Haematology Flashcards
(278 cards)
1
Q
what is the process of making red blood cells called
A
erythropoiesis
2
Q
life span of a RBC
A
120 days
3
Q
constituents of blood (%)
A
plasma (55%) & cellular (45%) - RBC WBC Platelets
4
Q
life span of a platelet
A
7 days
5
Q
life span of a WBC
A
7hrs
6
Q
where are erythrocytes produced
A
bone marrow
7
Q
where does eryptosis take place and what is it
A
spleen, liver and bone marrow - programmed cell death of RBC
8
Q
what is erythroprotein
A
hormone controlling rbc production
9
Q
stages of rbc production
A
pro erythroblast - erythroblast (10% die in BM) - late eryhtroblast (nucleus lost) - reticulocyte - erythrocyte (RBC)
10
Q
what makes up haemoglobin A
A
2 alpha 2 beta 4 oxygen per haemoglobin
11
Q
what is HbA2
A
2x alpha 2x delta
12
Q
what is HbF
A
2x alpha 2x gamma
13
Q
what cause o2 dissociation curve to shift right
increase or decrease O2 affinity?
A
decrease in pH (increase in H+)
increase in CO2
increase in temp
decrease in o2 affinity
14
Q
what does thrombin do
A
converts fibrinogen to fibrin (clot)
activates factor XIII to xIIIa
positive feedback on further thrombin production
15
Q
what factors does warfarin inhibit
A
10 9 7 2 (1972)
16
Q
what does heparin inhibit
A
factor Xa which converts prothrombin to thrombin
17
Q
what does plasmin do
A
cuts fibrin into fragments (D-Dimer) preventing clot growing
18
Q
which are the vit K dependent clotting factors
A
10 9 7 2 (1972)
19
Q
what does fibrinogen form cross links between
A
glycoproetin IIb/IIIa receptors on adjacent platelets
20
Q
what is indicated by a low reticulocyte count
A
problem = producition i.e haematinic deficiency (iron, folate)
21
Q
what is indicated by a high reticulocyte count
A
problem = removal
rbc lost or destroyed i.e bleeding or haemolytic anaemia as new rbc production is increased to compensate
22
Q
what is ferritin
A
iron storage protein
23
Q
what does a common myeloid progenitor cell go on to form
A
megakaryocyte-erythroid progenitor (i.e platelets and erythrocytes) or a granulocyte macrophage progenitor (basophil neutrophil eosinophil monocyte)
EVERYTHING NOT T AND B LYMPHOCYTES
24
Q
what does a common lyphoid progenitor cell go on to form
A
b cell progenitor (b lympohocyte – plasma cell) or t cell progenitor (t lyphocyte, natural killer cell)
25
what is the mean cell volume in microcytic anaemia
<80fL
26
mean cell vol in normocytic
80-96fL
27
mean cell vol in macrocytic anaemia
>96fL
28
low mcv and high rbc anaemia type?
thallassemia or other Hb abnormalities
29
low MCV and low RBC
Fe deficiency anaemia
lead poisoning
anaemia of chronic inflam
30
normal cell vol anaemias?
```
acute blood loss
anaeimia of chronic disease
haemolytic anaemia
chronic kidney disease ( as no EPO)
marrow infiltration/fibrosis
autoimmune rheumatic disease
```
31
high mcv bone marrow is megaloblastic
vit 12 or folate deficiency
32
high mcv bone marrow is normoblastic
liver disease
reticulocytosis
hypothryoidism
alcohol
33
why is anaemia often asymptomatic?
