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haematology Flashcards

Haematological diseases and their risk factors, pathology, clinical signs, diagnosis, and treatment. (145 cards)

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1
Q

causes of microcytic anaemia

A

iron deficiency
chronic disease
thalassaemia
vitamin B6 deficiency

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2
Q

how many menstruating women get iron deficiency anaemia?

A

15%

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3
Q

microcytic anaemia blood film

A

small erythrocytes

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4
Q

pathology of microcytic anaemia

A

low MCV
blood loss
malabsorption
hookworm

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5
Q

clinical sign of microcytic anaemia

A

koilonychia (spoon nails)

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6
Q

diagnosis of microcytic anaemia

A

blood film - microcytic
ferritin test
gastroscopy

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7
Q

treatment of microcytic anaemia

A

oral iron/ferrous sulfate

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8
Q

normal Hb range for males

A

131 - 166 g/L

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9
Q

normal Hb range for females

A

110 - 147 g/L

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10
Q

complications of anaemia

A 
myocardial fatty change
fatty change in liver
angina
claudication 
skin and nail atrophy
CNS cell death
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11
Q

normal MCV

A

80-100 fl

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12
Q

how many men are affected by normocytic anaemia?

A

44% of men over 85

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13
Q

causes of normocytic anaemia

A

acute blood loss
chronic disease
combined haematinic deficiency

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14
Q

pathology of normocytic anaemia

A 
normal MCV
acute blood loss
anaemia secondary to chronic bone disease
bone marrow failure
renal failure
hypothyroidism 
haemolysis
pregnancy
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15
Q

diagnosis of normocytic anaemia

A

normal MCV but decreased haemoglobin and haematocrit

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16
Q

causes of macrocytic anaemia

A

B12/folate deficiency
alcohol (liver disease)
hypothyroid
haematological (rare) - genetic disease

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17
Q

diagnosis of macrocytic anaemia

A

high MCV on blood film
Schilling test
IF/coeliac antibodies

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18
Q

causes of folate deficiency

A 
poor diet
malabsorption
increased demand (e.g. pregnancy)
medications
high exposure to UV
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19
Q

clinical sign of folate deficiency

A

macrocytosis

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20
Q

symptoms of folate deficiency

A 
loss of appetite
weight loss
weakness
heart palpitations
megaloblastic anaemia
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21
Q

