Haematology Flashcards
1
Q
Primary haemostasis sequence
A
Vessel damage -> exposes sub-endothelial collagen - platelets bind via VWF (via Glycoprotein 1b)
Following that: activation of platelets, release of ADP and TXA2 which stimulate platelet aggregation and form the plug
2
Q
Causes of failure (primary haemostasis)
A
Vascular factors
Low or absent VWF
Low or absent platelets
3
Q
Vitamin K dependent clotting factors
A
2,7,9,10 All produced in the liver
4
Q
Secondary haemostasis =
A
production of fibrin to form more stable clot.
5
Q
Extrinsic pathway (most important in the production of fibrin) measured time
A
PROTHROMBIN TIME (PTT)
6
Q
Intrinsic pathway (important in amplifying the response) measured time:
A
ACTIVATED PARTIAL PROTHROMBIN TIME (aPTT)
7
Q
Failure of intrinsic pathway can cause:
A
Bleeding into muscles and joints
8
Q
Common pathway (forms THROMBIN) measured time:
A
Thrombin time (TT)
9
Q
Anticoagulant proteins
A
Tissue factor inhibitor
Anti-thrombin 3
Protein C/S (inhibit factors 5&8)
10
Q
What do vascular disorders typically present as failure of : primary or secondary hemostasis (and how do they present?)
A
Primary: (bleeding, purpura, mucosal bleeding)
11
Q
Hereditary Haemorrhagic Telangiectasia (HHT)
A
Autosomal dominant condition.
Telangiectasia throughout body, especially on fingers, nose and surface of organs
Px. as upper GI bleed
12
Q
Scurvy bleeding ?
A
Peri-follicular
13
Q
Bruising syndrome px?
A
Tingling in arms and legs followed by painful easy bruising
14
Q
Normal platelet count
A
150-400
15
Q
Causes of decreased production of platelets
A
Anything that results in marrow suppression: Myelofibrosis Myelodysplasia Aplastic anaemia Leukaemia etc.
16
Q
Myelosuppressive drugs (2 therapies, 1 drug)
A
Chemotherapy, radiotherapy, Methotrexate
17
Q
Causes of increased destruction or use of platelets - conditions
A
ITP, TTP, HUS, DIC
Autoimmune: SLE, APLS
Hypersplensism: CCF, Cirrhosis, SLE
18
Q
Drug causes of increased destruction or use of platelets? -
A
Heparin, antibiotics (penicillin), sulphonamides, furosemide, digoxin, valproate
19
Q
ITP px./ mx.
A
AI condition with antibodies against platelets
Bloods: bleeding time increased
Platelets low
Bone marrow biopsy: increased megakaryocytes
Epistaxis, menorrhagia, easy bruising
Mx. Only if severe;
1) steroids
2) splenectomy
3) Azathioprine
20
Q
TTP - haematological emergency px. mx.
A
Fever, AKI, Mucosal bleeding, fluctuating consciousness.
Blood film: Schistocytes
Mx. Plasmapheresis
Steroids + Ig
Normal coagulation
21
Q
Abnormal platelets - bloods
Coagulation
Platelet count
Bleeding time
A
Coagulation: normal.
Platelet count: normal.
Bleeding time: increased
22
Q
Von Willebrand disease (autosomaml dominant condition) bloods:
A
Coagulation: increased aPTT, bleeding time.
Serum: low VWF, low factor 8. (VWF needed for factor 8)
23
Q
VWB disease mx.
A
Mild: Desmopressin
Severe haemorrhage: VWF concentrates
24
Q
What medication class should be avoided in VWBD
A
NSAIDS
25
Haemophilia A/B chromosome
| which is more common
8/9
| A
26
Haemophillia blood results
Coagulation: isolated increase in aPTT
Serum: low factor 9 or 8
27
Haemophillia Mx:
General: AVOID NSAIDS, IM injections
| Active bleeding = active infusions
28
Disseminated intravascular coagulation px.
MIXEd thrombosis and coagulation defect.
