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2a RRS > Haematology > Flashcards

Haematology Flashcards

1
Q

what is the process of making red blood cells called

A

erythropoiesis

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2
Q

life span of a RBC

A

120 days

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3
Q

constituents of blood (%)

A

plasma (55%) & cellular (45%) - RBC WBC Platelets

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4
Q

life span of a platelet

A

7 days

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5
Q

life span of a WBC

A

7hrs

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6
Q

where are erythrocytes produced

A

bone marrow

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7
Q

where does eryptosis take place and what is it

A

spleen, liver and bone marrow - programmed cell death of RBC

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8
Q

what is erythroprotein

A

hormone controlling rbc production

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9
Q

stages of rbc production

A

pro erythroblast - erythroblast (10% die in BM) - late eryhtroblast (nucleus lost) - reticulocyte - erythrocyte (RBC)

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10
Q

what makes up haemoglobin A

A

2 alpha 2 beta 4 oxygen per haemoglobin

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11
Q

what is HbA2

A

2x alpha 2x delta

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12
Q

what is HbF

A

2x alpha 2x gamma

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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

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14
Q

what does thrombin do

A

converts fibrinogen to fibrin (clot)
activates factor XIII to xIIIa
positive feedback on further thrombin production

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15
Q

what factors does warfarin inhibit

A

10 9 7 2 (1972)

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16
Q

what does heparin inhibit

A

factor Xa which converts prothrombin to thrombin

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17
Q

what does plasmin do

A

cuts fibrin into fragments (D-Dimer) preventing clot growing

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18
Q

which are the vit K dependent clotting factors

A

10 9 7 2 (1972)

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19
Q

what does fibrinogen form cross links between

A

glycoproetin IIb/IIIa receptors on adjacent platelets

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20
Q

what is indicated by a low reticulocyte count

A

problem = producition i.e haematinic deficiency (iron, folate)

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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

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22
Q

what is ferritin

A

iron storage protein

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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

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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)

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25
Q

what is the mean cell volume in microcytic anaemia

A

<80fL

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26
Q

mean cell vol in normocytic

A

80-96fL

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27
Q

mean cell vol in macrocytic anaemia

A

> 96fL

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28
Q

low mcv and high rbc anaemia type?

A

thallassemia or other Hb abnormalities

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29
Q

low MCV and low RBC

A

Fe deficiency anaemia
lead poisoning
anaemia of chronic inflam

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30
Q

normal cell vol anaemias?

A 
acute blood loss
anaeimia of chronic disease
haemolytic anaemia 
chronic kidney disease ( as no EPO)
marrow infiltration/fibrosis
autoimmune rheumatic disease
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31
Q

high mcv bone marrow is megaloblastic

A

vit 12 or folate deficiency

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32
Q

high mcv bone marrow is normoblastic

A

liver disease
reticulocytosis
hypothryoidism
alcohol

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33
Q

why is anaemia often asymptomatic?

A

a slow decrease in Hb allows for haemodynamic compenstation and enhanced o2 carrying capacity of the blood (curve shifts right as dissociates more readily)

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34
Q

classic symptoms of aneamia

A
  1. fatigue
  2. lethargy
  3. dyspnoea
  4. palpatations
  5. headache
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35
Q

classic signs of anaemia

A
  1. pallor
  2. pale mucous membranes
  3. tachycardia (compensatory)
  4. cardiac failure
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36
Q

what is anaemia the result of

A

low Hb concentraion due to low red cell mass OR increased plasma vol

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37
Q

microcytic examples

A

iron def
chronic disease
thalassaemia

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38
Q

normocytic examples

A

acute blood loss
anaemia of chronic disease
combined haematinic deficiency (nutrients req for rbc formation)

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39
Q

marcocytic examples

A

b12/folate defic
alcohol excess/liver disease
hypothyroid
haemtological (bone marrow infiltraion or failure)

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40
Q

anaemia consequences (in terms of oxygen)

A

tissue hypoxia as decreased o2 tranport

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41
Q

compensatory changes as a result of anaemia

A

increased tissue perfusion
increased rbc production
increase o2 transfer to tissues

