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haematology - anaemias
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- 1what is anaemia?low Hb conc
<13.5 men
<11 women - 2types of anaemia by cell size?microcytic
normocytic
macrocytic
haemolytic - 3what are the microcytic anaemias?low MCV:
IDA
thalassaemia
sideroblastic anaemia - rare - 4normocytic anaemias?acute blood loss
anaemia of chronic disease (or microcytic)
BM failure
renal failure
hypothyroid - or macro
haemolysis - or macro
pregnancy - 5when to suspect marrow failure?normocytic anaemia
low WCC
low plt - 6macrocytic anaemias?B12/folate deficiency
alcohol excess/ liver dz
reticulocytosis
cytotoxics eg hydroxycarbamide
myelodysplastic syndromes
marrow infiltration
hypothyroidism
antifolate drugs eg phenytoin - 7what are haemolytic anaemias?can be normo or macro
suspect if:
reticulocytosis >2% RBCs
mild macro
low haptoglobin
high bilirubin
high urobilogen
haemolysis causes prehepatic jaundice so you get mild jaundice but no bilirubin in urine - 8when to transfuse in anaemia?Hb <7
if acute cause eg haemorrhage w/ peptic ulcer may need to transfuse up to 8
if severe anaemia w/ HF - give packed cells SLOWLY w/ furosemide + check for rising JVP/crackles, if CCF worse stop.
can try unit exchange transfusion - 9what are the causes of IDA?blood loss
poor diet - babies + children/poverty
malabsorption - coeliac - cause of refractory
tropics - hookworm (GI blood loss) - 10signs of IDA?koilonychia
atrophic glossitis
angular cheilosis
post cricoid webs - plummer vinson - 11tests for IDA?micro hypo
anisocytosis + poikilocytosis
confirm w/ low ferritin + low serum iron w/ high TIBC
investigate for:
GI blood loss
gastroscopy
sigmoidoscopy
barium enema
colonoscopy
stool micro if hookworm possible
all above esp if no obvious cause - 12what to think about with ferritin?is an acute phase reactant
increases w/ infection, inflamm, malignancy etc - 13tx IDA?tx cause
oral iron eg ferrous sulphate 200mg/8h PO continued til Hb normal and for at least 3 months to replenish stores
if oral not a go then IV - 14s/e ferrous sulphate?nausea
abdo discomfort
diarrhoea
constipation
black stools - 15why might oral ferrous sulphate not be a go?compliance
functional ID in CRF
misdiagnosis - 16what is anaemia of chronic disease?2y anaemia
commonest in hosp pt, 2nd commonest worldwide after IDA
3 probs where hepcidin plays a role:
1. poor iron use in erythropoiesis
2. cytokine induced short RBC survival
3. low production of/response to EPO - 17causes of 2y anaemia?chronic infection
vasculitis
rheumatoid
malignancy
renal failure - 18tests ACD?normo normo mild anaemia
normal ferritin/ high
blood film
B12
folate
TSH
haemolysis tests
as may be mulitfactorial - 19tx of anaemia of chronic disease?underlying cause
EPO
parenteral iron - 20s/e EPO?flu like sx
HTN
mild elevated plt
thromboembolism - 21sideroblastic anaemia?microcytic hypochromic anaemia not responding to iron!