Thrombosis: multi-organ failure
Bleeding: large widespread bruising
oozing blood from mouth nose and cannulation sites
29
DIC causes:
Sepsis, trauma burns and surgery, ABO mismatch, Obstetric causes: Pre-eclampsia, abruption.
30
```
DIC bloods:
•D Dimers:
•Platelets:
•Bleeding time:
•PTT, APTT, TT:
```
D Dimers: high.
Platelets: low.
Bleeding time: increased.
PTT, APTT, TT: increased
31
```
Summary - causative pathologies for:
Increased bleeding time:
• Prolonged PTT:
• Prolonged APTT:
• Prolonged TT:
• Increased everything:
```
Increased bleeding time: platelet issues.
- Prolonged PTT: liver disease / warfarin.
- Prolonged APTT: haemophillia, VWD, heparin.
- Prolonged TT: heparin, advanced liver disease.
Increased everything: DIC.
32
Heparin MoA
POTENTIATES antithrombin 3
33
Clotting factors targeted by potentiated ATIII depend on type of heparin
Unfractioned:
LMWH:
UF: inhibits 2,9,10,11,12
LMWH inhibits 10a only
Renal excretion
34
Heparin which is more markedly increased : aPTT or PT
aPTT
35
Monitoring LMWH/UF Heparin
NONE REQUIRED
| UFH: aPTT
36
Heparin complications
| and long term..
```
Bleeding
HYPERKALAEMIA
Heparin induced thrombocytopenia
LONG term:
OSTEOPOROSIS
```
37
Heparin reversal drug:
Protamine sulphate (stopping alone is usually enough however)
38
Fundoparinex:
Target:
Route:
Indications:
Inhibits factor 10
S/C injection also
Generally same as LMWH but preferred for anti-coagulation in ACS.
Lower risk of HIT
39
Warfarin: inhibits?
Vit K reductase which inhibits the production of factors 2,7,9,10
40
Warfarin effects on coagulation:
Increased aPTT and PT, PT more profound than aPTT
41
CIs for Warfarin
```
Pregnancy
Non-thromboembolic strokes
Severe uncontrolled hypertension.
Severe liver or renal disease
Peptic ulcer disease or GI bleeds.
```
42
Drugs that potentiate warfarin:
```
Alcohol
Omeprazole
Erythromycin
Ciprofloxacin
Valproate
```
43
Drugs that reduce warfarin effects:
Phenytoin
Carbamazepine
Rifampicin
(these are all P450 inducers)
44
Warfarin: Medical Potentiators/inhibitors
Potentiate: febrile illness
hyperthyroidism, cardiac failure, liver/renal disease
Inhibitors: pregnancy, hypothyroidism
45
```
Warfarin reversal
INR >5, <6
INR 6-8
INR > 8 w/little bleeding
INR > 8 w/severe bleeding
```
INR >5, <6: Reduce dose
INR 6-8 w/little bleeding: Stop warfarin - restart at < 5
INR > 8 w/ little bleeding: Stop warfarin, oral vit K. restart <5
INR > 8 w/severe bleeding - Stop warfarin, Prothrombin complex concentrate. Beriplex FFP if available, IV vit K
46
Do NOACs require monitoring
NO
47
Virchow's triad
Stasis
Hypercoagulability
Vessel wall damage
48
Neutrophillia causes:
Acute inflammation/infection/haemorrhage, pregnancy, drugs: steroids (due to reducing marginization - the process in which neutrophils leave the blood.
49
Neutropenia causes
Aplastic anaemia, pancytopenia, drugs: carbimazole and clozapine
50
Eosinophils raised in ?
Churg-strauss, atopy, PARASITIC INFECTIONS, HODGKINS
51
Monocytes - (circulate for a couple days before entering tissues and becoming macrophages) raised in?
Chronic bacterial infections, SLE, RA, lymphoma, leakaemia
52
lymphocytes - raised in?
infection, malignancy, hyposplenism
53
How much iron is stored in the body (grams)
4 g
54
Female normal Hb:
| Pregnant female Hb:
>120
| >110
55
What is haematocrit a measure of?
how much of the volume of blood is RBCs - expressed as a percentage.
