Haematology Flashcards
(222 cards)
1
Q
Acquired risks of VTE? (13)
A
- Age
- Obesity
- Previous DVT/PE
- Immobilisation
- Major surgery
- Long distance travel
- Mlaignancy
- Pregnancy
- COCP
- HRT
- Anti-phospholipid syndrome
- Polycythaemia
- Thrombocythaemia
2
Q
Factor V Leiden
- who
- what
A
caucasian
resistance to Protein C, therefore increased clotting
3
Q
Inherited risk factors of VTE? (7)
A
- Antithrombin deficiency
- Protein C deficiency
- Protein S deficiency
- Factor V Leiden
- Prothrombin G20210A
- Lupus anticoagulant
- coag excess - VIII, II, fibrinogen
4
Q
DVT Prophylaxis
A
- TED stockings
2. sc LMWH
5
Q
Treatment of 1st VTE unknown cause?
A
Warfarin
6
Q
Action of Heparin?
A
potentiates antithrombin II
inactivates thrombin
inactivates factors 9, 10, 11
7
Q
Action of Warfarin?
A
inhibits reductase enzyme responsible for regenerating Vitamin K
Inhibits synthesis of factors 2, 7, 9, 10
8
Q
Warfarin side effects?
A
teratogenicity
9
Q
Action of Rivaroxiban?
A
direct inhibitor of factor Xa
10
Q
Target INR in AF?
A
2-3
11
Q
Target INR in recurrent VTE/PE?
A
2.5 - 3.5
12
Q
Target INR in mechanical heart valve?
A
3.5
13
Q
INR raised >5 no bleeding?
A
Stop Warfarin, restart when INR
14
Q
INR raised 5 - 8 and bleeding?
A
Stop Warfarin, Vit K slowly
15
Q
Anaemia in men?
A
Hb
16
Q
Anaemia in women?
A
Hb
17
Q
Symptoms of anaemia? (7)
A
- fatigue
- dyspnoea
- faintness
- palpitations
- headache
- tinnitus
- anorexia
18
Q
Leukoerythroblastic anaemia
- what
- morphological features (3)
- causes (4)
A
- anaemia of white and red cell precursors in the peripheral blood
- marrow infiltration
1. nucleated RBCs
2. myelocytes
3. poikilocytes “tear drop”
Causes
1. haematological malignancies
2. non-haematological malignancies
3. severe infection e.g. miliary TB
4. myelofibrosis
19
Q
Lab findings of haemolysis (5)
A
- anaemia
- reticulocytosis
- raised bilirubin
- raised LDH
- reduced haptoglobins
20
Q
Haemolytic anemia
- what is it?
- types (2)
A
breakdown of RBCs before their normal lifespan of 120 days
a) inherited
b) acquired 1) immune 2) non-immune
21
Q
Lab findings auto-immune haemolytic anaemia? (2)
A
- spherocytes
2. DAT +
22
Q
Types of non-immune acquire haemolytic anaemia? (2)
A
- Infection - malaria
2. Micro-angiopathic haemolytic anaemia
23
Q
Causes of microcytic anaemia? (4)
A
- IDA
- Anaemia of chronic disease
- Sideroblastic anaemia
- Thalassaemia
24
Q
Causes of normocytic anaemia? (7)
A
- acute blood loss
- anaemia of chronic disease
- bone marrow failure
- renal failure
- hypothyroidism
- haemolysis
- pregnancy
25
Causes of macrocytic anaemia? (7)
1. pregnancy
2. anti-folates
3. hypothyroidism
4. reticulocytosis
5. B12 or folate deficiency
6. cirrhosis
7. myelodysplastic syndromes
26
Causes of neutrophilia (5)
1. corticosteroids
2. neoplasm
3. inflammation
4. myeloproliferative disorders
5. bacterial infection
27
Causes of eosinophilia (4)
1. parasitic infection
2. allergic disease e.g. asthma, RA
3. neoplasm
4. drug reaction e.g. erythema multiforme
28
Signs of IDA (5)
1. Koilonychia
2. atrophic glossitis
3. anugular cheilosis
4. Plummer-Vinson syndrome
5. brittle hair & nails
29
Features of blood film in IDA? (4)
1. microcytic
2. hypochromic
3. anisocytosis
4. poikilocytosis
30
Causes of IDA? (5)
1. blood loss
2. increased utilisation e.g. pregnancy
3. decreased intake e.g. prematurity, dietary
4. decreased absorption e.g. coeliac
5. intravascualr haemolysis e.g. MAHA
31
Pathophysiology of anaemia of chronic disease
| - lab findings
- cytokine driven inhibition of RBC production