marrow - disease defining sideroblasts
tx - remove cause / pyridoxine + repeated transfusion - 22causes of sideroblastic anaemia?xlinked AR
myelodysplasia
excessive alcohol, pyridoxine deficiency, lead poisoning, copper deficiency
excess zinc
isoniazid, chloramphenicol, linezolid - 23what is poikilocytosis?abnormally shaped RBCs
- 24what is anisocytosis?blood cells unequal size
- 25what is a ring sideroblast?perinuclear ring iron granules
sideroblastic anaemia - poor erythropoiesis leading to increased iron absorption with iron loading in marrow + haemosiderosis (endocrine, liver,heart damage from iron deposition) - 26where do we see anisocytosis?megaloblastic anaemia,
thalassaemia
IDA - 27what are acanthocytes?spicules on RBCs
causes - splenomegaly, ALD, abetalipoproteinaemia, spherocytosis - 28what is basophilic RBC stippling?denatured RNA in RBCs
lead poisoning
megaloblastic
myelodysplasia
liver dz
haemoglobinopathy eg thalassaemia - 29what are blasts?nucleated precursor cells -not normally on periph blood film
myelofibrosis, leukaemia, malignancy - 30burr cells?echinocytes - RBC projections
- 31cabot rings?pernicious anaemia, lead poisoning, bad infections
- 32dimorphic picture on PBF?to pops of RBCs
seen in
mixed deficiency
post transfusion
1y sideroblastic anaemia - 33what are howell jolly bodies?DNA nuclear remnants in RBCs -usually removed by spleen
post splenectomy
hyposplenism eg SCD, coeliac, congenital, UC/CD, myeloproliferative dz, amyloid
megaloblastic anaemia - 34when is hypochromia seen?IDA
thalassaemia
sideroblastic - 35what are pappenheimer bodies?siderocyte granules containing iron
lead poisoning
carcinomatosis
post splenectomy - 36when is poikilocytosis seen?IDA
myelofibrosis
thalassaemia - 37when are reticulocytes seen?haemolyiss
haemorrhage - 38left shift?immature white cells - eg infection
- 39right shift?hypermature white cells- hypersegmented polymorphs
megaloblastic
uraemia
liver dz - 40what is rouleau formation?red cells stack together - worm like
causes raised ESR
chronic inflammation
paraproteinaemia
myeloma - 41spherocytes?hereditary spherocytosis + AIHA
- 42schistocytes?fragmented RBCs sliced by fibrin bands
in IV haemolysis
microangiopathic anaemia eg DIC
HUS
TTP
pre-eclampsia - 43what are target cells?mexican hat cells
RBCs w/ central staining, ring of pallor + outer rim of staining
liver dz
hyposplenism
thalassaemia
IDA sometimes - 44what are the causes of neutrophilia?bacterial infection
inflamm - MI, PAN
myeloproliferative disorders
drugs - steroids
disseminated malignancy
stress eg trauma, burn, haemorrhage, seizure - 45what is a normal neutrophil count?2-7.5
- 46causes of neutropenia?viral infection
drugs -chemo, cytotoxic, carbimazole, sulfonamides
severe sepsis
nuetrophil abs (SLE, haemolytic anaemia)
hypersplenism eg felty's
BM failure - 47what is the normal leukocyte count?1.5-4.5
- 48causes lymphocytosis?acute viral infxn
chronic infection eg brucella, hep, syph
leukaemia/ lymphoma esp CLL - 49what kinds of lymphocytes are seen in EBV infection?atypical
large clear cytoplasm w/ blue rim that flows around neighbouring rbcs - 50causes lymphopaenia?steroid tx
SLE
uraemia
legionnaire's
HIV
marrow infiltrate
post chemo/ radio - 51what is a normal CD4 count?537 - 1571
- 52normal eosinophil levels?0.04-0.4
- 53eosinophila causes?drug rxns eg erythema multiforme
allergies
asthma
parasitic infection
skin dz exp pemhigus, eczema, psoriasis, dermatitis herpetiformis
malignancy incl lymphoma/leukaemia
PAN
adrenal insufficiency
irradiation
convalescence post infection - 54what is HES?hypereosinophilc syndrome
no cause
high for > 6wks
end organ damage
tx - steroid PO + anti IL-5 mab
imantinib if genetic predisposition - 55causes of monocytosis?post chemo/radio
chronic infxn eg malaria, TB, brucellosis
malignancy - AML, hodgkin's
myelodysplasia - 56basophilia causes?myeloproliferative dz
viral infxn
IgE HS - urticaria
inflamm eg UC, RA - 57macrocytic anaemia causes?often due to alcohol w/o anaemia
megaloblastic - B12 def, folate def, cytotoxic drugs
non megloblastic - alcohol, reticulocytosis eg haemolysis, liver dz, hypothyroid, pregnant
