haem stuff to memorise Flashcards
microcytic anaemia mnemonic
(low MCV, small RBC) Thalassaemia Anaemia of chronic disease Iron def anaemia Lead poisoning Sideroblastic anaemia
normocytic anaemia mnemonic
(normal MCV, normal RBC) Anaemia of chronic disease Aplastic anaemia Acute blood loss Hypothyroidism Haemolytic anaemia
causes of macrocytic, megaloblastic
(impaired DNA synth - cannot divide so keeps growing into large abnormal cell)
B12/folate def
causes of macrocytic, normoblastic
alcohol, reticulocytosis, hypothyroidism, azathioprine
normal haemoglobin, normal MCV
120-165, 130-180 men
80-100 both
signs of iron def
pica, hair loss, angular cheilitis, spoon shaped nails, atrophic glossitis, brittle hair/nails
key sign for each: thalassaemia, CKD, haemolytic
thal - bone deformities
CKD - oedema, hypertension
haem - jaundice, splenomegaly
genetic patterns for thalassaemia, sickle cell, G6PD def, hereditary spherocytosis/elliptocytosis, von willebrand dis, haemophilia
thal - auto recess, sickle - auto recess, G6PD - X linked, hered sph - auto dom, vwf - auto dom, haemo - x linked
chromosomes for alpha and beta thal
alpha - 16
beta - 11
thal minor vs thal intermedia vs thal major?
1 norm 1 abnorm
2 abnorm
2 deletion
genetics of sickle cell
abnormal beta-globin gene on chrom 11
prophylaxis for sickle cell
penicllin V
drug that stimulates prod of what in sickle cell
hydroxycarbamide - stim prod of HbF (protective)
3 types of sickle cell crisis
vaso-occlusive (distal ischaemia)
splenic sequestration (RBC block flow in spleen)
aplastic (parvovirus 19, no RBC made)
treat sickle cell crises
keep warm, hydrate, treat infection, penile aspiration
diagnose sickle cell, thalassaemia
haemoglobin electrophoresis, screened at preg (dna test in thal, heel prick in sickle)
diagnose g6pd def
enzyme assay (defect on enzyme that is usually protective against reactive oxygen species)
meds to avoid in g6pd
primaquine ciprofloxacin nitrofurantoin trimethoprim sulfasalazine
where is iron absorbed
duodenum jejunum
drug that reduces iron absorption
PPI (crohns and coeliac also has this effect)
if no clear cause of iron def then what management?
gastroduodenoscopy and colonoscopy
3 types of treatment for iron def
- blood trans correct anaemia but not cause
- iron infusion eg. cosmofer (risk of anaphylaxis)
- oral iron eg. ferrous sulphate (can feed bacteria in sepsis, constip and black stool)
haemolytic anaemia on blood film? test for autoimmune
spherocytes
direct coombs test
general tests for anaemia
haem level mcv b12/folate ferritin (TIBC marker for transferrin) blood film
treat hered spherocytosis, elliptocytosis
folate suppl
splenectomy
cholecystectomy
temps for warm/cold autoimmune haemolytic anaemia
manage
warm - normal, idiopathic
cold - under 10 degrees, agglutination
manage both with transfusions, steroid, rituximab
splenectomy
paroxysmal nocturnal haemoglobinuria
mutation causes complement cascade to be activated = red urine in morning
treat - eculizumab targets C5
alloimmune haemolytic anaemia
disease of newborn
antib cross placenta causing immune resp
causes of pernicious anaemia
b12 def
insuf diet intake
signs of pern anaem
low mood peripheral neuropathy pins and needles less proprioception visual changes lemon yellow skin
antib in pern anaem
intrinsic factor antibod
gastric parietal cell antibod
why treat b12 before folate in pern anaem
subacute combined degeneration of the spinal cord
treat dietary insuf cause of pern anaem
cyanocobalamin
treat autoimmune cause of pern anaem
IM hydroxocobalamin
most toxic, dormant species of malaria
plasmodium falciparum
plas ovale & vivax
4 stages of malaria
sporozoites, hypnozoites (dorm), merozoites (mature), RBC rupture releasing more
invest malaria
blood film in EDTA bottle - 3 samples over 3 days bc 48hrs regen
uncomplicated oral treatment malaria
riamet
