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haem Flashcards

1
Q

von willebrand tx for bleeding

A

mild = transexamic acid

desmopression (DDAVP): increased levels of vWF by causing its release from weibel palade bodies in endothelial cells

factor VIII concentrate

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2
Q

pernicious anaemia path

A

antibodies against intrinsic factor and gastric parietal cells = cant absorb b12 at terminal ileum

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3
Q

causes of b12 defiiciency

A

pernicious anaemia
post gastrectomy
vegan or poor diet
terminal iluem disorders ie crohns or ileocecal resection

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4
Q

b12 features

A

macrocytic anaemia
glossitis
neuro - proporception and vib lost first then distal parathesia
mood disturbances

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5
Q

b12 def tx

A

IM hydroxocobalamin

no neuro sx = 3x a week for 2 weeks
neuro = alternate days until sx resolve

TREAT B12 BEFORE FOLATE to avoid subacute degene of spinal cord

maintenence
pernicious = 2-3mhtly injecttions forveer
diet related = 2x yearly inhections or oral

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6
Q

hereditory spherocytosis

A

autosomal dominant

spherical shape RBCs = get stuck in spleen = haemolytic anaemia

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7
Q

g6pd avoid what

A

fava beans cipro sulfonurea
trimethoprim
nitrofurantoin
anti malarials

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8
Q

henz bodies

A

blobs of denatured hb inside RBCs

g6pd and alpha thalassemia

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9
Q

autoimune haemolytic anaemia

A

Warm – common, idiopathic, CLL
Cold – secondary to lymphoma, leukaemia, SLE, etc.

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10
Q

Alloimmune haemolytic anaemia

A

Transfusions
New-born – rhesus disease

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11
Q

thalassaemia gene and path

A

autosomal recessive

= either defective alpha chains or beta chains
= fragile rbc breakdown and collected by spleen = splenomegaly

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12
Q

risk of iron with thalasaaemia (esp in B major)

A

iron overload

bc regular transfusion and
increased iron absorb at GI tract

iron chelation ie desferrioxamine

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13
Q

B thalassaemia major presentation

A

first year of life failure to thrive and hepatosplenomegaly
microcyitic anaemia

face changes:
Frontal bossing (prominent forehead)
Enlarged maxilla (prominent cheekbones)
Depressed nasal bridge (flat nose)
Protruding upper teeth

bc bone marrow under so much strain to produce extra red blood cells to compensate for the chronic anaemia that it expands enough to increase the risk of fractures and change the patient’s appearance

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14
Q

CLL

age
path
sx
ix

complications

A

over 60
too many B cells
often Asx
blood film = sludge cells
immunophenotyping

Richters transformation (b cell lymophoma)
warm haemolytic anaemia
hypogammaglobulinaemia
anaemia

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15
Q

what is richters transformation

A

CLL cells go into lymph node and change into high grade fast growing non hodgkins lymphoma

pt become v unwell suddenly

of of following sx
lymph node swelling
fever w/o infection
wt loss
night sweats
nausea
abdo pain

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16
Q

CML

phases

A

philadephia chromosome
(= excess acitivity of tyrosine kinase)

increase in granulocytes at different stages of maturation (neutro,baso,eosin) +/- thrombocytosis
decreased leukocyte alkaline phospatase

3 phases
chronic, accelerated, blast

accelerated = 10-20% of bone marrow is blast cell
blast =>20%

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17
Q

cml mx

A

imatinib
hydroxyurea
interferon alpha
bone marrow transplant

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18
Q

AML

A

adults anytime but usually middle age onwards
can be from myeloproliferative disorder ie polycythemia rubra vera or myelofibrosis
blast cells with AUER RODS

features of bone marrow failure; anaemia, neutropenia, thrombocytopenia, bonepain, splenomegaly

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19
Q

acute promyelocytic leukemia

A

type of AML (m3 subtype)

translocation of chromosome 15&17

presents young ie average age 25
DIC or thrombocytopenia at presentation
good prognosis

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20
Q

acute promyelocytic anaemia tx

A

all trans retinoic acid (ATRA)
chemo

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21
Q

CLL tx

A

tyrosine kinase inhibitors (e.g., ibrutinib)
Monoclonal antibodies (e.g., rituximab, which targets B-cells)

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22
Q

tumor lysis syndrome

A

high uric acid (deposit in tubule = AKi)
high potass (arthyth)
high phosphate
high creatinine

low calcium
arythmia
seizure

make sure good hydration and urine output b4 chemo –> allopurinol and rasburicase to supress uric if at risk

