Haem Flashcards
1
Q
Myeloma
what is it
A
cancer of the plasma cells (a type of B-lymphocyte that produces antibodies)
cancer in a specific type of plasma cell results in large quantities of a single type of antibody being produced
2
Q
Myeloma
what is multiple myeloma
A
where the myeloma affects multiple areas of the body
3
Q
Myeloma
what is monoclonal gammopathy of undetermined significance (MGUS)
A
where there’s an XS of a single type of antibody or antibody components without other features of myeloma or cancer
may progress to myeloma so routinely followed up
4
Q
Myeloma
what is smouldering myeloma
A
where there is progression of MGUS with higher levels of antibodies or antibody components
premalignant and more likely to progress to myeloma than MGUS
5
Q
Myeloma
what is Waldenstrom’s macroglobulinemia
A
a type of smouldering myeloma where there is XS IgM specifically
6
Q
Myeloma
why are they called B cells
A
they are found in the bone marrow
7
Q
Myeloma
pathophysiology
A
genetic mutation in plasma cells causing it to rapidly and uncontrollably multiply
they produce an abundance of immunoglobulins (usually IgG)
8
Q
Myeloma
what is a monoclonal paraprotein
A
a single type of abnormal protein
in this case, IgG produced by all the identical cancerous plasma cells
9
Q
Myeloma
what can be found in the urine
A
Bence Jones protein
light chains of the antibody in myeloma
10
Q
Myeloma
why is there anaemia, neutropenia and thrombocytopenia
A
Bone marrow infiltration from the cancerous plasma cells
causes suppression of the development of other blood cell lines
11
Q
Myeloma
how is myeloma bone disease caused
A
increased osteoclast activity and suppressed osteoblast activity
caused by cytokines release from the plasma cells and the stromal cells (other bone cells) when they are in contact with the plasma cells
12
Q
Myeloma
where are common places for myeloma bone disease to happen
A
the skull, spine, long bones and ribs
13
Q
Myeloma
what’s the name for patches of thin bone
A
osteolytic lesions
14
Q
Myeloma
what can osteolytic lesions lead to
A
pathological fractures
e.g. fractured femur or vertebra from minimal force
15
Q
Myeloma
why is there hypercalcaemia in myeloma bone disease
A
osteoclast activity causes calcium to be reabsorbed from the bone into the blood
16
Q
Myeloma
what can pts also develop
A
plasmacytomas
individual tumours made up of the cancerous plasma cells
they occur in the bones, replacing normal bone tissue or can occur outside bones in the soft tissue of the body
17
Q
Myeloma
why do pts develop renal impairment (4)
A
- high levels of Ig can block the flow through the tubules
- hypercalcaemia impairs renal function
- dehydration
- bisphosphonates can be harmful to the kidneys
18
Q
Myeloma
what is the normal plasma viscosity (or internal friction in the flow of blood)
A
between 1.3 and 1.7 times that of water
blood is 1,3 to 1.7 times thicker than water
19
Q
Myeloma
why is the plasma viscosity significantly higher
A
large amounts of Ig in the blood
20
Q
Myeloma
what issues can raised plasma viscosity cause
A
- easy bruising
- easy bleeding
- reduced or loss of sight
- purplish palmar erythema
- heart failure
21
Q
Myeloma
why may there be reduced sight
A
raised plasma viscosity cause vascular disease in the eye
22
Q
Myeloma
4 key features of myeloma for exams
A
CRAB
Calcium (elevated)
Renal failure
Anaemia (normocytic, normochromic) from replacement of bone marrow
Bone lesions/pain
23
Q
Myeloma
RFs (5)
A
- older age
- male
- black african ethnicity
- FH
- obesity
24
Q
Myeloma
when should you consider myeloma
A
in anyone >60 with
- persistent bone pain esp back
- or unexplained fractures
25
Myeloma
what initial inx would you perform if suspecting myeloma
- FBC: low WCC
- Ca: raised
- ESR: raised
- plasma viscosity: raised
26
Myeloma
if any of the initial inx are positive or myeloma is still suspected, what do you do
an urgent serum protein electrophoresis
and a urine Bence-Jones protein test
27
Myeloma
what initial inx would you perform when testing for myeloma
BLIP
Bence-Jones protein (request urine electrophoresis)
serum-free Light-chain assay
serum Immunoglobulins
serum Protein electrophoresis
28
Myeloma
which inx is necessary to confirm dx
bone marrow biopsy
29
Myeloma
what imaging is required to assess for bone lesions (in order of preference)
1. whole body MRI
2. whole body CT
3. skeletal survey (xray images of the full skeleton)
pts only require 1 inx
30
Myeloma
xray signs
- punched out lesions
- lytic lesions
- raindrop skull
31
Myeloma
what is raindrop skull
x ray sign caused by many punched out (lytic) lesions throughout the skull that give the appearance of raindrops splashing on a surface
32
Myeloma
1st line trx
combination of chemo:
- Bortezomid
- Thalidomide
- Dexamethasone
33
Myeloma
whilst on certain chemo regimes (e.g. thalidomide), what else do pts require
VTE prophylaxis with aspirin or LMWH as there is a higher risk of developing a thrombus
34
Myeloma
when can stem cell transplantation be used
as part of a clinicial trial where pts are suitable
35
Myeloma
mnx of myeloma bone disease
- bisphosphonates
- radiotherapy: can improve bone pain
- orthopaedic surgery: can stabilise bones or treat fractures
- cement augmentation
36
Myeloma
what is cement augmentation
injecting cement into vertebral fractures or lesions and can improve spine stability and pain
37
Myeloma
complications
- infection
- pain
- renal failure
- anaemia
- hypercalcaemia
- peripheral neuropathy
- spinal cord compression
- hyperviscocity
38
B12 insufficiency causes
- insufficient dietary intake of B12
| - pernicious anaemia
39
Pernicious anaemia
what is it
an autoimmune condition where antibodies form against the parietal cells or intrinsic factor
40
Pernicious anaemia
why do pts become B12 deficient
a lack of intrinsic factor prevents the absorption of B12
41
Pernicious anaemia
what produces intrinsic factor
parietal cells of the stomach
42
Pernicious anaemia
what does intrinsic factor do