other blood dz - myelodysplasia, myeloma, myeloproliferation, aplastic anaemia - 58what are megaloblasts?delayed nuclear maturation compared to cytoplasm
- 59tests in macrocytic anaemia?B12 + folate look same
blood film - hypersegmented polymorphs in B+F (target cells if liver dz)
other - LFTs, TFTs, serum B12, serum folate (or red cell folate- more reliable)
bone marrow bx - indicated if cause not found with other tests - 60what might a bone marrow bx show in macrocytic anaemia?megaloblasts
normoblasts - liver dz, hypothyroid
abnormal erythropoeisis - sideroblastic, leukaemia, aplasia
increased erthropoeisis - haemolysis - 61where do we get folate?greens
nuts
yeast
liver
stores last 4 months
maternal def - neural tube defects - 62causes of folate def?poor diet - poverty, alcoholic, elderly
increased demand eg pregnancy/ haemolysis/ malignancy/ inflamm/renal dialysis
malabsorption - coeliac, sprue
drugs, alcohol, anti-epileptics, MTX, trimethroprim - 63where is folate absorbed?duodenum
jejunum - 64tx of folate deficiency?underlying cause
folic acid 5mg/day PO 4 months, never w/o B12 unless pt has normal B12 as in low B12 states it can precipitate/worsen SACDC
prophylaxis in pregnancy - 65folate + HDmay be a link (unproven) between high homocysteine + IHD, folate reduces homocysteine.
- 66folate + cognition?if borderline deficiency - 88micrograms/day for 3 yrs can benefit - FACIT trial
- 67where is B12 found?meat
fish
dairy products
body stores good for 4 years
binds IF in stomach and is then absorbed in terminal ileum - 68what happens in B12 deficiency?thymidine synthesis + thus dna synthesis is slow so RBC production is slow in turn
- 69causes of B12 deficiency?dietary eg vegans
malabsorption - stomach (lack of IF): pernicious anaemia, post gastrectomy
-terminal ileum - ileal resection, CD, bacterial overgrowth, tropical sprue, tapeworm
congenital metabolic error - 70general features of B12 deficiency?anaemia sx
lemon tinge to skin from combo of pallor (anaemia) + mild jaundice (haemolysis)
glossitis - beefy red sore tongue
angular cheilosis (stomatitis) - 71neuro features of B12 def?irritable
depression
psychosis
dementia - 72what are the general features of anaemia?fatigue
SOB
faintness
palps
headache
tinnitus
anorexia
pallor, tachyC, flow murmur, cardiomegaly, retinal haemorrhage
HF - 73what is subacute combined degeneration of the spinal cord?insidious w/ peripheral neuropathy due to low B12:
combo of symmetrical dorsal column loss (sensory + LMN signs)
and
symmetrical CST loss (motor + UMN signs)
joint position and vibration sense can be affected 1st - ataxia, stiffness, wkness - 74what is the classical presentation of subacute combined degeneration of the spinal cord?triad - extensor plantars (UMN) + absent knee jerks (LMN) + absent ankle jerks (LMN)
falls at night - ataxia + reduced vision
pain and temp can be intact in even severe cases as STT preserved - 75what is pernicious anaemia?caused by AI atrophic gastritis w/ achlorhydria and lack of IF secretion
more in women,
>40
more blood group A - 76associations of PA?other AI dz
thryoid
vitiligo
addison's
hypoparathyroid
carcinoma of stomach - 3x more common - 77general tests in PA?low Hb
macrocytic
low WCC/plt if severe
low serum B12
low reticulocytes or normal
hyeprsegmented polymorphs
megaloblasts in marrow - 78specific PA test?parietal cell abs in 90%
IF ab - specific but low sens - 79tx of PA?cause if poss
if due to malabsorption - injections - hydroxocobalamin (B12) 1mg IM - maintenance is img Im every 3 months for life
dietary cause - oral B12 - 80how to know if PA is improving?transient reticulocytosis and macrocytosis after 4-5 days
- 81px B12 deficiency?supplements help peripheral neuropathy but little help for cord signs
tx asap - 82name some non-dietary, non AI causes of B12 deficiency?CD
coeliac
post gastric surgery
acid suppressors eg ranitidine
metformin
panc insufficiency - 83haemolytic anaemia basics?premature breakdown of RBCs before 120d
either intravascular or extravascular
can be asymptomatic if bone marrow compensates - 84where are the extravascular sites of haemolytic anaemia?liver, spleen, bone marrow macrophages
- 85what questions to ask in an approach to haemolysis?1. is there increased RBC breakdown?