56
At what Hb do you transfuse a pt.
>70
57
What are microcytic hypochromic anaemias caused by:
REDUCED HAEMOGLOBIN PRODUCTION
58
Causes of reduced Hb
Haem group:
Porphyrin ring:
Globin chains:
Haem group: Iron deficiency, anaemia of chronic disease
Porphyrin ring: Sideroblastic anaemia
lead poisoning
Globin chains: pyridoxine responsive anaemia
59
Management of iron deficiency anaemia:
| Screen for?
Iron replacement - 200mg ferrous sulphate or fumerate TDS.
| Coeliac disease
60
How long should treatment be given, minimum
3 months
61
How long should treatment be given after correction minimum
3 months
62
When to refer for OGD/colonoscopy in IDA
Iron deficiency anaemia W/ dyspepsia
ALL MEN with unexplained IDA.
IDA with rectal bleeding or symptoms of CRC
ALL WOMEN NOT MENSTRUATING and iron deficient
IDA not responding to treatment.
63
```
Sideroblastic anaemia (Xlinked condition or 2ry to chemo,lead poisoning or anti-TB drugs) -
part of Hb it affects?
```
Porphyrin ring
64
When should sideroblastic anaemia be considered a diagnosis
Iron studies:
Mx:
When microcytic anaemia does not respond to treatment
HIGH IRON FERRITIN AND TRANSFERRIN . Normal TIBC
Treat cause, regular blood transfusions
65
Thalassaemia effect on Hb production
Defective production of globin chains.
66
a-globin gene chromosome
16
67
α+ thalassemia trait:
1/4 copies of gene deleted - asymptomatic
68
α0 thalassemia trait
| Key finding on blood film
2/4 copies of gene deleted
Clinical: asymptomatic
FBC: mild anaemia
Blood film: HEINZ BODIES
69
α Thalassemia (HbH disease)
| px
3/4 copies lost. Causes increased production of HbH (abnormal haemoglobin)
Iron: normal or increased
General symptoms of anemia.
Symptoms of chronic haemolysis: jaundice, leg ulcers, hepatosplenomegaly.
Others: growth retardation, gallstones, iron overload
70
Diagnostic test for HbH
HPLC - high performance liquid chromatography.
71
Mx.
Folic acid supplementation -> to support increased erythropoiesis.
Mild: intermittent transfusion
Severe: regular transfusion
72
Hb Barts - hydrops fetalis
4/4 copies lost
| No functional alpla Hb = intra-uterine death
73
β Thalassemia chromosome
11
74
B chain only present in HbA, therefore:
Reduced HbA production and compensatory increases in other Hb
75
β thalassemia intermedia
thalassemia intermedia is the less severe form of clinically apparent B thalassemia most likely type to be misdiagnosed as IDA
px.
mx.
Mild/moderate anaemia +/- splenomegaly
Intermittent transfusions
76
```
β Thalassemia major
When does it present
Ix findings
FBC: Hb? MCV?
HPLC?
```
Skull XR: hair on end sign
First year of life (failure to thrive, severe anaemia.
HPLC: Very high: HbF.
• High: HbA2.
• Low HbA.
Skull XR: hair on end sign
77
β Thalassemia major Mx.
Life long transfusions
Folate supplementation.
- Life long transfusions + Iron chelators -> Deferiprone +/- splenectomy.
-> The main mortality is caused by iron overload from chronic transfusions.
78
Sickle cell pathophysiology
Point mutation in codon 6 of B globin gene -> alters structure of Hb -> Hbs. Hbs polymerises if exposed to low oxygen for prolonged periods. This distorts the red cell, damaging the RBC membrane.
79
Precipitants of sickle crisis?