essential for the absorption of B12 in the ileum
43
Pernicious anaemia
what neurological sx do pts present with in B12 deficiency
- peripheral neuropathy with numbness or paraesthesia (pins and needles)
- loss of vibration sense or proprioception
- visual changes
- mood or cognitive changes
44
Pernicious anaemia
1st line inx and dx
test for intrinsic factor antibody
| gastric parietal cell antibody can also be tested but is less helpful
45
B12 dietary deficiency mnx
PO cyanocobalamin
46
Pernicious anaemia
why is oral replacement inadequate
because the problem is with absorption rather than intake
47
Pernicious anaemia
trx
1mg IM hydroxycobalamin
3 times weekly for 2w, then every 3m
48
Pernicious anaemia
what do you correct first? folate or B12 deficiency
B12 deficiency because treating pts with folic acid can lead to subacute combined degeneration of the cord
49
Pernicious anaemia
FBC results
- macrocytic anaemia
- hypersegmented polymorphs on blood film
- low WCC + platelets
50
Pernicious anaemia
RFs (3)
- female
- autoimmune conditions: vitiligo, thyroid, T1DM, rheumatoid, addison's
- blood group A
51
Blood film findings
what is Anisocytosis
variation in size of the RBCs
52
Blood film findings
which condition is Anisocytosis seen in
- myelodysplasic syndrome
| - some forms of anaemia
53
Blood film findings
what are target cells
have a central pigmented area, surrounded by a ring of thicker cytoplasm on the outside
'bull's eye target'
54
Blood film findings
when can target cells be found
- in iron deficiency anaemia
| - in post-splenectomy
55
Blood film findings
what are Heinz Bodies
individual blobs seen inside RBCs caused by denatured globin
56
Blood film findings
where are Heinz Bodies seen in
- G6PD deficiency
| - alpha thalassaemia
57
Blood film findings
what are Howell-Jolly bodies
individual blobs of DNA material seen inside RBCs
normally this DNA material is removed from the spleen during circulation of RBCs
58
Blood film findings
when are Howell-Jolly bodies seen
- post-splenectomy
| - severe anaemia where the body is regenerating RBCs quickly
59
Blood film findings
what are reticulocytes
immature RBCs
60
Blood film findings
when is there an increase in reticulocytes
when there is rapid turnover in RBCS e.g. haemolytic anaemia
demonstrates that the bone marrow is active in replacing lost cells
61
Blood film findings
what are schistocytes and what do they indicate
fragments of RBCs
RBCs are being physically damaged by trauma during their journey through the blood vessels
may indicate networks of clots in small blood vessels
62
Blood film findings
when are schistocytes seen
- HUS
- DIC
- TTP
- replacement metallic heart valves
- haemolytic anaemia
63
Blood film findings
what are sideroblasts
immature RBCs that contain blobs of iron
they occur when the bone marrow is unable to incorporate iron into the haemoglobin molecules
64
Blood film findings
which condition may sideroblasts indicate
myelodysplasic syndrome
65
Blood film findings
what are smudge cells
ruptured WBCs that occur during the process of preparing the blood film due to aged or fragile WBCs
66
Blood film findings
what can smudge cells indicate
CLL
| chronic lymphocytic leukaemia
67
Blood film findings
what are spherocytes
spherical RBCs without the normal bi-concave disk space
68
Blood film findings
what do spherocytes indicate
- autoimmune haemolytic anaemia
| - hereditary spherocytosis
69
Haemolytic Anaemia
what is it
destruction of RBCs (haemolysis) leading to anaemia
70
Haemolytic Anaemia
what can inherited haemolytic anaemias cause
RBCs to be more fragile and break down faster than normal
leading to chronic haemolytic anaemia
71
Haemolytic Anaemia
name the inherited haemolytic anaemias (5)
- hereditary spherocytosis
- hereditary elliptocytosis
- thalassaemia
- sickle cell anaemia
- G6PD deficiency
72
Haemolytic Anaemia
what can acquired haemolytic anaemias lead to
increased breakdown of RBCs and haemolytic anaemia
73
Haemolytic Anaemia
name 5 acquired haemolytic anaemias
- autoimmune haemolytic anaemia
- alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
- paroxysmal nocturnal haemoglobinuria
- microangiopathic haemolytic anaemia
- prosthetic valve related haemolysis
74
Haemolytic Anaemia
features (3)
- anaemia
- splenomegaly
- jaundice
75
Haemolytic Anaemia
why is there splenomegaly
the spleen becomes filled with destroyed RBCs
76
Haemolytic Anaemia
why is there jaundice
biliruibin is released during the destruction of RBCs
77
Haemolytic Anaemia
what would the FBC count show
normocytic anaemia
78
Haemolytic Anaemia
what does the blood film show
schistocytes (fragments of RBCs)
79
Haemolytic Anaemia
what test would be positive in autoimmune haemolytic anaemia
Direct Coombs test
80
Haemolytic Anaemia
what is the most common inherited haemolytic anaemia in northern Europeans
Hereditary Spherocytosis
81
Haemolytic Anaemia
what is the inheritance pattern in Hereditary Spherocytosis
autosomal dominant
82
Haemolytic Anaemia
what does hereditary spherocytosis cause
sphere shaped RBCs that are fragile and easily break down when passing through the spleen
83
Haemolytic Anaemia
what does hereditary spherocytosis present with
- jaundice
- gallstones
- splenomegaly
84
Haemolytic Anaemia
Hereditary Spherocytosis: in the presence of parvovirus, what does hereditary spherocytosis present with
aplastic crisis: abnormal decrease in reticulocytes
85
Haemolytic Anaemia
Hereditary Spherocytosis: what is shown on the blood film
spherocytes
86
Haemolytic Anaemia
Hereditary Spherocytosis: dx
- FH
- clinical
- spherocytes on blood film
87
Haemolytic Anaemia
Hereditary Spherocytosis: what would the FBC show
raised mean corpuscular haem conc (MCHC)
raised reticulocytes due to rapid turnover of RBCs
88
Hereditary Spherocytosis
trx
- folate supplementation
- splenectomy
- cholecystectomy if gallstones are a problem
89
Hereditary Elliptocytosis
what is it
very similar to hereditary spherocytosis except that the red blood cells are ellipse shaped.