a slow decrease in Hb allows for haemodynamic compenstation and enhanced o2 carrying capacity of the blood (curve shifts right as dissociates more readily)
34
classic symptoms of aneamia
1. fatigue
2. lethargy
3. dyspnoea
4. palpatations
5. headache
35
classic signs of anaemia
1. pallor
2. pale mucous membranes
3. tachycardia (compensatory)
4. cardiac failure
36
what is anaemia the result of
low Hb concentraion due to low red cell mass OR increased plasma vol
37
microcytic examples
iron def
chronic disease
thalassaemia
38
normocytic examples
acute blood loss
anaemia of chronic disease
combined haematinic deficiency (nutrients req for rbc formation)
39
marcocytic examples
b12/folate defic
alcohol excess/liver disease
hypothyroid
haemtological (bone marrow infiltraion or failure)
40
anaemia consequences (in terms of oxygen)
tissue hypoxia as decreased o2 tranport
41
compensatory changes as a result of anaemia
increased tissue perfusion
increased rbc production
increase o2 transfer to tissues
42
pathological consequence of anaemia
```
myocardial fatty changes
liver fatty change
aggravates angina
atrophic changes to skin and nails
CNS cell death
```
43
what does MHCH stand for
mean corpuscular haemoglobin concentration
44
aetiology of iron deificency anaemia
blood loss (menorrhagia or GI tract - HOOKWORM)
decreased absorption
poor intake
pregnancy/breast feeding
45
why can serum ferritin levels APPEAR normal in iron deficiency anaemia
ferritin is a acute phase reactant and so levels increase in inflam or malignant disease so may appear normal even if Fe deficient
46
Dx of iron deficiency anaemia
FBC - hypochromic microctyic
serum ferritin low (may be normal as acute phase reactant)
reticulocyte count low
endocscopy (GI related bleed cause?)
clin history - heavy periods, diet, NSAIDs(GI bleed)
47
what would be suggestive of fe defic anaemia from a clinical history
low intake - diet
heavy periods
NSAIDs self medication ( GI bleed)
48
Mx of iron defi anaem and any SE
orally - ferous sulphate
| SE - black stools, nausea, diarr/constip
49
what does haemoglobinopathy mean
disorder of quantity (thalassaemia) or quality (sickle cell)
50
homozygous beta thalassaemia causes what defect in chain production
little of no normal beta chains produced with excess alpha chains
51
how does excess alpha chains in beta thalassaemia affect the body
they precipitate in erythroblats and RBCs and cause ineffective RBC production and breakdown (erythropoeisis and haemolysis)
combine with any other chain types produced increasing amounts of HbA2 (2x alpha 2xgamma) HbF(2x alpha 2xdelta) which also do this
52
if heterozygous beta thalassaemia what occurs
asymptomatic microctyosis with or without mild anaemia
53
causes of beta thalassaemia
mutation in beta globin genes - decrease beta chain production
54
causes of alpha thalassamemia
genetic DELETIONS in alpha globin chain controlling genes
55
4 deletions in alpha anaemia
no alpha chain synth = Hb barts (4x gamma) DEATH IN UTERO
56
3 deletions in alpha anaemia
decrease in alpha chains so low HbA2 levels - HBH (4x beta) disease - severe haemolytic anaemic and splenomegaly
57
2 deletions in alpha anaem
asympt carrier
mild anaemia
HBH (beta 4) may be seen on blood film
58
if both mild beta+ and alpha thalassamia what is the result and why
intermedia - decease in alpha chain precipitation and so balances with little beta production (both decreased) - less unmatched
59
clincical features of major homozygous beta thal
fail to thrive, chronic infections, bony abnormalities (skull bossing) hepatosplenomegaly
60
homozygous beta thallas, why severe anaemia presentation after first 3-6months of life
this is when should switch from foetal haemoglobin (HbF (2x alpha 2x beta) to adult HbA2 (2x alpha 2x beta) so gets worse as switch as no beta
61
Mx of thalassaemia
regular iron transfusions
long term folic accid supplements
bone marrow transplant
62
what is the risk of regular blood transfusions and how overcome it?