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23
Q

myeloproliferative disorders/ myelofibrosis what is it and what causes it

associated w which gene

A

proliferation of a single type of stem cell = bone marrow fibrosis = extramedullary haematopoeisis ie in liver and spleen

primary myelofibrosis
polycythaemia vera
essential thrombocytopenia

JAK2

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24
Q

blood film in myelofibrosis and ix

A

teardrop shaped rbcs
Anisocytosis (varying sizes of red blood cells)
Blasts (immature red and white cells)

bone marrow biopsy (dry) –> trephine biopsy

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25
polycythaemia vera proliferating cell line and blood finding
erthroid cells high hb (&neutrophilia &inc plts) low esr high leukocyte alkaline phos
26
Primary myelofibrosis proliferating cell line and blood finding
Haematopoietic stem cells low hb (high or low red/white cells)
27
essential thrombocythemia proliferating cell line and blood finding
Megakaryocyte high plts
28
myeloproliferative risk
potential to transform to AML
29
myeloproliferative disorders chemo tx
hydroxyurea ( hydroxycarbamide
30
polycythaemia tz
aspirin (bc thrombo events) venesection 1st line hydrocycarbamide phosphorus 32
31
myelodyspastic syndrome
mutation of myeloid cells = inadequate production of blood cells (either red white plts or 3) can transform in AML bone marrow biopsy
32
Antiplatelets - COX inhibitors
aspirin
33
antiplatlets P2Y12 inhibitors
clopidogrel, ticagrelor, prasugrel
34
antiplatelets Phosphodiesterase inhibitors
– dipyridamole
35
anticoags Indirect thrombin inhibitors
heparins fondaparinux
35
Anticoagulants - Direct thrombin inhibitors
dabigatran
36
anticoags direct Xa inhib
DOACS
37
what is FFP when is it given
part of blood that contains clotting factors – liver disease, massive transfusion, bleeding disorders
38
factor 7 deficiency =
prolonged PT and prolonged APTT
39
Factor 8, 9, 11, 12 (intrinsic pathway) deficiency
normal platelets, normal PT, prolonged APTT
40
factor 10 deficiency
PT prolonged and APTT prolonged
41
Budd-chiari syndrome
thrombosis in hepatic vein/IVC (outflow of liver) caused by hypercoaguable states (ie myeloprolif disroders)
42
budd chiari triad
abdo pain hepatomegaly ascites
43
Thrombotic thrombocytopenic purpura (TTP)
deficiency of ADAMTS-13 protein = tiny thrombi in micro vessels
44
pernicious anaemia risk of ca
inc risk gastric cancer
45
lemon tinge skin
pernicious anaemia bc pallor (anaemia) + jaundice (haemolysis) = lemon tinge
46
CML sx
anaemia: lethargy wt loss n sweating SPLENOMEGALY granulocytyes at diff stages +/- thrombocytosis (too many plts)
47
myelodysplasia anaemia type
normocytic
48
haemolytic anaemia bloods
normocytic anaemia inc reticulocyte count increased lactate dehydrongenase high billirubin
49
antiphosphlipid syndrome PE tx
LMWH then vit k anatg ie warfarin
50
what are bands cell
intermediate neutrophil ie half mature neutrophils
51
steps to differentiate between leukemias
1. Look at lymphocytes 2. Look at WBC 3. Others hint (blast cell/ bands) ie 1.Low Lymphocytes -> the answer should be AML or CML 2. WBC > 100 -> Chronic causes so Consider CML (rule out AML) 3. Presence of bands cell -> confirm CML blast= acute bands = chronic
52
why low lymphocytes in aml/cml
overprolif of myeloid cells (ie rbc plts granulocytes and monocytes) this crowds out bone marrow cells ie lymphocytes
53
INR 5-8 on warfarin no bleed, bleed and major bleed when restart
oral vit k IV Iv vit k and Prothrombin complex concentration restart when INR<5
54
ALL cause of haemorrhagi or thrombotic complications
DIC
55
pt stable with CVD but has AF
stop anti platelet ie clopidrgel start anticoagulant monotherapy
56
Post ACS/PCI and has af w stroke risk what meds
triple therapy (2antiplt 1 anticoag) 4wks to 6mths post event dual (1 of each) to complete 12 mths
57
p450 inhibitors
ie Increase INR SICKFACES.COM-Group Sodium valp Isonizid Cimetidine Ketoconazole Fluconazole Alcohol(binge) Chloramphenicol Erythromycin Sulfonamides (I fir inhibit) Ciprofloxacin OMEPRAZOLE METRONIDAZOLE Grapefruit juice
58
ALL poor prognostic factors
<2yo >10yo WBC>20 at diagnosis T or B cell surface markers non caucasion male
59
UFH monitoring
APTT
60
lMWH monitering
no regular monitoring needed but if needed ie renal imapirment, obesity, preg moniter anti factor Xa
61
why do you feeling of fullness/ constant satiety with cml
enlarged spleen compressing other organs
62
PE CXR
usually normal may see wedge shaped opacification
63
Howell jolly bodies what is it and seen in
blobs of DNA material inside RBCs - spleen usually supposed to remove them post splenectomy sickle cell severe anaemia