autosomal dominant
presentation + mnx the same
90
Haemolytic Anaemia
G6PD Deficiency: what is it
defect in the RBC enzyme, G6PD
91
Haemolytic Anaemia
G6PD Deficiency: whom is it more common in
Mediterranean and African patients
92
Haemolytic Anaemia
G6PD Deficiency: what is the inheritance pattern
autosomal recessive
93
Haemolytic Anaemia
G6PD Deficiency: what can crises be triggered by
- fava beans (broad beans)
- infections
- medications
94
Haemolytic Anaemia
G6PD Deficiency: presentation
- jaundice (usually in neonatal period)
- gallstones
- anaemia
- splenomegaly
95
Haemolytic Anaemia
G6PD Deficiency: what would the blood film show
Heinz bodies
96
Haemolytic Anaemia
G6PD Deficiency: diagnostic inx
G6PD enzyme assay
97
Haemolytic Anaemia
G6PD Deficiency: what medications trigger haemolysis
- primaquine (an antimalarial)
- ciprofloaxacin
- sulfonylureas
- sulfasalazine
- other sulphonamide drugs
98
patient turns jaundice and becomes anaemic after eating broad beans/ developing an infection/ given antimalarials. what is it
G6PD deficiency
99
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what is it
occurs when antibodies are created against the pt's RBCs
leading to destruction of the RBCs
100
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what are the 2 types based on
the temp at which the auto-antibodies function to cause the destruction of RBCs
101
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what is the more common type
warm type
102
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what is warm type
haemolysis occurs at normal or above normal temperatures
103
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): warm type cause
idiopathic
104
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what is cold type aka
cold agglutinin disease
105
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): pathophysiology of cold type
- at <10°C the antibodies attach themselves to RBCs and cause them to clump (agglutination)
- immune system activated against them and destroys RBCs
- they get filtered and destroyed by the spleen
106
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what is cold type often secondary to
- lymphoma
- leukaemia
- SLE
- mycoplasma, EBV, CMV, HIV
107
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): mnx
- blood transfusions
- prednisolone
- Rituximab
- splenectomy
108
Haemolytic Anaemia
Autoimmune Haemolytic Anaemia (AIHA): what is Rituximab
a monoclonal antibody against B cells
109
Haemolytic Anaemia
Alloimmune Haemolytic Anaemia: when does it occur (2 scenarios)
1) haemolytic transfusion reactions
| 2) haemolytic disease of the newborn
110
Haemolytic Anaemia
Alloimmune Haemolytic Anaemia: what are haemolytic transfusion reactions
foreign RBCs circulate pt's blood --> immune reaction --> RBCs destroyed
111
Haemolytic Anaemia
Alloimmune Haemolytic Anaemia: what is haemolytic disease of the newborn
foreign antibodies cross placenta from mum to fetus --> maternal antibodies target antigens on RBCs of fetus --> destruction of the RBCs in the fetus + neonate
112
Haemolytic Anaemia
what is Paroxysmal Nocturnal Haemoglobinuria
rare
when a specific genetic mutation in the haematopoietic stem cells in the bone marrow occurs during the pt's lifetime
113
Haemolytic Anaemia
Paroxysmal Nocturnal Haemoglobinuria: what does the mutation result in
loss of proteins on the surface of RBCs that inhibit the complement cascade
so there is activation of the compliment cascade on the surface of RBCs and destruction of RBCs
114
Haemolytic Anaemia
Paroxysmal Nocturnal Haemoglobinuria: what is the characteristic presentation
red urine in the morning containing Hb + haemosiderin
115
Haemolytic Anaemia
Paroxysmal Nocturnal Haemoglobinuria: what are they predisposed to
- thrombosis
| - smooth muscle dystonia (e.g. oesophageal spasm + erectile function)
116
Haemolytic Anaemia
Paroxysmal Nocturnal Haemoglobinuria: mnx
- Eculizumab
| - bone marrow transplantation (curative)
117
Haemolytic Anaemia
Paroxysmal Nocturnal Haemoglobinuria: what is Eculizumab
a monoclonal antibody that targets complement component 5 (C5) causing suppression of the complement system
118
Haemolytic Anaemia
Microangiopathic Haemolytic Anaemia (MAHA): what is it
where the small blood vessels have structural abnormalities that cause haemolysis of the blood cells travelling through them
119
Haemolytic Anaemia
Microangiopathic Haemolytic Anaemia (MAHA): cause
secondary to:
- haemolytic uraemic syndrome
- DIC
- TTP
- SLE
- cancer
120
Haemolytic Anaemia
Prosthetic Valve Haemolysis: how does it cause haemolytic anaemia
the valve churns up the cells and they break down
121
Haemolytic Anaemia
what is a key complication in prosthetic heart valves and why
haemolytic anaemia caused by turbulence around the valve and collision of RBCs with the implanted valve
122
Haemolytic Anaemia
Prosthetic Valve Haemolysis: mnx
- monitoring
- PO iron
- blood transfusion if severe
- revision surgery if severe
123
Myeloproliferative Disorders
what are 3 myeloproliferative disorders to remember
- Primary myelofibrosis
- Polycythaemia vera
- Essential thrombocythaemia
124
Myeloproliferative Disorders
what do these conditions occur due to
uncontrolled proliferation of a single type of stem cell
considered a type of bone marrow cancer
125
Myeloproliferative Disorders
what is primary myelofibrosis
proliferation of the haematopoietic stem cell
126
Myeloproliferative Disorders
what is Polycythaemia vera
proliferation of the erythroid cell line
127
Myeloproliferative Disorders
what is essential thrombocythaemia
proliferation of the megakaryocytic cell line
128
Myeloproliferative Disorders
what do they have the protentional to progress and transform into
acute myeloid leukaemia
129
Myeloproliferative Disorders
mutations in which genes?
- JAK2
- MPL
- CALR
130
Myeloproliferative Disorders
what is ruxolitinib
JAK2 inhibitor
131
Myeloproliferative Disorders
what is myelofibrosis
where the proliferation of the cell line leads to fibrosis of the bone marrow
the bone marrow is replaced by scar tissue
132
Myeloproliferative Disorders
what can cause myelofibrosis
- primary myelofibrosis
- polycythaemia vera
- essential thrombocythaemia
133
Myeloproliferative Disorders
why does fibrosis occur in the bone marrow in myelofibrosis
in response to cytokines that are released from the proliferating cells
134
Myeloproliferative Disorders
which particular cytokine is released from proliferating cells in myelofibrosis
fibroblast growth factor
135
Myeloproliferative Disorders
why can myelofibrosis lead to anaemia and leukopenia *(low WCC)
the fibrosis affects the production of blood cells
136
Myeloproliferative Disorders
Myelofibrosis: what is extramedullary haematopoiesis
when the bone marrow is replaced with scar tissue, the production of blood cells (haematopoiesis) starts to happen in other areas such as the liver and spleen
137
Myeloproliferative Disorders
Myelofibrosis: what can extramedullary haematopoiesis lead to
hepatomegaly and splenomegaly --> portal HTN
if occurs at spine --> spinal cord compression
138
Myeloproliferative Disorders
presentation
initially asymptomatic
systemic sx:
- fatigue
- weight loss
- night sweats
- fever
139
Myeloproliferative Disorders
signs and sx of underlying complications
- Anaemia (except in polycythaemia)
- Splenomegaly (abdominal pain)
- Portal hypertension (ascites, varices and abdominal pain)
- Low platelets (bleeding and petechiae)
- Thrombosis common in polycythaemia and thrombocythaemia
- Raised RBCs (thrombosis and red face)
- Low WCC (infections)
140
Myeloproliferative Disorders
Polycythaemia vera: 3 key signs on examination
1. conjunctival plethora
2. ruddy complexion
3. splenomegaly
141
Myeloproliferative Disorders
Polycythaemia vera: what is conjunctival plethora
excessive redness to the conjunctiva in the eyes
142
Myeloproliferative Disorders
FBC findings in polycythaemia vera
raised Hb
>185 in men
>165 in women
143
Myeloproliferative Disorders
FBC findings in primary thrombocythaemia
raised platelet count
| >600 x 10^9/l
144
Myeloproliferative Disorders
FBC findings due to primary MF or secondary to PV or ET
can give variable findings
- Anaemia
- Leukocytosis or leukopenia (high or low white cell counts)
- Thrombocytosis or thrombocytopenia (high or low platelet counts)
145
Myeloproliferative Disorders
what will the blood film show in myelofibrosis
- teardrop-shaped RBCs
- poikilocytosis
- blasts
146
Myeloproliferative Disorders
blood film: what is poikilocytosis
varying sizes of red blood cells
147
Myeloproliferative Disorders
blood film: what are blasts
immature red and white cells
148
Myeloproliferative Disorders
what is the test of choice to establish a dx
bone marrow biopsy
testing for JAK2, MPL and CALR genes can help guide management.