iron overload risk - give iron chelation (desferrioxamine) to prevent endocrine organ damage
63
diagnosis of thalassaemia on blood film
increased reticulocytes and nucleated RBCs
64
Dx of thalass methods
fbc blood film electrophoresis
65
thalassaemia electrophoreis results
show lack of HbA2
| HbF band Large
66
what infections cause anaemia of chronic disease
TB,
chronic inflamm (crohns, reumatoid arthritis, SLE)
malignancy
67
causes of anaemia of chronic disease
1. decrease in iron release from BM
2. decreased rbc survial
3. inadeq EPO response to anaemia
68
Dx of anaemia of chronic disease
low serum iron
| serum ferritin normal or increased due to inflam process
69
what is sideroblastic anaemia
inherited or aquired disorders characterised by refactory anaemia - accumulation of iron in mitochondria of erhythroblasts so causes disordered haem production
70
cx of sideroblastic anaemia
myeloid leukaemia, lead toxicity, alcohol xs
71
normocytic anaemia sub catergories x3
1. acute blood loss or haemorrhage
2. COMBINED haematinic deficiency (iron -micro and b12- macro)
3. anaemia of chronic disease
72
what system disorders is normocytic anaemia commonly seen
endocrine
73
macroctyic anaem aetiolgy x3
megaloblastic BM (pernicious and folate defic) and normoblastic (alcohol XS)
74
pathophysiology behind megaloblastic aneamiea
inhib of dna synthesis and so RBS dont progress onto mitosis - continuing growth WITHOUT DIVISION = MACRO
erthyroblasts with delayed nuclear matureation - megaloblasts
75
haematological findings in macro anaem
| how can this be combined to show an average mcv?
MCV >96fL (unless coexists with micro - dimorphic picture therefore average MCV = normocytic anaemia
76
what is deficient in pernicious anaemia
vitamin b12
77
where is vit b12 absorbed
terminal ileum bound to Intrinsic factor (IF)
78
what is IF secreted by
gastric parietal cells with H+
79
Cx of b12 deficiency
```
atrophic gastritis
crohns
gastrectomy
coeliac
pernicious anaemia
```
80
pathophysiology of b12 defic anaemia
autoimmune disorder destruction of parietal cells - decreasing IF production - cant bind to vit b12 so not absorbed. b12 required to make rbcs
81
CFs of pernicious anaemia
neurological problems - polyneuropathy, pschyiatric problems
glossitis (sore red tongue)
anaemia symptoms
82
diagnosis of pernicous anaemia
blood film - macrocytic rbs
autoantibody screen - IF antibodies
serum bilirubin - increase due to ineffective erythropoiesis
serum b12 - below 160ng/L
83
what do u see on the blood film for pernicious anaemia
macrocytic RBCs
84
what is serum b12 level in perncious anaemia Dx
below 160ng/L
85
Mx of pernicious anaemia
vitamin b12 tablets/injections (hydroxocobalamin)
86
complication of pernicious anaemai
heart failure, angina, neuropathy
87
where is folate absorbed
jejunum
88
Causes of folate deficiency anaemia
NUTRITION
1.poor intake
2. antifolate drugs (methotrexate)
XS UTILISATION
89
sources of folate in diet
green veg, liver, nuts
90
why may folate intake be poor?
anorexia - GI disease (crohns, coeliac, cancer)
91
when may folate be utilised excessively
1. physiological - pregancy,lactation
2. pathological - haemolysis, malignant disease (increased cell turnover), inflam disease
3. malabsorption - small bowel disease (minor)
92
what drug may cause folate defic
methotrexate = folate antagonist
93
does neuropathy occur in b12 or folate defic anaemia
b12 NOT folate
94
clinical features of folate deficiency
asymptomatic
anaemia symptoms (pallor, fatigue)
symptoms from underlying cause
glossitis can occur
95
Mx of folate defic anaemia
5mg folic acid daily
96
Dx of folate defic anaemia
blood film - megaloblasts
erythrocyte folate level
serum folate (>3 ug/L)
97
what area in world is alpha thalassaemia confined to
eastern med and far east
98
what is haemolytic anaemia caused by
increased destruction of RBCs - reduced survival time
99
when does haemolytic anaemia occur?