```
Hypoxia
Dehydration
Infection
Cold exposure
Stress/fatigue
```
80
Mx. Sickle crisis
```
Opiate analgesia
hydration
rest
oxygen
(Antibiotics if evidence of infection)
```
81
Long term Mx. Sickle cell
Hyposplenism -> reduce risk of infection, prophylactic penicillin. VACCINATION: pneumococcus,meningococcus, haemophilus
Folic acid supplementation (increased turnover so increased demand)
82
Macrocytosis threshold (MCV)
>100
83
Causes of megaloblastic macrocytic anaemia
Vit. B12 and FOLATE DEFICIENCY
84
B12 -> absorbed? via? how long does body store last
Absorbed in terminal ileum via intrinsic factor
| Store = 2-4 years
85
Folate -> absorbed? how long does body store last
Absorbed in the duodenum, a lot smaller store than B12 meaning that folate will become deficient first.
86
Pernicious anaemia -> describe? Auto-antibodies?
AUTOIMMUNE ATROPHIC GASTRITIS with reduction OF HCL and INTRINSIC FACTOR. m/c in women in their 40s with other AI conditions.
Anti-parietal cell = sensitive but not specific
Anti-intrinsic factor = specific but not sensitive
87
Common autoimmune condition that can cause B12 deficiency
Coeliac disease
88
Acute px of B12 def.
SUBACUTE DEGENERATION OF THE SPINAL CORD.
| Degeneration of CST and dorsal column.
89
Mx. B12/folate deficiency
ID and Tx. underlying cause:
FOLATE: 5mg for 4 months
B12: IM B12 COBALAMIN initially on alternate days then 3 monthly if appropriate.
RULE: folate should not be given ALONE due to failure to provide B12 causing/worsening SACD
90
Causes of non-megaloblastic macrocytosis
| All cause an increase in RBC size by altering the red cell membrane.
ALCOHOL
pregnancy
HYPOthyroidism
Liver disease
91
What is Reticulocytosis?
An increased number of circulating reticulocytes due to increased production of RBCs.
92
When is reticulocytosis seen
Haemolysis
Haemorrhage
Treatment of anaemia
Thalassemia (reticulocytosis but not macrocytosis)
93
Haemolysis = breakdown of RBCs before how many days
| Bone marrow response =
120
| Erythroid hyperplasia/reticulocytosis
94
What is extra-vascular haemolysis hallmarked by:
(EV haemolysis occurs in liver and spleen mainly)
Can be physiological or pathological
Hepatoplenomegaly
Unconjugated bilirubin
Urobilinogen
95
Intra-vascular haemolysis - always pathological
| Hallmarked by?
```
Schistocytes
Increased LDH
Decreased Haptogglobins
Haemoglobinaemia/ methealbuminaemia
haemosidenuria
```
96
Causes of EV and IV Haemolysis
EV = Thalassaemia, sickle cell disease, hereditary spherocytosis, AI haemolytic anaemia
Intravascular: Drug induced, ABO mismatch, G6PD deficiency, severe malaria, MAHA
97
Acquired haemolytic anaemia causes
Infection: malaria
| Drug-induced, autoimmune haemolytic anaemia.
98
Drug induced haemolytic anaemia
COOMBS postive
COOMBS negative
COOMBS positive: Penicillin, cephalosporin
| COOMBS negative: rituximab
99
Microangiopathic haemolytic anaemia:
Intravascular haemolysis due to mechanical disruption of RBCs leading to their fibrosis and release of extracellular components
CAUSES?
DIC, pre-eclampsia, TTP, HUS
100
Autoimmune haemolytic anaemia
| classified by hot and cold (based on temperature at which the ABs bind) Mx.
Management:
HOT =Steroids +/- splenectomy
COLD = keep warm, CHLORAMBUCIL
101
Hereditary haemolytic anaemias: (3)
G6PD deficiency
Hereditary spherocytosis
Haemoglobinopathies: thalassaemia, sickle cell
102
G6PD def. inheritance
X-linked
103
G6PD Presentation = rapid onset jaundice/ anaemia after exposure to
On blood film:
Acute illness
Broad beans
Drugs: ASPIRIN
HENNA
Heinz bodies, bite cells
104
Hereditary spherpocytosis Px: YOUNG child with family history of splenectomy w/ jaundice, splenomegaly, mild anaemia
Blood film?
Spherocytes
| Mx. Splenectomy
105
Haemolytic disease RBC blood picture
Polychromatic (colourful) macrocytosis with high reticulocytes
106
Pancytopenia : Fanconi anaemia inheritance
Autosomal recessive
107
Fanconi syndrome px.