64
schistocytes what are they when seen
fragments of RBCs - being damaged microangiopathic haemolytic anaemia (MAHA) eg: TTP,DIC, haemolytic uremic syndrome ( HUS)
65
sideroblasts
immature RBCs w nucleus surrounded by iron blobs - ie iron cant be incorporated in myelodydysplatic syndrome
66
smudge cells
ruptured wbcs CLL
67
spherocytes
sphere shaped RBCs hereditary spherocytosis autoimmune haemolytic anaemia
68
difference and why dont you moniter lwmh compared to UFH
LMWH has shorter longer half life = longer durations of action more predictable pharmacokinetics
69
epistaxis failed all emergency tx
sphenopalatine ligation in theatre
70
beta thallassemia bloods
mild hypochromic microcytic anaemia microcytosis disprop to anaemia ie crazy small size even compared to hb which isnt asss low
71
beta thalassaemia hb electropharesis
HbA2 HbA2 only in trait HbA2 and HbF in major
72
emergency surgery but on warfarin
stop warfarin (emergency or elective) if surg can wait (6-8hrs) = vit K 5mg IV emergency = 25-50 units/kg four factor prothrombin complex
73
hypersegmented polymorphs
pernicious anaemia
74
heparin induced thrombocytopenia (HIT)
antibodies against platelet factor 4 5-10 days post heparin tx
75
syphhilis how to tell if reinfection and tx
RPR if increases by more than 4 fold = no tx response or reinfection tx = benzathine penicillin G IM STAT dose
76
causes of false positive non treponemal tests (Cardiolipin ) (in syphyliis)
pregnancy SLE, anti-phospholipid syndrome tuberculosis leprosy malaria HIV
77
two types of tets for syphyliis and eg
non treponemal (non specific) - RPR - (venereal disease research lab) VDRL (become -ve after tx) Treponemal specific - TP EIA ( T.palladium enzyme immunoassay) - TPHA (t.p . HaemAgglutination ) (stay after disease)
78
ITP blood and tx
isolated thrombocytopenia 1st = pred IVIG if active bleeding or urgent prodecure required
79
hodgkins lymphoma sx associated w poor prognosis
B sx wt loss>10%in 6mths fever >38C night sweats
80
hodgkins lymphoma RF and sx
HIV, EBV B sx pruritis lympadenopathy painful w alcohol hepatosplenomegaly
81
hodgkins bloods and ix
bloods normocytic anaemia eosinophillia inc LDH LN biopsy - reed sternberg Imaging
82
hodgkins staging
ann arbour
83
complication of hodgkins
mediastinal LN enlargment =SVC obstruction
84
iron def anaemia signs and specific signs
conjuctival pallor tachyardia tachypneoa ejection systolic murmur angular cheilosis atrophic glossitis koilionychia
85
iron def iron studies
low transferrin sat low ferritin high TIBC
86
side effects ferrous sulphate
constipation diarohea nausea black stools
87
sickle cell pt what dx will be in hx
hydroxyurea folic acid penicillin ( bc autoinfarct of spleen - hyposplen = suseptible infections)
88
causes of sickle cell crisis
cold dehydration hypoxia acidosis infection
89
principles of tx w sickle crisis
oxygen analgesia iv fluids empirical abx
90
if pt as sickle cell and partner completelty normal likelehood of child having SICKLE TRAIT
100%
91
Sickle cell protective against what
malaria
92
why doesnt sickle show until 6 mths
High levels of foetal haemoglobin (HbF) which mask the effect of the disease until levels of HbF begin to fall at 6 months old.
93
what is multiple myeloma
malignant clonal prolif of B lymphocytic plasma cells = bare igG
94
MM sx
fatigue bruise easy recurrent infxn polydipsia bone pain
95
MM ix
blood - fbc ue ca2 igG + film urine electropharesis serum electropharesis Bm sampling XR
96
causes of splenomeg
Leukemia malaria liver cirrhosis w port htn mono haemolytic anameia
97
hoe to diff cml and aml on film
aml = immature blast cells cml = white cells in full spectrum of lineage band cells
98
cml breathru tx
imatinib tyrosine kinase inhib oral
99
immediate transfusion if hb
<7
100
signs of super low hb
pallor tachycardia tachypnoe flow murmur
101
early blood transfusion reactions
acute haemolytic reaction bacterial contamination allergic/anaphylaxis TRALI Fluid overload febrile non haemolytic massive transfusion
102
delayed tranfusion reactions
delayed haemolytic 1-7d post trans purpura 7-10d GvHD 4-30d fe overload chronic
103
pt transfuse instant reaction n temp
haemolytic anaemia --> DIC bact contam allergic wouldnt have fever
104
macrocytic anemia
megaloblastic b12 folate cytotox dx non alcohol, liver dz preg, hypothyroid
105
beta thal major name
cooleys anameia
106
confim mm
serum electopharesis
107
confirm thalasema
serum electopharesis
108
haemophillia a n b factor
a = factor viii b = 9
109
haemophilia mx
avoid nsaids n IM injections minor bleed =desmopression and tranxemic acid major = Purifies factor vii, FFP w VII,
110

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