when RBCs are destroyed before 120days in marcophages of bone marrow, liver and spleen
100
when can shorter RBC survival sometimes not cause anaemia?
if it is compensated!
| an increase in RBC production in the BM = compensated haemolytic disease
101
how can the BM compensate for haemolysis thus not have anaemia
1. increase proportion of cells undergoing erythropoeis
2. increase vol of active marrow
3. release immature red cells (reticulocytes) early
102
what can be seen on blood film of haemolytic anaemia
polychromasia due to residual ribosmal rna as reticuloctyes present as released early to compensate increased breakdwon
103
inherited causes of haemolytic anaemia
inherited - membranopathies=spherocytosis
enzymopathies
haemoglobinopatheis = thalassaemia, sickle cell
104
aquired casues of haemolytic anaemia
autoimmune
infections - malaria
secondary to systemic disease - renal and liver failure
105
symptoms of haemolytic anaeima
gall stones (XS bilirubin
106
what is there XS of in haemolytic anaemia
billirubin (Hb -- Haem + globin. Haem = iron + BILIRUBIN)
107
signs of haemolytic anaemia
jaundice (XS bilirubin)
leg ulcers
splenomegaly (where breakdown occurs)
108
why splenomegaly in haemolyitc anaemia
as increase in breakdown of RBC and occurs in spleen
109
Dx on blood film of haemolytic anaemia
spherocytes (if Cx=spherocytosis) and RETICULOCYTES (released early to keep up with increased breakdown)
110
FBC results in Haemolytic anaemia
low Hb
111
what is the osmotic fragility test and what used for
place RBCs in water and swell and lyse - if spehrocytes = less tolerant than normal concave
USED IN HAEMOLYTIC ANAEMIA - spherocytosis
112
what is Coombs test
direct antiglobulin that is negative in hereditary spherocytosis ruling out autoimmune haemolytic anaemia
113
Tx of haemolytic anaemia
folate and iron supplements
splenomectomy
immunosuppressives (if autoimmune Cx)
114
what is aplastic anaemia
bone marrow failure causing a decrease in pluripotent stem cells - lack of haemopoiesis
115
causes of bone marrow failure
congenital, aquired (aplastic anaemia) infections and cytotoxic drugs/radiation
116
Bone marrow failure - decrease in specific cells ultimately leading to (pancytopenia effects)
anaemia, bleeding, infection
117
clinical feautres of aplastic anaemia
```
increase bruising,
sucespt to infection,
epistaxis of nose, gum bleeding,
blood blisters in mouth,
skin = echymosis (discolouration due to bleeds under skin)
```
118
DDx of aplastic anaemia
megablastic anaemia, BM infiltraion (hogkins and non hodg lymphoma, myeloma etc)
FROM OTHER PANCYTOPENIA Cxs
119
Dx of aplastic anaemia BLOODS
FBC- Pancytopenia
| retuclocyte - low
120
characteristic Dx of aplastic anaemia on BM biopsy
hepatocellular marrow w increased fat spaces
121
Mx of aplastic anaemia
remove causative agent
haemopoeitc stem cells - blood/platelet transfusion
immunosuppresive therapy
stem cell and BM tranplant
122
Risk factors for DVT
```
immobilisation
long haul flights
obesity
pregnancy
age
```
123
CFs of DVT
calf pain - warm, swelling
ankle and pitting oedema
homans sign - pain on dorsiflexion
124
Dx of DVT