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42
Q

pathological consequence of anaemia

A 
myocardial fatty changes
liver fatty change
aggravates angina 
atrophic changes to skin and nails
CNS cell death
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43
Q

what does MHCH stand for

A

mean corpuscular haemoglobin concentration

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44
Q

aetiology of iron deificency anaemia

A

blood loss (menorrhagia or GI tract - HOOKWORM)
decreased absorption
poor intake
pregnancy/breast feeding

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45
Q

why can serum ferritin levels APPEAR normal in iron deficiency anaemia

A

ferritin is a acute phase reactant and so levels increase in inflam or malignant disease so may appear normal even if Fe deficient

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46
Q

Dx of iron deficiency anaemia

A

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)

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47
Q

what would be suggestive of fe defic anaemia from a clinical history

A

low intake - diet
heavy periods
NSAIDs self medication ( GI bleed)

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48
Q

Mx of iron defi anaem and any SE

A

orally - ferous sulphate

SE - black stools, nausea, diarr/constip

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49
Q

what does haemoglobinopathy mean

A

disorder of quantity (thalassaemia) or quality (sickle cell)

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50
Q

homozygous beta thalassaemia causes what defect in chain production

A

little of no normal beta chains produced with excess alpha chains

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51
Q

how does excess alpha chains in beta thalassaemia affect the body

A

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

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52
Q

if heterozygous beta thalassaemia what occurs

A

asymptomatic microctyosis with or without mild anaemia

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53
Q

causes of beta thalassaemia

A

mutation in beta globin genes - decrease beta chain production

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54
Q

causes of alpha thalassamemia

A

genetic DELETIONS in alpha globin chain controlling genes

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55
Q

4 deletions in alpha anaemia

A

no alpha chain synth = Hb barts (4x gamma) DEATH IN UTERO

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56
Q

3 deletions in alpha anaemia

A

decrease in alpha chains so low HbA2 levels - HBH (4x beta) disease - severe haemolytic anaemic and splenomegaly

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57
Q

2 deletions in alpha anaem

A

asympt carrier
mild anaemia
HBH (beta 4) may be seen on blood film

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58
Q

if both mild beta+ and alpha thalassamia what is the result and why

A

intermedia - decease in alpha chain precipitation and so balances with little beta production (both decreased) - less unmatched

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59
Q

clincical features of major homozygous beta thal

A

fail to thrive, chronic infections, bony abnormalities (skull bossing) hepatosplenomegaly

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60
Q

homozygous beta thallas, why severe anaemia presentation after first 3-6months of life

A

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

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61
Q

Mx of thalassaemia

A

regular iron transfusions
long term folic accid supplements
bone marrow transplant

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62
Q

what is the risk of regular blood transfusions and how overcome it?

A

iron overload risk - give iron chelation (desferrioxamine) to prevent endocrine organ damage

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63
Q

diagnosis of thalassaemia on blood film

A

increased reticulocytes and nucleated RBCs

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64
Q

Dx of thalass methods

A

fbc blood film electrophoresis

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65
Q

thalassaemia electrophoreis results

A

show lack of HbA2

HbF band Large

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66
Q

what infections cause anaemia of chronic disease

A

TB,
chronic inflamm (crohns, reumatoid arthritis, SLE)
malignancy

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67
Q

causes of anaemia of chronic disease

A
  1. decrease in iron release from BM
  2. decreased rbc survial
  3. inadeq EPO response to anaemia
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68
Q

Dx of anaemia of chronic disease

A

low serum iron

serum ferritin normal or increased due to inflam process

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69
Q

what is sideroblastic anaemia

A

inherited or aquired disorders characterised by refactory anaemia - accumulation of iron in mitochondria of erhythroblasts so causes disordered haem production

