Haem Laz Flashcards
(205 cards)
1
Q
iron deficiency anaemia blood film
A
target cells
‘pencil’ poikilocytes
2
Q
myelofibrosis blood film
A
tear-drop poikilocytes
3
Q
What do multipotent stem cells differentate into
A
- common myeloid progenitor
- common lymphoid progenitor
4
Q
what do commoon myeloid cells differentiate into
A
MYELOBLASTS (monocytes, neutrophils, basophils, oesinophils)
ERYTHROCYTES
MEGAKARYOCYTES
5
Q
What do common lymphoid progenitor differentiate into?
A
T cells
B cells > plasma cells
6
Q
What cells does AML affect
A
Common myeloid progenitor and myeloblast
7
Q
What cells does CML affect
A
Monocytes, neutrophils, basophils, oesinophils
8
Q
What cells does ALL affect
A
Common lymphoid progenitor
9
Q
What cells does CLL affect
A
B cells, T cells
10
Q
Summarise AML presentation
A
Adults
BM failure (pancyctopoena)
acute onset
Auer rods
11
Q
Summarise ALL presentation
A
Children
BM failure
Failure to thrive
12
Q
Summarise CML presentation
A
Adults
t(9,22) transolcation -** PHIILADELPHIA CHROMOSOME**
Often incidental finding, occasionally BM failure
FLAWS
splenomegaly (abdo discomfort)
13
Q
Summarise CLL presentation
A
- Adults
- Often incidental finding, occasionally BM failure
- non tender lymphadenopathy
- hepatosplenomegaly
- systemic symptoms: lethargy,malaise, weight loss, night sweats
- late stage –> features of BM failure (anaemia, easy brusing/bleeding, recurrent infections)
14
Q
what is richter’s transformation
A
when leukaemia cells enter the lymph node and change into high grade, fast growing non-Hodkin lymphoma
happens a lot in CLL
15
Q
ivestigations for CLL
A
- Bloods: FBC (high WCC, low: Hb, platelets, serum Ig, neutrophils ~ marrow infiltration), U+E, LFT, LDH
- blood film: SMUDGE CELLS (abnornally fragile lymphocytes)
- immunotypes: surface Ig, CD5, CD19, CD20, CD23
16
Q
What does MAHA stand for
A
Microangiopathic Haemolytic Anaemia
17
Q
WHat is MAHA
A
MECHANISM NOT DISEASE
RBC breakdown in small vessels
18
Q
What does HUS stand for
A
Haemolytic uraemic syndrome
19
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
20
Q
What is the triad of HUS and what othher Sx may occur
A
HUS: MAHA + thrombocytopoenia + renal failur
+ diarrhoea (due to e coli)
21
Q
What is TTP
A
Thrombotic thromobocytopoenic purpura
22
Q
reversal agent for warfarin
A
prothrombin complex (FFP if not available)
vitamin K
23
Q
reversal agent for dabigatran
A
Idarucizumab
24
Q
reversal agent for rivaroxaban and apixaban
A
andexanet alfa
25
What occurs in TTP
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
26
Sx of TTP
MAHA + Thrombocytopoenia + renal failure
+ RASH + CONFUSION (due to reduced brain perfusion)
27
What is DIC caused by
increased exposure to tissue factor, such as in
- pregnancy
- sepsis
- tumour
- pancreatitis
28
What does DIC stand for, and what occurs
Disseminated INtravascular Coagulaton
increased exposure to tssue factor
activation of clotting cascade
- increased platelet consumption > THROMBOCYTOPOENAI
- increased coag factor consumption > decreased coagulation factors
29
What is multiple myeloma
* **Cancer of plasma cells**
* genetic mutation as b cells differentiate into mature plasma cells--> excessive monoclonal Ig production (**paraprotein** ~ IgG or IgA)
30
What is the progression of monoclonal Ig disease
1. MGUS
2. Smouldering myeloma
3. MM
4. B cell leukaemia
31
how does MGUS present
NO CRAB SX
- M protein <30g/L
- BM plasma cells <10%
32
how does smouldering myeloma present
M protein >30g/L
BM plasma cells >10%
annual risk of progression to MM is 10%