- cytokines IFNs, TNF, IL1 reduced EPO receptor, and therefore EPO synthesis
- ferritin is HIGH as iron is sequestered in macrophage to deprive invading bacteria of iron
32
Causes of anaemia of chronic disease (4)
1. chronic infection
2. vasculitis
3. RA
4. Malignancy
33
Sideroblastic anaemia
- what
- causes (4)
- diagnosis
- inability to incorporate iron in to Hb resulting in iron loading
1. myelodysplastic disorders
2. chemotherapy
3. irradiation
4. alcohol excess
- ring sideroblasts
34
Acanthocytes (3)
Abetalipoproteinaemia
liver disease
hyposplenism
35
Basophilic RBC stippling (3)
accelerated erythropoiesis
1. lead poisoning
2. liver disease
3. haemoglobinopathy
36
Burr cells (3)
1. uraemia
2. GI bleeding
3. stomach carcinoma
37
Heinz bodies
Glucose-6-phosphate dehydrogenase deficiency
38
Howell-Jolly bodies (4)
1. post-splenectomy
2. hyposplenism
3. megaloblastic anaemia
4. hereditary spherocytosis
39
Polychromasia
premature or inappropriate release from bone marrow
40
High no of reticulocytes
haemolytic anaemia
41
Low no of reticulocytes
aplastic anaemia
42
Right shift of white cells (3)
hypermature white cells
megaloblastic anaemia
uraemia
liver disease
43
Rouleaux formation (3)
paraproteinaemia
myeloma
chronic inflammation
44
Schistocytes (3)
microangiopathic anaemia
DIC
HUS
45
Spherocytes (2)
hereditary spherocytosis
| autoimmune haemolytic anaemia
46
Stomacytes (3)
hereditary stomacytosis
high alcohol intake
liver disease
47
Target cells (4)
IDA
liver disease
hyposplenism
thalassemia
48
Megaloblastic anaemia
results from inhibition of DNA synthesis during RBC production
49
Vitamin B12 Deficiency
- causes (2)
- sources
- symptoms (8)
1) dietary
2) malabsorption
- meat, dairy products
1. glossitis
2. angular chelitis
3. irritability
4. depression
5. psychosis
6. dementia
7. peripheral neuropathy
8. paraesthiasiae
50
Pernicious anaemia
- what
- test
autoimmune atrophic gastritis with lack of intrinsic factor
| Schilling test
51
Folate deficiency
- causes (4)
- sources
1. poor diet
2. pregnancy
3. malabsorption
4. alcohol
green vegetables, nuts
52
Herditary spherocytosis
- inheritance pattern
- deficiency
- susceptibilities (2)
- Blood film
- Diagnostic test
- Sign
- autosomal dominant
- spectrin deficiency
1) parvovirus B19
2) gallstones
- spherocytes
- DAT -ve
- splenomegaly
53
Hereditary elliptocytosis
- inheritance pattern
- deficiency
- outcome
- autosomal dominant
- spectrin
asymptomatic to hydrops fetalis
54
G6PD Deficiency
- who
- inheritance pattern
- blood film
- precipitants (3)
- attack
- Mediterranean
- X-linked
- Heinz bodies, bite cells
1) broad beans
2) drugs - anti-malarials, sulphonamides, aspirin
3) acute infection
- rapid anaemia and jaundice
55
Pyruvate kinase deficiency
- inheritance pattern
- clinical features (3)
- autosomal recessive
1. neonatal jaundice
2. splenomegaly
3. haemolytic anaemia
56
single base mutation in sickle cell anaemia?
GAG - GTG
| Glutamine to valine
57
age of onset of sickle cell anaemia?
3-6 months, coincides with decreasing fetal Hb
58
features of sickle cell anaemia?
```
SICKLED MP
S - stroke
I - infection, Parvovirus B19
C - crises
K - kidney necrosis, nephrotic syndrome
L - liver, gallstones
E - eyes, retinopathy
D - dactilitis, impaired growth
M - mesenteric ischaemia
P - priapism
```
59
Clinical features of B thalassemia? (3)
1. frontal bossing
2. maxillary hypertrophy
3. hairs on end skull x-ray
60
Inherited haemolytic anaemia DAT/Coombs test?
DAT -ve
61
Acquired haemolytic anaemia DAT/Coombs test?