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70
Q

cx of sideroblastic anaemia

A

myeloid leukaemia, lead toxicity, alcohol xs

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71
Q

normocytic anaemia sub catergories x3

A
  1. acute blood loss or haemorrhage
  2. COMBINED haematinic deficiency (iron -micro and b12- macro)
  3. anaemia of chronic disease
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72
Q

what system disorders is normocytic anaemia commonly seen

A

endocrine

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73
Q

macroctyic anaem aetiolgy x3

A

megaloblastic BM (pernicious and folate defic) and normoblastic (alcohol XS)

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74
Q

pathophysiology behind megaloblastic aneamiea

A

inhib of dna synthesis and so RBS dont progress onto mitosis - continuing growth WITHOUT DIVISION = MACRO
erthyroblasts with delayed nuclear matureation - megaloblasts

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75
Q

haematological findings in macro anaem

how can this be combined to show an average mcv?

A

MCV >96fL (unless coexists with micro - dimorphic picture therefore average MCV = normocytic anaemia

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76
Q

what is deficient in pernicious anaemia

A

vitamin b12

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77
Q

where is vit b12 absorbed

A

terminal ileum bound to Intrinsic factor (IF)

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78
Q

what is IF secreted by

A

gastric parietal cells with H+

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79
Q

Cx of b12 deficiency

A 
atrophic gastritis
crohns
gastrectomy
coeliac
pernicious anaemia
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80
Q

pathophysiology of b12 defic anaemia

A

autoimmune disorder destruction of parietal cells - decreasing IF production - cant bind to vit b12 so not absorbed. b12 required to make rbcs

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81
Q

CFs of pernicious anaemia

A

neurological problems - polyneuropathy, pschyiatric problems
glossitis (sore red tongue)
anaemia symptoms

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82
Q

diagnosis of pernicous anaemia

A

blood film - macrocytic rbs
autoantibody screen - IF antibodies
serum bilirubin - increase due to ineffective erythropoiesis
serum b12 - below 160ng/L

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83
Q

what do u see on the blood film for pernicious anaemia

A

macrocytic RBCs

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84
Q

what is serum b12 level in perncious anaemia Dx

A

below 160ng/L

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85
Q

Mx of pernicious anaemia

A

vitamin b12 tablets/injections (hydroxocobalamin)

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86
Q

complication of pernicious anaemai

A

heart failure, angina, neuropathy

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87
Q

where is folate absorbed

A

jejunum

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88
Q

Causes of folate deficiency anaemia

A

NUTRITION
1.poor intake
2. antifolate drugs (methotrexate)
XS UTILISATION

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89
Q

sources of folate in diet

A

green veg, liver, nuts

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90
Q

why may folate intake be poor?

A

anorexia - GI disease (crohns, coeliac, cancer)

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91
Q

when may folate be utilised excessively

A
  1. physiological - pregancy,lactation
  2. pathological - haemolysis, malignant disease (increased cell turnover), inflam disease
  3. malabsorption - small bowel disease (minor)
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92
Q

what drug may cause folate defic

A

methotrexate = folate antagonist

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93
Q

does neuropathy occur in b12 or folate defic anaemia

A

b12 NOT folate

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94
Q

clinical features of folate deficiency

A

asymptomatic
anaemia symptoms (pallor, fatigue)
symptoms from underlying cause
glossitis can occur

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95
Q

Mx of folate defic anaemia

A

5mg folic acid daily

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96
Q

Dx of folate defic anaemia

A

blood film - megaloblasts
erythrocyte folate level
serum folate (>3 ug/L)

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97
Q

what area in world is alpha thalassaemia confined to

A

eastern med and far east

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98
Q

what is haemolytic anaemia caused by

A

increased destruction of RBCs - reduced survival time

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99
Q

when does haemolytic anaemia occur?

A

when RBCs are destroyed before 120days in marcophages of bone marrow, liver and spleen

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100
Q

when can shorter RBC survival sometimes not cause anaemia?

