haem Flashcards
1
Q
in IDA how long shld u take iron for
A
- for 3 months after levels have come up
2
Q
when might you see ferritin rise with low iron
A
- inflammation as ferritin is acute phase protein as well
3
Q
where is most dietary iron absorbed from
A
- duodenum
4
Q
IDA in postmenopausal/ male patients.. next step?
A
- UGI/LGI ix
5
Q
confirmed IDA, Next steps
A
- PMP/Men = UGI/LGI ix
- Coeliac screen
- h pylori screen
- urine test for blood = malignancy
6
Q
lmwh clearance mode
A
- renal
7
Q
half lfie lmwh
A
12hrs
8
Q
peak lmwh
A
4hrs
9
Q
antixa is measured after lmwh in which circumstances
A
- pregnancy
- renal failure
- obese
10
Q
UFH clearance mode
A
- reticuloendothelial
11
Q
UFH half life
A
1.5hrs
12
Q
how to give UFH
A
= IV infusion/sc
13
Q
how to monitor UFH
A
- APTT
14
Q
UFH reversal agent
A
- protamine
15
Q
what does appttr measure
A
- intrinsic pathway
16
Q
instrinsic pathway involves which factors
A
- common
- 12 and 11
17
Q
extrinsic pathway involves which factors
A
- common
- 7
18
Q
what is Heparin induced thrombocytopenia
A
= minor plt drop at 5days
- no rx
19
Q
what is rx for Heparin induced thrombocytopenia with thrombosis
A
- stop hep
= use danaparoid
20
Q
SE heparin
A
- HIT
- HITT
- Osteoporosis; duration and dose related
21
Q
warfarin SE
A
- Bleed
- skin necrosis due to protein c deficiency
- teratogen; coumarin embryopathy at 6- 12weeks with doses >5mg. also increases foetal loss due to haemorrhage
22
Q
what does protein c do
A
- stops fv and fv8 = anticoag
23
Q
CI to dabigatran
A
<30 crcl
active bleed
risk of bleed
prosthetic heart valve
24
Q
CI to rivaroxaban
A
crcl<15
active bleed
risk of bleed
prosthetic heart valve
25
CI to apixaban
crcl<15
acitve bleed
risk of bleed
26
sideroblastic anaemia inheritance pattern
= x linked
27
causes for acquired sideroblastic anaemia
- myelodysplasia
- myeloproliferative disorders
- myeloid leukaemia
- alcohol misuse and toxicity
- lead poisoning, copper deficiency
- vit b6 deficiency esp in isoniazid treatment
28
b12 or folate replacement first
- b12
| - always check b12 if folate low otherwise can cause damage
29
causes of acquired haemolytic anaemia
- AIHA
- Transfusion
- MAHA
- Systemic
30
b thalaessaemia inheritence pattern
- AR on chr 11, point mutation
| - extra hbf made as no beta for normal hba
31
beta thalassemia types
minor = b1 - 1 affected gene - reduced synthesis
intermedia = b2 - 2 affected genes from parents which reduce synthesis
major - 2 inherited genes both with lost trait - so none made
32
why do you get haemolysis in beta thalaessaemia
- too many alpha chains that are free so clump up and form inclusions and destroy rbc membrane = haemolysis
33
when does b thalaseemia major cause sx
- 3-6 months of life as hbf made till then
34
what iron issue can beta thalassemia patients have
- haemachromatosis
35
sx beta thalassemia
- those of anaemia
- FTT
- Chipmunk faces
- hair on end.
36
target cells = what anaemia
beta thaemolysis
37
dx GS for beta thalaessemia
- electrophoresis. b minor >3.5% hba2
38
rx beta thalassemia major/intermedia
- regular transfusions
but can cause iron voerload so worsen haemachromatosis. iron chelating agents, deferoxamine
- splenectomy
39
Heinz bodies and bite cells are indicative of what problem
- G5PD deficiency
40
G6PD inheritance pattern
- X linked
41
Pyruvate kinase deficiency inheritance pattern
- AT
42
warm AIHA involves which abs
IgG
43
what happens in paroxysmal nocturnal haemoglobinuria
- acquired disorder
| - increases destruction of Haem cells by complement due to lack of GPI on their membranes.
