haem Flashcards
what is in serum
glucose
electrolytes
protein
where is bone marrow
pelvis
vertebrae
ribs
sternum
where do blood cells develop
bone marrow
what does the pluripotent haematopoietic stem cell form
myeloid stem cell
lymphoid stem cell
what do megakaryocytes form
platelets
what does the myeloid stem cell form
->reticulocytes->RBC
->promyelocytes->monocytes->macrophages, neutrophils, eosinophils, mast cells, basophils
what do lymphoid stem cells form
-> B cell (plasma or memory)
-> T cell (CD4, CD8, NK)
lifespan RBC
3m
anisocytosis
variation size RBC
myelodysplasic syndrome
what are target cells found in
iron deficiency anaemia
post splenectomy
when are heinz bodies found
G6PD deficiency
alpha thalassaemia
heinz bodies
blob in RBC
howell jolly bodies
blobs DNA in RBC
when are howell jolly bodies found
post splenectomy
severe anaemia
reticulocytes
larger than RBC
mesh like
when are reticulocytes found
haemolytic anaemia
schistocytes
RBC fragments
when are schistocytes found
HUS
DIC
thrombotic thrombocytopenic purpura
sideroblasts
blobs iron in immature RBC
when are sideroblasts found
myelodysplasic syndrome
smudge clls
ruptured WBC (fragile)
when are smudge cells found
CLL
spherocytes
spherical RBC
when are spherocytes found
AI haemolytic anaemia
hereditary spherocytosis
normal Hb
F: 120-165
M: 130-180
normal MCV
80-100
causes microcytic anaemia
thalassaemia
chronic disease
iron deficiency
lead poisoning
sideroblastic
causes normocytic anaemia
acute blood loss
chronic disease
aplastic
haemolytic
hypothyroid
causes macrocytic anaemia
megaloblastic: B12/folate deficeincy
normoblastic: alcohol, reticulocytosis, hypothyroid, liver disease, azathoprine
sx anaemia
tired
SOB
headache
dizzy
palpitations
pallor
increased HR and RR
features iron deficiency anaemia
picca
hair loss
koilonychia
angular chelitis
atrophic glossitis
brittle hair and nails
features haemolytic anaemia
jaundice
ix anaemia
Hb
MCV
B12
Folate
ferritin
blood film
OGD
colonoscopy
BM biopsy
where is iron absorbed
duodenum and jejunum
transferrin saturation
= serum iron / total iron binding capacity
mx iron deficiency anaemua
if no cause: OGD and colonoscopy
oral ferrous suphate, iron infusion, blood transfusion
SE oral ferrous sulphate
constipation
black stool
cause pernicious anaemia
AI
Ab against parietal cells/intrinsic factor therefore B12 not absorbed
sx pernicious anaemia
peripheral neuropathy - numbness, paraesthesia
loss vibration sense
visual change
mood change
ix pernicious anaema
auto ab- intrinsic factor ab, gastric parietal cell ab
mx pernicious anaemia
1g IM hydroxycobalamin 3/wk for 2wk then every 3m
inherited causes haemolytic anaemia
hereditary spherocytosis
hereditary elliptocytosis
thalassaemia
SC anaemia
G6PD deficiency
acquired causes haemolytic anaemia
AI
alloimmune
paroxysmal nocturnal haemoglobinuria
microangiopathic
prosthetic valve related
features hereditary spherocytosis
AD
jaundice
gallstones
splenomegaly and aplastic crisis with parvovirus
increased MCHC and reticulocytes
mx hereditary spherocytosis
folate
splenectomy
features hereditary elliptocytosis
AD
jaundice
gallstones
splenomegaly and aplastic crisis with parvovirus
increased MCHC and reticulocytes
mx hereditary elliptocytosis
folate
splenectomy
inheritance G6PD deficiency
X linked recessive
mediteranean/african
triggers of crises in G6PD defeicny
infection
meds: primaquine, ciprofloxacin, sulfonylureas, sulfasalazine
broad beans
sx G6PD deficiency
jaundice
gallstones