A

if it is compensated!

an increase in RBC production in the BM = compensated haemolytic disease

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101
Q

how can the BM compensate for haemolysis thus not have anaemia

A
  1. increase proportion of cells undergoing erythropoeis
  2. increase vol of active marrow
  3. release immature red cells (reticulocytes) early
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102
Q

what can be seen on blood film of haemolytic anaemia

A

polychromasia due to residual ribosmal rna as reticuloctyes present as released early to compensate increased breakdwon

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103
Q

inherited causes of haemolytic anaemia

A

inherited - membranopathies=spherocytosis
enzymopathies
haemoglobinopatheis = thalassaemia, sickle cell

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104
Q

aquired casues of haemolytic anaemia

A

autoimmune
infections - malaria
secondary to systemic disease - renal and liver failure

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105
Q

symptoms of haemolytic anaeima

A

gall stones (XS bilirubin

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106
Q

what is there XS of in haemolytic anaemia

A

billirubin (Hb – Haem + globin. Haem = iron + BILIRUBIN)

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107
Q

signs of haemolytic anaemia

A

jaundice (XS bilirubin)
leg ulcers
splenomegaly (where breakdown occurs)

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108
Q

why splenomegaly in haemolyitc anaemia

A

as increase in breakdown of RBC and occurs in spleen

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109
Q

Dx on blood film of haemolytic anaemia

A

spherocytes (if Cx=spherocytosis) and RETICULOCYTES (released early to keep up with increased breakdown)

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110
Q

FBC results in Haemolytic anaemia

A

low Hb

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111
Q

what is the osmotic fragility test and what used for

A

place RBCs in water and swell and lyse - if spehrocytes = less tolerant than normal concave
USED IN HAEMOLYTIC ANAEMIA - spherocytosis

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112
Q

what is Coombs test

A

direct antiglobulin that is negative in hereditary spherocytosis ruling out autoimmune haemolytic anaemia

113
Q

Tx of haemolytic anaemia

A

folate and iron supplements
splenomectomy
immunosuppressives (if autoimmune Cx)

114
Q

what is aplastic anaemia

A

bone marrow failure causing a decrease in pluripotent stem cells - lack of haemopoiesis

115
Q

causes of bone marrow failure

A

congenital, aquired (aplastic anaemia) infections and cytotoxic drugs/radiation

116
Q

Bone marrow failure - decrease in specific cells ultimately leading to (pancytopenia effects)

A

anaemia, bleeding, infection

117
Q

clinical feautres of aplastic anaemia

A 
increase bruising, 
sucespt to infection, 
epistaxis of nose, gum bleeding,
blood blisters in mouth, 
skin = echymosis (discolouration due to bleeds under skin)
118
Q

DDx of aplastic anaemia

A

megablastic anaemia, BM infiltraion (hogkins and non hodg lymphoma, myeloma etc)
FROM OTHER PANCYTOPENIA Cxs

119
Q

Dx of aplastic anaemia BLOODS

A

FBC- Pancytopenia

retuclocyte - low

120
Q

characteristic Dx of aplastic anaemia on BM biopsy

A

hepatocellular marrow w increased fat spaces

121
Q

Mx of aplastic anaemia

A

remove causative agent
haemopoeitc stem cells - blood/platelet transfusion
immunosuppresive therapy
stem cell and BM tranplant

122
Q

Risk factors for DVT

A 
immobilisation
long haul flights
obesity 
pregnancy
age
123
Q

CFs of DVT

A

calf pain - warm, swelling
ankle and pitting oedema
homans sign - pain on dorsiflexion

124
Q

Dx of DVT

A 
D Dimer
Doppler US
Venogram for calf
FBC (platelets)
WELLS SCORE
125
Q

What criteria is used to assess probability of DVT

A

Wells score

126
Q

Why is D-dimer test used in DVT

A

d dimer is a breakdown product of fibrin

positive result = clot breaking down therefore suggestive of DVT

127
Q

DVT prevention

A

compression stocking

128
Q

Mx of DVT aim

A

to prevent pulmonary embolism

129
Q

what does low molecular weight heparin do and why preffered

A 
inhibits Xa (converts prothrombin to thrombin) - in clotting
low molecular weight more effective monitoring - reduce bleeding risk
130
Q