149
Myeloproliferative Disorders
what will bone marrow aspiration show
usually 'dry' as the bone marrow has turned to scar tissue
150
Myeloproliferative Disorders
Primary Myelofibrosis: mnx of mild disease with minimal sx
monitored but not actively treated
151
Myeloproliferative Disorders
Primary Myelofibrosis: mnx
- Allogeneic stem cell transplantation: potentially curative but carries risks
- Chemo: slow progression
- supportive: anaemia, splenomegaly, portal HTN
152
Myeloproliferative Disorders
Management of Polycythaemia Vera
1st line: Venesection (keep Hb in normal range)
aspirin: reduce thrombus formation
chemo: control the disease
153
Myeloproliferative Disorders
Management of Essential Thrombocythaemia
aspirin: reduce thrombus formation
chemo: control the disease
154
Myelodysplastic Syndrome
what is it
myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells
155
Myelodysplastic Syndrome
what does it cause
low levels of blood components that originate from the myeloid cell line:
- anaemia
- neutropenia
- thrombocytopenia
156
Myelodysplastic Syndrome
whom is it more common in
- >60 years
| - pts that have had pevious trx w/ chemo or radio
157
Myelodysplastic Syndrome
what is there an increased risk of it transforming into
acute myeloid leukaemia
158
Myelodysplastic Syndrome
presentation
may be asymptomatic and incidentally diagnosed based on FBC
anaemia: fatigue, pallor, SOB
neutropenia: frequent/severe infections
thrombocytopenia: purpura or bleeding
159
Myelodysplastic Syndrome
inx
FBC: anaemia, neutropenia, thrombocytopenia
blood film: blasts
160
Myelodysplastic Syndrome
diagnosis
bone marrow aspiration and biopsy
161
Myelodysplastic Syndrome
mnx
- watchful waiting
- blood transfusions if severely anaemic
- chemo
- stem cell transplantation
162
Lymphoma
what are they
a group of cancers that affect the lymphocytes inside the lymphatic system
these cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy)
163
Hodgkin’s Lymphoma
what is it caused by
proliferation of lymphocytes
164
Hodgkin’s Lymphoma
at what age does it peak
bimodal age distribution
| 20 and 75 years
165
Hodgkin’s Lymphoma
RFs (4)
- HIV
- epstein-barr virus
- autoimmune conditions: RA, sarcoidosis
- FH
166
Hodgkin’s Lymphoma
what is the key presenting symptom
lymphadenopathy
167
Hodgkin’s Lymphoma
describe the lymph nodes
may be enlarged in the neck, axilla, inguinal area
non-tender
rubbery
168
Hodgkin’s Lymphoma
what may some pts experience when drinking alcohol
pain in the lymph nodes
169
Hodgkin’s Lymphoma
what are the systemic symptoms
B symptoms:
- fever
- weight loss
- night sweats
170
Hodgkin’s Lymphoma
sx (excluding the B sx)
- fatigue
- itching
- cough
- SOB
- abdo pain
- recurrent infections
171
Hodgkin’s Lymphoma
what blood test is often raised
lactate dehydrogenase (LDH)
172
Hodgkin’s Lymphoma
why is raised LDH not diagnostic
because it's not specific and can be raised in other cancers and many non-cancerous diseases
173
Hodgkin’s Lymphoma
what is the key diagnostic test
lymph node biopsy
174
Hodgkin’s Lymphoma
what is the key finding from lymph node biopsy
Reed-Sternberg cells
175
Hodgkin’s Lymphoma
what are Reed- Sternberg cells
abnormally large B cells
that have multiple nuclei
that have nucleoli inside them
appearance of an owl with large eyes
176
Hodgkin’s Lymphoma
what inx can be used for diagnosing and staging
CT, MRI, PET
177
what staging is used for lymphomas
Ann Arbor
178
Lymphomas
Ann Arbor: Stage 1
confined to 1 region of lymph nodes
179
Lymphomas
Ann Arbor: Stage 2
>1 region but on the same side of the diaphragm
180
Lymphomas
Ann Arbor: Stage 3
affects lymph nodes both above and below the diaphragm
181
Lymphomas
Ann Arbor: Stage 4
widespread involvement inc non-lymphatic organs such as the lungs or liver
182
Hodgkin’s Lymphoma
key trx
chemo and radio
183
Hodgkin’s Lymphoma
what does chemo create a risk of
leukaemia and infertility
184
Hodgkin’s Lymphoma
what is the risk of radiotherapy
- creates a risk of cancer
- damage to tissues
- hypothyroidism
185
Non-Hodgkin's Lymphoma
what is Burkitt lymphoma associated with
- Epstein-Barr virus
- malaria
- HIV
186
Non-Hodgkin's Lymphoma
what does MALT lymphoma affect
the mucosa-associated lymphoid tissue, usually around the stomach
187
Non-Hodgkin's Lymphoma
what is MALT lymphoma associated with
H.pylori infection
188
Non-Hodgkin's Lymphoma
how does diffuse large B cell lymphomas often present as
a rapidly growing painless mass in pts over 65 years
189
Non-Hodgkin's Lymphoma
RFs (6)
- HIV
- Epstein-Barr Virus
- H.pylori (MALT lymphoma)
- Hep B or C infection
- exposure to pesticides
- FH
190
Non-Hodgkin's Lymphoma
which specific chemical is a RF
trichloroethylene used in several industrial processes
191
Non-Hodgkin's Lymphoma
mnx
- watchful waiting
- chemo
- monoclonal antibodies: rituximab
- radiotherapy
- stem cell transplantation
192
Leukaemia
what is it
cancer of a particular line of the stem cells in the bone marrow.