DAT +ve
62
Paroxysmal Cold Haemoglobinuria
- what
- cause
- antibodies
autoimmune haemolysis where haemoglobin is present in the urine
virus e.g. measles, syphilis, VZV
Donath-Landsteiner antibodies
63
Paroxysmal Nocturnal Haemoglobinuria
- what
- pathophysiology
- signs (2)
- test
```
acquired haemolysis
loss of GPI markers of RBCs
1. morning haemoglobinuria
2. thrombosis
- Ham's test
```
64
Microangiopathic Haemolytic Anaemia
- what
- blood film
- causes (4)
```
mechanical destruction of RBCs
schistocytes
1. HUS
2. TTP
3. DIC
4. pre-eclampsia
```
65
Thrombotic Thrombocytopenic Purpura
- what
- pentad of symptoms
- autoimmune platelet activation
1. MAHA
2. fever
3. renal impairment
4. neuro abnormalities
5. thrombocytopenia
66
Haemolytic Uraemic Syndrome
- cause
- pathophysiology
- who
- symptoms (2)
- E.Coli
- toxin damages endothelial cells
- children & elderly
1. renal impairment
2. diarrhoea
67
Normal platelet count
150 - 400
68
Intrinsic pathway of coagulation cascade?
```
Factor TwelvE
EleveN
NinE
EighT
Ten
```
69
Extrinsic pathway of coagulation cascade?
Enodthelial damage
Tissue Factor activation
Factor Seven
Ten
70
Common pathway of coagulation cascade?
Ten
| Two
71
Prothrombin is?
Factor II
72
Thrombin is?
Factor IIa
73
Antithrombin
inhibits clotting factors
74
Phases of clotting cascade? (4)
1. initiation
2. activation
3. propagation and thrombin burst
4. stable clot
75
Inhibitors of clotting cascade? (4)
1. Tissue factor pathway inhibitor
2. Protein C
3. Protein S
4. Antithrombin III
76
Monitoring of intrinsic pathway?
| - which drug?
Activated Partial Thromboplastin Time (APTT)
| - Heparin
77
Monitoring of extrinsic pathway?
| - which drug?
```
Prothrombin Time (PT)
- Warfarin
```
78
Monitoring of common pathway?
Thrombin Time (TT)
79
Fibrinolysis
- initiated by?
- outcome
- fibrin clot formation
| - plasmin lyses fibrin clot in to degradation products
80
what is TPA?
tissue plasminogen activator
| "clot-buster"
81
Types of bleeding disorders (4)
1. vascular defects
2. platelet defects
3. coagulation disorders
4. mixed
82
Symptoms of vascular and platelet disorders (3)
1. superficial bleeding into skin
2. bleeding of mucosal membranes
3. immediate bleeding after injury
83
symptoms of coagulation disorders (3)
1. prolonged bleeding
2. bleeding into deep tissues, muscles, joints
3. delayed, severe bleeding after injury
84
Osler-Weber- Rendu Syndrome
- inheritance pattern
- signs (3)
Hereditary Haemorrhagic Telangiectasia
- autosomal dominant
1. telangiectasia of mucous membranes
2. epistaxis
3. arteriovenous malformations
85
Ehlers Danlos
- what
- inheritance pattern
- signs
- connective tissue disorder
- autosomal dominant
1. hyper mobility
2. fragile skin
3. arteriovenous malformations
4. valvular heart disease
86
Acquired vascular defects (4)
1. senile purpura
2. infection - measles, meningococcal
3. steroids
4. scurvy - perifollicular haemorrhages
87
Causes of thrombocytopenia?
1. decreased production - Bone marrow failure
| 2. increased destruction - AITP, drugs
88
Auto Immune Thrombocytopenia Purpura
- what
- sign and symptoms (2)
antibodies against platelet antigens so platelets are destroyed by splenic macrophages
1. petichiae
2. menorrhagia
89
von Willebrand Disease
- what
- inheritance pattern
- symptoms (5)
- lab findings (3)
hereditary coagulation disorder - low platelet function
autosomal dominant
1. easy bruising
2. muscosal membrane bleeding
3. epistaxis
4. menorrhagia
5. prolonged bleeding
1. increased APTT
2. increased bleeding time
3. low factor VIII
90
Haemophilia A
- what
- which factor?
- inheritance pattern
- symptoms
- who
```
congenital condition of failure to generate fibrin to stabilise a platelet plug
Factor VIII deficiency
X-linked recessive
prolonged bleeding after surgery/trauma
M>F
```
91
Haemophilia B
- what
- which factor?