Action of warfarin

A

inhibits vit K dependent factors (1972) 10, 9, 7, 2

131
Q

Mx of DVT

A

low molec weight heparin
then warfarin
till INR within target range(2.5)

132
Q

what does INR stand for and what for

A

international normalised ration - used in DVT

133
Q

what kind of malignacny is myeloma

A

malignancy of plasma cells – progressve BM failure

134
Q

what does proliferation of plasma cells in myeloma cause proudction of

A

monoclonal immunoglobins (IgG or IgA)

135
Q

consequence of monoclonal immunoglobins

A

increased infection as no variation

136
Q

which more common type of immunoglobin

A

IgG (55%) IgA (20%)

137
Q

CF’s of myeloma

A

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
Q

which has pepperpot skull and lytic lesions

A

myeloma

139
Q

condition in which Bence Jones protein deposition

A

myeloma

140
Q

Dx of myeloma

A

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
Q

what is shown by bloods of myeloma

A 
low Hb (anaemia)
low antibodies 
high calcium (bone resorption)
high creatine (renal impairment)
142
Q

why is there renal impairment in myeloma

A

deposition of light chain paraproteins in the kidney

143
Q

how does myeloma cause anaemia

A

prolif of plasma cells in bone marrow = crowding out therefore decrease in no of RBC produced = anaemia

144
Q

what effect does myeloma have on bone activity

A

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
Q

2 types of lymphoma

A

hodkins and non hodgkins

146
Q

what is hidgkins lymphoma

A

malignancy of mature lymphocytes - w reed and sterberg cells

147
Q

what is the causes of hodgkins lymphoma

A

unknown - assos with Epstein Barr Virus and immunosupression (HIV, transplants etc)

148
Q

what is haematological condition is assos w Epstein barr virus

A

hodkins lymphoma

149
Q

what is ann arbor staging system used for

A

hodgkins lymphoma

150
Q

stage 1 ann arbor

A

single lymph node region

151
Q

stage 2 ann arbor

A

2+ lymph node regions SAME SIDE diaphragm

152
Q

stage 3 ann arbor

A

2+ lymph node region BOTH SIDES diaphramg (may involve spleen)

153
Q

stage 4 ann arbor

A

diffuse extra lymphatic disease (liver, bone marrow, lungs..)

154
Q

A or B in ann arbor meaning

A

A - absence of B symptoms

B - presence of B symptoms

155
Q

age distrib of hodgkins lymphoma

A

bimodal - young and elderly

156
Q

CF of hodgkins lymphoma

A 
B - symptoms 
painless enlarged non tender rubbery nodes - lymphadenopathy = worse with alcohol
cough (if compresses SVC)
hepatosplenomegaly 
anaemia symptoms (if BM infiltration)
157
Q

what are B symtpoms

A

weightloss, pyrrexia, night sweats, lethargy

158
Q

in what disease does alcohol make lymphadenopathy painful

A

hodkins lymphoma

159
Q

what is found characteristally in hodgkins but not non hodgkins lymphoma

A

reed steinberg cells

160
Q

what are reed steinberg cells

A

mirror image nuclei in lymph node biopsy

161
Q

Dx of hodgkins lymphoma

A

lymph node biopsy = reed sternberg cells
FBC - increased ESR (infection), Hb decreased
CXR - mediastinal widening if mass

162
Q

Non hodgkin lymphoma sub types based on cell

A

B cell (80%) and T cell (20%)

163
Q

classification of types of nonhodgkins lymphoma

A

v HIGH grade - Burkitts lymphoma
HIGH - diffuse large B cell lymphoma
LOW - follicular lymphoma

164
Q

CFs of non hodgkins lymphoma

A 
varied - 
superficial lymphadenopathy
systemic b symptoms
compression
Panctyopenia - and assos symtpoms (BM infiltration)
165
Q

Cx of non hodgkins lymphoma

A

H. pylori in MALT (mucosa assos lmphoid tissue) - in stomach
HIV
genetic - Wiskott aldrich syndrome
immunosuppressive drugs - (SLE, rheumatoid arthritis)

