Haem Flashcards
what is mcv for microcytic anaemia and what does it suggest
MCV < 80
insufficient haemoglobin production
what is mcv for normocytic anaemia and what does it suggest
mcv 80-100
decreased blood volume or decreased erythropoiesis
what is mcv for macrocytic anaemia and what does it suggest
mcv > 100
defective DNA synthesis and repair
clinical features of anaemia
pallor
dyspnoea on exertion
fatigue
tachycardia (bcos need more blood)
koilonychia (iron deficiency)
pica
what would iron studies for iron deficiency anaemia show
low ferritin (confirms IDA)
raised transferrin
increased TIBA
what would iron studies show for anaemia of chronic disease
high ferritin
low transferrin
reduced TIBC
raised ESR
increased hepcidin
what investigation would you use to diagnose thalassaemia
haemoglobin electrophoresis
what investigation would you use to diagnose sickle cell disease
haemoglobin electrophoresis
what does increased HbA2 suggest
beta thalassemia trait
what does blood film show for iron deficiency anaemia
pencil poikilocytes
hypochromic and microcytic red cells
target cells
anisopoikilocytosis
investigations for macrocytic anaemia
vit b12 and folate levels
homocysteine and methylmalonic acid levels
what do hypersegmented neutrophils on blood film suggest
megaloblastic anaemia (b12/folate deficiency)
test for autoimmune haemolytic anaemia
DAT test (aka Coombs test)
lab results for haemolytic anaemia
(and first line treatment)
raised unconjugated bilirubin
raised LDH (raised when intracellular contents released)
raised reticulocytes
low haptoglobin
spherocytes on blood film
may be warm (IgG antibodies, SLE, CLL) or cold (IgM antibodies, mycoplasma, mononucleosis, lymphoma)
Tx with steroids (prednisolone)
management for anaemia
blood transfusion with RBCs if severe
nutrient replacement
IDA > ferrous sulfate
Ix for microcytic anaemia
FBC
blood film
blood chemistry
iron studies
mnemonic for types of microcytic anaemias
Find Those Small Cells Last
Fe deficiency
Thalassemia
Sideroblastic
Chronic disease
Lead poisoning
causes of iron deficiency anaemia
decreased intake:
-eating disorders
-dietary restrictions
-food insecurity
decreased absorption
-coeliac disease
-surgical resection of GI tract
-bariatric surgery
-excessive dietary calcium
-tannates
-oxalates
increased need and growth
-pregnancy, lactation
-growing children
increased loss of blood
complications of iron deficiency anaemia
high output heart failure
angina
cardioresp failure
impaired growth and development
signs and symptoms of iron deficiency anaemia
split into decreased oxygen to tissues and effects on epithelium
decreased oxygen to tissues
-pallor
-fatigue
-exertional dyspnoea
-angina
-compensation: palpitations, increased pulse, increased cardiac output, tachypnoea, shunting of blood to vital organs
effects on epthelium
-glossitis
-scaling, fissuring, dryness, lip scaling
-koilonychia
-oesophageal stricture
lab results for IDA
low RBC count
low/normal reticulocytes
low hb, haematocrit
hypochromic-microcytic erythrocytes
- low MCV, low MCH, low MCHC
blood film shows rbc with central pallor, anisocytosis, poikilocytosis, target cells
iron studies show:
decreased serum iron
decreased ferritin
high transferrin
increased total iron binding capacity
treatment for IDA
PO iron supplements (ferrous sulfate)
parenteral iron for severe persistent anaemia or intolerance or non adherence to PO iron
increase dietary iron
vit C increases absorption
calcium DECREASES absorption
blood transfusion
components of HbF
2 alpha globin + 2 gamma globin
components of HbA
2 alpha globin + 2 beta globin
components of HbA2
2 alpha globin + 2 delta globin
ethnic group beta thalassaemia most commonly seen
Mediterranean descent
ethnic group associated with alpha thalassaemia
asian and african
what types of beta thalassaemia are there
beta globin chains are coded by two alleles so two forms of the disease
beta thalassaemia minor (one defective allele)
beta thalassaemia major (two defective alleles)
what are the types of alpha thalassaemia
alpha globin chains are coded by four alleles so four forms of the disease
silent carrier (one defective)
alpha thalassaemia trait (two defective)
haemoglobin H disease (three defective)
