anticoagulation in VTE are a

A

DOAC, warfarin or LMWH.

238
Q

do DOACs require monitoring

A

no

239
Q

DOACs not suitable if

A

severe renal impairment, pregnancy or antiphopsholipid syndrome

240
Q

what is the first lien in patients with antiphosphilipid syndrome for DVT/PE

A

warfarin

241
Q

what is the first line anticoagulant in pregnancy

A

LMWH

242
Q

syndrome involves obstruction to the outflow of blood from the liver caused by thrombosis in the hepatic veins or inferior vena cava. It is associated with hypercoagulable states (e.g., myeloproliferative disorders). It presents with a classic triad of:

Abdominal pain
Hepatomegaly
Ascites

A

Budd-Chiari

243
Q

usual imaging for Budd chiari syndrome

A

doppler ultrasonography

My haem (8 decks)

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