Haem Flashcards
Mnemonic for macrocytosis?
FAT RBC
Fetus Alcohol Thyroid (hypo) Reticulocytosis B12/Folate Cirrhosis
Cells that have granules of iron accumulated in the mitochondria surrounding the nucleus
Sideroblasts - they can be called ring sideroblasts if the mitochondria form a ring around the nucleus.
Perls’ Prussian blue stain turns blueish purple. What does it indicate the presence of?
Iron
How can the causes of sideroblastic anaemia be categorised?
Congenital
Acquired
Acquired reversible
What are causes of acquired reversible sideroblastic anaemia?
Excess alcohol
Pyidoxine (B6) deficiency
Lead poisoning
Copper deficiency
How can you treat sideroblastic anaemia?
Remove the cause Consider Pyridoxine (B6) to promote RBC production
What is the general mechanism in Anaemia of chronic disease?
IL-6 production
- -> promote hepcidin production by liver
- -> reduces ferroportin activity
- -> reduces transfer of iron to circulation
What are the Iron, TIBC and Ferritin levels like in Anaemia of Chronic Disease?
Iron: Low
TIBC: Low
Ferritin: High
What is the most common antibody involved with warm autoimmune haemolytic anaemia?
IgG
What type of antibody is usually involved in cold autoimmune haemolytic anaemia?
IgM
Is it the indirect or direct Coombs test that is used when testing for autoimmune haemolytic anaemia?
direct
Is it the indirect or direct Coombs test that is used when testing blood for transfusion?
indirect
Basic steps in a direct Coombs test?
- patient blood sample
- wash it
- add Coombs reagent (antihuman antibodies)
- agglutination = positive result
Basic steps in an indirect Coombs test?
- recipient serum
- add donor blood
- add Coombs reagent (antihuman antibodies)
- agglutination = positive
What is the inheritance pattern of beta-thalassaemia?
autosomal recessive
What impact does beta-thalassaemia have on chain synthesis and haemoglobin types?
Reduced beta-chain synthesis, excess alpha-chains.
Under production of haemoglobin A
What is the chain composition of haemoglobin A?
two alpha
two beta
What is the chain composition of haemoglobin A2?
two alpha
two delta
What is the chain composition of haemoglobin F?
two alpha
two gamma
dysphagia iron deficiency anaemia cheilosis glossitis oesophageal webs
Plummer-Vinson syndrome
Role of ferritin in the body?
store/deposit iron in a safe form (i.e. in cells away from invading pathogens)
Low iron
High TIBC
Low ferritin
Iron deficiency
Low iron
Low TIBC
High ferritin
Anaemia of chronic disease
High iron
Low TIBC
High ferritin
Chronic haemolysis
Haemachromatosis (could also have normal TIBC)
High iron
High TIBC
Normal ferritin
Pregnancy
High iron
Normal TIBC
High ferritin
Sideroblastic anaemia
Haemachromatosis
Causes ofa macrocytic megaloblastic anaemia?
B12 or folate deficiency
cytotoxic drugs
A type of anaemia due to an inhibition of DNA synthesis during red blood cell production
Megaloblastic anaemia (the folate/B12 is needed for the nucleic acid recycling)
Large immature red blood cells
Howell-jolly bodies
hypersegmented polymorphs
Megaloblastic anaemia
spectrin or ankyrin defects
Hereditary spherocytosis
just spectrin defect
Hereditary elliptocytosis
Sickle cell patient with parvovirus B19
Aplastic crisis
E. Coli
impaired renal function
diarrhoea
thrombocytopenia
Haemolytic uraemic syndrome
ADAMTS13
enzyme for cleaving vWF
Pathogenesis of TTP?
- enzyme (ADAMTS13) that cleaves vWF is inhibited
- more vWF
- more platelet clotting
- RBCs pass through clots, undergo shear stress –> rupture –> anaemia and schistocyte formation
Anaemia Jaundice Thrombocytopenia Schistocytes Neurological symptoms Renal impairment Fever
Thrombotic Thrombocytopenic Purpura
What releases factor VIII from vWF
thrombin
gpIb/IX/V
on platelets, vWF binds to these
What does vWF bind to on the blood vessel?
exposed collagen in the endothelial cells
Condition with defective/low levels of GpIIb/IIIa?
(Glanzmann’s) thrombasthenia
gpIIb/IIIa
on platelets, form crosslinks between platelets and binds fibrinogen
What do platelets secrete once activated?