haemoglobin bart disease (four defective alleles)
clinical features of beta thalassaemia
minor - mild microcytic hypochromic anaemia, symptomatic
major- severe haemolytic anaemia, hepatosplenomegaly, skeletal deformities
clinical features of alpha thalassaemia
silent carrier - asymptomatic
alpha thalassaemia trait - microcytic hypochromic red cells, no anaemia
haemoglobin H (HbH) disease - microcytic hypochromic anaemia with anaemia
haemoglobin bart disease - intrauterine death
Ix for thalassaemia
Hb - electrophoresis to check HbA2 levels and HbH
FBC (microcytic hypochromic anaemia)
raised reticulocytes
interpretation of Hb electrophoresis for thalassemia
minor alpha thalassemia:
normal or low MCV/MCH, normal or low HbA2, normal HbF
HbH disease:
low MCV/MCH, normal or low HbA2, normal or high HbF, HbH present
beta thalassemia:
low MCV/MCH, high HbA2, high HbF, absent HbH
management for thalassaemia
thalassaemia major - lifelong transfusion therapy - may lead to iron overload and organ failure so iron chelation therapy with desferrioxamine (deferoxamine)
genetic counselling and screening tests for relatives
folic acid supplements to support erythropoiesis
consultation with cardiology, other specialties
ongoing monitoring
complications of beta thalassaemia
haemolytic, microcytic, hypochromic anaemia:
- chronic leg tissue hypoxia
- leg ulcers
- high output heart failure
- hypermetabolic state > nutritional deficiency
extramedullary haematopoiesis:
- bone marrow hyperplasia which can lead to structural malformations
haemolysis
- increased bilirubin leads to gallstones
iron overload
- myocardium > arrythmia, restrictive cardiomyopathy, heart failure
- endocrinopathies
- liver cirrhosis and hepatocellular cancer
- renal insufficiency
treatment related complications
signs and symptoms of thalassemia
hypoxia:
-systemic: pallor, fatigue, activity intolerance
-cardiac: low bp, tachycardia, arrythmias
chronic haemolysis:
- jaundice, dark urine, hepatosplenomegaly
lab results thalassaemia
reduced serum Hb
hypochromic microcytic erythrocytes
blood smear (poikilocytosis, anisocytosis, erythroblasts, target cells)
increased LDH
increased unconjugated bilirubin
decreased haptoglobin
increased serum iron and ferritin
how does chronic disease cause anaemia
infective organism wants to utilise body’s iron
cytokines switch off iron transport via hepcidin in response
increases iron storage and decreases its presence in serum
is anaemia of chronic disease normocytic or microcytic
usually normocytic but eventually become microcytic when iron is depleted
point mutation for sickle cell disease
beta globin gene, Chr 11
autosomal recessive
sickling of cells is predisposed by
hypoxia
dehydration
acidosis
infection
lab results for sickle cell disease
blood film:
Howell-Jolly Bodies
Sickled cells
vaso-occlusion, haemolysis, splenic sequestration > decreased haemoglobin, haematocrit, RBC count
reticulocytosis
increased WBC count
what happens during sickle cell crisis
acute painful crisis
stroke
sequestration crisis
chronic cholecystitis
management of sickle cell crisis
mnemonic - Sickle Acute Painful Crisis
Supportive oxygen
Antibiotics (if needed)
Pain relief
Cannula (IV fluids) and Crizanlizumab for prevention
Exchange blood transfusion
Splenectomy
Cholecystectomy
Diagnostic tests for sickle cell anaemia
Hb electrophoresis
blood film
complications of sickle cell anaemia
dactylitis
acute chest syndrome
haemolytic anaemia
priapism
aplastic crisis
management of sickle anaemia
conservative:
-trigger avoidance
-vaccination
medical:
-vaccinations
-hydroxyurea (increases cell deformability, decreases RBC endothelial adhesion, reduces sickling)
-hydroxycarbamide
-prophylactic Abx
- L-glutamate (reduced sickling)
- pain management
surgical:
-bone marrow transplant (curative)
what type of anaemia is sickle cell disease
normocytic haemolytic anaemia
causes of megaloblastic anaemia
cobalamin and/or folate deficiency
b12 deficiency
impaired DNA synthesis during erythropoiesis
insufficient diet or increased requirements
malabsorption
what causes pernicious anaemia
vitamin b12 deficiency secondary to intrinsic factor (IF) deficiency
signs and symptoms of megaloblastic anaemia
fatigue, activity intolerance, pallor