Short stature
| missing limbs/radii
108
Which cancer is Fanconi syndrome assoc. with an increase in?
Acute Myeloid Leukaemia
109
Aplastic anaemia - group of conditions where there is damage to bone marrow that results in instability of stem cells to generate cells.
Normochromic normocytic anaemia
Causes:
Irradiation
Infection: parvovirus B19
Drugs: chloramphenicol, carbamazepine, cytotoxics
110
Aplastic anaemia mx:
Identify and treat underlying cause +/- splenectomy: supportive treatment: red cell/ platelet transfusion
Infection prophylaxis.
111
Paraprotein diseases examples:
Blood film:
```
Myeloma
MGUS
amyloidosis
B cell lymphoma
Chronic lymphocytic anaemia
Waldenstroms Macroglobinaemia
```
Roulex formation
112
Multiple myeloma: Malignant proliferation of B cells within the bone marrow. Px?
Myeloma should always be considered in AKI if patient is greater than 50 or has anaemia or hypercalcemia
As direct effect on bone: bone pain, usually back, osteoporosis and pathological fractures
Hypercalcaemia: bones,groans, moans, psychiatric undertones
Aplastic anaemia: anaemia, bleeding, bruising, recurrent infections.
Renal impairment: AKI or CKD
Hyperviscosity: reduced vision and cognition
113
```
Ix.
Blood film:
Serum electrophoresis:
urine electrophoresis:
skeletal survey:
```
Blood film: Rouleaux formation
Serum electrophoresis: paraprotein
urine electrophoresis: BENCE JONES PROTEIN
skeletal survey: PEPPERPOT SKULL VERTEBRAL COLLAPSE.
114
Myeloma prognostic investigation:
B2 microglobin
115
Myeloma management:
Active tx. - immunosupression and chemotherapy
Dexamethasone
Melphalin
Thaidomide/lenalidomide
116
MGUS - monoclonal gammopathy of unknown significance
defined as less than X paraprotein?
Is there evidence of myeloma or end-organ damage
Can this progress to myeloma
X < 30g
Bone marrow plasma cells <10%
No
Yes
117
```
Amyloidosis px:
Kidney
Liver
GI
Heart
Nerves
Skin
```
```
Nephrotic syndrome
Hepatomegaly
Macroglossia, malabsorption
Restrictive cardiomyopathy
autonomic and peripheral neuropathy
peri-orbital purpura = characteristic
```
118
Amyloidosis diagnostic finding.
| Mx.
Apple green birefringence with congo red stain
| essentially none and pt. tend to die in a few years
119
What are B cell symptoms
Fever
Wt. loss
Night sweats
120
Waldenstroms macroglobinaemia:
what antibody does it concern ?
Main pathological outcome ?
Mx.
IgM
IgM mediated hyperviscosity
Chemotherapy: Chlorambucil
Hyperviscosity: Leucophoresis and plasmapharesis
121
```
Leukaemia classification based on?
Onset:
Cell lineage:
Immunophenotyping:
Cytogenics:
```
Onset: acute or chronic
Cell lineage: lymphoid or myeloid
Immunophenotyping: antigen expressed
Cytogenics: mutations present in the cells
122
```
Acute lymphoid leukaemia:
most common cancer in what age group?
main risk factors:
Px?
Examination:
```
CHILDREN (2-8)
Downs syndrome, irradiation (esp. during pregnancy)
Px: Anaemia, thrombocytopenia (easy bruising), Leukopenia (recurrent infections, not feeling well)
Hepatosplenomegaly, lymphadenopathy, orchidomegaly
123
```
Acute lymphoid leukaemia ix:
Bloods
Blood film
Bone marrow biopsy
additional investigations?
```
bloods: normocytic normochromic, neutropenia, leukocytosis (although WCC Is high, they are NON- functioning)
>20% blasts
>20% blasts
Immunophenotyping/cytogenetics
CT CAP/ head - look for infiltration
124
ALL Mx:
Curative intent with long term chemotherapy.
| Cure rate = 90% in children
125
Acute Myeloid leukaemia features similar infiltration of tissues outside of the bone marrow as ALL (skin, gums, lymph nodes, liver and spleen) with one exception - what is it?