193
Leukaemia
what are the 4 main types
- Acute myeloid leukaemia
- Acute lymphoblastic leukaemia
- Chronic myeloid leukaemia
- Chronic lymphocytic leukaemia
194
Leukaemia
pathophysiology
genetic mutation in one of the precursor cells in the bone marrow
excessive production of a single type of abnormal white blood cell
suppression of the other cell lines causing underproduction of other cell types.
pancytopenia
195
Leukaemia
what are the progressive ages of the different leukaemias from 45-75
“ALL CeLLmates have CoMmon AMbitions”
<5 and >45 - ALL
>55 - CLL
>65 - CML
>75 - AML
196
Leukaemia
presentation
non-specific:
- fatigue
- fever
- failure to thrive
- pallor
- petechiae, abnormal bruising
- abnormal bleeding
- lymphadenopathy
- hepatosplenomegaly
197
Leukaemia
what is petechiae caused by
bleeding under the skin due to thrombocytopenia (low platelets)
198
Leukaemia
Ddx of petechiae, a non-blanching rash
- Leukaemia
- Meningococcal septicaemia
- Vasculitis
- Henoch-Schonlein Purpura
- ITP
- Non-accidental injury
199
Leukaemia
initial inx
FBC within 48h of suspected leukaemia
200
Leukaemia
diagnostic inx
bone marrow biopsy
201
Leukaemia
what blood test is often raised
lactate dehydrogenase (LDH)
202
Leukaemia
what may CXR show
infection or mediastinal lymphadenopathy
203
Leukaemia
what does bone marrow aspiration involve
taking a liquid sample full of cells from within the bone marrow
204
Leukaemia
what does bone marrow trephine involve
taking a solid core sample of the bone marrow
provides a better assessment of the cells and structure.
205
Leukaemia
what does a bone marrow biopsy involve
local anaesthetic and a specialist needle. Taken from iliac crest
206
Leukaemia
what is the difference between Bone marrow trephine and aspiration
Samples from bone marrow aspiration can be examined straight away
a trephine sample requires a few days of preparation.
207
Acute Lymphoblastic Leukaemia
which type of lymphocyte is usually acutely proliferated
B-lymphocytes
208
Acute Lymphoblastic Leukaemia
epidemiology
- most common cancer in children
- peaks around 2-4y
- can affect adults >45
209
Acute Lymphoblastic Leukaemia
what condition is it associated with
Down's syndrome
210
Acute Lymphoblastic Leukaemia
what would the blood film show
blast cells
211
Acute Lymphoblastic Leukaemia
which chromosome is it associated with
Philadelphia chromosome (t(9:22) translocation)
212
Chronic Lymphocytic Leukaemia
what is it
chronic proliferation of B-lymphocytes
213
Chronic Lymphocytic Leukaemia
what condition can it cause
warm autoimmune haemolytic anaemia
214
Chronic Lymphocytic Leukaemia
what can it transform into
high-grade lymphoma (Richter's transformation)
215
Chronic Lymphocytic Leukaemia
what would the blood film show
'smear' or 'smudge' cells
occurs during the process of preparing the blood film where aged or fragile white blood cells rupture and leave a smudge on the film.
216
Chronic Myeloid Leukaemia
what are the 3 typical phases
chronic phase
accelerated phase
blast phase
217
Chronic Myeloid Leukaemia
what happens in the chronic phase
can last 5y
asymptomatic
diagnosed incidentally with a raised WCC
218
Chronic Myeloid Leukaemia
what happens in the accelerated phase
occurs when the abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%)
symptomatic: anaemia, thrombocytopenia and become immunocompromised
219
Chronic Myeloid Leukaemia
what happens in the blast phase
an even higher proportion of blast cells and blood (>30%).
severe sx: pancytopenia
often fatal
220
Chronic Myeloid Leukaemia
what is the cytogenetic change that is characteristic of CML
Philadelphia chromosome (t(9:22) translocation)
221
what is the most common acute leukaemia in adults
Acute Myeloid Leukaemia
222
Acute Myeloid Leukaemia
it can be the result of a transformation from which conditions
myeloproliferative disorder such as polycythaemia ruby vera or myelofibrosis.
223
Acute Myeloid Leukaemia
what will blood film show
a high proportion of blast cells
Auer rods inside cytoplasm
224
Leukaemia
mnx
chemo + steroids primarily
radiotherapy, bone marrow transplant, surgery
225
Leukaemia
complications of chemo
- Failure
- Stunted growth and development in children
- Infections due to immunodeficiency
- Neurotoxicity
- Infertility
- Secondary malignancy
- Cardiotoxicity
- Tumour lysis syndrome
226
Leukaemia
what is tumour lysis syndrome caused by
the release of uric acid from cells that are being destroyed by chemotherapy
227
Leukaemia
how does tumour lysis syndrome cause AKI
The uric acid can form crystals in the interstitial tissue and tubules of the kidneys
228
Leukaemia
mnx of tumour lysis syndrome
- Allopurinol or rasburicase: reduced uric acid levels
| - monitor K and phosphate as these are released
229
Leukaemia
tumour lysis syndrome: what can high phosphate lead to
low calcium
230
what is the most common inherited cause of abnormal bleeding (haemophilia)
Von Willebrand Disease
231
Von Willebrand Disease
cause
- many different underlying genetic causes
- most are autosomal dominant
- absence or malfunctioning of VWF, a glycoprotein
232
Von Willebrand Disease
what are the different types and which one is most severe
Type 1,2 and 3
3 is most severe
233
Von Willebrand Disease
presentation
unusually easy, prolonged or heavy bleeding:
- bleeding gums with brushing
- epistaxis
- menorrhagia
- heavy bleeding during surgical operations
234
Von Willebrand Disease
dx
combination of:
- hx of abnormal bleeding
- FH
- bleeding assessment tools
- lab inx
235
Von Willebrand Disease
mnx
does not require day to day treatment. Mnx for response to major bleeding or trauma:
- desmopressin
- IV VWF
- Factor VIII
236
Von Willebrand Disease
how may women with VWD that suffer from heavy periods be managed
- Tranexamic acid
- Mefanamic acid
- Norethisterone
- COCP
- Mirena coil
- Hysterectomy if severe
237
Thrombocytopenia
what is it
low platelet count.