anaemia
splenomegaly
types AI haemolytic anaemia
warm: at normal/above temp = idopathic
cold (<10 degrees): agglutination = secondary to lymphoma, leukaemia, SLE, EBV, CMV, HIV
mx AI haemolytic anaemia
blood transfusion
pred
rituximab
splenectomy
cause alloimmune haemolytic anaemia
transfusion rxn
haemolytic disease newborb
cause paroxysmal nocturnal haemoglobinuria
gene mutation in life results in activation complement cascade
features paroxysmal nocturnal haemoglobinuria
red urine in morning
risk VTE
smooth muscle dystoniam
mx paoxysmal nocturnal haemoglobinuria
eculizimab
BM transplant
cause microangiopathic haemolytic anaemia
vessel abnormality damages RBC
secondary to HUS, DIC, TTP, SLE, cancer
cause prosthetic valve haemolysis
valve churns up RBC
mx prosthetic valve haemolyssi
oral iron
transfusion
revision surgery
inheritance thalassaemia
AR
features thalassaemia
microcytic anaemia
fatigue
pallor
jaundice
gallstones
splenomegaly
poor growth
pronounced forehead and malar eminences
ix thalassaemia
FBC
Hb electrophoresis
DNA
why is there iron overload in thalassaemia
faulty RBC
transfusions
features iron overload
fatigue
liver cirrhosus
impotence
HF
arrhythmia
DM
osteoporosis
mx iron overload
iron chelation
alpha thalassaemia mutation
chrom 16
mx alpha thalssaemia
transfusion
splenectomy
BM transplant
beta thalassaemia mutation
chrom 11
types beta thalassaemia
minor: 1 normal and 1 abnormal gene
intermedia: 2 abnormal beta globin
major: homozygous for deletion
mx intermediate beta thalassaemia
transfusion
mx major beta thalassaemia
regular transfusion
BM transplant
splenecomy
what is HbF replaced by
HbA at 6w
inheritance SC anaemia
AR
abnormal gene for beta globin on chrom 11
SC trait genes
1 copy faultygene
complications SC disease
anaemia
increased risk infection
stroke
avsascular necrosis
pulmonary HTN
priapism
CKD
SC crisis
vaso-occlusive/painful crisis
splenic sequestration crisis
aplastic crisis
acute chest syndrome
triggers SC crisis
infection
dehydration
cold
cause vaso-occlusive crisis SC
sickle cells block capillaries
features splenic sequestration crisis SC
severe anaemia
hypovolaemic shock
mx splenic sequestration crisis SC
blood transfusion
fluid
what happens in aplastic crisis SC
triggered by parvovirus B19
no new RBC made ->anaemia
causes acute chest syndrome SC
infective or non infective (vaso-occlusive, fat emboli)
features acute chest syndrome SC
fever
resp sx
new infiltrates on CXR
mx SC disease
avoid dehydration
vaccinations
abx prophylaxis - penicillin V
hydroxycarbamide - increaes HbF
blood transfusion
BM transplant
what is leukaemia
cancer BM
mutation results in overproliferation of 1 cell type
sx leukaemia
fatigue
fever
FTT
pallor
petechiae/bruising
abnormal bleeding
lymphadenopathy
hepatosplenomegaly
ix leukaemia
FBC
blood film
increased LDH
BM biopsy
CT/MRI/CXR
associattions ALL
<5 or >45
down syndrome
philadelphia chromosome
most common leukaemia adults
CLL
features CLL
warm haemolytic anaemia
richters transformation to lymphoma
smudge/smear cells
phases CML
chronic: asx - 5y
accelerated
blast
chromosome CML
philadelphia
what can cause AML
transformation from myeloproloiferative disorder
Blood film AML
auer rods
mx leukaemia
chemo
steroids
BM transplant
SE chemo
reduced growth
infection
neurotoxicity
inferitlity
seconadry maligancy
cardiotoxixity
tumour lysis syndrome (release uric acid ->AKI)
lymphoma
cancers of lymphocytes
RF hodgkins lymphoma
20y or 75y
HIV
EBV
RA
fhx
features hodgkins lymphoma