- inheritance pattern
congenital condition of failure to generate fibrin to stabilise a platelet plug
Christmas Disease
Factor IX deficiency
X-linked recessive
92
Acquired causes of defects in haemostasis (3)
1. liver failure
2. DIC
3. Vitamin K deficiency
93
Causes of DIC (5)
1. malignancy
2. sepsis
3. toxins
4. obstetric complications
5. trauma
94
Pathophysiology of DIC
TF is released in the circulation causing binding with coagulation factors and intitation of the extrinsic pathway triggering small clots to form within blood vessels throughout the body -> multiorgan failure
consumption and depletion of coagulation factors and platelets
95
Clinical picture of bone marrow failure (3)
1. anaemia
2. thrombocytopenia
3. neutropenia
96
Signs of bone marrow failure (7)
```
hepatomegaly
splenomegaly
lymphadenopathy
bone pain
CNS involvement
easy bruising
gum hypertrophy
```
97
Acute Myeloid Leukaemia
- what
- who
- common gene mutation
neoplastic process of bone marrow and blood cells causing a block in maturation of white blood cells therefore an increase in BLAST cells
adulthood
RUNX1
98
Acute Promyelocytic Leukaemia
| - association
subtype of acute leukaemia
| DIC
99
Blood film finding associated with AML?
Auer Rods
100
Cytochemistry staisn associated with AML? (2)
1. myeloperoxidase
| 2. Sudan black stain
101
Monocytic AML
| - associations (3)
1. petichiae
2. gum infiltration
3. hypokalaemia
102
Cause of retinal haemorrhage in AML?
if WBC is very high causes hyperviscosity
103
Acute Lymphoblastic Leukaemia
- who
- types
- prognosis
```
male, caucasian CHILDREN
commonest cancer in children
T-cell lineage 15%
B-cell lineage 85%
85% of children cured
```
104
Clinical Features of ALL? (9)
ALO BOFFIN
A-anaemia
L-lymphadenopathy (thymic enlargement)
O - organomegaly (splenomegaly, hepatomegaly
```
B-bleeding (easy bruising, petichiae)
-bony pain
-brain (CNS involvement)
O-orchidomegaly
F-fever
F-fatigue
I-infection
N-neutropenia
```
105
Investigations in ALL?
Male Caucasian Children Love Big Rocket Blasts
```
L-LP look for blasts
B-biopsy look for blasts
-FBC lymphocytosis
R-X-ray, CT - mediastinal & abdominal lymph nodes
B-blast cells
```
106
Subtype of ALL with good prognosis?
hyperdipoloidy
107
Miantenance of chemotherapy in
a) boys?
b) girls?
boys - 3 years
| girls - 2 years
108
Increased risk of AML/ALL?
Down's syndrome
109
Neonates in comparison to adults...
1. A higher Hb
2. A lower WBC
3. Smaller red blood cells
4. The same percentage of haemoglobin F
have a higher Hb
110
Complications of Sickle cell anaemia? (5)
```
Hand-foot syndrome
Hyposplenism
Red cell aplasia
Splenic sequestration
Stroke
```
111
Complications of sickle cell anaemia more common in adults than children?
Hyposlenism due to previous splenic sequestration or splenectomy
112
```
Siblings with sickle cell anaemia present simultaneously with anaemia and a low reticulocyte count?
Splenic sequestration
2. Parvovirus B19 infection
3. Folic acid deficiency
4. Haemolytic crisis
5. Vitamin B12 deficiency
```
Parvovirus B19 infection
113
A 6-year-old Afro-Caribbean boy presents with chest and abdominal pain; Hb is 63 g/l, MCV 85 fl and blood film shows sickle cells—likely diagnosis?
1. Sickle cell trait
2. Sickle cell anaemia
3. Sickle cell/beta thalassaemia
Sickle Cell Anaemia
114
What additional therapy to blood transfusion will a child with B thalassemia require?
Iron chelation - desferrioxamine to stop iron overload
115
Clinical signs of haemolysis? (4)
1. jaundice
2. splenomegaly
3. increased unconjugated bilirubin
4. increased reticulocytes
116
Chronic Myeloid Leukaemia
- what
- who
- presentation
uncontrolled proliferation of myeloid cells
middle aged 40-60years M>F
picked up on routine bloods
117
CML
| Signs & symptoms (9)
```
fatigue
weight loss
fever
sweats
gout
bruising
hepatomegaly
splenomegaly
anaemia
```
118
Chromosomal translocation present in CML
Philadelphia chromosome translocation 9;22 creating BCR-ABL fusion gene with tyrosine kinase activity
119
Treatment of CML in chronic phase?