NO CNS INFILTRATION
126
Key finding in ALL marrow biopsy
AUER RODS
127
ALL Mx:
```
Intense chemotherapy regimen
5 cycles given one week apart
common drugs: Daunorubicin cytrabine
stem cell transplant ->
APL ALTRA transretinoic acid - pretty much curative
```
128
Chronic Lymphocytic leukaemia:
| Associations?
Autoimmune haemolytic anaemia
| ITP
129
CLL occurs almost exclusively in which age group
| Most common leukaemia in adults.
>50 years
130
CLL investigations:
Bloods:
Electrophoresis:
immunophenotyping:
Normochromic normocytic anaemia
low neutrophils, platelets
high WCC -> lymphocytes
hypoggammaglobinaemia
CD19/20
131
CLL rule of 1/3rds
1/3 don't progress
1/3 will progress with time
1/3 actively progressing at presentation
132
CLL mx:
Not actively progressing: observation
actively progressing: Chemotherapy: cyclophosphamide, Fludarabine
+/- stem cell transplant
133
Chronic Myeloid Leukaemia: pathognominic mutation:
Philadelphia chromosome 9:22 results in a abnormal tyrosine kinase
134
CML presents as a slow onset of
Weight loss, fatigue, fever and night sweats
Gout -> increased red cell turnover
Abdominal pain/ bloating -> massive splenomegaly
135
CML Ix: marrow biopsy
Hyper cellular marrow
136
CML Mx:
TYROSINE KINASE INHIBITORS: IMATINIB
| does not cure disease but completely controls it.
137
```
Lymphoma general investigations:
Bloods:
Diagnostic:
Immunological:
Imaging:
```
Bloods: FBC, LDH, blood film.
Diagnostic = Excisional lymph node biopsy
immunological: cytogenics, immunophenotyping
imaging: CXR, CTCAP, PET, CT - PET CT is best for staging and for assessing treatment response in HL.
138
Hodgkins lymphoma: percentage of of lymphomas
10%
139
Pathognomonic CD30+ cell of Hodgkins lymphoma:
Reed sternberg cell
140
Differentiating feature of HL (apparently)
Lymph node pain on DRINKING ALCOHOL
141
Mx Hodgkins Lymphoma.
```
CURATIVE INTENT w/ chemotherapy +/- radio +/- anti-CD30
ABVD:
o A Adriamycin.
o B Bleomycin.
o V Vincristine.
o D Dacarbazine.
```
142
Hodgkins lymphoma: CD..antigen?
| Non-Hodgkins lymphoma (NHL) CD..?
```
HL = CD30
NHL = CD20
```
143
Types of NHL.
Burkitts
Follicular
Mantle
144
Key facts:
Burkitts:
Follicular:
Mantle:
Burkitts Usually seen in African children who are EBV +.
Typically presents as a large tumour on face.
Follicular: High grade B cell lymphoma.
Mantle: Low grade B cell lymphoma
145
Extra nodal disease is more common in NHL: examples?
- CNS.
- Spleen: splenomegaly.
- Marrow: marrow failure.
- Skin: popular rash, usually T cell.
- Waldeyers ring: difficulty breathing.
146
Mx. NHL:
Non-curative and serves to induce remission only -> chronic relapsing-remitting course.
```
RCHOP:
Rituximab - anti-CD20 monoclonal antibody
Cyclophosphamide
Hydroxyduanorubicin
O - Vincristine
P - Prednisolone
```
147
```
Lymphoma lymph nodes =
Tender?
Consistency?
Surface?
Inflammation?
Tethered?
```
```
Non-tender,
rubbery and soft
smooth
no inflammation
not tethered
```
148
```
Causes of hypersplenism:
Infective
Inflammatory
Congestion
Haematological
```
Infective: malaria, leishmaniasis,EBV
Inflammatory: rheumatoid, SLE
Congestion: heart failure, liver cirrhosis
Haematological: lymphoma, leukaemia, myeloproliferative disorders, thalassaemia, spherocytosis
149
Felty's syndrome (3)
sequestration of blood cells in the spleen
Mx?