**no CRAB SX**
33
How does mutliple myeloma present
CRAB SX
M protein >30g/L
>60% plasma cells in BM
>1 focal lesion on MRI
34
What symptoms occur with MM
CRABBI
**Calcium** elevated (stones, bones, groans, moans)
**Renal** failure (dehydration/thirst. due to amyloidosis or renal stones)
**Anaemia** (BM crowding suppressing erthropoesis)
**Bone** pain and lesions (lytic)
**Bleeding** (BM crowding leads to thrombocytopenia)
**Infection** (Reduced normal Ig --> inc infection risk)
35
what Ix myst you get in MM
1. **FBC, U+Es, bone profile (Ca) , ESR, serum b2-microglobulin** (most important survival prognostic marker)
2. **Blood film** (rouleaux formation)
3.. Serum / urine electrophoresis (**bence jones protein** in urine)
4. Whole body low dose CT/**MRI**
5. **Bone marrow aspirate and biopsy**
36
What does Serum / urine electrophoresis show in MM
shows a single monoclonal band (MONOCLONAL GAMMOPATHY)
37
what proteins are present in MM
Bence Jones proteins
38
management of multiple myeloma
supportive for CRAb symptoms (bisphosphonates)
**induce remission + HSCT**
39
what is myelodysplasia
abnormal differentiation of cells along myeloid line
| this causes a BM disorder with PANCYTOPOENIA and FUNCTONALLY IMMATURE CELLS
40
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
41
causes of MICROCYTIC anaemia
TAILS
```
Thalassaemia
Anaemia of Chronic Disiease
Iron Deficiency anaemia
Lead poisoning
Sideroblastic anaemia
```
42
causes of NORMOCYTIC anaemia
```
Iron deficiency (early)
Chronic disease (early)
Marrow failure
Renal failure
Aplastic anaemia, Acute blood loss
Leukaemia, Myelofibrosis
```
43
causes of MACROCYTIC anaemia
*megaloblastic*
- **B12 /folate** deficiency
- Anti-folate drugs - **phenytoin, methotrexate**
- Cytotoxic drugs - **hydroxycarbamide**
*Normoblastic* (**RALPH**)
* **Reticulocytosis**
* **Alcoholism**
* **Liver disease**
* **Pregnancy**
* **Hypothyroidism**
* **Myelodysplastic sydrome**
44
what size are RBC in haemolytic anaemia and why
MACROCYTIC or normocytuic
45
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)
46
investigation for Hereditary spherocytosis
clincal diagnosis
EMA binding test
47
management for Hereditary spherocytosis
o Acute haemolytic crisis: **supportive treatment, transfusion** if necessary
o Longer term treatment: **folate replacement and splenectomy**
48
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
49
what are the two types of immune-mediated anaemia
AIHA (warm and cold)
50
What test can you do for AIHA
Coomb's test / direct antiglobulin test
51
Explain features of WARM AIHA
GHANA: IgG, 37 deg
spherocytes
outside of europe: EXTRAvascular haemolysis
52
explain features of COLD AIHA
Mountain: IgM
under 37 deg
inside europe: INTRAvascular
53
what is tumour lysis syndrome
cancer cells undergo rapid cell lysis -->metabolic and electrolyte abnormalities
54
what causes tumour lysis syndrome
recent chemotherapy, causing destruction of lots of cells
55
what are electroolytes like in tumour lysis syndrome
**HIGH POTASSIUM + HIGH PHOSPHATE + HIGH URATE + LOW CALCIUM**
56
complications of tumour ysis syndrome
* ↑Urea: **acute renal failure**
* ↑PO4: **nephrocalcinosis and urinary obstruction**
* ↑K: **cardiac arrhythmia**
* ↓Ca: **seizure, convulsions**
57
which cancers are at high risk of tumour lysis syndrome
* Haematological malignancies characterised by a high proliferating rate --> **Non-Hodgkin's lymphoma, ALL, AML**
58
scoring system for TLS
Cairo-Bishop scoring system
59
what does positive clinical TLS require
At least one of the following.