238
Thrombocytopenia
what can it be split into
problems with production or destruction
239
Thrombocytopenia
causes (problems with production)
- sepsis
- B12 or folic acid deficiency
- liver failure causes reduced thrombopoietin production in the liver
- leukaemia
- myelodysplastic syndrome
240
Thrombocytopenia
causes (problems with destruction)
- medications (valproate, methotrexate, isotretinoin, antihistamines, PPI)
- alcohol
- ITP
- TTP
- HIT
- HUS
241
Thrombocytopenia
presentation
- easy or spontaneous bruising
| - prolonged bleeding times
242
Thrombocytopenia
what is a platelet count below 10 x 10^9/L high risk for
spontaneous bleeding:
- Spontaneous intracranial haemorrhage
- GI bleeds
243
Thrombocytopenia
Ddx of abnormal or prolonged bleeding
- thrombocytopenia
- Haemophilia A and B
- VWD
- DIC (usually secondary to sepsis)
244
Thrombocytopenia
what is Immune Thrombocytopenic Purpura (ITP) aka
autoimmune thrombocytopenic purpura
idiopathic thrombocytopenic purpura
primary thrombocytopenic purpura.
245
Thrombocytopenia
what is ITP
- antibodies are created against platelets
- this causes an immune response against platelets
- resulting in destruction of platelets and a low platelet count
246
Thrombocytopenia
mnx options of ITP
- prednisolone
- IV IG
- Rituximab
- splenectomy
247
Thrombocytopenia
safety net advice for pts with ITP
seek help if signs of bleeding:
- persistent headaches
- melaena
- control BP
- suppress menstrual periods
248
Thrombocytopenia
what is Thrombotic Thrombocytopenic Purpura (TTP)
tiny blood clots develop throughout the small vessels (microangiopathy)
using up platelets and causing thrombocytopenia, bleeding under the skin and other systemic issues
249
Thrombocytopenia
why do blood clots develop in TTP
problem with the protein, ADAMTS13
250
Thrombocytopenia
TTP: what does the protein, ADAMTS13 normally do
- inactivates vWF
- reduced platelet adhesion to vessel walls
- reduces clot formation
251
Thrombocytopenia
TTP: why is there haemolytic anaemia
The blood clots in the small vessels break up red blood cells
252
Thrombocytopenia
TTP: what can deficiency in ADAMS13 be due to
- inherited genetic mutation
| - autoimmune: antibodies are created against the protein
253
Thrombocytopenia
TTP: trx
- plasma exchange
- steroids
- rituxumab
254
Thrombocytopenia
what is Heparin induced thrombocytopenia (HIT)
the development of antibodies against platelets in response to exposure to heparin
255
Thrombocytopenia
HIT: which protein on the platelets do the heparin induced antibodies specifically target
platelet factor 4 (PF4)
they become anti-PF4/heparin antibodies.
256
Thrombocytopenia
heparin with low platelets forms unexpected blood clots. What could this be
HIT
257
Thrombocytopenia
HIT: dx
testing for the HIT antibodies in the patients blood
258
Thrombocytopenia
HIT: mnx
- stop heparin
| - use alternative anticoagulant
259
Haemophilia
what is Haemophilia A caused by
a deficiency in factor VIII
260
Haemophilia
what is Haemophilia B caused by
caused by a deficiency in factor IX.
261
Haemophilia
what is Haemophilia B aka
Christmas Disease
262
Haemophilia
what is the mode of inheritance for A and B
X linked recessive
263
Haemophilia
who does it usually affect and why
males because men only require one abnormal copy as they only have one X chromosome.
Women require abnormal copies on both their X chromosomes
264
Haemophilia
presentation
most cases present in neonates or early childhood
- spontaneous haemorrhage
- intracranial haemorrhage, haematomas and cord bleeding
- abnormal bleeding
265
Haemophilia
how may severe haemophilia present
Spontaneous bleeding into joints (haemoathrosis) and muscles
266
Haemophilia
dx
- bleeding scores
- coagulation factor assays
- genetic testing
267
Haemophilia
mnx
replace clotting factor 8 or 9 by IV infusion
prophylactically or in response to bleeding
268
Haemophilia
complication of factor 8 or 9 IV infusion mnx
formation of antibodies against the clotting factor resulting in the treatment becoming ineffective
269
Haemophilia
mnx of acute episodes of bleeding or prevention of excessive bleeding during surgical procedure
- Infusions of the affected factor (VIII or IX)
- Desmopressin to stimulate the release of von Willebrand Factor
- Antifibrinolytics such as tranexamic acid
270
Anaemia
causes of microcytic anaemia
TAILS
- Thalassaemia
- Anaemia of chronic disease
- Iron deficiency anaemia
- Lead poisoning
- Sideroblastic anaemia
271
Anaemia
causes of normocytic anaemia
3As and 2Hs
- Acute blood loss
- Anaemia of chronic disease
- Aplastic anaemia
- Haemolytic anaemia
- Hypothyroidism
272
Anaemia
types of macrocytic anaemia
megaloblastic or normoblastic
273
Anaemia
what is megaloblastic anaemia the result of
vitamin deficiency causes
impaired DNA synthesis preventing the cell from dividing normally
Rather than dividing it keeps growing into a larger, abnormal cell
274
Anaemia
causes of megaloblastic anaemia
- B12 deficiency
| - folate deficiency
275
Anaemia
causes of normoblastic macrocytic anaemia
- alcohol
- hypothyroidism
- reticulocytosis (usually from haemolytic anaemia or blood loss
- liver disease
- drugs e.g. azathioprine
276
Anaemia
generic sx
- Tiredness
- SOB
- Headaches
- Dizziness
- Palpitations
- Worsening of other conditions (angina, HF or peripheral vascular disease)
277
Anaemia
sx specific to iron deficiency anaemia
- Pica
| - hair loss
278
Anaemia
what is pica
dietary cravings for abnormal things such as dirt
279
Anaemia
generic signs of anaemia
- pale skin
- conjunctival pallor
- tachycardia
- raised resp rate
280
Anaemia
specific signs of iron deficiency
- Koilonychia
- Angular chelitis
- Atrophic glossitis
- brittle hair and nails
281
Anaemia
what is Koilonychia
spoon shaped nails
282
Anaemia
what is atrophic glossitis
a smooth tongue due to atrophy of the papillae
283
Anaemia
what is a sign in haemolytic anaemia
jaundice
284
Anaemia
what sign may there be in thalassaemia
bone deformities
285
Anaemia
what can indicate CKD
Oedema, hypertension and excoriations on the skin
286
Anaemia
initial inx
- Haemoglobin
- MCV
- B12
- Folate
- Ferritin
- Blood film
287
Anaemia
further inx for a GI cause of unexplained iron deficiency anaemia
urgent cancer referral for suspected GI cancer
OGD
288
Anaemia
further inx if the cause is unclear
bone marrow biopsy