malarone
quinine sulphate
doxycycline
complicated/severe malaria treat
- IV artesunate
2. Quinine dihydrochloride
malaria prophyl and side effects
malarone
doxycycline - sun and take longer
mefloquine - hallucinations, seizures
hodgkins lymph RF
fam hist
EBV
HIV
autoimmune cond
large lymph nodes, non-tender
pain after alcohol
hodgkins lymphoma
treat hodgkins and diag test
ABVD chemo, lymph node biop showing reed sternberg cells
mutation in JAK2 indicates which dis and what may it lead to
polycythaemia vera - prolif of erythroid cell, can become AML, myelofibrosis
hepatosplenomegaly from rbc being produced there to compensate
treat polycythaemia vera
1st venesection
aspirin and chemo
ALL pres, assoc, key bits
child
downs syn
blast cells
CLL pres, assoc, key bits
warm autoimmune haem anaem can become high grade lymph = richters trans smear/smudge cells treat with rituximab most common in adults
CML pres, assoc, key bits
chronic phase (5yrs)
accelerated phase
blast phase - worse symptoms
philadelph chrom 9:22 translocation
AML pres, assoc, key bits
most common acute in adults
polycythaemia ruby vera becomes AML
blast cells - auer rods
define pancytopenia
anaemia, leukopenia, thrombocytopenia
tumour lysis syndrome cause and treat
release of uric acid during chemo - crystals in interstitial tissue and tubules of kidney = aki
treat with allopurinol, rasburicase
monitor calcium phosphate potass
treat vW dis
desmopressin
vWF infused
clot factor 8
heavy period - tranexamic acid
presentation of haemophilia and their factors
haem a - factor 8
haem b - factor 9
severe bleeding, haematoma, umbilical cord, intercran haemorrhage, bleed into joints and muscles
invest haemophilia
coag factor assay
bleeding score
gen testing
treat hameophilia
intraven infusion = clotting factors replaced but can develop antib against them
desmopressin to stim release of vwf
5 features of myeloma mnemonic
OLD Calcium (elevated) Renal failure Anaemia (normocytic, normochromic) from replacement of bone marrow. Bone lesions/pain
myeloma?
cancer of plasma b cells - produce immunoglobulins which infiltrate the bone marrow
myeloma antib protein?
bence jones protein
myeloma rf
black, old, male, fma hist, obese
4 initial tests when suspect myeloma
if posi then what?
FBC (low white blood cell count in myeloma) Calcium (raised in myeloma) ESR (raised in myeloma) Plasma viscosity (raised in myeloma)
posi = urgent serum protein electrophoresis and a urine Bence-Jones protein test
4 initial invest for myeloma mnemonic
B – Bence–Jones protein (request urine electrophoresis)
L – Serum‑free Light‑chain assay
I – Serum Immunoglobulins
P – Serum Protein electrophoresis
then whole body mri (skeletal survey if they cant hack it)
signs on xray for myeloma
Punched out lesions
Lytic lesions
“Raindrop skull”
first line treat myeloma
chemo with:
Bortezomid
Thalidomide
Dexamethasone
- high risk of develop thrombus so also need venous thromboembolism prophylaxis
improve bone comp in myeloma?
bisphosphonates - suppress osteoclast activity.
Radiotherapy to bone lesions can improve bone pain.
Orthopaedic surgery can stabilise bones
Cement augmentation - injecting cement into vertebral fractures or lesions to stabilise
complications of myeloma
Infection Pain Renal failure Anaemia Hypercalcaemia Peripheral neuropathy Spinal cord compression Hyperviscocity
treat immune thrombocytopenic purpura
prednisolone
iv immunoglobulins
rituximab
splenectomy
treat thrombotic thrombocytopenic purpura
prednisolone
rituximab
plasma exchange
which protein impairment in TTP
ADAMTS 13 cannot inactivate vWF so extra platelet adhesion
define thrombocytopenia and causes
low platelet count
itp, ttp, hit, sodium valproate, alcohol
describe heparin induced thrombocytopenia
antib against platelets develop in response to heparin
anti PF4/heparin antib
swap to diff anticoag!!