- >1.5x ULN creatinine
- cardiac arrythmia / sudden death
- seizure
60
management of tumour lysis syndrome
1. Urgent management of cardiac arrhythmia or seizure:
o **IV Calcium Gluconate 1g bolus
o ECG monitoring**
2. **IV fluid resuscitation ± Furosemide** (could worsen hyperuricaemia)
3. **IV Rasburicase 0.2 mg/kg bolus**
4. **PO Aluminium hydroxide**: 15-30 mL OD: phosphate binder
5. **Potassium correction**
6. **Renal Dialysis** if persistent HTN, severe acidosis or uremic encephalopathy
61
Prophylaxis treatment of tumour lysis syndrome
* if high risk: IV allopurinol or IV rasburicase (not together)
* if low risk: PO allopurinol
62
when do you ned to transfuse packed RBC
No ACS : for Hb <70
| ACS: Hb <80
63
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
64
what is a THROMBOPHILIA
LOVES THROMBI > propensitiy to develop clots
65
causes of thrombophilia - inherited
Factor V Leiden
Prothrombin gene mutation
Protein C/S deficiency
Antithrombin III deficiency
66
causes of thrombophilia - aquired
antiphospholippid syndrome
| Drugs (COCP)
67
what is heparin inducted thrombocytopoenia (HIT)
a PROTHROMBOTIC condition
68
how does HIT ooccur
AB form against complexes of platelet factor 4 and hepatin > induce platelet activation > low platelets but LOTS OF THROMBI FORMATION
69
Classic HIT presentaton
>50% reduction in platelets
Thrombosis
Skin allergy
70
How do you manage HIT
STOP HEPARIN, chaange to Argatroban (thrombin inhibitor)
71
what category are anti D antibodies
IgG (dont cause direct agglutination - cause a delayed haemolytic transfusion reaction instead)
72
what are antibodies against A, B on blood
IgM against normal RBC antigens
| IgG against atypical RBC antigens
73
List acute transfusion reactions (<24h)
```
Acute haemolytic reactions (ABO incompatibility)
Allergic / anaphylactic
Infection (bacterial)
Febriile non haemolytic reaction
Respiratory (TACO, TRALI)
```
74
what occurs in sickle cell anameia
defective beta globin gene (glutamine to valine), which leads to abnormal folding of RBC into sickle shape
75
What is mode of inheritance of sickle cell
AR
76
types of sickle cell crisis
**BISH AV**
* **Bone pain crisis** - microvascular occlusion to bones of fingers
* **Infarctive crisis**
* **Splenic sequestration crisis** -> Shock and severe anaemia
* **Haemolytic crisis**
* **Aplastic crisis** -> Parvovirus B19 infection -> huge drops in haematocrit
* **Vaso-occlusive crisis**- microvascular occlusion to any part of body
77
clinical features of sickle cell diseae
**anaemia, jaundice, infection**
SICKLED
* Splenomegaly -> sequestration crisis
* Infarction: stroke, spleen, AVN, leg ulcers, BM
* Crises: pulmonary, mesenteric, pain
* Kidney disease
* Liver, Lung disease
* Erection – priapism due to vaso-occlusion
* Dactylitis - often child's 1st presentation
78
how can vaso-occlusive crises present
o Hand-foot syndrome: swelling of feet and hands in infants
o Acute chest syndrome: chest pain, cough, wheeze, fever, hypoxia, tachypnoea
o Avascular necrosis of femoral head -> SEVERE HIP PAIN
79
how to treast sickle cell crisis
1. oxygen
2. IV fluids
3. IV analgesia (opiods)
80
what occurs in thalassaemiai
reduced haemoglobin synthesiis
81
which type of malaria is most fatal
Plasmodium falciparum
82
what other types of less fatal malaria exist
Plasmodium vivax
ovale
knowlesi
83
describe symptoms of P falciparum
```
cyclical fevers (every 48h)
splenomegaly
neuro involvement (altered GCS and seizures)
DV
metabolic acidosis
shock
```
84
how do you treat malaria falciparum
mild: oral MALARONE / artemisin combination therapyu
severe: IV Artesunate
85
how do you treat other types of malaria
chloroquine
86
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
```
87
how do you investigate for malaria
3 thick and thin blood films (thick: presence; thin: species)
Malaria rapid antigen detection test
88
How do you prevent malaria
Quinine prophylaxis
89
typhoid fever cause
salmonella typhi / paratyphi