```
D Dimer
Doppler US
Venogram for calf
FBC (platelets)
WELLS SCORE
```
125
What criteria is used to assess probability of DVT
Wells score
126
Why is D-dimer test used in DVT
d dimer is a breakdown product of fibrin
| positive result = clot breaking down therefore suggestive of DVT
127
DVT prevention
compression stocking
128
Mx of DVT aim
to prevent pulmonary embolism
129
what does low molecular weight heparin do and why preffered
```
inhibits Xa (converts prothrombin to thrombin) - in clotting
low molecular weight more effective monitoring - reduce bleeding risk
```
130
Action of warfarin
inhibits vit K dependent factors (1972) 10, 9, 7, 2
131
Mx of DVT
low molec weight heparin
then warfarin
till INR within target range(2.5)
132
what does INR stand for and what for
international normalised ration - used in DVT
133
what kind of malignacny is myeloma
malignancy of plasma cells -- progressve BM failure
134
what does proliferation of plasma cells in myeloma cause proudction of
monoclonal immunoglobins (IgG or IgA)
135
consequence of monoclonal immunoglobins
increased infection as no variation
136
which more common type of immunoglobin
IgG (55%) IgA (20%)
137
CF's of myeloma
CRAB
C-calcium increase (hypercalciemia+hyperviscosity due to paraproteinaemia - confusion)
R- renal impair
A - anaemia
B - bone lytic lesions (pepperpot skull, osteoporosis, back ache (spinal cord compression)
138
which has pepperpot skull and lytic lesions
myeloma
139
condition in which Bence Jones protein deposition
myeloma
140
Dx of myeloma
protein electrophoresis - monoclonal band
BM aspiration - XS plasma
urinalysis - high paraprotein
x-ray - pepperpot skull, lytic lesions (long bones), fractures (osteoporosis)
FBC - anaemia hypercalcaeimia low antibodies
UE - high creatine as renal impairment
141
what is shown by bloods of myeloma
```
low Hb (anaemia)
low antibodies
high calcium (bone resorption)
high creatine (renal impairment)
```
142
why is there renal impairment in myeloma
deposition of light chain paraproteins in the kidney
143
how does myeloma cause anaemia
prolif of plasma cells in bone marrow = crowding out therefore decrease in no of RBC produced = anaemia
144
what effect does myeloma have on bone activity
imbalance between osteoclastic and osteoblastic activity
osteoclasts activated by RANKL (from malig myeloma cells)
osteblasts inhib by DKK1
esp long bones/skull/vertebrae (spinal cord compression)
145
2 types of lymphoma
hodkins and non hodgkins
146
what is hidgkins lymphoma
malignancy of mature lymphocytes - w reed and sterberg cells
147
what is the causes of hodgkins lymphoma
unknown - assos with Epstein Barr Virus and immunosupression (HIV, transplants etc)
148
what is haematological condition is assos w Epstein barr virus
hodkins lymphoma
149
what is ann arbor staging system used for
hodgkins lymphoma
150
stage 1 ann arbor
single lymph node region
151
stage 2 ann arbor
2+ lymph node regions SAME SIDE diaphragm
152
stage 3 ann arbor
2+ lymph node region BOTH SIDES diaphramg (may involve spleen)
153
stage 4 ann arbor
diffuse extra lymphatic disease (liver, bone marrow, lungs..)
