Haem Laz Flashcards
1
Q
What do multipotent stem cells differentate into
A
- common myeloid progenitor
- common lymphoid progenitor
2
Q
what do commoon myeloid cells differentiate into
A
MYELOBLASTS (monocytes, neutrophils, basophils, oesinophils)
ERYTHROCYTES
MEGAKARYOCYTES
3
Q
What do common lymphoid progenitor differentiate into?
A
T cells
B cells > plasma cells
4
Q
What cells does AML affect
A
Common myeloid progenitor and myeloblast
5
Q
What cells does CML affect
A
Monocytes, neutrophils, basophils, oesinophils
6
Q
What cells does ALL affect
A
Common lymphoid progenitor
7
Q
What cells does CLL affect
A
B cells, T cells
8
Q
Summarise AML presentation
A
Adults
BM failure (pancyctopoena)
acute onset
Auer rods
9
Q
Summarise ALL presentation
A
Children
BM failure
Failure to thrive
10
Q
Summarise CML presentation
A
Adults
t(9,22) transolcation - PHIILADELPHIA CHROMOSOME
Often incidental finding, occasionally BM failure
Tx IMATINIB
11
Q
Summarise CLL presentation
A
Adults
Often incidental finding, occasionally BM failure
SMUDGE CELLS
12
Q
What does MAHA stand for
A
Microangiopathic Haemolytic Anaemia
13
Q
WHat is MAHA
A
MECHANISM NOT DISEASE
RBC breakdown in small vessels
14
Q
What does HUS stand for
A
Haemolytic uraemic syndrome
15
Q
What is HUS caused by
A
E coli 0157:H7 which produces shiiga like toxins
this causes endothelial injury in glomerular vessels
leading to platelet plug formation > THROMBOCYTOPOENIA
RCs destroyed as they try to get past > MAHA
Poor kiidney perfusion > renal failure
16
Q
What is the triad of HUS and what othher Sx may occur
A
HUS: MAHA + thrombocytopoenia + renal failur
+ diarrhoea (due to e coli)
17
Q
What is TTP
A
Thrombotic thromobocytopoenic purpura
18
Q
What occurs in TTP
A
ADAMTS 13 is reduced (unkown cause, cancer, pregnancy)
AdamTS13 is usually used to break dowon vWF multimers
This means that there are excessve vWF multipmers > form platelet plug > platelet consumton, RC destruction, poor end organ perfusion
19
Q
Sx of TTP
A
MAHA + Thrombocytopoenia + renal failure
+ RASH + CONFUSION (due to reduced brain perfusion)
20
Q
What is DIC caused by
A
increased exposure to tissue factor, such as in
- pregnancy
- sepsis
- tumour
- pancreatitis
21
Q
What does DIC stand for, and what occurs
A
Disseminated INtravascular Coagulaton
increased exposure to tssue factor
activation of clotting cascade
- increased platelet consumption > THROMBOCYTOPOENAI
- increased coag factor consumption > decreased coagulation factors
22
Q
What is multiple myeloma
A
Cancer of plasma cells
resulting in excessive monoclonal Ig production
23
Q
What is the progression of monoclonal Ig disease
A
- MGUS
- Smouldering myeloma
- MM
- B cell leukaemia
24
Q
how does MGUS present
A
NO CRAB SX
- M protein <30g/L
- BM plasma cells <10%
25
how does smouldering myeloma present
M protein >30g/L
BM plasma cells >10%
annual risk of progression to MM is 10%
no CRAB SX
26
How does mutliple myeloma present
CRAB SX
M protein >30g/L
>60% plasma cells in BM
>1 focal lesion on MRI
27
What symptoms occur with MM
```
CRAB
calcium elevared
renal failure
anaemia
bone lesions (lytic)
```
28
what Ix myst you get in MM
1. FBC, UE, bone profile, ESR, serum Ca
2. Serum / urine electrophoresis
3. Whole body low dose CT
4. Bone marrow aspirate and biopsy
29
What does Serum / urine electrophoresis show in MM
shows a single monoclonal band (MONOCLONAL GAMMOPATHY)
30