lymphadenopathy: rubbery, pain with alcohol
B sx: fever, wt loss, night sweats
ix findings hodgkins lymphoma
increased LDH
lymph node biopsy=reed sternburg ells
mx hodgkins lymphoma
chemo and radio
staging for lymphomas
ANN ARBOR
1. 1 region of lymph nodes
2. >/= 1 region same side of diaphragm
3. lymph nodes above and below diaphragm
4. non lymph organs e.g. lungs
e.g. non hodgkins lymphoma
burkitt
MALT - mucosa associated lymphoid tissue
diffuse large B cell
RF burkitt lymphoma
EBV
malaria
HIV
RF MALT lymphoma
H.pylori
features diffuse large B cell lymphoma
> 65y
painless
myeloma
cancer of plasma cells
sx myeloma
CRAB
increased calcium
renal failure
anaemia
bone lesions and pain
RF myeloma
age
M
african
Fhx
ix myeloma
bloods: reduced WCC, increased Ca, increased ESR
increased plasma viscocity
urine elctrophoresis
serum free light chain assay
serum Ig
serum protein electrophoresis
BM biopsy
XR
CT/MRI/bone scan
finding urine electrophoresis myeloma
bence jones protein
XR findings myeloma
punched out lytic lesions
pepperpot skull
mx myeloma
chemo
stem cell transplant
VTE mx
bisphosphonates
what are myeloproliferative disorders
proliferation 1 stem cell
genes associated with myeloproliferative disorders
JAK2
MPL
CACR
features myeloproliferative disorders
systemic sx
anaemia
splenomegaly
portal HTN
ix myeloproliferative disorders
FBC
blood film
BM biopsy
what can myeloproliferative disorders lead to
myelofibrosis - therefore haematopoesis in liver and spleen istead
types myeloproliferative disorders
primary myelofibrosis
polycythaemia rubra vera
essential thromocytopenia
features primary myelofibrosis
haematopoetic stem cell
anaemia
teardrop RBC
anisocytosis
blasts
mx primary myelofibrosis
chemo
allogenic stem cell transplant
Ix findings polycytheamiea rubra vera
erythroid cells
increased Hb
mx polycythaemia rubra vera
venesection
aspirin
chemo
features essential thrombocytopenia
megakaryocyte
increased plateleyts
mx essential thrombocytopenia
aspirin
chemo
myelodysplastic syndrome
myeloid BM cells dont mature properly -> anaemia, neutropenia, thrombocytopenia
RF myelodysplastic syndrome
> 60
prev chemo/radio
thrombocytopenia
low platelets
causes thrombocytopenia
production: sepsis, reduced B12/folate, liver failure, leukaemia, myelodysplastic syndrome
destruction: meds (valproate, methotrexate, isotretinoin, antihistamines, PPI), alcohol, ITP, thrombottic thrombocytopenic purpura, herparin induced thrombocytopenia, HUS
what is ITP
Ab against platelets
mx ITP
pred
IVIg
rituximab
splenectomy
what is thrombotic thrombocytopenix purpura
clots in small vessels
mx thrombotic thrombocytopenic purpura
plasma exchange
steroids
rituximab
features heparin induced thrombocytopenia
HIT Ab
how does thrombocytopenia present
bleeding
bruising
cause von wilebrand diseae
AD inheritance
defective VWF
types von willebrand disease
1-3
3 most sev
sx von willebrand disease
bleeding gums
epistaxis
menorrhagia
fhx
mx von willebrand disease
desmopressin (stimulates VWF)
factor VII/VWF infusion
usually dont need daily mx, only whe surgery
inheritance haemophilia
X-linked recessive
so males
types haemophilia
A: reduced VIII
B: reduced: IX
sx haemophilia
bleeding-major haemorhage (intracranial, joints, muscles)
mx haemophilia
IV infusion factor VIII/IX
desmopressin
tranexamic acid
what do thrombophilias predispose patient to
blood clots
e.g. thrombophilias
antiphospholipid syndrome
antithrombus deficiency
protein C or S deficiency
factor V leiden