Imantinib - tyrosine kinase inhibitor
Hydroxycarbamide
a-interferon
120
Chronic Lymphblastic Leukaemia
- what
- who
- where
lymphoproliferative disease
elderly population M>F
bone marrow
121
Small lymphocytic leukaemia (SLL)
- what
- where
lymphoproliferative disease
| lymph nodes
122
Clinical features of CLL (4)
| Association with CLL (2)
1. painless lymphadenopathy
2. anaemia
3. thrombocytopenia
4. weight loss, low grade fever, night sweats
Autoimmunity AIHA, ITP
123
Evan's Syndrome
autoimmunity associated with CLL
124
Good prognostic feature in CLL?
hypermutated Ig gene
125
Bad prognostic feature in CLL?
raised LDH
126
Staging of CLL?
Binet Staging
A - High WBC, 3 LNs
C - anaemia, thrombocytopenia
127
Hodgkin's Lymphoma
- percentage of lymphoma
- who
- association
- clinical presentation
- B constitutional symptoms (5)
- 20%
- bimodal age incidence 1. 20-29years 2. >60years
- EBV
- symmetrical painless lymphadenopathy
- pain in LNs after alcohol
1. weight loss
2. night sweats
3. pruritis
4. fever
5. fatigue
128
Blood film finding in Hodgkins Lymphoma
Reed-Sternberg binucleate cells
129
Staging of Hodgkins lymphoma
Stage 1 - one LN region
Stage 2 - >2 LN regions SAME sides of diaphragm
Stage 3 - >2 LN regions OPPOSITE sides of diaphragm
Stage 4 - extranodal sites
A - no constitutional symptoms
B - constitutional symptoms
130
Chemotherapy in Hodgkins lymphoma
```
AVBD
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
```
131
Non-Hodgkins lymphoma
- what
- percentage of lymphomas
- all lymphomas other than Hodgkins
| - 80%
132
Burkitts Lymphoma
- cell type
- 3 types
- mutation
- high or low grade?
- histology
- chemotherapy
B cell
1. Endemic
2. Sporadic
3. Immuno-deficiency
c-myc oncogene overexpression
high grade
starry-sky appearance
rituximab
133
Endemic Burkitts Lymphoma
- who
- where
- association
- presentation
children/teenagers
equatorial Africa
EBV
jaw involvement
134
Sporadic Burkitts Lymphoma
- where
- association
- presentation
outside of Africa
EBV
no jaw involvement
135
Immunodeficiency Burkitts Lymphoma
| - who (2)
1. HIV patients
| 2. post-transplant patients
136
Follicular Lymphoma
- cell type
- histology
- characteristics
- B cell
- nodular appearance
- indolent and incurable
137
MALT/Marginal Zone Lymphoma
- who
- associations (2)
middle-aged
1. H.pylori
2. Sjogren's syndrome
138
Richter's syndrome
CLL transforms into a large B cell lymphoma with poor prognosis
139
Diffuse Large B Cell Lymphoma
- who
- histology
middle-aged and elderly
| large sheets of lymphoid cells
140
Mantle Cell lymphoma
- who
- nature of cancer
- histology
middle-aged men
aggressive
angular nuclei
141
Anaplastic large cell lymphoma
- cell type
- who
- histology
T cell
children and young adults
large epithelioid lymphocytes
142
Coeliac disease is associated with which lymphoma?
Enteropathy-associated T cell lymphoma
143
Multiple Myeloma
- what
- who
- production of?
neoplasia of plasma cells
middle aged - elderly
high incidence in Afro-Caribbean
paraproteins IgG
144
Signs and symptoms in Multiple Myeloma? (CRAB)
C - hyperCalcemia
R - Renal impairment - amyloidosis, nephrotic syndrome
A - Anaemia
B - Bones - osteolytic lesions, fractures
145
Clinical findings in Multiple Myeloma? (5)
1. dense, narrow single monoclonal band of serum electrophoresis
2. rouleaux on blood film
3. Bence Jones proteins in urine
4. High ESR
5. >10% plasma cells in bone marrow
146
MGUS Monoclonal Gammaglobinopathy of Unknown Significance
147
Smoldering Myeloma (2)
plasma cells >10% but no CRAB symptoms
148
Waldenstrom's Macroglobinaemia
- what
- who
- which antibody?
- symptoms (3)
lymphoproliferative low grade non-hodgkins lymphoma
elderly men
IgM
1. weight loss
2. fatigue
3. hyperviscosity syndrome (spontaneous bleeding, retinopathy, neuropathy)
149
Systemic Amyloidosis
- which antibody?
- investigation
- presentation (5)
Ig light chain paraprotein deposition in tissues
Congo Red stain to apple green birefringence
1. macroglossia
2. carpal tunnel syndrome
3. peripheral neuropathy
4. HF
5. renal failure
150
What are myelodysplastic syndromes?