2. is there increased RBC production
3. is the haemolysis intravascular or extravascular?
4. why? - 86how to tell if increased RBC breakdown?normo or macrocytic anaemia
high bilirubin - unconjugated from haem breakdown ie prehepatic jaundice
high urobilogen - no urinary conj bilirubin
high serum LDH as released from RBCs - 87how to tell if increased RBC production?reticulocytosis causing macrocytosis + polychromasia
- 88what are the features of extravascular haemolysis?splenic hypertrophy + splenomegaly
- 89features of intravascular haemolysis?increased free plasma Hb - from RBCs
methaemalbuminaemia
decreased plasma haptoglobin - as mopping up free plasma Hb then removed by liver
haemoglobinuria - red brown urine w/o RBCs
haemosiderinuria - prussian blue staining a week after onset - chronic haemolysis - 90what are the causes of haemolytic anaemia?acquired - immune + non immune
hereditary - enzyme/membrane defects or haemoglobinopathy - 91what are the acquired immune causes of haemolytic anaemia?coombs test +ve = drug induced/ AIHA/ paroxysmal cold haemoglobinuria
coombs test -ve - 92acquired non immune causes of haemolyitic anaemia?microangiopathic haemolytic anaemia (MAHA)
infection
paroxysmal noctural haemoglobinuria - 93drug induced immune mediated cooms +ve causes of haemolytic anaemia?RBC autoab formation - penicillin
immune complex formation - quinine - 94what is AIHA (coombs +ve)?autoab causing mostly extravascular haemolysis + spherocytosis
classified by optimal binding temperature to RBCs:
warm AIHA
cold AIHA - 95warm AIHA?IgG mediated
bind at 37*C
tx - steroids/IS +/- splenectomy - 96cold AIHA?IgM mediated, bind @ <4*C
chronic anaemia worse in cold, often w/ raynaud's/ acrocyanosis
tx = keep warm/ chlorambucil - 97causes of AIHA?most idiopathic
2y WAIHA - lymphoproliferative dz eg CLL, lymphoma, drugs, AI eg SLE
2y CAIHA - may follow infxn eg mycoplas/EBV - 98what is paroxysmal cold haemoglobinuria?viruses/syph
cause - donath landsteiner ab sticking to RBCs in cold - complement mediated haemolysis on rewarming. - 99what are the causes of AHIA w/ -ve coomb's?AIH
hep B / C
post flu/ other vax
drugs - piperacillin, rituximab - 100what infections cause haemolytic anaemia?malaria-
RBC lysis - 101what is paroxysmal nocturnal haemoglobinuria?rare acquired stem cell disorder
haemolysis, marrow failure, thrombophilia
visceral thrombosis/ pulm emboli- poor px
dx - urinary haemosidern
tx - anticoagulation, eculizumab has a role so less SCT needed. - 102what enzyme defect hereditary dz can cause haemolytic anaemia?G6PD deficiency (glucose 6 phosphate dehydrogenase)
pyruvate kinase deficiency - 103what is G6PD deficiency?X linked
RBC enzyme defect
men - mediterranean, africa, middle east
most no sx BUT can have oxidative crisis precipitated by drugs (primaquine, sulfonamides, aspirin)/illness
attack - rapid anaemia + jaundice
film - bite + blister cells
tx - avoid precipitants eg henna, transfuse if severe - 104what is pyruvate kinase deficiency?AR
low ATP production causes less RBC survival
homozyg - neonate jaundice, later haemolysis w/ splenomegaly + jaundice
dx enzyme assay
tx - often none. splenectomy - 105membrane defects that cause hereditary haemolytic anaemia?hereditary spherocytosis - AD, spherical RBCs trapped in spleen - extravasc haemolysis (splenomegaly, jaundice) - dx film/ high bilirubin
hereditary elliptocytosis - AD - film - mostly no sx - may protect from malaria 10% severe phenotype death in utero.