hyperhomocysteinaemia
prothrombin gene variant
RF VTE
immobile
surgery
long haul flights
pregnancy
oestrogen HRT/COCP
malignancy
polycythaemia
SLE
thrombophilia
DVT sx
unilateral leg swelling (>3cm compared to the other side 10cm below tibial tuberisty)
dilated superficial veins
tender
VTE ix
wells score
d-dimer
doppler
CTPA
mx VTE
LMWH (dalteparin)
then warfarin or NOAC (apixaban, rivaroxaban)
ix unprovoked DVT
CXR
FBC
Ca
LFTs
urine dip
CT abdo pelvis
mammogram
antiphospholipd Ab
budd chiari syndrome
clot in hepatic vein -> hepatitis
sx budd chiari syndrome
abdo pain
hepatomegaly
ascites
mx budd chiari syndrome
heparin/warfarin
pancytopenia
anaemia, thrombocytopenia, and leukopenia
agranulocytosis FBC
depleted levels of basophils and eosinophils
universal donors
In adults, group O red cells can be universally donated (due to lack of A or B antigens on their surface) and group AB plasma can be universally donated (due to lack of anti-A or anti-B antibodies)
vit k deficiency/warfarin use coag screen
PT/INR: increased
APTT: increased
bleeding time: norm
platelet count: norm
haemophilia coag screen
PT/INR: norm
APTT: increased
bleeding time: norm
platelet count:norm
von willebrands coag screen
PT/INR: norm
APTT: norm/increased
bleeding time: increased
platelet count: norm
DIC coag screen
PT/INR: norm
APTT: norm
bleeding time: increased
platelet count: decreased
ITP coag screen
PT/INR: norm
APTT: norm
bleeding time: increased
platelet count: decreased
TTP coag screen
PT/INR: norm
APTT: norm
bleeding time: increased
platelet count: decreased
HUS coag screen
PT/INR: norm
APTT: norm
bleeding time: increased
platelet count: decreased
how do ITP, HUS and TTP differentiate on coag screen
THEY DONT
PT/INR: norm
APTT: norm
bleeding time: increased
platelet count: decreased
Common causes of acute leukocytosis
Reactive: infection, inflammation, post-surgery
Steroids: stress response (i.e. endogenous steroids) or medication (i.e. exogenous steroids)
Haematological: acute leukaemias
Common causes of chronic leukocytosis:
Reactive: chronic infection, smoking
Haematological: leukaemia, certain subtypes of lymphoma
Hyposplenism: typically mild
Pregnancy
Common causes of leukopenia
Infection: can be seen as a transient phenomenon in viral illness or as a result of consumption in sepsis
Medications: antibiotics, immunosuppressants, anti-epileptics, cytotoxic agents (e.g. chemotherapy)
B12/folate deficiency
Autoimmune disease
Iron deficiency
HIV (any cytopenia could be due to HIV)
Racial variation: middle eastern and black patients can have lower baseline neutrophil counts which are not pathological
Bone marrow failure: often this will be seen alongside low platelets and low haemoglobin
Causes of lymphocytosis include:
Viral infection
Smoking
Hyposplenism/post-splenectomy
Malignancy: leukaemia and certain types of lymphoma
Pertussis: rates are increasing in the U.K. with decreased vaccination rates
Causes of lymphopenia
infection
Infection
Older age (rarely clinically significant in this context)
Alcohol excess
HIV
Autoimmune disease
Bone marrow disease
Medications: cytotoxic agents, immunosuppressants
Renal failure
Congenital immunodeficiency disorders (e.g. Wiskott-Aldrich syndrome)
Common causes of monocytosis
Bacterial infection
Autoimmune disease
Steroids
Causes of a very low monocyte count
Acute infection
Steroids
Bone marrow failure
Cytotoxic agents (e.g. chemotherapy)
Hairy cell leukaemia
Causes of eosinophilia
Allergies/atopy