289
Iron Deficiency Anaemia
where is iron mainly absorbed
in the duodenum + jejunum
290
Iron Deficiency Anaemia
what is required to keep the iron in the soluble form
ferrous (Fe2+)
acid from the stomach
291
Iron Deficiency Anaemia
why can PPIs interfere with iron absorption
When the acid drops it changes to the insoluble ferric (Fe3+) form
292
Iron Deficiency Anaemia
how can coeliac disease or Crohn's disease cause inadequate iron absorption
they causes inflammation of the duodenum or jejunum which is where iron is mainly absorbed
293
Iron Deficiency Anaemia
causes
- blood loss (most common in adults)
- dietary insufficiency
- poor iron absorption
- increased requirements in pregnancy
294
Iron Deficiency Anaemia
how does iron travel around the blood
as ferric ions (Fe3+) bound to a carrier protein called transferrin
295
Iron Deficiency Anaemia
what is Total iron binding capacity (TIBC)
the total space on the transferrin molecules for the iron to bind
thus directly related to the amount of transferrin in the blood
296
Iron Deficiency Anaemia
what is transferrin saturation
the proportion of the transferrin molecules that are bound to iron
= Serum Iron / Total Iron Binding Capacity
297
Iron Deficiency Anaemia
what is ferritin
the form that iron takes when it is deposited and stored in cells
298
Iron Deficiency Anaemia
when is extra ferritin released from cells
in inflammation e.g. infection or cancer
299
Iron Deficiency Anaemia
what does low ferritin in the blood suggest
iron deficiency
300
Iron Deficiency Anaemia
what does high ferritin in the blood suggest
difficult to interpret but likely to be related to inflammation rather than iron overload
301
Iron Deficiency Anaemia
can a patient with a normal ferritin still have iron deficiency anaemia
yes
302
Iron Deficiency Anaemia
why is serum iron on its own not a very useful measure
Serum iron varies significantly throughout the day with higher levels in the morning and after eating iron containing meals
303
Iron Deficiency Anaemia
what can be used as a marker for how much transferrin is in the blood
Total iron binding capacity (easier test to perform than measuring transferrin)
304
Iron Deficiency Anaemia
do TIBC and transferrin increase or decrease in iron deficiency
increase
305
Iron Deficiency Anaemia
do TIBC and transferrin increase or decrease in iron overload
decrease
306
Iron Deficiency Anaemia
what gives a good indication of the total iron in the body
transferrin saturation
307
Iron Deficiency Anaemia
what 2 things can increase the values of all serum ferritin, iron, TIBC and transferrin saturation
- Supplementation with iron
| - Acute liver damage (lots of iron is stored in the liver)
308
Iron Deficiency Anaemia
inx in an adult without a clear underlying cause
OGD and colonoscopy
309
Iron Deficiency Anaemia
mnx from least invasive to most invasive
1. PO iron e.g. ferrous sulfate 200mg TDS
2. iron infusion e.g. cosmofer
3. blood transfusion
310
Iron Deficiency Anaemia
SEs of ferrous sulfate
- constipation
- black coloured stool
unsuitable where malabsorption is the cause of the anaemia
311
Iron Deficiency Anaemia
SEs of iron infusion
- anaphylaxis
avoid during sepsis as iron 'feeds' bacteria
312
Thalassaemia
what does normal Hb consist of
2 alpha and 2 beta-globin chains.
313
Thalassaemia
what does defect in alpha-globin chains lead to
alpha thalassaemia
314
Thalassaemia
what does defects in beta-globin chains lead to
beta thalassaemia
315
Thalassaemia
mode of inheritance
autosomal recessive
316
Thalassaemia
why is there splenomegaly
RBCs are more fragile and break down more easily
the spleen collects all the destroyed RBCs and swells
317
Thalassaemia
why do pts have a pronounced forehead and malar eminences (cheekbones).
The bone marrow expands to produce extra RBCs to compensate for the chronic anaemia
which causes a susceptibility to fractures and prominent features
318
Thalassaemia
potential signs + sx
- Microcytic anaemia
- Fatigue
- Pallor
- Jaundice
- Gallstones
- Splenomegaly
- Poor growth and development
- Pronounced forehead and malar eminences
319
Thalassaemia
dx
- FBC: microcytic anaemia
- Haemoglobin electrophoresis: globin abnormalities
- DNA testing: genetic abnormality
320
Thalassaemia
who are offered a screening test for thalassaemia
pregnant women at booking
321
Thalassaemia
why does iron overload occur
- faulty creation of RBCs
- recurrent transfusions
- increased absorption of iron in response to anaemia
322
Thalassaemia
mnx of iron overload
- monitoring serum ferritin
- limiting transfusions
- iron chelation
323
Thalassaemia
effects of iron overload
similar to haemochromatosis:
- fatigue
- liver cirrhosis
- infertility + impotence
- HF
- arthritis
- diabetes
- osteoporosis + joint pain
324
Thalassaemia
what is alpha-thalassaemia and what Ch is affected
defects in alpha-globin chains.
The gene coding for this protein is on Ch16.
325
Thalassaemia
mnx of alpha-thalassaemia
- monitor FBC
- monitor for complications
- blood transfusions
- splenectomy may be performed
- bone marrow transplant can be curative
326
Thalassaemia
what is beta-thalassaemia and what Ch is affected
defects in beta-globin chains.
The gene coding for this protein is on Ch11
327
Thalassaemia
the gene defects in beta-thalassemia can either consist of....
abnormal copies that retain some function or deletion genes where there is no function in the beta-globin protein at all
328
Thalassaemia
what are the 3 types of beta-thalassaemia
- Thalassaemia minor
- Thalassaemia intermedia
- Thalassaemia major
329
Thalassaemia
what is beta thalassaemia minor
carriers: 1 abnormal + 1 normal gene
330
Thalassaemia
sx of beta thalassaemia minor
mild microcytic anaemia
331
Thalassaemia
mnx of beta thalassaemia minor
require monitoring and no active treatment.
332
Thalassaemia
what is beta thalassaemia intermedia
two abnormal copies of the beta-globin gene
can either be 2 defective genes
or 1 defective gene and 1 deletion gene
333
Thalassaemia
sx of beta thalassaemia intermedia
- more significant microcytic anaemia
- monitoring
- occasional blood transfusions
- If they need more transfusions they may require iron chelation to prevent iron overload.
334
Thalassaemia
what is beta thalassaemia major
homozygous for the deletion genes. They have no functioning beta-globin genes at all.