90
how is typhoid transmitted
faeco oral
91
sx typhus
```
fever
bradycardia
headache
dry cough
weight loss, anorexia
GI sx (diarrhoea or constipation)
```
92
classical derm presentation of typhuss
rose spots
93
Mx typhus
IV ceftriaxone
94
where is typhus still found
pakistan
india
bangladesh
95
organism that causes dengue fever
arbiirus flavivirus - aedes aegiptii mosquito
96
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
97
how do you treat dengue
supportive (fluids and monitoring) >ITU
98
what are 'Tear-drop' poikilocytes found in
myelofibrosis
99
what is ITP
**Immune Thrombocytopenic Purpura**
* **antibodies against GLPIIb/IIIa** > thrombocytopoenia
100
how does ITP present
* easy bruising
* mucosal bleeding, menorrhagia, epistaxis
* petechiae, purpura
* after an infection
101
how do you manage ITP
oral pred
| IVIG
102
how does acute haemolytic reaction present
DURING/RIGHT AFTER TRANSFUSION
- fever
- abdo pain
- hypotension
103
how do you confirm an acute haemolytic reaction
COOMBS +ve
104
How do you manage acute haemolytic reaction
- stop transfusion
| - fluid resus
105
what is the most common inherited BLEEDING DISORDER
von Willenbrand disease
106
mode of inheritance of vWD
AD
107
what is the normal role of vWF
to promote platelet adhesion to damaged endothelium
108
types of vWD
1. Reduced quantity vWF
2. poor quality vWF
3. total lack vWF
109
vwd investigations
* FBC: normal platelets
* **prolonged bleeding time**
* **APTT may be prolonged**
* **factor VIII levels** may be moderately **reduced**
* **defective platelet aggregation with ristocetin**
110
vwd management
tranexamic acid
desmopressin (releases intracellular stores of vWF)
vWF and factor 8 concentrate
111
what is Neutropenic Sepsis and when does it usually occur
- **Temperature > 38°C** or any **symptoms and/or signs of sepsis**, in a person with an absolute **neutrophil count ≤ 0.5 x 109/L**
- Most commonly occurs **7-14 days after chemotherapy**
112
mx neutropoenic sepsis
**IV piperacillin + tazobactam** = tazocin
113
what drughs cause haemolysis in G6PD deficiency
Ciprofloxacin
| S drugs: sulphoonamides, sulphonylurea, sulphasalazine
114
how do you confirm G6pD deficiency
G6PD levels now and in 3 months
115
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)
116
which type of vWD are AD and which are AR?
AD: type 1 and 2
AR: type 3 (total lack vWF)
117
how can you manage vWF
tranexaminc acid, desmopressin (which induces vWF release), F8 concentratee
118
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
119
what does X ray head show iin MM
a RAINDROP SKULL (similar to pepper pot skull in 1HPTH)
120
sx of polycythaemia
hyperviscosity: **headache, light headedness, visual changes, fatigue, dyspnoea, DVT/PE/MI/STROKE**
histamine release: **aquagenic pruritus, peptic ulceration, splenomegaly**
tenderness or burning sensation in fingers and toes ( **Erythromelalgia**)
**plethroric**
121
What are blood findings on polycythaemia
Raised Hb (>16.5 g/dL M; >16.0 g/dL F)
Raised Htc
122
investigations for Polycythaemia Rubra Vera
* FBC and blood film
* JAK2 mutation testing
* Serum ferritin - usually low
* renal and liver function tests
123
what mutation is important in distinguishing the cause of polycythaemia
**JAK2** V617F +ve = polycythaemia rubra vera
| JAKV617F -ve = True polycythaemiia (hypoxia, renal disease, tumour --> m
124
what does polycythaemia vera risk progressing to
myelofibrosis
| AML
125
mx polycythaemia
* **aspirin** (to reduce risk of thrombosis)
* **regular venesection**
* Cytoreductive therapy: **Hydroxycarbamide** (if raised platelet count and/or marked leukocytosis)
126
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)
127
how to manage anaphylacitic blood transfusion reaction
* Stop the transfusion
* IM adrenaline
* ABC (oxygen and fluids)
128
what is the decond fastest reaction to blood transfusion that can occur
Acute haemolytic reaction(due to ABO incompativiliy, IgM mediagted) - within mins
