haem Flashcards

1
Q

in IDA how long shld u take iron for

A
  • for 3 months after levels have come up
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2
Q

when might you see ferritin rise with low iron

A
  • inflammation as ferritin is acute phase protein as well
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3
Q

where is most dietary iron absorbed from

A
  • duodenum
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4
Q

IDA in postmenopausal/ male patients.. next step?

A
  • UGI/LGI ix
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5
Q

confirmed IDA, Next steps

A
  • PMP/Men = UGI/LGI ix
  • Coeliac screen
  • h pylori screen
  • urine test for blood = malignancy
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6
Q

lmwh clearance mode

A
  • renal
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7
Q

half lfie lmwh

A

12hrs

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8
Q

peak lmwh

A

4hrs

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9
Q

antixa is measured after lmwh in which circumstances

A
  • pregnancy
  • renal failure
  • obese
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10
Q

UFH clearance mode

A
  • reticuloendothelial
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11
Q

UFH half life

A

1.5hrs

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12
Q

how to give UFH

A

= IV infusion/sc

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13
Q

how to monitor UFH

A
  • APTT
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14
Q

UFH reversal agent

A
  • protamine
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15
Q

what does appttr measure

A
  • intrinsic pathway
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16
Q

instrinsic pathway involves which factors

A
  • common

- 12 and 11

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17
Q

extrinsic pathway involves which factors

A
  • common

- 7

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18
Q

what is Heparin induced thrombocytopenia

A

= minor plt drop at 5days

- no rx

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19
Q

what is rx for Heparin induced thrombocytopenia with thrombosis

A
  • stop hep

= use danaparoid

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20
Q

SE heparin

A
  • HIT
  • HITT
  • Osteoporosis; duration and dose related
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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
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22
Q

what does protein c do

A
  • stops fv and fv8 = anticoag
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23
Q

CI to dabigatran

A

<30 crcl
active bleed
risk of bleed
prosthetic heart valve

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24
Q

CI to rivaroxaban

A

crcl<15
active bleed
risk of bleed
prosthetic heart valve

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25
Q

CI to apixaban

A

crcl<15
acitve bleed
risk of bleed

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26
Q

sideroblastic anaemia inheritance pattern

A

= x linked

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27
Q

causes for acquired sideroblastic anaemia

A
  • myelodysplasia
  • myeloproliferative disorders
  • myeloid leukaemia
  • alcohol misuse and toxicity
  • lead poisoning, copper deficiency
  • vit b6 deficiency esp in isoniazid treatment
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28
Q

b12 or folate replacement first

A
  • b12

- always check b12 if folate low otherwise can cause damage

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29
Q

causes of acquired haemolytic anaemia

A
  • AIHA
  • Transfusion
  • MAHA
  • Systemic
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30
Q

b thalaessaemia inheritence pattern

A
  • AR on chr 11, point mutation

- extra hbf made as no beta for normal hba

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31
Q

beta thalassemia types

A

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

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32
Q

why do you get haemolysis in beta thalaessaemia

A
  • too many alpha chains that are free so clump up and form inclusions and destroy rbc membrane = haemolysis
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33
Q

when does b thalaseemia major cause sx

A
  • 3-6 months of life as hbf made till then
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34
Q

what iron issue can beta thalassemia patients have

A
  • haemachromatosis
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35
Q

sx beta thalassemia

A
  • those of anaemia
  • FTT
  • Chipmunk faces
  • hair on end.
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36
Q

target cells = what anaemia

A

beta thaemolysis

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37
Q

dx GS for beta thalaessemia

A
  • electrophoresis. b minor >3.5% hba2
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38
Q

rx beta thalassemia major/intermedia

A
  • regular transfusions
    but can cause iron voerload so worsen haemachromatosis. iron chelating agents, deferoxamine
  • splenectomy
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39
Q

Heinz bodies and bite cells are indicative of what problem

A
  • G5PD deficiency
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40
Q

G6PD inheritance pattern

A
  • X linked
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41
Q

Pyruvate kinase deficiency inheritance pattern

A
  • AT
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42
Q

warm AIHA involves which abs

A

IgG

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43
Q

what happens in paroxysmal nocturnal haemoglobinuria

A
  • acquired disorder

- increases destruction of Haem cells by complement due to lack of GPI on their membranes.