Parasitic infection
Autoimmune disease (e.g. vasculitis)
Medications (e.g. antibiotics, anti-epileptics, allopurinol)
Gastrointestinal disease (e.g. eosinophilic oesophagitis)
Respiratory disease (e.g. asthma)
Malignancy (any solid organ or haematological)
Causes of basophilia
Allergic reactions/atopy
Iron deficiency
Chronic inflammation
Hypothyroidism
Infection
Haematological malignancies (myeloproliferative disorders)
Causes of an elevated blast count
Acute leukaemia
Myeloproliferative disorders
Reactive (severe infection or treatment with G-CSF)
Cytotoxic agents (chemotherapy)
Causes of acute thrombocytopenia
Consumption (e.g. infection, bleeding)
Acute viral infection
Medications (e.g. antibiotics, anti-epileptics, cytotoxic agents)
Disseminated intravascular coagulation/microangiopathic haemolytic anaemia (e.g. TTP, HUS)
Heparin-induced thrombocytopenia (HIT)
Immune thrombocytopenic purpura (ITP)
Pregnancy: pre-eclampsia/HELLP syndrome
Causes of chronic thrombocytopenia:
Hypersplenism
Cirrhosis
Alcohol excess
Medications (e.g. anti-epileptics, cytotoxic agents)
ITP
Autoimmune disease
B12/folate deficiency
Iron deficiency
HIV
Hepatitis B/C
Haematological disease
Bone marrow failure
Causes of thrombocytosis
Reactive: inflammation/infection
Myeloproliferative disorders: typically essential thrombocythaemia although any myeloproliferative disorder can elevate platelet counts
Iron deficiency
Hypospenlism/post-splenectomy
Underlying malignancy: likely secondary to underlying inflammatory processes
indications poor prognosis in hodkins lymphoma
lymphopenia
leukpcytosis
rasied ESR
raised LDH
low alubmin
== aggressive cancer
sx non haemolytic febrile transfusion rxn
shivering
fever
headache
nausea
flushing
tachycardia
30-60m after start
PATIENT IS HOT BUT WELL
MOST COMMON RXN
cause non-haemolytic febrile transfusion rxn
alluimunised recipitant produces cytokines due to donor lukocytes/HCA antigens
mx non-haemolytic febrile transfusion rxn
slow transfusion
monitor regularly
paracetamol
what is acute haemolytic transfusion rxn also known as
ABO incompatability
cause acute haemolytic transfusion rxn
IgM mediated
sx acute haemolytic transfusion rxn
PATIENT IS SICK
fever
hypotension
agitation
flushing
chest/abdo pain
bleeding/DIC
AKI
within mins of starting
mx acute haemolytic transfusion rxn
STOP TRANSFUSION
A-E
supportive
features bacterial contaamination blood transfusion
fever
hypotension
rigors=septic shcok
features delayed haemolytic transfusion rxn
anaemia
jaundice
haemoglobinuria
usually 4-8d after but up to 4w
mx bacterial contamination blood products
stop transfusion
broad spectrum abx
sepsis 6
mx delayed haemolytic transfusion rxn
ix
monitor renal funt
features transfusion related acute lung injury
acute resp distress syndrome
dyspnoea
cough
CXR=white out
occurs <6h - usually 2h after start
mx transfusion related acute lung injury
stop transfusion
oxygen
supportive care - may need ICU
features transfusion associated circulatory overload
dyspnoea
hypoxaemia
tachycardia
increased JVP
basal creps
mx transfusion associated circulatory overload
stop transfusion
treat as acute LVF: furosemide, oxygen
cause non anaphylactic allergic transfusion rxn
plasma protein incompatability
sx non anaphylactic allergic transfusion rxn
urticaria and itch
mx non anaphylactic allergic transfusion rxn
stop or slow transfusion depending on severity
chlorpheniramine
sx anaphylactic transfusion rxn
bronchospasm
cyanosis
hypotension
soft tissue swelling