compensatory mechanisms: increased heart rate, bounding pulse
jaundice, splenomegaly
from neuronal demyelination: numbness, glove and stocking paraesthesia, weakness
hyporeflexia
rombergs +ve
lab results megaloblastic anaemia
increased MCV and MCH
hypersegmented neutrophils
anisocytosis and poikilocytosis
decreased serum hb and haematocrit
decreased serum b12 or folate levels
increased homocysteine and methylmalonic acid
causes of non-megaloblastic macrocytic anaemia
Myelodysplasia
Hypothyroidism
Liver disease
Alcohol
features of haemolytic anaemia
scleral icterus
pale conjunctivae and skin
blood test results haemolytic anaemia
Hb low
Haptoglobin low
Unconjugated bilirubin raised
LDH raised
Causes of hereditary haemolytic anaemias
membrane - hereditary spherocytosis
enzymes- G-6-P-D deficiency
haemoglobin - sickle cell, thalassaemia
causes of g6pd deficiency
genetic - x linked recessive
certain drugs, fava beans
infection
oxidative stress > build up of free radicals
signs and symptoms of g6pd deficiency
asymptomatic until exposed to oxidative stress
acute haemolysis:
-pallor, jaundice, dark urine
-abdo/back pain due to increased splenic activity
favism
investigations and results for g6pd deficiency
decreased hb
heinz bodies
bite cells
elevated bilirubin
elevated LDH
decreased haptoglobin
reticulocytosis
screening
g6pd assay
what could parvovirus b19 cause
aplastic crisis
signs and symptoms of hereditary spherocytosis
jaundice
anaemic signs and symptoms
splenomegaly
ix for hereditary spherocytosis
osmotic fragility test + coombs test negative
low hb
reticulocytosis
spherocytosis
schistocytes
elevated bilirubin, elevated ldh, decreased haptoglobih
treatment for hereditary spherocytosis
splenectomy
blood transfusions
phototherapy
folic acid supplements
what do you find on blood film for microangiopathic haemolytic anaemia
sheared RBCs - schistocytes
what do you find on blood film for microangiopathic haemolytic anaemia
sheared RBCs - schistocytes
haemolytic uraemic syndrome triad
thrombocytopenia
microangiopathic haemolytic anaemia
acute kidney injury
typical patient profile for haemolytic uraemic syndrome
child infected with shiga toxin-producing e.coli with bloody diarrhoea
what is atypical haemolytic uremic syndrome
no preceding diarrhoea
lab results for haemolytic uremic syndrome
requires triad
proteinuria, haematuria
schistocytes on blood film
differential diagnosis for haemolytic uraemic syndrome
TTP - measure ADAMTS13 activity in plasma
DIC - DIC panel (pTT, INR, d-dimer, fibrinogen)
abx for haemolytic uraemic syndrome
none!
shiga-like toxin clears in days to week, abx not recommended as dead bacteria potentially release more toxins
what is disseminated intravascular coagulation
severe concurrent clotting and bleeding
causes of DIC
pancreatitis
sepsis
obstetric complications
cancers
trauma
ABO reaction
features of DIC
bleeding features:
- petechiae
- ecchymoses
- haematuria
clotting features:
- prolonged APPT
- prolonged PT
haemolytic features:
- jaundice
- conjunctival pallor
+ features of underlying pathology
lab results for DIC
low platelets
low Hb
low fibrinogen
low clotting factors
increased PT
increased aPPT
increased FDPs
increased d-dimer
schistocytes due to MAHA
cause thrombotic thrombocytopaenic purpura
defunct ADAMTS-13 enzyme
clinical signs for TTP
ADAMTS:
Antiglobulin negative
Decreased platelets
AKI
MAHA
Temperature
Swinging CNS signs
DIC management
treat underlying disease
platelet + coagulation factors transfusion
anticoagulation - heparin
complication for DIC
acute renal failure
life-threatening haemorrhage
haemothorax
gangrene
blood cancer classification
leukaemia:
- myeloid
- lymphoid
lymphoma:
- Hodgkin
- non-Hodgkin
other:
- myelodysplasia
- myeloma
- polycythaemia vera, myelofibrosis, etc
which cells are affected acute myeloid leukaemia
rapid proliferation of MYELOBLASTS
these crowd out bone marrow so you don’t get downstream production of neutrophils and other granulocytes
risk factors for AML
incidence increases with age
down’s syndrome
irradiation
anti-cancer drugs
signs and symptoms AML
bone marrow failure: pallor, bleeding, infections
tissue infiltration: swollen gums, mild splenomegaly
Auer rods on cytology
signs of neutropenia, anaemia, thrombocytopenia