129
how does febrile non haemolytic transfusion reaction pesent
rise in temp by 1 degree without circulatory collapse
| +- chilld and rigors
130
how do you manage febrile non haemolytic transfusion reaction
stop or slow transfusion, give paracetamol
131
what kind of transfusion is most likely to cause bacterial contamination
platelet transfusion
132
platelet transfusion indications
- **clinically significant bleeding (haematemesis, melaena, prolonged epistaxis) + platelet count < 30 x 109/L**
- If **severe bleeding/bleeding at critical sites: threshold < 100 x 109/L**
- If **not bleeding: threshold < 10 x 109/L**
133
how do you manage TACO
IV furosemide
slow / stop transfusion
oxygen
134
how do you manage TRALI
stop transfusion, supportive
135
how doees a delayed type haemolytic transfusion reeaction present
same as acute but milder
| occurs within one week of transfgusion
136
how does GvHD present
**within one week of transfusion**
donor lymphotes recognise HLA as foreign --> attack
- gut (**diarrhoea**)
- liver (**liver failure**)
- skin (**rash and skin desquamation**)
- BM.
also get **fever**
137
causes of MASSIVE SPLENOMEGALY
```
myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher's syndrome
```
138
how do you remove the risk of GvHD and what patients need this adaptation
by **IRRADIATE** the blood products (deplete them of T lymophocytes)
patients with **hodgkin /immunocompromised/neonates <28days**
139
what patients need CMV neg blood
intrauterine / neonates / preg
140
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
141
what are the four types of hodgkins lymphoma
Nodular sclerosing
Mixed cellularity
lymphocyte predominant
lymphocyte depleted
142
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. = MIRROR IMAGE NUCLEI
143
what is the staging system of hodgkins
ANN ARBOUR
144
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
145
what are B symptoms in hodgkins
fever >38
night sweats
unintentional WL >10% body weight in 6 months
146
clincal features of hodgkin's lymphoma
lymphadenopathy (75%)
* most commonly in the **neck** (cervical/supraclavicular) > axillary > inguinal
* usually **painless, non-tender, asymmetrical**
* **alcohol-induced** lymph node **pain**
pruritis --> skin excoriation marks
hepatomeglay +/- splenomegaly
147
investigations for hodgkins lymphoma
FBC: **normocytic anaemia, high wcc, high neutriophils and eosinophils**
**high ESR, CRP and LDH** (released during cell turonvover)
**Lymph node biopsy** = mirror image nuclei/RS cell
148
what do B symptoms suggest in terms of prognosis
POOR
149
management of hodgkins lymphoma
* 1st line = **chemotherapy** (ABVD)
* +/- **radiotherapy**
* refractory and relapsed disease after therapy = chemo, radio --> **autologous HSCT**
150
how do you manage an allergic reaction to the transfusion
stop the transfussion
| administer antihistamine
151
which type of hodgkins has the best prognosis
Lymphocyte predoiminant
152
what part of the intestine is iron absorbed in
in the DUODENUM
153
what part of the intestine is folate absorbed in
JEJUNUM
154
what part of the intestine is B12 absorbed in
ILEUM
155
what vitamin will someone with al ilieocaecal resection from chrons be deficient in
B12
156
blood findings for EBV glandular fever
RAISED lymphocytes, no neutrophils
157
sx glandular fever
fatigue
| recurrent tonsillitis
158
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
159
how do you invesitgate lymphoma
- FBC, CRP ESR, blood film, LDH
- exisional node biopsy
- CT chesst abdo pelvis (staging)
- HIV test
160
what is the most aggressive NHL
Burkitt's
161
who does Burkitts occur in
young immunosuppressed
| EBV and HIV associations
162
what are high grade NHL
DLBCL and mantle cell lymphoma
163
what is the MOST COMMON type of lymphoma
DLBCL
164
what virus is DLBCL asociated with
EBV
165
types of low grade lympha
follicluar
malt
SLL/CLL
166
how do you treat CML
Imatinib (tyrosine kinase inhib)
167
