haem Flashcards
von willebrand tx for bleeding
mild = transexamic acid
desmopression (DDAVP): increased levels of vWF by causing its release from weibel palade bodies in endothelial cells
factor VIII concentrate
pernicious anaemia path
antibodies against intrinsic factor and gastric parietal cells = cant absorb b12 at terminal ileum
causes of b12 defiiciency
pernicious anaemia
post gastrectomy
vegan or poor diet
terminal iluem disorders ie crohns or ileocecal resection
b12 features
macrocytic anaemia
glossitis
neuro - proporception and vib lost first then distal parathesia
mood disturbances
b12 def tx
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
hereditory spherocytosis
autosomal dominant
spherical shape RBCs = get stuck in spleen = haemolytic anaemia
g6pd avoid what
fava beans cipro sulfonurea
trimethoprim
nitrofurantoin
anti malarials
henz bodies
blobs of denatured hb inside RBCs
g6pd and alpha thalassemia
autoimune haemolytic anaemia
Warm – common, idiopathic, CLL
Cold – secondary to lymphoma, leukaemia, SLE, etc.
Alloimmune haemolytic anaemia
Transfusions
New-born – rhesus disease
thalassaemia gene and path
autosomal recessive
= either defective alpha chains or beta chains
= fragile rbc breakdown and collected by spleen = splenomegaly
risk of iron with thalasaaemia (esp in B major)
iron overload
bc regular transfusion and
increased iron absorb at GI tract
iron chelation ie desferrioxamine
B thalassaemia major presentation
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
CLL
age
path
sx
ix
complications
over 60
too many B cells
often Asx
blood film = sludge cells
immunophenotyping
Richters transformation (b cell lymophoma)
warm haemolytic anaemia
hypogammaglobulinaemia
anaemia
what is richters transformation
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
CML
phases
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%
cml mx
imatinib
hydroxyurea
interferon alpha
bone marrow transplant
AML
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
acute promyelocytic leukemia
type of AML (m3 subtype)
translocation of chromosome 15&17
presents young ie average age 25
DIC or thrombocytopenia at presentation
good prognosis
acute promyelocytic anaemia tx
all trans retinoic acid (ATRA)
chemo
CLL tx
tyrosine kinase inhibitors (e.g., ibrutinib)
Monoclonal antibodies (e.g., rituximab, which targets B-cells)
tumor lysis syndrome
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
myeloproliferative disorders/ myelofibrosis what is it and what causes it
associated w which gene
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
blood film in myelofibrosis and ix
teardrop shaped rbcs
Anisocytosis (varying sizes of red blood cells)
Blasts (immature red and white cells)
bone marrow biopsy (dry) –> trephine biopsy
polycythaemia vera proliferating cell line and blood finding
erthroid cells
high hb (&neutrophilia &inc plts)
low esr
high leukocyte alkaline phos
Primary myelofibrosis proliferating cell line and blood finding
Haematopoietic stem cells
low hb
(high or low red/white cells)
essential thrombocythemia proliferating cell line and blood finding
Megakaryocyte
high plts
myeloproliferative risk
potential to transform to AML
myeloproliferative disorders chemo tx
hydroxyurea ( hydroxycarbamide
polycythaemia tz
aspirin (bc thrombo events)
venesection 1st line
hydrocycarbamide
phosphorus 32
myelodyspastic syndrome
mutation of myeloid cells = inadequate production of blood cells (either red white plts or 3)
can transform in AML
bone marrow biopsy
Antiplatelets
- COX inhibitors
aspirin
antiplatlets P2Y12 inhibitors
clopidogrel, ticagrelor, prasugrel
antiplatelets Phosphodiesterase inhibitors
– dipyridamole
anticoags Indirect thrombin inhibitors
heparins fondaparinux
Anticoagulants
- Direct thrombin inhibitors
dabigatran
anticoags
direct Xa inhib
DOACS
what is FFP when is it given
part of blood that contains clotting factors – liver disease,
massive transfusion, bleeding disorders
factor 7 deficiency =
prolonged PT and prolonged APTT
Factor 8, 9, 11, 12 (intrinsic pathway) deficiency
normal platelets, normal PT, prolonged APTT
factor 10 deficiency
PT prolonged and APTT prolonged
Budd-chiari syndrome
thrombosis in hepatic vein/IVC (outflow of liver)
caused by hypercoaguable states (ie myeloprolif disroders)
budd chiari triad
abdo pain
hepatomegaly
ascites
Thrombotic thrombocytopenic purpura (TTP)
deficiency of ADAMTS-13 protein
= tiny thrombi in micro vessels
pernicious anaemia risk of ca
inc risk gastric cancer
lemon tinge skin
pernicious anaemia
bc pallor (anaemia) + jaundice (haemolysis) = lemon tinge
CML sx
anaemia: lethargy
wt loss n sweating
SPLENOMEGALY
granulocytyes at diff stages +/- thrombocytosis (too many plts)
myelodysplasia anaemia type
normocytic
haemolytic anaemia bloods
normocytic anaemia
inc reticulocyte count
increased lactate dehydrongenase
high billirubin
antiphosphlipid syndrome PE tx
LMWH then vit k anatg ie warfarin
what are bands cell
intermediate neutrophil
ie half mature neutrophils