hereditary ovalocytosis/ stomatocytosis - rare - refer
all coombs -ve, all need folate, splenectomy may help. - 106what haemoglobinopathies might cause haemolytic anaemia?SCD
thalassaemia - 107what is a coombs test?antiglobulin test
direct - blood sample - washed RBCs are incubated w/ antihuman ab (coombs reagent) - RBCs agglutinate and coombs reagent forms links between RBCs
indirect - used in prenatal testing and pre blood transfusion - detects ab against RBCs that are free in serum (incubated w/ RBCs of known antigenicity) - if agglutination - +ve - 108how might the blood film help in haemolytic anaemia?hypo micro - thalassaemia
sickle cells
schistocytes - MAHA
abnormal cells - haematological malgnancy
spherocytes - AIHA/ hereditary spherocytosis
elliptocytes
heinz bodies/ bite cells - G6PD def - 109what does a +ve coombs test mean?immune cause of HA
- 110what is MAHA?mechanical disruption of RBCs in circulation
intravascular haemolysis + schistocytes - 111causes of MAHA?HUS
TTP
DIC
pre-eclampsia
eclampsia - 112tx MAHA?cause
transfusion
plasma exchanage - 113what is sickle cell anaemia?AR
abnormal beta globin chains
Glu-val substitution at position 6
produces HbS instead of HbA
africans
homozygotes have SCD, and hetrozygotes have sickle cell trait - 114SCD vs traittrait cause no sx and protects against malaria
but in hypoxia eg unpressurised aircraft/anaesthesia - vaso-occlusive events can occur
SCD has sx - 115pathogenesis of SCD?HbS polymerises when deoxygenated - sickle cells which are fragile and haemolyse, and block small blood vessels
- 116tests for SCD?haemolysis variable
Hb 6-9
increased reticulocytes
increased bilirubin
film - sickle cells + target cells
sickle solubility test +ve but doesn't distinguish HbSS and AS (hetero)
Hb electrophoresis - confirms and distinguishes zygosity - 117ideally SCD should be diagnosed...at birth from cord blood
so can have pneumococcal prophylaxis - vaccine - 118signs/sx of SCD?vaso-occlusive crisis
aplastic crisis
sequestration crisis - 119what is a vaso-occlusive crisis?microvascular occlusion
pain triggered by cold, dehydration, infection or hypoxia
<3yrs hands and feet affected - dactylitis
occlusion can cause mesenteric ischaemia mimicking an acute abdomen
CNS infarct in 10% children - stroke, seizure, cognitive defect - transcranial doppler US can assess risk and BT can reduce risk
avascular necrosis of the femoral head
leg ulcers
low flow priaprism - 120aplastic crisis?due to parvovirus B19
sudden reduction in marrow production esp RBCs
self limiting
may need transfusion - 121sequestration crisis?children as spleen has not yet undergone atrophy
blood pooling in spleen/ liver + organomegaly, severe anaemia, shock
need urgent transfusion - 122complications of SCD?splenic infarct before 2yrs from microvascular occlusion - leads to increased susceptibility to infection - 40% childhood SCD deaths caused this way- zinc supplements
poor growth
CRF
gallstones
retinal disease
iron overload/ blood borne infection after repeated transfusion
lung damage - hypoxia-> fibrosis->pulm HTN - 123mangt chronic dz SCD?hydroxycarbamide if frequent crises
prophylaxis - abx/vax