what proteins are present in MM
Bence Jones proteins
31
what is myelodysplasia
abnormal differentiation of cells along myeloid line
| this causes a BM disorder with PANCYTOPOENIA and FUNCTONALLY IMMATURE CELLS
32
what is myelofibrosis
clonal BM disorder characterised by fibrous scar tissue deposition
overexpansion of clone in BM > fibrous scar tissue deposits in reesponse
>> PANCYTOPOENIA, TEAR DROP CELLS; DRY TAP; MASSIVE SPLENOMEG to compensate for low RBC
33
causes of MICROCYTIC anaemia
TAILS
```
Thalassaemia
Anaemia of Chronic Disiease
Iron Deficiency anaemia
Lead poisoning
Sideroblastic anaemia
```
34
causes of NORMOCYTIC anaemia
```
Iron deficiency (early)
Chronic disease (early)
Marrow failure
Renal failure
Aplastic anaemia, Acute blood loss
Leukaemia, Myelofibrosis
```
35
causes of MACROCYTIC anaemia
Alcoholics May Have Liver Failure
```
Alcoholism
Myelodysplastic sydrome, MM
Hypothyroid, haemolytic anaemia
Liver Failure
Folate / B12 def
```
36
what size are RBC in haemolytic anaemia and why
MACROCYTIC or normocytuic
37
what are causes of haemolytic anaemia
INHERITED: defect of RBC which cauze them to be broken down
- membrane (hereditary spherocytosis)
- cytoplasm / enzyme (G6PD deficiency)
- Haemoglobin (SCD or thalassaemia)
Aquired (defect in environment the RBC are in, either immune or non immune mediated)
38
why must you be careful measuring ferritin in IDA?
if no illness, fair to check
| if ill - be aware that FERRITIN is an ACUTE PHASE PROTEIN so check TIBF, TTF, iron instead
39
what are the two types of immune-mediated anaemia
AIHA (warm and cold)
40
What test can you do for AIHA
Coomb's test / direct antiglobulin test
41
Explain features of WARM AIHA
GHANA: IgG, 37 deg
spherocytes
outside of europe: EXTRAvascular haemolysis
42
explain features of COLD AIHA
Mountain: IgM
under 37 deg
inside europe: INTRAvascular
43
what causes tumour lysis syndrome
recent chemotherapy, causing destruction of lots of cells
44
what are electroolytes like in tumour lysis syndrome
HIGH K+
hgh phosphate
low calcium
45
what does positive clinical TLS require
At least one of the following.
- >1.5x ULN creatinine
- cardiac arrythmia / sudden death
- seizure
46
Mx tumour lysis syndrome
```
IV allopurinol (if high risk)
PO allopurinol (if low risk) - can be given prophylactically
```
47
when do you ned to transfuse packed RBC
No ACS : for Hb <70
| ACS: Hb <80
48
what level do you need to maintain platelets at for:
- pre-procedure
- pre-procedure if surgery at critical site
- no active bleedsing / planned surgery
- pre-procedure: >50
- pre-procedure if surgery at critical site >100
- no active bleedsing / planned surgery >10
49
what is a THROMBOPHILIA
LOVES THROMBI > propensitiy to develop clots
50
causes of thrombophilia - inherited
Factor V Leiden
Prothrombin gene mutation
Protein C/S deficiency
Antithrombin III deficiency
51
causes of thrombophilia - aquired
antiphospholippid syndrome
| Drugs (COCP)
52
what is heparin inducted thrombocytopoenia (HIT)
a PROTHROMBOTIC condition
53
how does HIT ooccur
AB form against complexes of platelet factor 4 and hepatin > induce platelet activation > low platelets but LOTS OF THROMBI FORMATION
54
Classic HIT presentaton
>50% reduction in platelets
Thrombosis
Skin allergy
55
How do you manage HIT
STOP HEPARIN, chaange to Argatroban (thrombin inhibitor)
56
what category are anti D antibodies