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44
Q

sx paroxysmal nocturnal haemaglobinuria

A
  • VTE
  • haem anaemia
  • pancytopenia
  • dark urin in the morning
  • aplastic canaemia
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45
Q

how to dx paroxysmal nocturnal haemoglobinuria

A
  • flow cytometry of CD59/CD55 = gs

- hams test; acid induced haemolysis

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46
Q

rx paroxysmal nocturnal haemoglobinuria

A
  • transfuse
  • anticoag
  • eculizamab - mab for C5
  • STCRT
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47
Q

where is folate absorbed in the body

A
  • jejunum
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48
Q

symptoms of folate deficiency

A
  • ulcers
  • growing hair
  • anaemia sx
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49
Q

what one iron studies marker is raised in anaemia of chronic disease but normal in IDA

A

hepcidin

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50
Q

which coagulation factor rises in the acute phase of systemic disease

A
  • FV8
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51
Q

ddx for left shift in leukocytes (more immature cells)

A
  • infection
  • malignancy
  • haemolytic crisis
  • MI
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52
Q

ddx for right shift in leukocytes

A

absence of youn or imamture wbcs

  • b12 and folate deficiency,
  • chronic uraemia
  • liver dsiease
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53
Q

tumour lysis syndrome abnormal blood results

A
  • hyperk
  • hyperphosphataemia
  • hyperuricaemia
  • hypoca
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54
Q

tumour lysis syndrome rx

A
  • agressive pre hydration
  • allopurinol/rasburicase
  • manage hyperk
  • dont replace ca
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55
Q

hyperviscosity syndrome triad of sx

A
  • mucosal bleed
  • blurry vision
  • neuro disturbance
56
Q

hyperviscosity rx

A
  • urgent plasmapheresis or leukopheresis
57
Q

how does acute promyelocytic leukaemia present

A
  • leucopenia and bruising; DIC
58
Q

chr transloaction in acute promyelocytic leukaemia

A
  • chr 15:17, PML RARA
59
Q

how to treat acute promyelocytic leuakemia

A
  • ATRA and correct coagulopathy
60
Q

CRABBI sx of myeloma

A
  • Calcium is high
  • Renal failure
  • Anaemia and pancytopenia
  • Bone disease; osteolytic lesions
  • bleeding
  • infection
61
Q

NICE guidance, who to suspect myeloma in

A

> 60 with back pain or bone pain or unexplained fractures

62
Q

intitial ix for myeloma

A
  • fbc
  • u and e
  • Calcium
  • Esr
  • plasma viscosity

then if positive = bence jones and electrophoresis

63
Q

what group of ix is done for myeloma diagnosis. clue = BLIP

A
  • 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
Q

rx multiple myeloma

A
  • 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
Q

diagnostic criteria for multiple myeloma

A
  • Monoclonal plasma cells in th BM >10%
  • Monoclonal protein in serum or urine
  • end organ damage
66
Q

criteria for MGUS

A
  • <10 % PLASMA CELLS
  • ABSENCE OF BONE LESIONS, ANAEMIA OR OTHER SX
  • <30G/L SERUM MONOCLONAL PROTEINS
67
Q

WHAT IS AMYLOIDOSIS

A
  • extraprotein deposits. due to inability of proteases to appropriately cleave or abnormal cleavage.
68
Q

what is AL amyloidosis = systemic

A
  • Amyloidosis with light chains
69
Q

what is AA amyloidosis = primary

A
  • misfolded protein becomes amyloid a in serum
70
Q

sx amyloidosis

A
- low albumin
and proteinuria 
- oedema 
- hyperlipdiaemia as albumin normally inhibits lipid production
- restrictive cardiomyopathy
71
Q