ADP
TXA2
increase gpIIb/IIIa expression on platelets
Thrombocytopenia
Bruises and petechiae
Normal bone marrow
No iatrogenic cause
Auto-immune thrombocytopenic purpura
Long aPTT Long Thrombin time Long bleeding time Low platelets Increased D-dimer
DIC
Anaemia
Pancytopenia
t(15;17)
Acute promyelocytic leukaemia
Anaemia
Pancytopenia
Myeloperoxidase and Sudan black stain positive
AML
Anaemia
Pancytopenia
Myeloperoxidase and Sudan black stain negative
ALL
t(9;22)
less than 5% blasts
What’s the diagnosis and treatment?
CML - chronic phase
Treatment = Tyrosine Kinase inhibitor, e.g. imatinib
t(9;22)
greater than 20% blasts
What’s the diagnosis and treatment?
CML - Blast phase
Treatment =
- Tyrosine Kinase inhibitor, e.g. imatinib
- induction chemo (as for AML like cytarabine plus a ‘rubicin’)
- all-SCT
t(9;22)
between 10 and 20% blasts
What’s the diagnosis and treatment?
CML - accelerated phase
Treatment=
- Tyrosine Kinase inhibitor, e.g. imatinib
- consider allo-SCT plus induction chemo like cytarabine with a ‘rubicin’
Old person
Lymphocytosis
Smear cells
Flow cytometry demonstrating coexpression of CD5 and CD19
CLL
Binet staging
CLL
CD30 positivity
Anaplastic large-cell lymphoma
t(2;5)
Anaplastic large-cell lymphoma
HTLV-1 infection
Adult T cell leukaemia
Lymphoma associated with mycosis fungoides
Cutaneous T Cell lymphoma
Myelodysplastic syndrome:
Blood:Anaemia
No blasts
BM: Erythroid dysplasia with less than 5% blasts
Refractory anaemia
Myelodysplastic syndrome:
Blood: Anaemia
No blasts
BM: Erythroid dysplasia with greater than 15% ringed sideroblasts
Refractory anaemia with ringed sideroblasts
Myelodysplastic syndrome:
Blood: Cytopenia in more than/equal to 2 cell lines
BM: Dysplasia in more than 10% cells in/greater than 2 cell lines
Refractory cytopenia with multilineage dysplasia
Myelodysplastic syndrome:
Blood: Cytopenia in more than/equal to 2 cell lines
BM: Dysplasia in more than 10% cells in/greater than 2 cell lines with ringed sideroblasts
Refractory cytopaenia with multilineage dysplasia and ringed sideroblasts
Myelodysplastic syndrome:
Blood: Cytopenia
Less than 5% blasts
No Auer rods
BM: Dysplasia and 5-9% blasts
Refractory anaemia with excess blasts - 1
Myelodysplastic syndrome:
Blood: Cytopenia or 5-19% blasts or Auer rods
BM: Dysplasias, 10-19% blasts or Auer rods
Refractory anaemia with excess blasts - II
Myelodysplastic syndrome:
Blood: Anaemia, normal or increased platelets
BM: Megakaryocytes with hypolobated nuclei and
MDS with 5q syndrome
Myeloproliferative disorder which is Philadelphia chromosome negative
Polycythemia vera
Myelofibrosis
Essential thrombocytosis
Blurred vision headaches Plethoric - congested with blood Gout Splenomegaly Raised Hb and HCT Low EPO
Polycythaemia rubra vera
Treatment of polycythaemia rubra vera?
Venesection
Hydroxycarbamide
Aspirin
JAK2 mutation
raised Hb and HCT
Low EPO
Polycythaemia rubra vera
Megakaryocytes dominate the BM
maybe associated with JAK2
Thrombosis
Essential thrombocythaemia
Tear drop poikilocytes
Leukoerythroblasts
Bm fibrosis
Myelofibrosis
Transfusion:
Urticaria
Mild allergic reaction
Transfusion:
Mild fever only
Febrile non-haemolytic transfusion reaction
Transfusion:
Bleeding
Dark urine
ABO incompatibility
Transfusion:
Swelling
Wheeze
Severe allergic reaction
Transfusion:
high fever
tachycardia
Bacterial infection
Transfusion:
Fever
Breathless
normal CVP
TRALI
Transfusion:
Fever
Breathless
Raised CVP
TACO/fluid overload
Transfusion
leukocyte antibodies in transfused plasma
TRALI