diagnosis of folate deficiency

A

blood film

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22
Q

treatment of folate deficiency

A

improve diet
oral vitamin supplements
treat specific cause if secondary

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23
Q

complications of folate deficiency if pregnant

A

neural tube defect in baby

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24
Q

risk factors for sickle cell anaemia

A 
family history (recessive autosomal)
African descent
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25
pathology of sickle cell anaemia
HbS polymerises when deoxygenated deformation of erythrocytes blockage of small vessels
26
signs of sickle cell anaemia
microvascular occlusion
27
symptoms of sickle cell anaemia
muscle and bone pain strokes vaso-occlusive crisis - pain triggered by cold
28
treatment of sickle cell anaemia
``` transfusion in aplastic crisis hydroxycarbamide (increase in HbF) prophylactic active artificial immunity antibiotics genetic counselling ```
29
complications of sickle cell anaemia
``` aplastic crisis chronic renal failure sequestration crisis (atrophic spleen) splenic infarction (immune-compromisation) sickle cell chest syndrome ```
30
causes of autoimmune haemolytic anaemia
broadly idiopathic | can be secondary to another disease
31
pathology of autoimmune haemolytic anaemia
antibody mediated phagocytosis of erythrocytes
32
signs of autoimmune haemolytic anaemia
breathlessness
33
diagnosis of autoimmune haemolytic anaemia
rule out other causes of anaemia evidence of haemolysis serological tests Coomb's test
34
treatment of autoimmune haemolytic anaemia
blood transfusion splenectomy immunosuppressant (e.g. rituximab) if secondary, treat primary illness
35
type of antibodies involved in autoimmune haemolytic anaemia
IgG and IgM
36
causes of thrombosis
Virchow's triad: changes in vessel surface changes in blood flow changes in blood constituents
37
why is thrombosis more likely after surgery or an MI?
blood flow is slower
38
thrombus definition
solid mass of blood constituents formed within the vascular system in life
39
venous thrombosis is most commonly due to
stasis
40
why are immobile patients more likely to get thrombosis?
muscular pressure decreases --> venous return also decreases
41
thrombophlebitis
the inflammatory reaction when a vein is thrombosed
42
phlebothrombosis
when an inflamed vein forms a thrombus
43
pathology of arterial thrombosis
usually superimposed on atheroma | generates turbulence --> loss of intimal cells --> clot formation with fibrin deposition and platelet clumping
44
propagation
thrombi grow in the direction of blood flow
45
signs of thrombosis
local pain and local inflammation
46
treatment of thrombosis
anticoagulants (aspirin, warfarin)
47
complications of arterial thrombosis
distal tissue infarction
48
complications of venous thrombosis
oedema | embolism
49
leukaemia risk factors
smoking ionising radiation Down's syndrome prior chemotherapy
50
who does acute myeloid leukaemia tend to affect?
older people who have had treatment for other cancers
51
who does ALL tend to affect?
young children
52
what does leukaemia come from?
haematopoietic stem cells
53
subtypes of leukaemia
acute lymphoblastic leukaemia chromic lymphoblastic leukaemia acute myeloid leukaemia chronic myeloid leukaemia
54
which mutation is associated with chronic myeloid leukaemia?
Philadelphia translocation
55
where does tumorigenesis occur in leukaemia?
bone marrow
56
signs of leukaemia
``` petechiae (easy bruising) enlarged spleen/liver fever pale skin low platelet count anaemia immune suppression (--> sores and opportunistic infection) neurological symptoms ```
57
acute leukaemia diagnosis
>20% of WBCs in marrow are blasts
58
what can indicate leukaemia relapse before anything else?
MRD testing
59
diagnosis of leukaemia
lymph node biopsy XR and MRI FBC bone marrow biopsy
60
treatment for acute myeloid leukaemia
chemotherapy
61
what can be used to treat some variations of leukaemia?
tyrosine kinase inhibitors
62
treatment for leukaemia
chemotherapy bone marrow transplant targeted therapy
63
differential diagnosis for acute leukaemia
post operative changes | sepsis
64
who does Hodgkin's lymphoma affect?
bimodal peak (young and old)
65
risk factors for lymphoma
HIV EBV HTLV-1 H.pylori
66
what cells are present in Hodgkin's lymphoma?
Reed-Sternberg cells
67
lymphoma
cancerous proliferation of lymphocytes predominantly in lymph nodes, but can present anywhere in the body
68
signs of lymphoma
compression syndromes
69
symptoms of lymphoma
``` fatigue lump nodal disease night sweats weight loss ```
70
diagnosis of lymphoma
``` lymph node biopsy bone marrow biopsy flow cytometry immune-phenotyping blood films PET scan blood test ```
71
first line treatment for lymphoma
watch and wait
72
treatment for Hodgkin's lymphoma stage 1-2
combination chemotherapy and radiotherapy
73
treatment for Hodgkin's lymphoma stage 3-4
long term combination chemotherapy
74
treatment for low grade non-Hodgkin's lymphoma
radiotherapy and monoclonal antibodies
75
treatment for high grade non-Hodgkin's lymphoma
combined chemotherapy and monoclonal antibodies (rituximab or zevalin)
76
complications of lymphoma treatment
infertility
77
rituximab
monoclonal antibody that is anti-CD20 (CD20 is found on B cells)
78
multiple myeloma
cancerous condition of bone marrow plasma cells
79
risk factors for multiple myeloma
MGUS protein in blood | obesity
80
pathology of multiple myeloma
cloned malignant plasma cells produce immunoglobulins high osteoclast activity light chain deposition in kidneys clonal expansion --> MGUS --> early myeloma (smouldering myeloma) --> late myeloma and end organ damage
81
symptoms of multiple myeloma
``` bone pain bleeding frequent infections tiredness malaise ```
82
signs of multiple myeloma
``` CRAB Calcium (high) Renal Anaemia Bone disease ```
83
diagnosis of multiple myeloma
Rouleaux stacks - aggregated RBCs in blood film high ESR monoclonal protein in blood and urine (Bence Jones protein) light chain deposition in kidneys pepper pot skull evidence of bone marrow plasma cells in excess of 10%
84
treatment of multiple myeloma