154
A or B in ann arbor meaning
A - absence of B symptoms
| B - presence of B symptoms
155
age distrib of hodgkins lymphoma
bimodal - young and elderly
156
CF of hodgkins lymphoma
```
B - symptoms
painless enlarged non tender rubbery nodes - lymphadenopathy = worse with alcohol
cough (if compresses SVC)
hepatosplenomegaly
anaemia symptoms (if BM infiltration)
```
157
what are B symtpoms
weightloss, pyrrexia, night sweats, lethargy
158
in what disease does alcohol make lymphadenopathy painful
hodkins lymphoma
159
what is found characteristally in hodgkins but not non hodgkins lymphoma
reed steinberg cells
160
what are reed steinberg cells
mirror image nuclei in lymph node biopsy
161
Dx of hodgkins lymphoma
lymph node biopsy = reed sternberg cells
FBC - increased ESR (infection), Hb decreased
CXR - mediastinal widening if mass
162
Non hodgkin lymphoma sub types based on cell
B cell (80%) and T cell (20%)
163
classification of types of nonhodgkins lymphoma
v HIGH grade - Burkitts lymphoma
HIGH - diffuse large B cell lymphoma
LOW - follicular lymphoma
164
CFs of non hodgkins lymphoma
```
varied -
superficial lymphadenopathy
systemic b symptoms
compression
Panctyopenia - and assos symtpoms (BM infiltration)
```
165
Cx of non hodgkins lymphoma
H. pylori in MALT (mucosa assos lmphoid tissue) - in stomach
HIV
genetic - Wiskott aldrich syndrome
immunosuppressive drugs - (SLE, rheumatoid arthritis)
166
what is burkitts lymphoma assos with
Epstein barr virus
167
Mx of low grade non hodgkins
chemo - rituximab NO CURE (SE:fever, rigors)
168
Mx of high grade non hodgkins
chemo/radiation 30% CURE RATE
169
which has worse prognosis low or high grade non hodkgins
low has no cure
170
what drug is used to manage low grade non hodgkins lymphoma and SE
rituximab - se=fevor, rigor
171
what is assos w reed steinberg cells
hodgkins lymphoma
172
what is assos with philadelphia chromosome
chronic myeloid leukaemia (CML)
173
what is a risk of chemo
tumour lysis syndrome
174
what is tumor lysis syndrome
caused by the breakdown of cancer cells which release their cell contents and cyrstalizes and deposits in the kidneys.
causes kidney damage/fails to function
175
what is commonly given alongside chemo and why (as a preventative)
allopurinol - to prevent tumor lysis syndrome
176
which leukaemias does having downs syndrome increase the risk of
AML (before 5yo)
| ALL (over 5yo)
177
which leukaemia is predominantly a child malignancy
ALL acute lymphoid leukaemia
178
which leukaemia is assos with ionising radiation
CML chronic myeloid leukaemia
179
what is seen on the FBC of non hodgkins lymphoma
panctyopenia
180
what is given to prevent tumor lysis syndrome
allopurinol
181
what is allopurinol and what does it prevent
prevents turmour lysis syndrome and gout (think crystals)
182
what can cause a GI bleed leading to iron defiency anaemia
hookworm
183
which leukamia is a disease of the elderly
AML, CLL
184
leukamia of middle age/elderly
CML
185
what is CML specifically assosiated with
philadelphia chromosome
186
what is the philadelphia chromosome
translocation 9;22 causing BCR-ABL gene fusion - protein activates thyrosine kinase causing increase myeloid division - increase chance of mutations
187
symptoms of CML
sweating weight loss fever abdo pain
188
signs of CML
anaemia, hepatosplenomegaly (feel full)
| lymphadenopathy if blast crisis - AML
189
what would you see on blood film in CML
mature myeloid precursors
190
FBC of CML
```
low Hb (normochromic normocytic anaemia)
high WCC
```
191
Mx 1st line of CML
IMATINIB - tyrosine kinase inhib if Ph+
192
Mx 2nd line of CML
dasatinib, nilotinib
193
Mx 3rd line of CML
chemo - if acute
194
Mx of CML if young
stem cell transplant - only CURE
195
what is Imatinib and when is it used
a tyrosine kinase inhib used in CML if philadelphia chromosome positive
196
what would cytogenetic test be used to show in CML
if philadelphia chromosome positive (BCR-ABL gene fusion)
197
what is acute myeloid leukaemia a disease of
myeloid progenitor cells
198
which is the commonest leaukaemia of adults
acute myeloid leukaemia
199
what are the 3 types of AML
monoblast AML,
megakaryoblast AML,
erythroblast AML
200
what age does downs increase risk for AML
pre 5yo
201
why is DIC a complication of AML
retinoic acid receptor dysfunction causes build up of promyelocytes = lots of AUER RODS increasing coag risk
202
what are clincial features of AML
gum hypertrophy
anaemia infection bleeding (BM marrow failure)
hepatosplenomegaly
203
investigations in AML
```
clotting screen (DIC)
BM aspirate (blast cells - auer rods in cytoplasm)
```
204
what would you see in BM aspirate of AML
auer rods in myeloblast cells
205
is progression of AML fast or slow
fast
206
tx of AML
| prognosis good or bad?