IgG (dont cause direct agglutination - cause a delayed haemolytic transfusion reaction instead)
57
what are antibodies against A, B on blood
IgM against normal RBC antigens
| IgG against atypical RBC antigens
58
List acute transfusion reactions (<24h)
```
Acute haemolytic reactions (ABO incompatibility)
Allergic / anaphylactic
Infection (bacterial)
Febriile non haemolytic reaction
Respiratory (TACO, TRALI)
```
59
what occurs in sickle cell anameia
defective beta globin gene (glutamine to valine), which leads to abnormal folding of RBC into sickle shape
60
What is mode of inheritance of sickle cell
AR
61
what occurs in thalassaemiai
reduced haemoglobin synthesiis
62
which type of malaria is most fatal
Plasmodium falciparum
63
what other types of less fatal malaria exist
Plasmodium vivax
ovale
knowlesi
64
describe symptoms of P falciparum
```
cyclical fevers (every 48h)
splenomegaly
neuro involvement (altered GCS and seizures)
DV
metabolic acidosis
shock
```
65
how do you treat malaria falciparum
mild: oral MALARONE / artemisin combination therapyu
severe: IV Artesunate
66
how do you treat other types of malaria
chloroquine
67
classical general S/S of malaria
```
onset 7-10 days after inoculation
fever (cyclical or continuous with spikes)
DV
flu like sx
jaundice
anaemia
drowsiness, confission
```
68
how do you investigate for malaria
3 thick and thin blood films (thick: presence; thin: species)
Malaria rapid antigen detection test
69
How do you prevent malaria
Quinine prophylaxis
70
typhoid fever cause
salmonella typhi / paratyphi
71
how is typhoid transmitted
faeco oral
72
sx typhus
```
fever
bradycardia
headache
dry cough
weight loss, anorexia
GI sx (diarrhoea or constipation)
```
73
classical derm presentation of typhuss
rose spots
74
Mx typhus
IV ceftriaxone
75
where is typhus still found
pakistan
india
bangladesh
76
organism that causes dengue fever
arbiirus flavivirus - aedes aegiptii mosquito
77
sx dengue
short incubation period
Primary infection: headache, sunburn rash, fever and myalgia
Secondnary infection: DENGUE HAEMORRHAGIC FEVER, very unwell with hypotennsion and massive haemorrhages
78
how do you treat dengue
supportive (fluids and monitoring) >ITU
79
what are 'Tear-drop' poikilocytes found in
myelofibrosis
80
what is ITP
Immune Thrombodytopoenia
essentially antibodies against GLPIIb/IIIa > thrombocytopoenia
81
how does ITP present
petechhiae, purpura, bleeding
82
how do you manage ITP
oral pred
| IVIG
83
how does acute haemolytic reaction present
DURING/RIGHT AFTER TRANSFUSION
- fever
- abdo pain
- hypotension
84
how do you confirm an acute haemolytic reaction
COOMBS +ve
85
How do you manage acute haemolytic reaction
- stop transfusion
| - fluid resus
86
what is the most common BLEEDING DISORDER
von Willenbrand isease
87
mode of inheritance of vWD
AD
88
what is the normal role of vWF
to promote platelet adhesion to damaged endothhelium
89
types of vWD
1. Reduced quantity vWF
2. poor quality vWF
3. total lack vWF
90
mx neutropoenic sepsis
Tazocin
91
what drughs cause haemolysis in G6PD deficiency
Ciprofloxacin
| S drugs: sulphoonamides, sulphonylurea, sulphasalazine
92
how do you confirm G6pD deficiency
G6PD levels now and in 3 months
93
what transfusion reaction is someone with IgA deficiency more at risk of
ANAPHYLACTIC REACTION (because they are more likely to have anti-IgA that attack the donor blood)
94
which type of vWD are AD and which are AR?