166
Q

what is burkitts lymphoma assos with

A

Epstein barr virus

167
Q

Mx of low grade non hodgkins

A

chemo - rituximab NO CURE (SE:fever, rigors)

168
Q

Mx of high grade non hodgkins

A

chemo/radiation 30% CURE RATE

169
Q

which has worse prognosis low or high grade non hodkgins

A

low has no cure

170
Q

what drug is used to manage low grade non hodgkins lymphoma and SE

A

rituximab - se=fevor, rigor

171
Q

what is assos w reed steinberg cells

A

hodgkins lymphoma

172
Q

what is assos with philadelphia chromosome

A

chronic myeloid leukaemia (CML)

173
Q

what is a risk of chemo

A

tumour lysis syndrome

174
Q

what is tumor lysis syndrome

A

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
Q

what is commonly given alongside chemo and why (as a preventative)

A

allopurinol - to prevent tumor lysis syndrome

176
Q

which leukaemias does having downs syndrome increase the risk of

A

AML (before 5yo)

ALL (over 5yo)

177
Q

which leukaemia is predominantly a child malignancy

A

ALL acute lymphoid leukaemia

178
Q

which leukaemia is assos with ionising radiation

A

CML chronic myeloid leukaemia

179
Q

what is seen on the FBC of non hodgkins lymphoma

A

panctyopenia

180
Q

what is given to prevent tumor lysis syndrome

A

allopurinol

181
Q

what is allopurinol and what does it prevent

A

prevents turmour lysis syndrome and gout (think crystals)

182
Q

what can cause a GI bleed leading to iron defiency anaemia

A

hookworm

183
Q

which leukamia is a disease of the elderly

A

AML, CLL

184
Q

leukamia of middle age/elderly

A

CML

185
Q

what is CML specifically assosiated with

A

philadelphia chromosome

186
Q

what is the philadelphia chromosome

A

translocation 9;22 causing BCR-ABL gene fusion - protein activates thyrosine kinase causing increase myeloid division - increase chance of mutations

187
Q

symptoms of CML

A

sweating weight loss fever abdo pain

188
Q

signs of CML

A

anaemia, hepatosplenomegaly (feel full)

lymphadenopathy if blast crisis - AML

189
Q

what would you see on blood film in CML

A

mature myeloid precursors

190
Q

FBC of CML

A 
low Hb (normochromic normocytic anaemia)
high WCC
191
Q

Mx 1st line of CML

A

IMATINIB - tyrosine kinase inhib if Ph+

192
Q

Mx 2nd line of CML

A

dasatinib, nilotinib

193
Q

Mx 3rd line of CML

A

chemo - if acute

194
Q

Mx of CML if young

A

stem cell transplant - only CURE

195
Q

what is Imatinib and when is it used

A

a tyrosine kinase inhib used in CML if philadelphia chromosome positive

196
Q

what would cytogenetic test be used to show in CML

A

if philadelphia chromosome positive (BCR-ABL gene fusion)

197
Q

what is acute myeloid leukaemia a disease of

A

myeloid progenitor cells

198
Q

which is the commonest leaukaemia of adults

A

acute myeloid leukaemia

199
Q

what are the 3 types of AML

A

monoblast AML,
megakaryoblast AML,
erythroblast AML

200
Q

what age does downs increase risk for AML

A

pre 5yo

201
Q

why is DIC a complication of AML

A

retinoic acid receptor dysfunction causes build up of promyelocytes = lots of AUER RODS increasing coag risk

202
Q

what are clincial features of AML

A

gum hypertrophy
anaemia infection bleeding (BM marrow failure)
hepatosplenomegaly

203
Q

investigations in AML

A 
clotting screen (DIC)
BM aspirate (blast cells - auer rods in cytoplasm)
204
Q

what would you see in BM aspirate of AML

A

auer rods in myeloblast cells

205
Q

is progression of AML fast or slow

A

fast

206
Q

tx of AML

prognosis good or bad?