chemo (w breaks for bone marrow regeneration)
BM transplant
Supportive (i.e anticoag etc)
POOR PROGNOSIS
207
what causes swollen gums in AML
build up of monoblasts causing gum hypertrophy
208
what is CLL
chronic malignancy of mature lymphoid cells (mainly B)
| =accumulation of B cells that have escaped apoptosis
209
who is most likely to get CLL
men, elderly,fam history of ALL and CLL
210
risk factor of CLL
family history of ALL and CLL
211
what is characteristic of CLL on blood film
```
smudge cells (fragile membrane of abnormal lymphocytes disrupted)
lymphocytosis
```
212
clincial feature of CLL
lymphadenopathy, hepatospenomegaly, LUQ discomfort (big spleen), "rubbery enlarged non tender nodes"
213
staging of CLL
Binet and rai -
less than 3 enlarged = A
more than 3 enlarged = B
anaemia &/or thrombocytopenia = C
214
rule of thirds in CLL
1/3 dont progress
1/3 slow
1/3 acute
215
Tx of CLL
chemo and radio ( radio to decrease lymphadenopathy and splenomegaly)
rituximab (SE=fever)
216
SE of ritxumab and when used
in NON hodgkins lymphoma and in CLL - SE = nausea
217
ALL is a malignancy of what cells
lymphoid progenitor cells (B and T cells)
| arrests maturation = uncontrolled proliferation of immature b and t cells
218
ALL RFs
downs (5yo plus)
| ionising radiation
219
symptoms of ALL
bone pain, infections (neutropenia)/fever, bleeding, headache, CN palsies, bruising
220
Dx of ALL
lumbar puncture - cns involvment?
FBC - low RBCs low Platelets
Blood film - BLAST CELLS
221
what do blasts cells look like on blood film of ALL
bigger, immature nucleus
222
signs on ALL
lymphadenopathy
hepatosplenobegaly
CNS involvment
223
Cure rate of ALL
70-90% children
| 40% in adults
224
Mx of ALL
chemo (+ ALLOPURINOL (TLS)) then consolidation then BM tranplant and steroids
prophyalxis antibiotics as neutropenia
225
what is thrombocytopenia
reduction of platelets
226
2 types of thrombocytopenia
immune thrombocytopenic purpura (ITP)
| thrombotic thrombocytopenic purpura (TTP)
227
general causes of thrombocytopenia
a decrease in production in BM
an increase in platelet destruction
platelet sequestration in an enlarged spleen
228
what regulates platelet formation
TPO (thrombopoeitin)
229
what are platelets
megakaryocyte fragments (anucleate)
230
what can cause a decrease in platelet production
bone marrow failure, myeloma, aplastic annaemia, hiv
231
what can cause XS destruction/increased platelet consumption
immune thrombocytopenic purpura,
heparin
DIC
SLE
232
causes of sequestraion of platelets
splenomegaly
hypersplenism
thrombotic thromboyctyopenic purpura
233
which is more common ITP or TTP
ITP
234
what is ITP
immune destruction of platelet cells
235
what is TPP
etensive microvascular clots in small blood vessels - organ damage and low platelets
236
causes of ITP
immune - post viral infection or immunisation - PRIMARY
| malignancies and HIV - SECODNARY
237
Cf's of ITP
easy bruising
purpura
epistaxis
menorrhagia
238
CF's of TPP
```
easy bruising
purpura
epistaxis
menorrhagia
CEREBRAL DYSFUNCTION
```
239
who is more likely to get ITP acute onset posst viral infection?