AD: type 1 and 2
AR: type 3 (total lack vWF)
95
what do ix for vWF reveal
prolonged APTT
| prolonged bleeding time
96
how can you manage vWF
tranexaminc acid, desmopressin (which induces vWF release), F8 concentratee
97
important ix in MM and their findinga
- serum / urine electrophoresis (shows monoclonal gammopathy of IgG or IgA and Bence-Jonees protein)
- FBC, UE, Ca, bone profile, blood film, ESR
- bone marrow aspirate and biopsy
- whole body CT/MRI
- X ray head
98
what does X ray head show iin MM
a RAINDROP SKULL (similar to pepper pot skull in 1HPTH)
99
sx of polycythaemia
hyperviscosity: headache, light headedness, visual changes, fatigue, dyspnoea
histamine release: aquagenic pruritus, peptic ulceration, splenomegaly
100
What are blood findings on polycythaemia
Raised Hb and Htc
101
what mutation is iportant in distinguishing the cause of polycythaemia
JAKV617F +ve = polycythaemia vera
| JAKV617F -ve = True polycythaemiia (hypoxia, renal disease, tumour) or pseudopolycythaemia (dehydration)
102
what does polycythaemia vera risk progressing to
myelofibrosis
| AML
103
mx polycythaemia
venesection
| aspirin (to reduce risk of thrombosis)
104
what is the FASTEST reaction to blood transfusion that can occur
anaphylactic reaction (SOB, wheeze, facial oedema. IMMEDIATE)
or allergic (may be mild - just pruritic rash)
105
what is the decond fastest reaction to blood transfusion that can occur
Acute haemolytic reaction(due to ABO incompativiliy, IgM mediagted) - within mins
106
sx Acute haemolytic reaction
1. Fever
2. Abdo pain
3. low BP
107
how do you mx Acute haemolytic reaction
stop transfusion
| fluid resus
108
how does febrile non haemolytic transfusion reaction pesent
rise in temp by 1 degree without circulatory collapse
| +- chilld and rigors
109
how do you manage febrile non haemolytic transfusion reaction
stop or slow transfusion, give paracetamol
110
what kind of transfusion is most likely to cause bacterial contamination
platelet transfusion
111
how do you manage TACO
IV furosemide
slow / stop transfusion
oxygen
112
how do you manage TRALI
stop transfusion, supportive
113
how doees a delayed type haemolytic transfusion reeaction present
same as acute but milder
| occurs within one week of transfgusion
114
how does GvHD present
within one week of transfusion
- rash
- skin desquamation
- diarrhoea
- fever
115
causes of MASSIVE SPLENOMEGALY
```
myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher's syndrome
```
116
how do you remove the risk of GvHD and what patients need this adaptation
by IRRADIATING
patients with hodgkin /immunocompromised/neonates <28days
117
what patients need CMV neg blood
intrauterine / neonates / preg
118
what do you give in pts with IDA who cannot tolerate oral iron / time interval is too short
give IV iron 1g
| repeat 1 week later
119
how does ITP present
petechiae
purpura
bleeding q
120
how do you manage ITP
Oral pred
| IVIG
121
what are the four types of hodgkins lymphoma
Nodular sclerosing
Mixed cellularity
lymphocyte predominant
lymphocyte depleted
122
what cells are classical of hodgkins and what do they looi like
```
REED STERNBERG (binucleated Owl's eye cells)
= large multinucleate cells with prominent eosinophilic nucleoli.
```
123
what is the staging system of hodgkins
ANN ARBOUR
124
explain ann arbour staging
1: 1 site on one side of the diaphragm
2: >1 site, on one side of diaphragm
3: both sides of diaphragm
4: BM, lungs, liver involvement
125
what are B symptoms in hodgkins
fever >38
night sweats
unintentional WL >10% body weight in 6 months
126
what do B symptoms suggest in terms of prognosis
POOR
127
how do you manage an allergic reaction to the transfusion
stop the transfussion
| administer antihistamine
128
which type of hodgkins has the best prognosis
Lymphocyte predoiminant
129
what part of the intestine is iron absorbed in
in the DUODENUM
130
what part of the intestine is folate absorbed in
JEJUNUM
131
what part of the intestine is B12 absorbed in
ILEUM
132
what vitamin will someone with al ilieocaecal resection from chrons be deficient in
B12
133
blood findings for EBV glandular fever
RAISED lymphocytes, no neutrophils
134
sx glandular fever
fatigue
| recurrent tonsillitis
135
how is hodgkins different to NHL
Hodgkin's lymphoma has:
- alcohol-induced pain in the node
- 'B' symptoms typically occur earlier
- Extra-nodal disease rare
136
how do you invesitgate lymphoma
- FBC, CRP ESR, blood film, LDH
- exisional node biopsy
- CT chesst abdo pelvis (staging)
- HIV test
137
what is the most aggressive NHL
Burkitt's
138
who does Burkitts occur in
young immunosuppressed
| EBV and HIV associations
139
what are high grade NHL
DLBCL and mantle cell lymphoma
140
what is the MOST COMMON type of lymphoma
DLBCL
141
what virus is DLBCL asociated with
EBV
142
types of low grade lympha
follicluar
malt
SLL/CLL
143
how do you treat CML
Imatinib (tyrosine kinase inhib)
144
what lymphoma has a starry sky appearance on LN buopsy
Burkitts
145
what findings does waldenstrom's macroglobilinaemia have
monoclonal IgM paraproteinaemia
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance
the pentameric configuration of IgM increases serum viscosity
hepatosplenomegaly
lymphadenopathy
146
how must you manage beta thalassaemia major
LIFELONG BLOOD TRANSFUSIONS
| + desferroxamine to avoid iron overload
147
what electrolyte abnormality may occur following transfusion of large volume RBC
HYPERKALAEMIA (because packed RBC have high potassium)
148
what is the most common inherited thrombophilia, and what is pathophy
FACTOR V LEIDEN (heterozygous)
| -- due to resistance to Protein C
149
what does the presence of band cells indicate
CML
150
what kind of leukaemia does LOW LYMPHOCYTES point to
AML or CML
151
what is sideroblastic anaemia
inability to form haem > form ring sideroblasts instead
152
causes of sideroblastic anaemia
```
congenital
myelodysplasia
alcohol
lead
anti TB meds
```
153
ix in sideroblastic anaemia
```
FBC (hypochromic microcytic)
iron studies (raised ferritin, raised iron, raised transferrin sat)
blood film (Basophilic stippling)
BM staining (ring sideroblasts)
```
154
what is aplastic anaemia
pancytopoenia + hypoplastic BM
155
causes of aplastic anaemia
congenital
- Fanconi
- dyskeratosis congenita
drugs
- cytotoxics
- chloramphenicol
- sulphonamides
- phenytoin
viral
- parvovirus
- hepatitis
156
which pathway does PT measure
the EXTRINSIC PATHWAY (i.e. the short one, activated by tissue factor)
157
which pathway does APTT measure
the INTRINSIC PATHWAY (long)
158
what factors are involved in the INTRINSIC PATHWAY
12, 11, 9, 8, 10
159
which factors are involved in EXTRINSIC PATHWAY B
tissue factor
| F7
160
explain the common pathway
PROTHROMBIN converts to THROMBIN (by F10a)
FIBRINOGEN converts to FIBRIN (by thrombin)
FIBRIN cross links the clot
161
what are causes of prolonged PT only (i.e. issue is in the extrinsic pathway)
inherited (F7 deficiency)
| Aquired (mild viit K, liver disease, warfarin, DIC)
162
what are causess of prolonged APTT ONLY (issue in intrinsic pathhway)
deficiency of any of the factors invlved
| vWD
163
causes of prolonged PT AND APTT
deficiency of fibrinogen, prothrombin, F5, F10
liver disease
DIC
anticoagulants
164
what is heparin MoA
inhibits antithrombin (whhich blocks thrombin)
165
how many globin chains is each Hb made up of
4 globin chains
166
what are the types of Hb and which globin chains do they contain
```
HbF= 2 alpha, 2 gamma (foetal, drops at 3m of life)
HbA = 2alpha, 2 beta (adult)
HbA2 = 2alpha, 2 delta (small portion of adult)
```
167
what occurs in beta thalassaemia
beta globin deficiency !! > free alpha chains form inclusions > haemolysis of RBC
haemolysis means
- raised bilirubin > jaundice
- raised iron > haemochromatosis
increased RBC production to compensate > HEPATOSPLENOMEGALY
168
what sx do you get with beta thalassaemia then
jaundice, haemochromatosis
| hepatosplenomegaly
169
what are blood findings of thalassaemia
microcytic anaemia
target cells
raised iron
170
what are the combinations of beta blobin mutations you can get in thalassaemia
```
beta+ = less than normal
beta0 = no beta chains
```
171
what are the types of beta thalassaemia
beta thalassaemia major = beta0beta0
beta thal intermedia ? beta+beta+
beta thal minor = beta+beta or beta0beta
172
how does beta thalaasaemia present on electrophoresis
low HbA
| raised HbF, HbA2
173
what type of thalassaemia is incompativle with life
alphha thalassaemia major = Hb Barts (x4 alpha globin gene deletion) > hydrops fetalis, death in utero