Ix amyloidosis

A
  • congored stain post biopsy = pink +ve

- under poalrised light = apple green

72
Q

what is gauchers disease

A
  • enzyme disorder = accumulation of lipid in bone marrow and organs
73
Q

RF for Hodgkins lymphoma

A
  • HIV
  • EBV
  • RA/ sarcoidosis
  • FHX
74
Q

sx for hodgkins lymphoma

A
  • non tender rubbery LN. worse pain with alcohol + b sx.
  • cough
  • itchy
  • recurrent infection
75
Q

ix hodgkins

A
  • VBG - LDH

- LN biopsy; reed sternberg

76
Q

ann arbor staging hodkins lymphoma

A

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
Q

rx hodgkins lymphoma

A
  • chemo –> leukaemia and infertility

- RDT –> Cancer, hypoTH

78
Q

burkitt lymphoma is associated with which RF

A
  • EBV and HIV
79
Q

Diffuse large B cell lymphoma sx

A
  • painless rapidly enalrging mass
80
Q

RF non hidgkins lymphoma

A
  • HIV
  • EBV
  • Hpylori if MALY
  • Hep b /c
  • trichlorethylene
  • fhx
81
Q

hodgkins lymphoma age group

A
  • bimodal
  • <25
  • > 50
82
Q

what type of lymphoma would hyperpigmentaion indicate

A
    • t cell
83
Q

SE of RDT earyl

A
  • fatigue
  • skin reaction
  • n and v
  • mucositis
  • Diarrhoea
  • dysphagia
  • cystitis
  • BM supression
84
Q

SE of RDT late

A
  • organ involvement
85
Q

4 subtypes of hodgkins lymphoma

A
  • nodular sclerosing
  • mixed cellularity
  • lymphocyte deplted
  • lymphocyte rich
86
Q

which subtype of hodgkins lymphoma has the best prognosis and is more common in women

A
  • modular sclerosing
87
Q

testicular mass can be found in which type of lymphoma

A
  • non hodgkins
88
Q

what does burkitts lymphoma look like on biopsy

A
  • starry sky appearance
89
Q

complications of lymphomas

A
  • BM infitration
  • SVC obstriction
  • mets
  • SCC
90
Q

2 types of burkitts lymphoma

A
  • endemic; african = maxilla and mandible

- sporadic = abdo tumorus. common in HIV

91
Q

what gene translocation is in burkitts lymphoma

A
  • cmyc gene. t8:14
92
Q

which b cell non hodgkins lymphoma is more likely to be completely cured - indolent (low grade) or aggressive (high grade)?

A
  • aggressive
93
Q

drugs used to treat aggressive high grade b cell non hidgkins lymphoma

A
  • rituximab and chemo
94
Q

What type of leukaemia do you get gum hypertrophy and parotid enlargement in

A
  • AML
95
Q

philadephia chr is in which leukaemia mostly

A
  • CML
96
Q

enlarged rubbery non tender lymph nodes are found in which leukaemia

A
  • chronic lympocytic - CLL
97
Q

gout occurs in which leukaemia

A
  • CML
98
Q

auer rods are in which type of leukaemia

A
  • AML
99
Q

smudge cells are in which type of leukaemia

A
  • CLL
100
Q

which leuakemia is related to myelodysplastic syndrome-

A

AML

101
Q

rx CML with ph chr

A
  • Imatinib
102
Q

CLL is associated with which haemolytic anaemia

A
  • Warm aiha
103
Q

Warm AIHA what is the defect in rbc

A
  • spherocytosis
104
Q

In Cold AIHA , what is the associations and triggers

A

associated;
- raynauds, acrocyanosis

trigger
- ebc, mycoplasma pneumonia

105
Q

in Paroxysmal nocturnal haemoglobinuria what antibody is seen

A
  • donath lansteiner - biphasic
106
Q

what is a typical exam q for paroxysmal nocturnal haemaglobinuria

A
  • 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
Q

pentad sx for TTP

A
  • thrombocytopenia
  • fever
  • renal failure
  • evidenc eof MAHA on blood film
  • confusion
108
Q