A

chemo (w breaks for bone marrow regeneration)
BM transplant
Supportive (i.e anticoag etc)
POOR PROGNOSIS

207
Q

what causes swollen gums in AML

A

build up of monoblasts causing gum hypertrophy

208
Q

what is CLL

A

chronic malignancy of mature lymphoid cells (mainly B)

=accumulation of B cells that have escaped apoptosis

209
Q

who is most likely to get CLL

A

men, elderly,fam history of ALL and CLL

210
Q

risk factor of CLL

A

family history of ALL and CLL

211
Q

what is characteristic of CLL on blood film

A 
smudge cells (fragile membrane of abnormal lymphocytes disrupted)
lymphocytosis
212
Q

clincial feature of CLL

A

lymphadenopathy, hepatospenomegaly, LUQ discomfort (big spleen), “rubbery enlarged non tender nodes”

213
Q

staging of CLL

A

Binet and rai -
less than 3 enlarged = A
more than 3 enlarged = B
anaemia &/or thrombocytopenia = C

214
Q

rule of thirds in CLL

A

1/3 dont progress
1/3 slow
1/3 acute

215
Q

Tx of CLL

A

chemo and radio ( radio to decrease lymphadenopathy and splenomegaly)
rituximab (SE=fever)

216
Q

SE of ritxumab and when used

A

in NON hodgkins lymphoma and in CLL - SE = nausea

217
Q

ALL is a malignancy of what cells

A

lymphoid progenitor cells (B and T cells)

arrests maturation = uncontrolled proliferation of immature b and t cells

218
Q

ALL RFs

A

downs (5yo plus)

ionising radiation

219
Q

symptoms of ALL

A

bone pain, infections (neutropenia)/fever, bleeding, headache, CN palsies, bruising

220
Q

Dx of ALL

A

lumbar puncture - cns involvment?
FBC - low RBCs low Platelets
Blood film - BLAST CELLS

221
Q

what do blasts cells look like on blood film of ALL

A

bigger, immature nucleus

222
Q

signs on ALL

A

lymphadenopathy
hepatosplenobegaly
CNS involvment

223
Q

Cure rate of ALL

A

70-90% children

40% in adults

224
Q

Mx of ALL

A

chemo (+ ALLOPURINOL (TLS)) then consolidation then BM tranplant and steroids
prophyalxis antibiotics as neutropenia

225
Q

what is thrombocytopenia

A

reduction of platelets

226
Q

2 types of thrombocytopenia

A

immune thrombocytopenic purpura (ITP)

thrombotic thrombocytopenic purpura (TTP)

227
Q

general causes of thrombocytopenia

A

a decrease in production in BM
an increase in platelet destruction
platelet sequestration in an enlarged spleen

228
Q

what regulates platelet formation

A

TPO (thrombopoeitin)

229
Q

what are platelets

A

megakaryocyte fragments (anucleate)

230
Q

what can cause a decrease in platelet production

A

bone marrow failure, myeloma, aplastic annaemia, hiv

231
Q

what can cause XS destruction/increased platelet consumption

A

immune thrombocytopenic purpura,
heparin
DIC
SLE

232
Q

causes of sequestraion of platelets

A

splenomegaly
hypersplenism
thrombotic thromboyctyopenic purpura

233
Q

which is more common ITP or TTP

A

ITP

234
Q

what is ITP

A

immune destruction of platelet cells

235
Q

what is TPP

A

etensive microvascular clots in small blood vessels - organ damage and low platelets

236
Q

causes of ITP

A

immune - post viral infection or immunisation - PRIMARY

malignancies and HIV - SECODNARY

237
Q

Cf’s of ITP

A

easy bruising
purpura
epistaxis
menorrhagia

238
Q

CF’s of TPP

A 
easy bruising
purpura
epistaxis
menorrhagia
CEREBRAL DYSFUNCTION
239
Q

who is more likely to get ITP acute onset posst viral infection?