steps to differentiate between leukemias
- Look at lymphocytes
- Look at WBC
- 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
why low lymphocytes in aml/cml
overprolif of myeloid cells (ie rbc plts granulocytes and monocytes)
this crowds out bone marrow cells ie lymphocytes
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
ALL cause of haemorrhagi or thrombotic complications
DIC
pt stable with CVD but has AF
stop anti platelet ie clopidrgel
start anticoagulant monotherapy
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
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
ALL poor prognostic factors
<2yo >10yo
WBC>20 at diagnosis
T or B cell surface markers
non caucasion
male
UFH monitoring
APTT
lMWH monitering
no regular monitoring needed
but if needed ie renal imapirment, obesity, preg
moniter anti factor Xa
why do you feeling of fullness/ constant satiety with cml
enlarged spleen compressing other organs
PE CXR
usually normal
may see wedge shaped opacification
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
schistocytes what are they when seen
fragments of RBCs - being damaged
microangiopathic haemolytic anaemia (MAHA) eg: TTP,DIC, haemolytic uremic syndrome ( HUS)
sideroblasts
immature RBCs w nucleus surrounded by iron blobs - ie iron cant be incorporated in
myelodydysplatic syndrome
smudge cells
ruptured wbcs
CLL
spherocytes
sphere shaped RBCs
hereditary spherocytosis
autoimmune haemolytic anaemia
difference and why dont you moniter lwmh compared to UFH
LMWH has shorter longer half life = longer durations of action
more predictable pharmacokinetics
epistaxis failed all emergency tx
sphenopalatine ligation in theatre
beta thallassemia bloods
mild hypochromic microcytic anaemia
microcytosis disprop to anaemia ie
crazy small size even compared to hb which isnt asss low
beta thalassaemia hb electropharesis
HbA2
HbA2 only in trait
HbA2 and HbF in major
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
hypersegmented polymorphs
pernicious anaemia
heparin induced thrombocytopenia (HIT)
antibodies against platelet factor 4
5-10 days post heparin tx
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
causes of false positive non treponemal tests
(Cardiolipin ) (in syphyliis)
pregnancy
SLE, anti-phospholipid syndrome
tuberculosis
leprosy
malaria
HIV
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)
ITP blood and tx
isolated thrombocytopenia
1st = pred
IVIG if active bleeding or urgent prodecure required
hodgkins lymphoma sx associated w poor prognosis
B sx
wt loss>10%in 6mths
fever >38C
night sweats
hodgkins lymphoma RF and sx
HIV, EBV
B sx
pruritis
lympadenopathy
painful w alcohol
hepatosplenomegaly
hodgkins bloods and ix
bloods
normocytic anaemia
eosinophillia
inc LDH
LN biopsy - reed sternberg
Imaging
hodgkins staging
ann arbour
complication of hodgkins
mediastinal LN enlargment =SVC obstruction
iron def anaemia signs
and specific signs
conjuctival pallor
tachyardia
tachypneoa
ejection systolic murmur
angular cheilosis
atrophic glossitis
koilionychia
iron def iron studies
low transferrin sat
low ferritin
high TIBC
side effects ferrous sulphate
constipation
diarohea
nausea
black stools
sickle cell pt what dx will be in hx
hydroxyurea
folic acid
penicillin ( bc autoinfarct of spleen - hyposplen = suseptible infections)
causes of sickle cell crisis
cold
dehydration
hypoxia
acidosis
infection
principles of tx w sickle crisis
oxygen
analgesia
iv fluids
empirical abx
if pt as sickle cell and partner completelty normal likelehood of child having SICKLE TRAIT
100%
Sickle cell protective against what
malaria
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.
what is multiple myeloma
malignant clonal prolif of B lymphocytic plasma cells
= bare igG
MM sx
fatigue
bruise easy
recurrent infxn
polydipsia
bone pain
MM ix
blood - fbc ue ca2 igG + film
urine electropharesis
serum electropharesis
Bm sampling
XR
causes of splenomeg
Leukemia
malaria
liver cirrhosis w port htn
mono
haemolytic anameia
hoe to diff cml and aml on film
aml = immature blast cells
cml = white cells in full spectrum of lineage band cells
cml breathru tx
imatinib
tyrosine kinase inhib
oral
immediate transfusion if hb
<7
signs of super low hb
pallor
tachycardia
tachypnoe
flow murmur
early blood transfusion reactions
acute haemolytic reaction
bacterial contamination
allergic/anaphylaxis
TRALI
Fluid overload
febrile non haemolytic
massive transfusion
delayed tranfusion reactions
delayed haemolytic 1-7d
post trans purpura 7-10d
GvHD 4-30d
fe overload chronic
pt transfuse instant reaction n temp
haemolytic anaemia –> DIC
bact contam
allergic wouldnt have fever
macrocytic anemia
megaloblastic
b12 folate cytotox dx
non
alcohol, liver dz
preg, hypothyroid
beta thal major name
cooleys anameia
confim mm
serum electopharesis
confirm thalasema
serum electopharesis
haemophillia a n b factor
a = factor viii
b = 9
haemophilia mx
avoid nsaids n IM injections
minor bleed =desmopression and tranxemic acid
major = Purifies factor vii, FFP w VII,