Heterogenous group of progressive disorders of
1) ineffective proliferation
2) ineffective differentiation
of abnormally maturing myeloid stem cells
151
Myelodysplastic syndrome
- who
- risk
- how are they diagnosed?
- blasts
- elderly
- transformation into AML
- incidental finding of routine bloods
152
Clinical features of myelodysplastic disorder (4)
1. BM failure - infection, fatigue, easy bleeding
2. Pseudo-Pelger-huet anomaly -hyposegmented neutrophils
3. sideroblasts
4. micromegakarycytes
153
Pseudo-Pelger-huet anomaly is associated with?
Myelodysplastic syndrome
154
Classification of Myelodysplastic Syndrome (5)
1. Refractory Anaemia with/without sideroblasts
2. Refractory Cytopenia with multilineage dysplasia
3. Refractory Anaemia with Excess Blasts I (5-10%) II (11-20%)
4. MDS with 5q deletion
5. Unclassified MDS - with fibrosis, or childhoos MDS
155
Death from myelodysplastic syndrome?
1/3 bleeding
1/3 infection
1/3 leukaemia
156
Bone Marrow Failure
damage to or suppression of haematopoietic pluripotent stem cells
157
Primary Bone Marrow Failure (2)
a) inherited
| b) idiopathic
158
Secondary Bone Marrow Failure (5)
1. malignancies
2. radiation
3. drugs - chemo, Abx, thiazides
4. viruses
5. auto-immune - SLE
159
Inherited causes of Aplastic Anaemia (4)
1. Fanconi Anaemia
2. Dyskeratosis Congenita
3. Schwachman-Diamond Syndrome
4. Diamond-Blackfan Syndrome
160
Fanconi Anaemia
- who
- inheritance pattern
- cytopenia?
- clinical features (5)
- children aged 5-10yrs
- autosomal recessive
- PANCYTOPENIA
1. short stature
2. skin pigmentation - cafe-au-lait spots
3. microophthalmia
4. abnormal thumbs
5. renal malformations
161
Dyskeratosis Congenita
- inheritance pattern
- characterised by (3)
- genetic mutation
```
X-linked
1. Bone Marrow Failure
2. Cancer predisposition
3. Somatic abnormalities
telomere shortening
```
162
Triad of Dyskeratosis Congenita
1. nail dystrophy
2. leukoplakia
3. skin pigmentation
163
Diamond-Blackfan Syndrome
- who
- cytopenia?
neonates - 1 year
| pure red cell aplasia
164
Schwachman-Diamond Syndrome
- inheritance pattern
- cytopenia
- risk association
- clinical features (5)
```
autosomal recessive
neutropenia
AML
1. short stature
2. pancreatic dysfuncion
3. hepatic impairment
4. endocrine dysfunction
5. skeletal abnormalities
```
165
Myeloproliferative disorder
clonal prolieration of one or more haematopoeitic line of cells i.e increased production of mature cells
166
Mutation associated with Philadelphia chromosome negative disorders?
JAK2
167
Polycythaemia lab results? (4)
1. raised red cell mass
2. raised Hb
3. raised red cell count
4. raised packed cell volume
168
Causes of pseudo-polycythaemia (4)
1. Burns
2. Dehydration
3. D&V
4. smoking
169
Causes of secondary polycythaemia? (3)
RAISED EPO
1. renal Ca
2. high altitude
3. chronic hypoxia
170
Polycythaemia Rubra Vera
- mutation
- predominate precursor cell
- how is it diagnosed?
- who?
JAK2
erythroid precursors
routine bloods
elderly
171
Clinical Features of Polycythaemi arubra Vera (10)
| - why?
HYPERviscosity HYPERvolaemia HYPERmetabolism
1. blurred vision
2. headache
3. plethoric red nose
4. gout - high RBC turnover high uric acid
5. stroke
6. retinal vein engorgement
7. splenomegaly
8. erythromelagia
9. aquagenic pruritis - itching on water contact
10. peptic ulcers
172
Lab results in Polycythaemia Rubra Vera?