44
sx paroxysmal nocturnal haemaglobinuria
- VTE
- haem anaemia
- pancytopenia
- dark urin in the morning
- aplastic canaemia
45
how to dx paroxysmal nocturnal haemoglobinuria
- flow cytometry of CD59/CD55 = gs
| - hams test; acid induced haemolysis
46
rx paroxysmal nocturnal haemoglobinuria
- transfuse
- anticoag
- eculizamab - mab for C5
- STCRT
47
where is folate absorbed in the body
- jejunum
48
symptoms of folate deficiency
- ulcers
- growing hair
- anaemia sx
49
what one iron studies marker is raised in anaemia of chronic disease but normal in IDA
hepcidin
50
which coagulation factor rises in the acute phase of systemic disease
- FV8
51
ddx for left shift in leukocytes (more immature cells)
- infection
- malignancy
- haemolytic crisis
- MI
52
ddx for right shift in leukocytes
absence of youn or imamture wbcs
- b12 and folate deficiency,
- chronic uraemia
- liver dsiease
53
tumour lysis syndrome abnormal blood results
- hyperk
- hyperphosphataemia
- hyperuricaemia
- hypoca
54
tumour lysis syndrome rx
- agressive pre hydration
- allopurinol/rasburicase
- manage hyperk
- dont replace ca
55
hyperviscosity syndrome triad of sx
- mucosal bleed
- blurry vision
- neuro disturbance
56
hyperviscosity rx
- urgent plasmapheresis or leukopheresis
57
how does acute promyelocytic leukaemia present
- leucopenia and bruising; DIC
58
chr transloaction in acute promyelocytic leukaemia
- chr 15:17, PML RARA
59
how to treat acute promyelocytic leuakemia
- ATRA and correct coagulopathy
60
CRABBI sx of myeloma
- Calcium is high
- Renal failure
- Anaemia and pancytopenia
- Bone disease; osteolytic lesions
- bleeding
- infection
61
NICE guidance, who to suspect myeloma in
>60 with back pain or bone pain or unexplained fractures
62
intitial ix for myeloma
- fbc
- u and e
- Calcium
- Esr
- plasma viscosity
then if positive = bence jones and electrophoresis
63
what group of ix is done for myeloma diagnosis. clue = BLIP
- Bence hones
- Light chain assay serum
- Immunoglobulins serum
- Protein electropheoresis
and biopsy BM = dx. rouleux formation on peripheral blood film. in bipsy - too many plasma cells
64
rx multiple myeloma
- chemo
- SCT
- VTE prophylaxis if hypervisocse/ certain chemo = LMWH/aspirin
- bonnes; bisphosphonates
- RDT for pain
- surgery
- cement augmentation into fractures or lesions foe stabiltiy and pain
65
diagnostic criteria for multiple myeloma
- Monoclonal plasma cells in th BM >10%
- Monoclonal protein in serum or urine
- end organ damage
66
criteria for MGUS
- <10 % PLASMA CELLS
- ABSENCE OF BONE LESIONS, ANAEMIA OR OTHER SX
- <30G/L SERUM MONOCLONAL PROTEINS
67
WHAT IS AMYLOIDOSIS
- extraprotein deposits. due to inability of proteases to appropriately cleave or abnormal cleavage.
68
what is AL amyloidosis = systemic
- Amyloidosis with light chains
69
what is AA amyloidosis = primary
- misfolded protein becomes amyloid a in serum
70
sx amyloidosis
```
- low albumin
and proteinuria
- oedema
- hyperlipdiaemia as albumin normally inhibits lipid production
- restrictive cardiomyopathy
```
71
Ix amyloidosis
- congored stain post biopsy = pink +ve
| - under poalrised light = apple green
72
what is gauchers disease
- enzyme disorder = accumulation of lipid in bone marrow and organs
73
RF for Hodgkins lymphoma
- HIV
- EBV
- RA/ sarcoidosis
- FHX
74
sx for hodgkins lymphoma
- non tender rubbery LN. worse pain with alcohol + b sx.
- cough
- itchy
- recurrent infection
75
ix hodgkins
- VBG - LDH
| - LN biopsy; reed sternberg
76
ann arbor staging hodkins lymphoma
Stage 1: Confined to one region of lymph nodes.
Stage 2: In more than one region but on the same side of the diaphragm (either above or below).
Stage 3: Affects lymph nodes both above and below the diaphragm.
Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.
77
rx hodgkins lymphoma
- chemo --> leukaemia and infertility
| - RDT --> Cancer, hypoTH
78
burkitt lymphoma is associated with which RF
- EBV and HIV
79
Diffuse large B cell lymphoma sx
- painless rapidly enalrging mass
80
RF non hidgkins lymphoma
- HIV
- EBV
- Hpylori if MALY
- Hep b /c
- trichlorethylene
- fhx
81
hodgkins lymphoma age group
- bimodal
- <25
- >50
82
what type of lymphoma would hyperpigmentaion indicate
- - t cell
83
SE of RDT earyl
- fatigue
- skin reaction
- n and v
- mucositis
- Diarrhoea
- dysphagia
- cystitis
- BM supression
84
SE of RDT late
- organ involvement
85
4 subtypes of hodgkins lymphoma
- nodular sclerosing
- mixed cellularity
- lymphocyte deplted
- lymphocyte rich
86
which subtype of hodgkins lymphoma has the best prognosis and is more common in women
- modular sclerosing
87
testicular mass can be found in which type of lymphoma
- non hodgkins
88
what does burkitts lymphoma look like on biopsy
- starry sky appearance
89
complications of lymphomas
- BM infitration
- SVC obstriction
- mets
- SCC
90
2 types of burkitts lymphoma
- endemic; african = maxilla and mandible
| - sporadic = abdo tumorus. common in HIV
91
what gene translocation is in burkitts lymphoma
- cmyc gene. t8:14
92
which b cell non hodgkins lymphoma is more likely to be completely cured - indolent (low grade) or aggressive (high grade)?