what is acute promyelocytic leukaemia
hyper-aggressive subtype of AML
genetic translocation - fuses RAR alpha gene with PML gene
Faggot cells on cytology- lots of Auer rods
what cells are proliferated in ALL
lymphoblasts
most common childhood blood cancer
ALL
risk factors ALL
childhood
genetics
radiation
influenza
signs and symptoms of ALL
bone marrow failure: pallor, bleeding, infections
tissue infiltration: lymphadenopathy, hepatosplenomegaly, swollen testes, tender bones
how do signs and symptoms of ALL differ from AML
lymph node swelling common in ALL unlike AML
B symptoms often present in ALL (fever, night sweats, weight loss)
hyperproliferation of which cell types in CML
myeloid stem cells (granulocyte precursor)
3 stages of CML
chronic phase
accelerated phase
blast crisis
risk factors for CML
male
Philadelphia chromosome (BCR-ABL1 fusion gene)
signs and symptoms of CML
up to 50% asymptomatic
massive splenomegaly
hypermetabolic symptoms:
- weight loss, malaise, sweating
bone marrow failure:
- pallor, bleeding, infections
hyperviscosity symptoms: (too many rbcs)
- thrombotic events, headaches
pathophysiology of CLL
progressive accumulation of functionally incompetent lymphocytes caused by failure of apoptosis
ALL bone marrow biopsy result
> 20% lymphoblasts on bone marrow biopsy
risk factors CLL
male
genetics
signs and symptoms of CLL
50% asymptomatic
occasionally non-tender lymphadenopathy
occasional bone marrow failure symptoms
how is CLL usually diagnosed
routine blood test - lymphocytosis
smear/smudge cells on blood film
leukaemia investigations to order
FBC
- unusual cell counts
LDH
- elevated in cancer
Blood smear
- to look at cells
Bone marrow aspirate
- enables conclusive diagnoses
CXR
- to look for mediastinal lymphadenopathy (ALL)
Immunophenotyping
- looking at cell markers and antigens to enable leukaemia classification
how do patients with lymphoma present
lump + systemic symptoms (FLAWS)
hodgkins lymphoma risk factors
bimodal age distribution (peaks between 20-30 and >50)
50% association with EBV (warning: EBV presence non specific)
hodgkins lymphoma sign and symptoms
painless enlarging neck mass which may become painful after alcohol consumed
B symptoms
non tender rubbery lymphadenopathy with splenomegaly +/- hepatomegaly
neutrophilia
diagnosis of Hodgkins lymphoma
Reed-Stenberg cells on lymph node biopsy
these are bi-nucleate lymphocytes
Who’s going to ace their exams
chelsea, hannah, maz and wese
risk factors for non Hodgkins lymphoma
EBV, HIV, SLE, Sjorgens
incidence increases with age
signs and symptoms of non-Hodgkins lymphoma
painless enlarging mass in neck, axilla or groin
B symptoms (tho less common than HL)
organ involvement - skin rashes, headache, hepatosplenomegaly
neutropenia
diagnosis of non hodgkins lymphoma
no reed-sternberg cells on lymph node biopsy
Burkitt lymphoma usually cancer of what cells
b lymphocytes
risk factors of Burkitt lymphoma
strong association with EBV infection
chronic malaria reduces resistance to EBV
HIV
presentation of burkitt lymphoma
rapidly enlarging lymph node in jaw
what does microscopy show in burkitt lymphoma
starry sky appearance
how is lymphoma staged
Ann Arbor staging system
stage 1: single lymph node region or single organ
stage 2: two or more lymph node regions same side of diaphragm
stage 3: two or more lymph node regions above or below the diaphragm
stage 4: widespread disease, multiple organs with or without lymph node involvement
cause of tumour lysis syndrome
metabolic abnormalities that arise as result of cancer treatment
release of contents from lysed cells causes a constellation of symptoms
symptoms of tumour lysis syndrome and how they arise
released phosphate:
- forms calcium phosphate crystals
- leads to kidney failure and hypocalcaemia
released potassium:
- causes hyperkalaemia
- leads to arrythmia
released uric acid:
- forms urate crystals
- leads to gout
which cells are proliferated in multiple myeloma
do not be fooled - not myeloid lineage
proliferation of plasma cells
what do cancerous plasma cells produce in multiple myeloma
monoclonal immunoglobulin (IgG or IgA)
risk factors for multiple myeloma
age > 70
Afro-Caribbean
ionising radiation
agricultural work (benzene, herbicides)
HIV