febrile kids risk septicaemia - OP ceftriaxone can avoid admission
BMT can cure but controversial - 124prevention SCD?genetic counselling
prenatal testing
parent education can prevent deaths - 125how to manage an SCD crisis?prompt generous analgesia IV opiates
crossmatch blood, FBC, blood cultures, MSU/CXR if high temp/chest signs
IVI rehydrate
keep warm
O2 by mask if paO2 low/sats low
blind abx if fever/unwell/chest signs - cephalosporin
transfuse
plasma exchange - if rapidly worsening - 126what is acute chest syndrome in SCD?pulm infiltrates involving complete lung segments
pain, fever, tachyp, wheeze, cough
serious
main infiltrate cause is fat embolism from bone marrow/ or chlamydia infxn - 127tx acute chest syndrome in SCD?O2
analgesia
blind abx (ceph + macrolide) until culture results
bronchodilators if wheeze
transfusion
exchange if severe
ventilate if can't keep paO2 above 9.2kPa on RA - 128what are the thalassaemias?genetic unbalanced Hb synthesis
under/no production of one globin chain
RBC membrane damage - haemolysis in marrow
mediterranean, far east - 129what types of thalasssaemia are there?beta: minor/trait, intermedia, major (cooley's anaemia)
alpha - 130what causes the beta thalassaemias?point mutation in b globin genes on ch 11
- 131what tests to do in thalassaemia?FBC
MCV
film
iron
HbA2
HbF
Hb electrophoresis
MRI can monitor myocardial siderosis - 132beta thalassaemia minor/trait?carrier
no sx
mild anaemia >9
can worsen in pregnancy
often confused w/ IDA - 133beta thalassaemia intermedia?moderate anaemia but doesn't need transfusion
poss splenomegaly
causes: mild homozygous b thal mutations/ co inheritance of b thal trait + other Hbpathy
sickle cell b thalassaemia- similar picture to SCD - 134cooley's anaemia?b thal major
both b-globin genes abnormal
presents in 1st yr - severe anaemia + failure to thrive
head shape - skull bossing (from extramedullary erythropoeisis)
hepatosplenomegaly - haemolysis
osteopenia
skull XR - hair on end sign from increased marrow activity - 135tx osteopenia in b thal major?zolendronic acid
- 136tx of beta thal major?lifelong BT needed
iron overload at 10yrs w/ endocrine failure (pituitary, thyroid, DM), liver dz, cardiotoxicity - 137film in beta thal major?hypo micro
target cells
nucleated RBCs
HbF is high, Hb A absent on electrophoresis. - 138tx of the beta thals?fitness, healthy diet + folate supplements
reg lifelong transfusion
iron chelators to prevent overload - deferipone +desferrioxamine
large dose ascorbic acid to increase iron in UOP
splenectomy if persistent hypersplenism - after 5yrs
hormone replacement for endo conditions
BMT can cure - 139s/e of iron chelators?pain
deaf
cataract
retinal damage
higher risk yersinia - 140what are the alpha thalassaemias?mainly caused by gene deletions ch 16 - 4 genes.
if all deleted - death in utero (bart's hydrops)
HbH dz if 3 genes deleted - moderate anaemia + haemolysis (hepatosplenomegaly, leg ulcers, jaundice)
2 genes deleted - asymptomatic carrier
one gene gone - normal.
Información
- Descripción
- anaemia, ddx for blood characteristics, haemolytic anaemia, thalassaemia, SCD
- Creado por
- roatf1
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