A

children

240
Q

diagnosis of ITP and TPP (and one difference)

A

low platelets, high megakaryocytes,

ITP has platelet antibodies

241
Q

Tx of ITP

A 
oral corticosteroids (immunosupp)
splenectomy
tranexamic acid (inhib firbin breakdown)
242
Q

Tx of TTP

A

urgent plasma exchange (replace ADAMTS 13 and remove antibody)
immunosuppression
NOT PLATELETS

243
Q

what do u not give in TTP

A

platelets

244
Q

what causes TTP

A

inhib of enzyme that breaks down clots by breaking down vWF

245
Q

what is deep vein thrombosis

A

thrombus formation in vein

246
Q

RF of DVT

A

immobiliation
obesity
long haul flight
pregnancy

247
Q

CFs of DVT

A

calf pain (warm, swelling, tender)
ankle oedema
pitting oedema
Homans sign - pain on dorsiflexion

248
Q

Dx of DVT

A

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
Q

what score is assoc with DVT

A

Wells score

250
Q

prevention of DVT

A

compression stockings

251
Q

Mx of CVT

A

low molecular weight heparin
warfarin
anticoag

252
Q

aim of DVT Mx

A

prevent pulmonary embolism

253
Q

how does LMW heparin work

A

inactivates factor Xa (prothrombin to thrombin)

254
Q

why LMW heparin not normal

A

more effective monitoring therefore less bleed risk

255
Q

how does warfarin work as an anticoag

A

antagonises vit K dep clotting factors 10 9 7 2 (1972)

256
Q

what is target INR for DVT mx (what does it stand for?)

A

2.0-3.0 (international normalised ratio)

257
Q

what is polycythaemia

A

increased Hb, packed cell vol and RBCs = thicker blood and increased clotting

258
Q

what is the gene mutation associated with polycythaemia rubra vera

A

JAK2 gene mutation - excessive proliferation of progenitors

259
Q

what are causes of primary polycythaemia

A

increased sensitivity to EPO so increased RBC

260
Q

what cause of secondary polycythaemia

A

more circualting EPO

261
Q

Cfs of polycythaemia rubera vera

A 
plethoric appearance (red face)
thrombosis
headache
itching (POST HOT BATH)
vertigo
262
Q

what is the cause of primary famililal and congen polycythaemia

A

EPOR gene mutation so receptor activated for longer

263
Q

what is a cause of secondary polycythaemia

A

chronic hypoxia
poor oxygen delivery (altitude)
abnormal RBC struct
tumours secreting EPO - adrenal, renal cell carcinoma

264
Q

Cf’s of secondary polycythaemia

A 
asympt
easy bleeding bruising, 
gout (as increased cell turnover)
fatigue dizziness headaches
palpable spleen and liver
265
Q

Mx of polycythaemia rubra vera

A

aspirin (reduce clotting as thick blood)

and venesection

266
Q

Mx of secondary polycythaemia

A

tx cause

267
Q

dx of polcythaemia

A

FBC- haemocrit level
BM biopsy
genetic testing - JAK2 gene

268
Q

complications of polcythaemia

A

thrombosis as thicker blood = slower

haemorrhage

269
Q

what is apparent polycythameia

A

normal rbc count but lower plasma vol (hypovolaemia) so raised PCV (packed cell vol)

270
Q

what type of polycythaemia has normal rbc count but hypovolaemia

A

apparent polycythaemia

271
Q

chronic hypoxia, EPO secreting tumours and altitude are causes of what kind of polycythaemia

A

secondary = more circulating EPO

272
Q

plethoric appearance, itching POST HOT BATH - which condition buzz words

A

Polycythaemia rubra vera

273
Q

what does DIC stand for

A

disseminated intravasc crisis

274
Q

what is DIC

A

out of control haemostasis -

generation of fibrin within BVs and coag factor/platelet consumption leading to secondary activation of fibrolysis

275
Q

when does DIC occur

A

sepsis, malignancy, trauma, intravasc haemolysis

276
Q

patho of DIC

A

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
Q

Dx of DIC

A

D-dimer
increased FDPs (fibrinolytic activity)
PTT prolonged
low platelets

278
Q

how to treat DIC

A

underlying cause
maintain blood vol (tranfusions etc)
activated C protein

2a RRS (26 decks)

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