- aggressive
93
drugs used to treat aggressive high grade b cell non hidgkins lymphoma
- rituximab and chemo
94
What type of leukaemia do you get gum hypertrophy and parotid enlargement in
- AML
95
philadephia chr is in which leukaemia mostly
- CML
96
enlarged rubbery non tender lymph nodes are found in which leukaemia
- chronic lympocytic - CLL
97
gout occurs in which leukaemia
- CML
98
auer rods are in which type of leukaemia
- AML
99
smudge cells are in which type of leukaemia
- CLL
100
which leuakemia is related to myelodysplastic syndrome-
AML
101
rx CML with ph chr
- Imatinib
102
CLL is associated with which haemolytic anaemia
- Warm aiha
103
Warm AIHA what is the defect in rbc
- spherocytosis
104
In Cold AIHA , what is the associations and triggers
associated;
- raynauds, acrocyanosis
trigger
- ebc, mycoplasma pneumonia
105
in Paroxysmal nocturnal haemoglobinuria what antibody is seen
- donath lansteiner - biphasic
106
what is a typical exam q for paroxysmal nocturnal haemaglobinuria
- child goes out to play and comes into warm house and starts developing sx. because it is biphasic - ab binds to rbc at 37 degrees and lyses at 20 degrees. trigger = viral infection
107
pentad sx for TTP
- thrombocytopenia
- fever
- renal failure
- evidenc eof MAHA on blood film
- confusion
108
blood results in TTP
- low plt
- low hb
- high bili
- high LDH
- high creat
- +/-ve trops
109
genetic defect in ttp
= adams13 = cleaving protease for vwf which is involved in ttp clotting, has an ab igg
110
ttp rx
- high dose steroids
- palsma exchange
- rituximab
- blood
- folic acid
- when recovered; aspirin and lmwh
111
what hip problem are those with SCD prone too
- avascular necrosis; steroids, core decomrpess and replace
112
do we give abx prophylaxis in SCD
- Lifelong OR full adherance tille age 5 = pen/erythro
113
what preventative durg other than abx shld you give SCD pts
- folic acid
114
f/up programme for SCD
- EVERY 3/12 in first 2 yrs then 6/12 3-5 yrs then annual
115
when is hydroxucarbamide given to patient with SCD
- >3 EPISODES WITH HOSPITAL admission in 12months
- pain interferes with QOL
- 2 episodes of chest syndrome in 12 month
116
extra haemolytic manifestations of thalassemia
- ostoeporosis
- hypoth
- - hypopth
- dm
- HPA axis issues
- hypogonadism
- iron overload
117
which part of the coagulation path does warfarin target
- extrinsic pathway
118
function of protein c and s
- inactivate factor 5 and 8
119
Why is there a higher thrombosis risk in cocp
- acquired increase in resistance to activated protein c
- reduced protein s levels
- reduced antithrombin levels
120
ITP rx
- if sympto or plt <20 = pred
121
drugs that cause hamolytic anaemia
- methyldopa
- quinine
- NSAIDs
- interferon
122
HUS triad
- AKI
- thrombocytopenia
- microangiopathic haemolytic anaemia = MAHA
123
hereditary spherocytosis dx
- osmotic fragility is no longer diagnostic
- if FH , typical sx, and lab investigations - spherocytes on blood film, high MCHC, high reticulocytes; no other tests
- if need further tests; EMA binding and cryohaemolysis test
124
Hereditary spherocytosis rx
- folate replacce
| - splenectomy
125
Paroxysmal nocturnal haemoglobinuria GS
- HAMS TEST; acid induced haemolysis
126
causes of absolute polycythaemia
primary = polycythaemia vera
secondary either hypoxia or inappropraite epo secretion
- hypoxia causes; high altitude, chronic lung disease, cyanotic congenital HD, heavy smoking
- epo - HCC, RCC
127
polycythaemia rubra vera symptoms
- headaches
- tinnitus
- dizzy
- visual changes
- facial plethora
- gout due to urate excess from b/down rbc
- itchy post hot bath
- erythromyalgia (also in essential thrombo)
- burning fingers and toes
- splenomegaly
- thrombosis both arterial and venous
128
PCV rx
- venesect
- if high risk e.g. >60 = hydroxycarbamide/urea
- alpha interferon in child bearing age women
- aspirin lifelong 75
129
essential thrpmbocythaemi rx
- aspirin
| - hydroxycarbamide in high risk
130
teardrop cells are in which disease
- myelofibrosis
131
what kind of biopsy is done in Myeloproliferative disorders
- trephine ; as cant aspirate due to dry tap
132
define essential thrombocythaemia
- >2 months of plt>450
133
livedo reticularis occurs in which myeloprolif disorder
- Essential thrombocythaemia
134
myelofibrosis sx
- constitutional sx
| - splenomegaly
135
what is the scoring system used for myelodysplastic syndromes
- cytopenias
- cytogenetics
- blast %
= RIPSS - prognostic
136
ITP epidemiology
- woman; chronic
| - kids - acute, 2 weeks post infection. goes away