children
240
diagnosis of ITP and TPP (and one difference)
low platelets, high megakaryocytes,
| ITP has platelet antibodies
241
Tx of ITP
```
oral corticosteroids (immunosupp)
splenectomy
tranexamic acid (inhib firbin breakdown)
```
242
Tx of TTP
urgent plasma exchange (replace ADAMTS 13 and remove antibody)
immunosuppression
NOT PLATELETS
243
what do u not give in TTP
platelets
244
what causes TTP
inhib of enzyme that breaks down clots by breaking down vWF
245
what is deep vein thrombosis
thrombus formation in vein
246
RF of DVT
immobiliation
obesity
long haul flight
pregnancy
247
CFs of DVT
calf pain (warm, swelling, tender)
ankle oedema
pitting oedema
Homans sign - pain on dorsiflexion
248
Dx of DVT
D-dimer (if neg=exclude signif venous clot = a firbin degradation production)
Doppler US
Calf venogram - dye n xray
US COMPRESSION TEST - popliteal vein
249
what score is assoc with DVT
Wells score
250
prevention of DVT
compression stockings
251
Mx of CVT
low molecular weight heparin
warfarin
anticoag
252
aim of DVT Mx
prevent pulmonary embolism
253
how does LMW heparin work
inactivates factor Xa (prothrombin to thrombin)
254
why LMW heparin not normal
more effective monitoring therefore less bleed risk
255
how does warfarin work as an anticoag
antagonises vit K dep clotting factors 10 9 7 2 (1972)
256
what is target INR for DVT mx (what does it stand for?)
2.0-3.0 (international normalised ratio)
257
what is polycythaemia
increased Hb, packed cell vol and RBCs = thicker blood and increased clotting
258
what is the gene mutation associated with polycythaemia rubra vera
JAK2 gene mutation - excessive proliferation of progenitors
259
what are causes of primary polycythaemia
increased sensitivity to EPO so increased RBC
260
what cause of secondary polycythaemia
more circualting EPO
261
Cfs of polycythaemia rubera vera
```
plethoric appearance (red face)
thrombosis
headache
itching (POST HOT BATH)
vertigo
```
262
what is the cause of primary famililal and congen polycythaemia
EPOR gene mutation so receptor activated for longer
263
what is a cause of secondary polycythaemia
chronic hypoxia
poor oxygen delivery (altitude)
abnormal RBC struct
tumours secreting EPO - adrenal, renal cell carcinoma
264
Cf's of secondary polycythaemia
```
asympt
easy bleeding bruising,
gout (as increased cell turnover)
fatigue dizziness headaches
palpable spleen and liver
```
265
Mx of polycythaemia rubra vera
aspirin (reduce clotting as thick blood)
| and venesection
266
Mx of secondary polycythaemia
tx cause
267
dx of polcythaemia
FBC- haemocrit level
BM biopsy
genetic testing - JAK2 gene
268
complications of polcythaemia
thrombosis as thicker blood = slower
| haemorrhage
269
what is apparent polycythameia
normal rbc count but lower plasma vol (hypovolaemia) so raised PCV (packed cell vol)
270
what type of polycythaemia has normal rbc count but hypovolaemia
apparent polycythaemia
271
chronic hypoxia, EPO secreting tumours and altitude are causes of what kind of polycythaemia
secondary = more circulating EPO
272
plethoric appearance, itching POST HOT BATH - which condition buzz words
Polycythaemia rubra vera
273
what does DIC stand for
disseminated intravasc crisis
274
what is DIC
out of control haemostasis -
| generation of fibrin within BVs and coag factor/platelet consumption leading to secondary activation of fibrolysis
275
when does DIC occur
sepsis, malignancy, trauma, intravasc haemolysis
276
patho of DIC
procoagulants released in response to sepsis
clot forms
increased platelet and coag factor consumption = spon bleeding tendencies
CLOT form = systemic activation of clotting cascade
microvasc thrombosis
organ failure
277
Dx of DIC
D-dimer
increased FDPs (fibrinolytic activity)
PTT prolonged
low platelets
278
how to treat DIC
underlying cause
maintain blood vol (tranfusions etc)
activated C protein