335
Thalassaemia
sx and signs of beta thalassaemia major
- severe microcytic anaemia
- splenomegaly
- bone deformities
- failure to thrive in early childhood
336
Thalassaemia
mnx of beta thalassaemia major
- regular transfusions
- iron chelation
- splenectomy
- Bone marrow transplant can potentially be curative.
337
Sickle Cell Anaemia
at around 6w of age, HbF is replaced with what
HbA
338
Sickle Cell Anaemia
what abnormal variant of Hb do pts have
HbS which causes RBCs to be an abnormal sickle shape
339
Sickle Cell Anaemia
mode of inheritance
autosomal recessive
340
Sickle Cell Anaemia
what Ch is affected
there is an abnormal gene for beta-globin on Ch11
341
Sickle Cell Anaemia
what is sickle-cell trait
1 copy of the abnormal gene for beta-globin on Ch11
usually asymptomatic
342
Sickle Cell Anaemia
what is sickle-cell disease
2 abnormal copies of the abnormal gene for beta-globin on Ch11
343
Sickle Cell Anaemia
who is sickle cell disease more common in
pts from areas traditionally affected by malaria such as Africa, India, the Middle East and the Caribbean
344
Sickle Cell Anaemia
why is it more common in pts from areas affected by malaria
having sickle cell trait reduces the severity of malaria this there is a selective advantage
345
Sickle Cell Anaemia
who is offered a sickle cell gene test
- pregnant women at risk of being carriers
| - newborn screening heel prick test at 5d of age
346
Sickle Cell Anaemia
complications
- anaemia
- increased risk of infection
- stroke
- avascular necrosis in large joints
- pulmonary hypertension
- priapism
- CKD
- sickle cell crises
- acute chest syndrome
347
Sickle Cell Anaemia
general mnx
- avoid dehydration + other triggers of crisis
- ensure vaccines are up to date
- penicillin V prophylaxis
- hydroxycarbamide
- blood transfusion for severe anaemia
- bone marrow transplant can be curative
348
Sickle Cell Anaemia
general mnx: why is hydroxycarbamide given
to stimulate production HbF.
it does not lead to sickling of RBCs.
This has a protective effect against sickle cell crises and acute chest syndrome.
349
Sickle Cell Anaemia
what is sickle cell crises
an umbrella term for a spectrum of acute crises related to the condition
350
Sickle Cell Anaemia
what can sickle cell crises be triggered by
- infection
- dehydration
- cold or significant life events
351
Sickle Cell Anaemia
mnx of sickle cell crises
supportive:
- low threshold for admission to hospital
- treat any infection
- keep warm
- keep well hydrated
- paracetamol + ibuprofen
- penile aspiration in priapism
352
Sickle Cell Anaemia
what is vaso-occlusive crisis (aka painful crisis)
sickle shaped blood cells clogging capillaries causing distal ischaemia
353
Sickle Cell Anaemia
signs + sx of vaso-occlusive crisis (aka painful crisis)
- dehydration
- raised haematocrit
- pain
- fever
- priapism (emergency)
354
Sickle Cell Anaemia
what is splenic sequestration crisis
RBCs blocking blood flow within the spleen
355
Sickle Cell Anaemia
signs + sx of splenic sequestration crisis
- enlarged + painful spleen
- severe anaemia
- circulatory collapse (hypovolaemic shock)
356
Sickle Cell Anaemia
mnx of splenic sequestration crisis
blood transfusions and fluid resuscitation to treat anaemia and shock
357
Sickle Cell Anaemia
what prevents splenic sequestration crisis
splenectomy
358
Sickle Cell Anaemia
what is aplastic crisis
a temporary loss of the creation of new blood cells
359
Sickle Cell Anaemia
what is aplastic crisis commonly triggered by
parvovirus B19
360
Sickle Cell Anaemia
sign of aplastic crisis
significant anaemia
361
Sickle Cell Anaemia
mnx of aplastic crisis
supportive
blood transfusions if necessary
usually resolves spontaneously within a week
362
Sickle Cell Anaemia
a diagnosis of acute chest syndrome requires___ (2)
1. fever or resp sx
| 2. with new infiltrates seen on CXR
363
Sickle Cell Anaemia
what can acute chest syndrome be due to
infection (pneumonia or bronchiolitis) or
non-infective causes (pulmonary vaso-occlusion or fat emboli)
364
Sickle Cell Anaemia
mnx fo acute chest syndrome
- abx or antivirals for infections
- blood transfusions for anaemia
- incentive spirometry (machine that encourages effective + deep breathing)
- artificial ventilation
365
VTE
what is Budd-Chiari syndrome
where a blood clot (thrombosis) develops in the hepatic vein, blocking the outflow of blood
it causes acute hepatitis
366
VTE
how does Budd-Chiari syndrome present
1. abdo pain
2. hepatomegaly
3. ascites
367
VTE
mnx of Budd-Chiari Syndrome
- anticoagulation (heparin or warfarin)
- investigating for the underlying cause of hyper-coagulation
- treating hepatitis
368
why may Rasburicase (recombinant xanithine oxidase) be given before starting chemo
to protect against urate build up from tumour lysis syndrome
369
hypochromic, microcytic anaemia but high serum ferritin and iron levels. What is it
sideroblastic anaemia
370
Positive green birefringence of rectal tissue with Congo red staining
what is it
amyloidosis
371
what are roleaux
stacks of aggregated red blood cells
372
when does roleaux occur
when the plasma protein concentration is high, such as in multiple myeloma
373
"white out" on a chest x-ray following a blood transfusion. What is it
Transfusion-related acute lung injury (TRALI)
374
what are burr cells
red blood cells with small, regularly distributed projections across the cell surface.
375
causes of burr cells
- liver disease
- vitamin E deficiency
- end-stage renal disease
- pyruvate kinase deficiency
376
what is non-haemolytic febrile transfusion reaction
occurs around 30-60 mins after transfusion has begun and causes fever and shivers
377
why is there splenomegaly and thrombocytopenia in oesophageal varices
portal hypertension results in splenic enlargement and hyperfunction, and thus platelet sequestration
378
what is the most common inherited thrombophilia
factor V Leiden
379
what is factor V Leiden caused by
mutation in clotting factor V, which becomes resistant to inactivation by protein C
380
features of factor V leiden
Homozygosity increases the risk of VTE 50-fold and heterozygosity increases the risk 5-fold
381
woman has essential thrombocytopenia but is asthmatic so can't take aspirin as trx. What trx is next
Hydroxyurea: suppresses the bone production of platelets in the bone marrow which should reduce her risk of VTE
382
what would FBC show in normoblastic macrocytic anaemia
low platelets because of liver disease in alcoholism?