thalidomide dexamethasone stem cell replacement therapy radiotherapy
85
complications of multiple myeloma
``` peripheral neuropathy (may be from treatment) amyloidosis infection (main cause of death from MM) ```
86
malaria vector
female anopheles mosquito
87
Malaria falciparum causes what percentage of malaria cases?
50%
88
signs of malaria
``` flu-like symptoms haemolytic anaemia fever jaundice paroxysm (sudden changes between hot and cold) can be cerebral enlarged spleen high bilirubin low platelet count ```
89
diagnosis of malaria
antigen RDT | thick and thin blood films
90
malaria treatment
PO quinine | primaquine for hypnozoite clearance (check G6PD/pregnancy status)
91
complication of Malaria malariae
quartan fever
92
risk factors for polycythaemia
``` dehydration hypertension obesity smoking hypoxia altitude lung disease cyanotic heart disease inappropriate increased EPO secretion ```
93
primary polycythaemia
polycythaemia rubra vera
94
95% of cases of polycytheamia are associated with which mutation?
JAK2 mutation
95
pathology of polycythaemia
clonal proliferation of haematopoietic myeloid stem cells in the bone marrow which retain the ability to differentiate into erythrocytes
96
polycythaemia
too many red blood cells
97
relative polycythaemia
decrease in plasma volume | normal RBC mass
98
absolute polycythaemia
increase in RBC mass normal plasma volume can be primary of secondary
99
signs of polycythaemia
splenomegaly facial plethora thrombosis
100
symptoms of polycythaemia
``` headaches dizziness tinnitus burning sensation in fingers and toes itching ```
101
diagnosis of polycythaemia
raised red cell mass on 51Cr studies and splenomegaly in the setting of a normal PaO2 is diagnostic decreased serum EPO
102
FBC results for patient with polycythaemia
``` increased RCC increased Hb increased HCT increased PCV ± increased WBCs and platelets ```
103
treatment of polycythaemia
``` aim to keep HCT <0.45 to decrease thrombosis risk aspirin alpha-interferon venesection bone marrow suppressive drugs ```
104
risk factors for haemophilia
x-linked inheritance pattern - therefore greater risk for males 30% new mutations
105
cause of haemophilia A
factor VIII deficiency
106
cause of haemophilia B
factor IX deficiency
107
symptoms of haemophilia
bleeding into joints severe arthropathy haematomas
108
diagnosis of haemophilia
increased APTT | decreased factor assay
109
treatment of haemophilia
genetic counselling recombinant factors desmopressin avoid NSAIDs
110
acquired haemophilia
a bleeding diathesis causing big mucosal bleeds | caused by antibodies that suddenly appear and interfere with factor VIII
111
risk factors for Von Willebrand's disease
genetic - autosomal dominant or recessive
112
pathology of Von Willebrand's disease
decreased VWF
113
signs of Von Willebrand's disease
platelet disorder signs bruising epistaxis menorrhagia
114
diagnosis of Von Willebrand's disease
increased APTT increased bleeding time decreased clotting factors decreased VWF
115
treatment or Von Willebrand's disease
desmopressin | VWF concentrate given for surgery
116
risk factors for immune thrombocytopaenia
``` pregnancy female viral infection (primary) cancer (secondary) HIV (secondary) ```
117
immune thrombocytopaenia pathology
anti platelet autoantibodies coat platelets
118
who does acute immune thrombocytopaenia affect?
children, following an infection
119
who does chronic immune thrombocytopaenia affect?
women
120
signs of immune thrombocytopaenia
petechiae
121
symptoms of immune thrombocytopaenia
``` bleeding epistaxis menorrhagia purpura bruising haematomas ```
122
complications of immune thrombocytopaenia
subarachnoid haemorrhage | intracerebral haemorrhage
123
diagnosis of immune thrombocytopaenia
megakaryocytic in marrow | anti platelet auto-antibodies
124
treatment of immune thrombocytopaenia
``` IV immunoglobulin (to temporarily raise platelet count) rituximab tranexamic acid inhibits breakdown of fibrin (may help with the bleeding) ```
125
risk factors for thrombotic thrombo-cytopaenic purpura (EMERGENCY)
lupus secondary often idiopathic
126
pathology of thrombotic thrombocytopenic purpura
reduction of a protease which chops VWF in half before entering circulation --> bit bits of VWF stick to platelets --> extensive microscopic clots in small vessels of the body causing low platelet count results in multi-organ damage
127
signs of thrombotic thrombocytopaenic purpura
``` fever changes in mental status thrombocytopaenia reduced renal function haemolytic anaemia hypertension ```
128
symptoms of thrombotic thrombocytopaenic purpura
``` tiredness confusion headaches seizures stroke-like episodes ```
129
which protease is deficient in thrombocytopaenic purpura?
ADAMTS13
130
diagnosis of thrombocytopaenic purpura
thrombotic microangiography | genetic tests for abnormal ADAMST13 genes
131
treatment of thrombocytopaenic purpura
plasma exchange glucocorticoids rituximab cyclophosphamide
132
complications of thrombocytopaenic purpura
sudden death (often within 24hrs)
133
thalassaemia
abnormal erythrocytes due to unequal amount of Hb chains
134
what may carriers of thalassaemia have?
slightly smaller RBCs
135
risk factors for thalassaemia
genetic
136
pathology of thalassaemia
unbalanced production of Hb chains - divided into alpha and beta thalassaemia
137
when does beta thalassaemia major present?
when the child stops making foetal Hb
138
signs of thalassaemia
splenomegaly yellow skin slow growth in children bone problems
139
symptoms of thalassaemia
tiredness | pale skin
140
diagnosis of thalassaemia
``` FBC MCV blood film HbF electrophoresis ```
141
complications of thalassaemia
blood transfusions may cause iron overload --> iron deposition in organs --> haemachromatosis e.g. myocardial siderosis from iron overload (do MRI to diagnose)
142
treatment of thalassaemia
blood transfusions splenectomy hormonal replacement marrow transplant (chance of cure)
143
pathology of neutropenia due to underproduction
marrow failure marrow infiltration marrow toxicity
144
pathology of neutropenia due to increased removal
autoimmune Felty's syndrome cyclical
145
common cause of neutropenia
chemotherapy

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