Rheumatoid arthritis, splenomegaly and LOW WCC
| treat cause +/- splenectomy
150
```
Myeloproliferative disorders:
RBC:
WCC:
Platelets:
FIbroblasts:
```
Polycythaemia rubra vera
Chronic myeloid leukaemia
Essential thrombocythaemia
Myelofibrosis
151
Myeloproliferative disorder diagnosis when there is no reactive cause for:
splenomegaly or thrombosis +/-
high granulocytes
high platelets
high Hb
152
Polycythaemia rubra vera
An increase in red cell mass described as
Absolute:
Relative:
Absolute: increased red cell mass from increased red cell production - hallmarked by an increase in packed cell volume.
Primary = PRV
Secondary: COPD smoking, increased EPO production (renal and hepatic carcinoma)
Relative = increase in red cell mass due to plasma volume depletion -> no increase in red cell volume is seen: caused by alcohol, obesity, dehydration
153
PRV mutation
JAK-2 (results in loss of auto-inhibtion and stimulation of RBC in absence of stimuli)
154
Diagnostic PRV; Itch when?
Itch after warm shower +/- eryhtromelalgia
155
Mx. PRV ?
Reduce risk of thrombosis:
low risk = venesection + aspirin
high risk: hydroxycarbamide + aspirin (>60 y/o or previous thrombosis)
156
Essential thrombocythaemia: platelets >?
>450
157
Causes of Thrombocythaemia:
| clonal proliferation of MEGAKARYOCYTES
bleeding, infection, malignancy, trauma/surgery, splenectomy and hyposplensim
158
Thrombocythaemia mutations
JAK2 or CALR
159
Thrombocythaemia Mx.
Low risk = aspirin
| High risk = aspirin + hydroxycarbamide
160
Myelofibrosis blood film finding:
Leukoerythroblastosis and TEAR DROP SHAPED RBC
161
Myelofibrosis management:
Supportive (allopurinol if hyperuricaemic
| active treatment + bone marrow transplant or Ruxolitinib (JAK 2 inhibitor)
162
Blood transfusions What diseases are all donations screened for?
unable to screen for?
Malignancy, HIV, hepatitis, syphillis, HTLV
| CJD
163
Red cells
shelf life:
once out of fridge needs to be transfused within?
35 days
| 4 hours
164
Platelets:
Shelf life:
Once out storage use by?
5 days
| 1 hour
165
```
FFP
Stored at?
For up to?
Use within?
Use in: DIC and warfarin overdose.
```
-30 degrees
2 years
4 hours
166
Cryopreciptate: factors?
8/9 VWF, fibrinogen
167
Anti-D uses:
Antepartum haemorrhage/ prophylaxis of rhesus negative mother
168
PT complex
Used in vitamin K deficiency -
warfarin
liver disease
169
Acute haemolytic transfusion reaction mediated by Ig?
| Causes severe hypotension, DIC, AKI and shock
IgM
170
Delayed haemolytic reaction (5-10 days after transfusion)
| m/c in pts. with previous transfusions Px.
Jaundice, sudden drop in Hb and AKI
171
White cell complications: febrile, non haeolytic px.
Fever, rigors
172
White cell complications: TRALI (transfusion related lung injury).
SOB, Cough, tachypnoea, bilateral crepitations
CXR?
Mx.
CXR = white out
| Mx. stop transfusion, high flow oxygen, admit to ITU.
173
Anaphylaxis
ABCDE
Adrenaline dose
hydrocortisone dose chloramphenamine dose
Adrenaline 0.5 ml 1:1000 IM
Hydrocortisone - 200mg IV
Chloramphenamine - 10 mg IV
174
Frequent transfusions: iron overload - minimised by?
Iron chelators: Deferiprone
175
Splenectomy pts. which organism are they still vulnerable to despite anti-biotic prophylaxis:
Haemophilus influenzae
