Haem Flashcards
ALL:
- Most common in children less than 5 years old, associated with Down’s Syndrome
CLL:
Smudge cells, Warm haemolytic anaemia, Richter’s transformation(from CLL to an aggressive high grade b-lymphoma
AML:
Auer rods and high number of blast cells, transformation from a Myeloproliferative disorder(eg. polycythaemia vera)
CML:
Philadelphia Chromosome, has 3 phase: long chronic(several years), accelerated(blast cells take up proportion of blood cells), blast phase
Leukaemia presenting feature:
- Bleeding under the skin due to thrombocytopenia causing non-blanching lesions
Leukaemia Investigations:
- FBC within 48 hours
- Bone marrow Biopsy= for definitive diagnosis (Gold standard)
Leukaemia general mx:
- Chemotherapy
- Targeted therapy(mainly used in CLL- tyrosine kinase inhibitors eg. ibrutinib and monoclonal antibodies eg. rituximab)
Haemophilia: (2 types, inherited X–linked recessive blood disorder therefore mainly affect Males as only have 1 chromosome
A- deficiency in Factor 8
B(Christmas Disease)- deficiency in Factor 9
Haemophilia Presentation:
is a severe bleeding disorder; can result in spontaneous bleeding without trauma and bleeding from eg. internal organs, into joints, intracranial haemorrhage
Haemophilia Inv:
Bleeding score, coagulation factor assays, genetic testing
Haemophilia Mx:
- Give Clotting factors 8/9 depending on type as an IV infusion.
Von Willebrand disease:
- Is the most common inherited cause of abnormal and prolonged bleeding
-deficiency of Von-Willebrand factor(important in platelet adhesion and aggregation) - 3 types: 1(partial deficiency), 2(reduced function), 3(complete deficiency)
Mx: Tranexamic acid for mild bleeding, Desmopressin
Thrombocytopenia= Low platelet count:
either:
- reduced platelet production
- increased platelet destruction
ITP(Immune Thrombocytopenia Purpura):
TTP(Thrombotic Thrombocytopenia Purpura):
ITP: Antibodies are created against platelets, presents with Purpura (non-blanching reasons due to bleeding under the skin). Mx: Prednisolone(steroids), IV immunoglobulin, Rituximab(monoclonal antibody that targets B cells- good for autoimmune related conditions.
TTP:
- Tiny thrombus develop through blood vessels using up platelets. Causes: Thrombocytopenia, purpura, tissue ischaemia/end organ damage
(ADAMTS13 protein),
Myelodysplastic syndromes:
- Form of cancer caused by mutation of myeloid cells and has the potential to transform into AML.
-Presents with: Anaemia, Neutropenia(low neutrophils), Thrombocytopenia-bleeding and purpura (low platelets)
Inv: Bone marrow biopsy is required to confirm diagnosis
Myeloproliferative disorder:
-Uncontrolled proliferation of a single type of blood cell, could transform into AML
- JAK2 mutation
- Main ones to remember(3):
1) Primary Myelofibrosis- low Hb, Mx: JAK2 inhibitor, supportive mx, chemotherapy
2) Polycythaemia Vera- High Hb, gout and thrombosis is a complication of the condition
3) Essential thrombocythaemia High platelet
Blood film: Tear-dropped shaped RBC, Anisocytosis(varying sizes of RBCs), Blasts (immature red and white cells)
Polycythaemia Vera (type of myeloproliferative disorder):
- Red face, conjunctival plethora(red insides of eye), splenomegaly, hypertension
- Bone marrow biopsy to confirm diagnosis
Mx: venesection, aspirin, chemo(hydroxycarbamide)
Myeloma:
Reversal agent for Dabigratran(anticoagulant):
-Idarucizumab
-Other reversal agent: Protamine to reverse=heparin, Vitamin K=warfarin