Haem Flashcards
Heparin & warfarin MoA
Heparin - Prevents activation factors 2,9,10,11
Warfarin - Affects synthesis of factors 2,7,9,10
Acute intermittent porphyria tx
avoiding triggers
acute attacks
IV haematin/haem arginate
IV glucose
Acute promyelocytic leukaemia associations
associated with t(15;17)
fusion of PML and RAR-alpha genes
Auer rods (seen with myeloperoxidase stain)
DIC or thrombocytopenia often at presentation
Antiphospholipid syndrome mx in pregnancy
low-dose aspirin - at pregnancy start
low molecular weight heparin - once fetal heart is seen on ultrasound
Warm vs Cold AIHA
Warm
- most common
- IgG
- haemolytic at body temp, at extravascular (spleen)
- Mx: treat underlying, steroids (+/- rituximab)
Cold
- IgM, at 4 deg C
- complement mediated, intravasc
- Raynaud’s and acrocynaosis
- steroids work less well
Beta-thalassaemia major ix & mc
HbA2 & HbF raised
HbA absent
(B thalassemia trait: HbA2 raised & mild hypochromic, microcytic anaemia)
repeated transfusion
this leads to iron overload → organ failure
iron chelation therapy is therefore important (e.g. desferrioxamine)
Target cells in
Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease
Spherocytes
Hereditary spherocytosis
Autoimmune hemolytic anaemia
Basophilic stippling
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
Heinz bodies
G6PD deficiency
Alpha-thalassaemia
thresholds for transfusion:
Patients without ACS- 70 g/L
Patients with ACS- 80 g/L
in a non-urgent scenario, a unit of RBC is usually transfused over 90-120 minutes
Burkitt’s lymphoma associations
c-myc gene translocation
t(8:14).
Epstein-Barr virus (EBV)
microscopic: ‘starry sky’ appearance
tumour lysis syndrome after chemo. (Rasburicase given to prevent)
Complications of CLL
anaemia
hypogammaglobulinaemia leading to recurrent infections
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)
Chronic myeloid leukaemia mx
imatinib is now considered first-line treatment
- inhibitor of the tyrosine kinase associated with the BCR-ABL defect
- very high response rate in chronic phase CML
DVT mx
DVT likely
- proximal leg vein USS w/in 4hrs
- if negative -> D dimer, if negative -> alternative dx
- If delay -> interim anticoagulation
- If USS -ve but D dimer +ve -> stop interim anticoagulant, repeat USS 6-8 days later
DVT unlikely
- D dimer w/in 4hrs
- If positive -> proximal leg vein USS w/in 4hrs
types of thrombophilia
Factor V Leiden (activated protein C resistance) - most common
Prothrombin gene mutation
Protein C deficiency
Protein S deficiency
Antithrombin III deficiency
G6PD deficiency ix
G6PD enzyme assay levels should be checked around 3 months after an acute episode of hemolysis
G6PD deficiency associations
Mediterranean and Africa, most common
X-linked recessive, male
broad (fava) beans,
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs
Heinz bodies, Bite and blister cell,
intravascular haemolysis
Philadelphia chromosome
t(9;22) -> BCR-ABL gene - tyrosine kinase activity in excess of normal
CML
Hereditary angioedema mx
Acute:
IV C1-inhibitor concentrate, fresh frozen plasma (FFP) if this is not available
(adrenaline, antihistamines, or glucocorticoids to not help)
prophylaxis: anabolic steroid Danazol may help
Hodgkin’s lymphoma types
( Reed-Sternberg cell)
Nodular sclerosing - Most common (around 70%),
Lymphocyte predominant - Best prognosis
ITP mx
1st. oral prednisolone
2nd or active bleeding/ urgent invasive procedure - pooled normal human immunoglobulin (IVIG)
Platelet transfusion tx criteria
platelet count of <30 x 10^9 w significant bleeding, higher is severe
Platelet transfusion for thrombocytopenia before surgery/ an invasive procedure. Aim for plt levels of:
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
>100×109/L if surgery at critical site
A threshold of 10 x 10^9 in no bleeding or. procedure
Polycythaemia vera mx
aspirin
venesection
chemotherapy
sickle cell disease ix & mx
haemoglobin electrophoresis
Longer-term management
- hydroxyurea
- pneumococcal polysaccharide vaccine every 5 years
Crisis:
- analgesia (opiates), rehydrate, oxygen, antibiotics ifinfection
- exchange transfusion
Clinical tumor lysis syndrome
Laboratory tumour lysis syndrome plus one or more of the following:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure
high potassium and high phosphate level in the presence of a low calcium.
(patients are higher risk should receive either allopurinol or rasburicase before chemo)