presentation of multiple myeloma (hint: cancer star sign symbol)
CRAB
Calcium high: bones, stones, abdo groans
Renal impairment: worse prognosis
Anaemia: due to bone marrow crowding
Bone lesions: increased osteoclast activation (back/rib pain)
monoclonal Ig also crowds out production of normal polyclonal Ig causing infections
multiple myeloma investigations
bloods:
-raised ESR, CRP, urea, Cr, Ca
- normal ALP (distinguish form bone malignancy where ALP high)
blood film:
- Rouleaux (rbc stacked like coins) formation
serum/urine electrophoresis:
- Bence Jones proteins
bone marrow aspirate:
- increased plasma cells > 10%
x-ray:
- to assess osteolytic lesions
what is monoclonal gammopathy of unknown significance
pre-malignant condition with accumulation of some monoclonal plasma cells
absent CRAB features
what is myelodysplasia
group of syndromes where the immature blood cells do not mature normally
difference between primary and secondary myelodysplasia
primary: intrinsic bone marrow problem
secondary: previous chemotherapy/radiotherapy
myelodysplasia signs and symptoms
may initially be asymptomatic
chronic pancytopaenia (anaemia, neutropenia, thrombocytopaenia)
presentation of haemophilia
usually presents early in life or after surgery/trauma
deep bleeding and bruising (secondary haemostasis)
- haemarthrosis
- haematoma
- excessive bruising and haematuria
x-linked recessive (male)
which clotting factor deficient in haemophilia A
factor 8
which clotting factor deficient in haemophilia B
factor 9
diagnosis of haemophilia
prolonged APTT (factor 8 + 9 in intrinsic pathway)
factor assay to confirm diagnosis
which clotting factor does vWF stabilise
factor 8
what is type 1 von willebrand syndrome
reduced levels of normal vWF
autosomal dominant
what is type 2 von willebrand syndrome
adequate levels of abnormal vWF
autosomal dominant
what is type 3 von willebrand syndrome
complete lack of vWF and reduced factor 8
autosomal recessive
presentation von willebrand syndrome
superficial bleeding
bruising, epistaxis, menorrhagia
prolonged gum bleeding after dental procedures
prolonged bleeding from minor wounds
type 3 more severe and causes deep bleeding into joints and soft tissues
diagnosis of von willebrand syndrome
depends on type
prolonged bleeding time
prolonged APTT and normal PT (factor 8 reduction)
reduced vWF (except in type 2)
normal platelets
difference between acute overt DIC and chronic non-overt DIC
Acute overt:
emergency and life-threatening
significant platelet and clotting factor depletion > bleeding
Chronic non-overt:
slower rate, time for compensatory responses
hypercoagulable state but less bleeding
what is haemochromatosis
autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation
risk factors for haemochromatosis
genetics
male
> 40 yrs
types of haemochromatosis and how they are caused
primary:
- hereditary (autosomal recessive)
- mutations on the HFE gene (hint: H(igh) Fe)
- most common form
secondary:
- caused by iron overload
- transfusion related
clinical features of haemochromatosis
most asymptomatic
triad (fatigue, arthralgia, erectile dysfunction)
signs of secondary diabetes
tanning (bronze pigmentation)
liver - cirrhosis, hepatomegaly
cardiac failure
loss of libido due to hypogonadism
Ix for haemochromatosis
kinda opposite to IDA results
high serum iron
serum transferrin saturation >45%
transferrin low
raised serum ferritin
low TIBC
genetic tests
management of haemochromatosis
dietary change (low iron diet)
therapeutic phlebotomy: (1st line)
- regular venesection weekly
- target ferritin and transferrin levels
drug-induced iron chelation with deferoxamine (2nd line)
complication of haemochromatosis
cirrhosis
diabetes mellitus
heart failure
hepatocellular carcinoma (may need USS)
hypogonadism
what is Wilson’s disease
autosomal recessive disorder leading to excess copper deposition in tissues hence LOW COPPER IN SERUM
presentation of Wilson’s disease
onset usually between 10-25 yrs old
combination of liver and neurological disease
liver - hepatitis, cirrhosis
neurological - basal ganglia degeneration, asterixis, chorea, dementia
Kayser-Fleischer rings
classification of haemophilia based on severity
mild:
- haematomas following severe trauma
- factor 8/9 activity between 5-50%
moderate:
- haematomas following mild trauma
- factor 8/9 activity between 1-5%
severe:
- spontaneous haematomas
- factor 8/9 activity <1%
management of haemophilia
substitution of clotting factors (factor concentrates)
desmopressin - may be given for mild haemophilia A. triggers release of vWF leading to increase of factor 8
antifibrinolytic therapy (aminocaproic acid or tranexamic acid)
management of von willebrand disease
tranexamic acid for mild bleeding
desmopressin
factor 8 concentrate
causes of hyposplenism
operative splenectomy
sickle cell anaemia
coeliac disease
CLL
IBD
bone marrow transplant
congenital asplenia
indications for splenectomy
trauma
spontaneous rupture
hypersplenism
neoplasia
most common organisms associated with severe infection in hyposplenism
NHS or No Happy Spleen
N.meningitidis
H.influenzae type b
S.pneumoniae (pneumococcus)
presentation of hyposplenism
sepsis following splenectomy due to infection (pyrexia, hypotension, tachycardia)
Ix for hyposplenism
blood film:
howell jolly bodies, target cells, siderocytes
imaging:
USS, CT, MRI
following splenectomy - thrombocytosis (may need prophylactic aspirin)
Mx for hyposplenism
immunisations
prophylactic antibiotics (penicillin or amoxicillin) - recommended in patients at high risk of pneumococcal infections
what are the myeloproliferative disorders
CML
polycythaemia vera
essential thrombocytosis
primary myelofibrosis
what is essential thrombocytosis
aka primary thrombocythaemia
chronic myeloproliferative disorder associated with sustained dysregulated megakaryote proliferation in the bone marrow increasing the number of circulating platelets causing thrombocytosis
risk factors for essential thrombocytosis
50-70 years
JAK2 mutation
main conditions with massive splenomegaly
CML and myelofibrosis
signs and symptoms of myelofibrosis
massive splenomegaly
pancytopenia
dry tap - failure of bone marrow aspirate
tear drop poikiloctes (dactrocytes) on blood film
clinical features of myleproliferative disorders
erythromelalgia - burning pain and dusky congestion of extremities, pain worse with heat
splenomegaly
arterial and venous thrombosis
bleeding
Ix for myeloproliferative disorders
FBC - platelet count > 450 x 10^9/L
JAK2 mutation testing
Mx of myeloproliferative disorders
hydroxycarbamide - used to reduce platelet count
antiplatelet therapy - aspirin
causes of pancytopenia
aplastic anaemia
myelofibrosis
multiple myeloma
methotrexate
leukaemia
chemotherapy
radiotherapy
b12 or folate deficiency
lymphoma
what is polycythaemia
increased hb conc
difference between relative and absolute (true) polycythaemia
relative: normal cell mass but low plasma volume caused by dehydration or stress
absolute: increased cell mass
difference between polycythaemia vera (primary) and secondary polycythaemia
primary is EPO independent
secondary driven by excess EPO
risk factor for polycythaemia vera
Budd-Chiari syndrome
what can polycythaemia vera lead to
AML or myelofibrosis
causes on increase in EPO
appropriate: chronic hypoxia
inappropriate: hepatocellular carcinoma, renal carcinoma, EPO abuse by athletes
clinical features of polycythaemia
constitutional symptoms - weight loss, fatigue, sweating
hyperviscosity syndrome - mucosal bleeding + neurological symptoms + visual changes
facial redness
tenderness or painful burning and redness of fingers palm, heels or toes
pruritis - worse when skin touches water
splenomegaly
Ix and results for polycythaemia
hb elevated
hct elevated
JAK2 gene mutation screen
elevated platelets
decrease EPO in polycythaemia vera
increased EPO in secondary polycythaemia (check for EPO secreting tumours)
Mx for polycythaemia
phlebotomy (venesection)
antiplatelet prophylaxis (aspirin)
cytoreductive therapy
JAK2 inhibition (Ruxolitinib)
common anticoagulants
oral:
vit K antagonist (warfarin)
DOACs (apixaban)
parenteral:
heparin
how does warfarin work
vit K antagonist
vik K responsible for production of factors 2,7,9,10
has biggest effect on factor 7 hence prolonged PT
can be reversed by replacement of vit K
warm autoimmune haemolytic anaemia is associated with which immunoglobulin
IgG
warm weather makes me Glad
cold autoimmune haemolytic anaemia is associated with which immunoglobulin
IgM
cold weather makes me Mad