Hb high
HCT high
Plts high
WCC high
EPO low
173
Essential Thrombocythaemia
- dominant cell in BM
- mutation
- who?
megakaryocytes (-> plts)
JAK2
~30 years F=M
~55 year F>M
174
Clinical features of Essential Thrombocythaemia? (9)
```
DVT/PE
Stroke
MI
Gangrene
Haemorrhage
Erythromelagia
Splenomegaly
Dizziness
Headaches
```
175
Management of essential thrombocythaemia?
| - side effects (2)
Anagrelide
1. palpitations
2. flushing
176
Myelofibrosis
myeloproliferative disease whereby myeloproliferation causes fibrosis of the bone marrow and ultimatley leads to replacement of bone marrow with collagenous tissue
177
Myelofibrosis
- who
- cytopenia
- prognosis
- elderly
- pancytopenia
- 2-5 years
178
Clinical Features of Myelofibrosis (7)
1. hepatomegaly
2. splenomegaly
3. fatigue
4. weight loss
5. dyspnoea
6. Budd-Chiari syndrome
7. night sweats
179
Myelofibrosis
Blood film? (2)
Bone Marrow? (2)
Blood film - poikilocytes, leukoerythroblasts
| BM - "dry tap" collagen fibrosis
180
Antibodies present in blood type A?
| Antigens present in blood type A?
Anti-B
| A antigen
181
Antibodies present in blood type B?
| Antigens present in blood type B?
Anti-A
| B antigen
182
Antibodies present in blood type AB?
| Antigens present in blood type AB?
No antibodies
| A and B antigen
183
Antibodies present in blood type O?
| Antigens present in blood type O?
Anti-A, Anti-B
| No antigens
184
Most common blood groups in the UK?
A & O
185
Haemolytic Disease of the newborn
- how?
- common antibody
- other antibodies (4)
If a Rh negative patient is transfued with Rh positive blood they can form Anti-D antibodies
No acute problem
However, if later during pregnancy a Rh negative woman encounters fetal cells either during pregnancy or delivery, and the Anti-D is high... it can destroy the fetal red cells with the corresponding antigen.
Resulting in
i) anaemia
ii) jaundice
iii) hydrops fetalis
Anti-D
Anti-c, anti-K IgG ABO
186
Indications for platelets transplant? (3)
1. Massive transfusion aim plts >75
2. Prevent bleeding post-chemo
3. Prevent bleeding post-surgery
187
Indications for FFP transplant? (3)
| - what is it?
1. Massive transfusion blood loss >150L/min
2. DIC with bleeding
3. Liver disease + risk
contaisn clotting factors
188
Febrile Non-Haemolytic Transfusion Reaction
- symptoms (3)
- when
- management
- cause
- Rise of temp 1*C during or soon after transfusion, rigors, chills
- Acute
189
Allergic Transfusion Reactions
- symptoms (2)
- when
- management
- cause
- type of transfusion
- who
- mild urticaria, wheeze
| - acute
190
Wrong ABO Blood
- symptoms (7)
- when
- cause
- management (4)
- Restless, chest/ loin pain, fever, vomiting, flushing, collapse, haemoglobinuria
- Acute
191
Delayed Haemolytic Transfusion Reaction
- symptoms (4)
- when
- cause
- management (3)
- high bilirubin, low Hb, high retics haemoglobinuria
- over next few days > 24hrs
- patients who are transfused can develop an ‘immune’ antibody to one of the ‘foreign’ RBC antigens they were exposed to, but not tested for ... if the patient has another transfusion with RBCs expressing the same ‘foreign’ antigen the ‘immune’ antibodies cause RBC destruction
= EXTRAVASCULAR HAEMOLYSIS
- repeat x-match: detect new Ab
May need further transfusion
Treat renal failure
192
Anaphylaxis following transfusion
- what
- when
- symptoms (4)
- causes (3)
- Severe, life-threatening reaction soon after start of transfusion
- acute
193
Transfusion associated Cardiac Overload (TACO)
- what
- when
- symptoms (5)
- who
- causes
- pulmonary oedema due to fluid overload
| - acute
194
Transfusion Related Acute Lung Injury (TRALI)
- what & symptoms (3)
- when
- who
- cause (4 steps)
- prevention (2)
- Acute dyspnoea with hypoxia and bilateral pulmonary infiltrates during or within 6 hours of transfusion, not due to circulatory overload or other likely causes
- acute TRAIL ... leave a trail of anti-leucocyte antibodies"
1. Donor anti-leucocyte antibodies (HLA or anti-granulocyte Abs)
2. Interact with patient’s leucocyte antigens
3. Aggregates of white blood cells get stuck in the pulmonary small capillaries
4. Release neutrophil proteolytic enzymes and toxic oxygen metabolites causes lung damage
- don't give FFP from female donors, stop unnecessary use of FFP
195
Delayed Non-Immune adverse reactions to transfusions? (3)
1. Bacterial infection
2. Viral infection
3. Iron overload
196
Presentation of delayed bacterial transmission?