blood results in TTP

A
  • low plt
  • low hb
  • high bili
  • high LDH
  • high creat
  • +/-ve trops
109
Q

genetic defect in ttp

A

= adams13 = cleaving protease for vwf which is involved in ttp clotting, has an ab igg

110
Q

ttp rx

A
  • high dose steroids
  • palsma exchange
  • rituximab
  • blood
  • folic acid
  • when recovered; aspirin and lmwh
111
Q

what hip problem are those with SCD prone too

A
  • avascular necrosis; steroids, core decomrpess and replace
112
Q

do we give abx prophylaxis in SCD

A
  • Lifelong OR full adherance tille age 5 = pen/erythro
113
Q

what preventative durg other than abx shld you give SCD pts

A
  • folic acid
114
Q

f/up programme for SCD

A
  • EVERY 3/12 in first 2 yrs then 6/12 3-5 yrs then annual
115
Q

when is hydroxucarbamide given to patient with SCD

A
  • > 3 EPISODES WITH HOSPITAL admission in 12months
  • pain interferes with QOL
  • 2 episodes of chest syndrome in 12 month
116
Q

extra haemolytic manifestations of thalassemia

A
  • ostoeporosis
  • hypoth
    • hypopth
  • dm
  • HPA axis issues
  • hypogonadism
  • iron overload
117
Q

which part of the coagulation path does warfarin target

A
  • extrinsic pathway
118
Q

function of protein c and s

A
  • inactivate factor 5 and 8
119
Q

Why is there a higher thrombosis risk in cocp

A
  • acquired increase in resistance to activated protein c
  • reduced protein s levels
  • reduced antithrombin levels
120
Q

ITP rx

A
  • if sympto or plt <20 = pred
121
Q

drugs that cause hamolytic anaemia

A
  • methyldopa
  • quinine
  • NSAIDs
  • interferon
122
Q

HUS triad

A
  • AKI
  • thrombocytopenia
  • microangiopathic haemolytic anaemia = MAHA
123
Q

hereditary spherocytosis dx

A
  • 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
Q

Hereditary spherocytosis rx

A
  • folate replacce

- splenectomy

125
Q

Paroxysmal nocturnal haemoglobinuria GS

A
  • HAMS TEST; acid induced haemolysis
126
Q

causes of absolute polycythaemia

A

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
Q

polycythaemia rubra vera symptoms

A
  • 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
Q

PCV rx

A
  • venesect
  • if high risk e.g. >60 = hydroxycarbamide/urea
  • alpha interferon in child bearing age women
  • aspirin lifelong 75
129
Q

essential thrpmbocythaemi rx

A
  • aspirin

- hydroxycarbamide in high risk

130
Q

teardrop cells are in which disease

A
  • myelofibrosis
131
Q

what kind of biopsy is done in Myeloproliferative disorders

A
  • trephine ; as cant aspirate due to dry tap
132
Q

define essential thrombocythaemia

A
  • > 2 months of plt>450
133
Q

livedo reticularis occurs in which myeloprolif disorder

A
  • Essential thrombocythaemia
134
Q

myelofibrosis sx

A
  • constitutional sx

- splenomegaly

135
Q

what is the scoring system used for myelodysplastic syndromes

A
  • cytopenias
  • cytogenetics
  • blast %

= RIPSS - prognostic

136
Q

ITP epidemiology

A
  • woman; chronic

- kids - acute, 2 weeks post infection. goes away