what lymphoma has a starry sky appearance on LN buopsy
Burkitts
168
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
169
how must you manage beta thalassaemia major
LIFELONG BLOOD TRANSFUSIONS
| + desferroxamine to avoid iron overload
170
what electrolyte abnormality may occur following transfusion of large volume RBC
HYPERKALAEMIA (because packed RBC have high potassium)
171
what is the most common inherited thrombophilia, and what is pathophy
FACTOR V LEIDEN (heterozygous)
| -- due to resistance to Protein C
172
what does the presence of band cells indicate
CML
173
what kind of leukaemia does LOW LYMPHOCYTES point to
AML or CML
174
what is sideroblastic anaemia
inability to form haem > form ring sideroblasts instead
175
causes of sideroblastic anaemia
```
congenital
myelodysplasia
alcohol
lead
anti TB meds
```
176
ix in sideroblastic anaemia
```
FBC (hypochromic microcytic)
iron studies (raised ferritin, raised iron, raised transferrin sat)
blood film (Basophilic stippling)
BM staining (ring sideroblasts)
```
177
what is aplastic anaemia
pancytopoenia + hypoplastic BM
178
causes of aplastic anaemia
congenital
- Fanconi
- dyskeratosis congenita
drugs
- cytotoxics
- chloramphenicol
- sulphonamides
- phenytoin
viral
- parvovirus
- hepatitis
179
which pathway does PT measure
the EXTRINSIC PATHWAY (i.e. the short one, activated by tissue factor)
180
which pathway does APTT measure
the INTRINSIC PATHWAY (long)
181
what factors are involved in the INTRINSIC PATHWAY
12, 11, 9, 8, 10
182
which factors are involved in EXTRINSIC PATHWAY B
tissue factor
| F7
183
explain the common pathway
PROTHROMBIN converts to THROMBIN (by F10a)
FIBRINOGEN converts to FIBRIN (by thrombin)
FIBRIN cross links the clot
184
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)
185
what are causess of prolonged APTT ONLY (issue in intrinsic pathhway)
deficiency of any of the factors invlved
| vWD
186
causes of prolonged PT AND APTT
deficiency of fibrinogen, prothrombin, F5, F10
liver disease
DIC
anticoagulants
187
what is heparin MoA
inhibits antithrombin (whhich blocks thrombin)
188
how many globin chains is each Hb made up of
4 globin chains
189
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)
```
190
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
191
what sx do you get with beta thalassaemia then
jaundice, haemochromatosis
| hepatosplenomegaly
192
what are blood findings of thalassaemia
microcytic anaemia
target cells
raised iron
193
what are the combinations of beta blobin mutations you can get in thalassaemia
```
beta+ = less than normal
beta0 = no beta chains
```
194
what are the types of beta thalassaemia
beta thalassaemia major = beta0beta0
beta thal intermedia ? beta+beta+
beta thal minor = beta+beta or beta0beta
195
how does beta thalaasaemia present on electrophoresis
low HbA
| raised HbF, HbA2
196
what type of thalassaemia is incompativle with life
alphha thalassaemia major = Hb Barts (x4 alpha globin gene deletion) > hydrops fetalis, death in utero
197
what is the definitive investigation to sickle cell disease
haemoglobin electrophoresis
198
sudden anaemia and a low reticulocyte count indicates?
parvovirus
199
High reticulocyte count + severe anaemia?
sickle cell anaemia
200
a high reticulocyte count is caused by?
acute sequestration and haemolysis
201
what is priapism
a persistent penile erection --> lasting longer than 4 hours and is not associated with sexual stimulation.
202
initial investigation for priapism
**Cavernosal blood gas analysis** --> differentiate between ischaemic and non-ischaemic
* ischaemic priapism **pO2 and pH would be reduced** whilst **pCO2 would be increased.**
203
first-line treatment of priapism
aspirate blood from the cavernosa AND inject a saline flush to help clear viscous blood that has pooled.
204
anaemia of chronic disease iron studies
low iron
low/normal TIBC
normal/high ferritin
205
investigations for Polycythaemia Rubra Vera
* FBC and blood film