1. Haemolysis
| 2. Anaphylaxis
197
Transfusion Associated Graft-Versus-Host Disease
- who (2)
- cause
- prevention
1. immunocompramised patients
2. donor is HLA-matched or HLA-similar
- donor's blood contains some viable lymphocytes, they should be recognised as "foreign" by recipient, if recipient is unable to do this...lymphocytes are not recognised and destroyed
- lymphocytes recognise same antigen tissue as "foreign" - > Causes severe diarrhoea, liver failure, skin desquamation, bone marrow failure death weeks to months post transfusion
- irradiate donors blood before transfusion to ensure no lymphocytes are capable of dividing
198
Post-transfusion Purpura
- what
- when
- cause
- management
development of purpura within 7-10 days of transfusion
delayed > 24 hrs
HPA-1a negative patients previously immunised by pregnancy or transfusion, causes autoimmune destruction of platelets
IVIG
199
Prevention of Anti-D sensitisation? (2)
1. Must always transfuse RhD negative females of child bearing potential with RhD negative blood
2. Can give intra-muscular injection of anti-D immunoglobulin, at times when mother is at risk of a fetomaternal bleed e.g. at delivery
- must be within 72 hrs
200
Evenst when FetoMaternal Haemorrhage is likely to occur (5)
1. spontaneous miscarriages if surgical evacuation needed and therapeutic abortions
2. amniocentesis and chorionic villous sampling
3. abdominal trauma (falls and car accidents)
4. external cephalic version (turning the fetus)
5. stillbirth or intrauterine death
201
Test for fetomaternal haemorrhage?
Kleihauer Test
202
Changes in maternal FBC during pregnancy? (4)
1. mild anaemia
2. macrocytosis
3. neutrophilia
4. thrombocytosis
203
Why mild anaemia during pregnancy?
- dilutionary effct
- red cell mass increases
- plasma volume increases
- overall increased total iron but decreased concentration
- due to increased iron requirement during pregnancy
204
How much does plasma volume increase during pregnancy?
150%
205
How much does red cell mass increase during pregnancy?
120-130%
206
Consequence of iron deficiency during pregnancy?
IUGR
207
Consequence of folate deficiency during pregnancy?
neural tube defects
208
Recommended iron supplement dose during pregnancy?
60 mg RDA
209
Recommended folic acid supplement dose during pregnancy?
400mcg RDA
210
Level of expected thrombocytopenia during pregnancy?
175-199 x 10*9/L
211
Causes of thrombocytopenia during pregnancy? (5)
1. Gestational/Physiological
2. Pre-eclampsia
3. ITP
4. Microangiopathic syndromes e.g. APLS, HUS, SLE
5. Other e.g. haematological malignancies, DIC
212
Why is pregnancy a hypercoagulable state? (3)
1. increased thrombin generation
2. increased fibrin cleavage
3. reduced fibrinolysis
213
Highest risk of PE at what gestation?
40 - 46 weeks
214
Post Partum Haemorrhage definition
> 500mL blood loss
215
Amniotic Fluid Embolism
- what
- why
- presentation (4)
- fetal debris e.g. amniotic fluid, hair etc enters maternal circulation and triggers an allergic-type repsonse
- coagulation changes during pregnancy predispose to DIC
1. sudden onset shivers
2. vomiting
3. shock
4. DIC
216
Newborns in contrast to adults have
a) A higher Hb
b) A lower WBC
c) Smaller red blood cells
d) The same percentage of haemoglobin F
a) a higher Hb
217
Haemoglobin A
- globin chains
- when is it present (4)
a2b2
1. late fetus
2. infant
3. child
4. adult
218
Haemoglobin A2
- globin chains
- when is it present? (3)
a2theta2
1. infant
2. child
3. adult
219
Haemoglobin F
- globin chains
- when is it present? (2)
a2gamma2
1. fetus
2. infant
220
Why does sickle cell anaemia not manifest at birth?
Clinical features become manifest as gamma chain production and haemoglobin F synthesis decrease and betaS and haemoglobin S production increase
221
Complications of Sickle Cell Anaemia that are more common in adults than in children?
a. Hand-foot syndrome
b. Hyposplenism
c. Red cell aplasia
d. Splenic sequestration
e. Stroke
b. hyposplenism because recurrent infarction has left the spleen small and fibrotic
222
Siblings with sickle cell anaemia present simultaneously with severe anaemia and a low reticulocyte count—likely diagnosis?
a. Splenic sequestration
b. Parvovirus B19 infection
c. Folic acid deficiency
d. Haemolytic crisise
e. Vitamin B12 deficiency
b. Parvovirus B19 Infants and children with sickle cell disease initially have no immunity to parvovirus B19—their first exposure leads to pure red cell aplasia
