Haem: Interactive Cases Flashcards
% Myeloblast that is abnormal
% Lymphoblasts abnormal
How to interpret FBC
B cell markers
CD19 - epitope for car t cells
CD20 - epitope for rituximab
T cell markers
All are CD3+
CD4 - Helper
CD8 - Cytotoxic
CD5
Markers of lympod differentiation
TdT marker of immature T an B lymphoblasts
Surface IgG is a marker of mature cells
List some general clinical signs of anaemia.
- Pale mucous membranes
- Tachycardia
- Cardiomegaly/CCF
List some specific clinical signs of anaemia.
- Koilonychia
- Glossitis (B12 deficiency - B12 is needed for epithelial cell replacement)
- Jaundice (haemolysis)
List some causes of microcytic anaemia.
- Iron deficiency
- Thalassemia
- Anaemia of chronic disease
List some investigationsthat are used for anaemia.
- FBC
- Blood film
- Reticulocyte count
- Haemoglobin electrophoresis
- Iron studies
List some physiological triggers for reticulocytosis.
- Haemolysis
- Haemorrhage
- Haematinics (agents that stimulate RBC production)
List some conditions in which reticulocytes are absent.
- Inadequate haematinics
- Bone marrow failure
- Acute major haemorrhage (reticulocyte response takes at least 6 hours)
What are the typical haemoglobin electrophoresis findings of thalassemia?
- High HbA2 and HbF
List some blood film features of iron deficiency anaemia.
- Pencil cells
- Anisocytosis
- Poikilocytosis
- Hypochromic
List some causes of pancytopaenia.
- Aplastic anaemia
- Leukaemia
- Infiltration (e.g. lymphoma, carcinoma)
- Drugs (e.g. chemotherapy)
- B12/folate deficiency
List some investigations for pancytopaenia.
- Blood film
- Vitamin B12 and folate
- Bone marrow biopsy
What is a defining feature of myeloid cells?
- Auer rods
- NOTE: the presence of Auer rods means that cytochemistry and immunophenotyping is not necessary
Which type of major infection is classically seen in AML?
Gram-negative septicaemia
What are the principles of management of AML?
- Supportive - red cell and platelet transfusions, isolation
- Chemotherapy
Normal vs Myeloma pathology
List some causes of thrombocytopaenia.
- Defect in platelet production - drugs, bone marrow disorders (e.g. leukaemia, myelodysplasia)
- Increased platelet destruction - ITP, DIC, heparin-induced thrombocytopaenia
List some investigations for thrombocytopaenia.
- Coagulation screen
- Blood film
- Bone marrow aspirate
- ANA/RAPA/anti-platelet antibodies (check for rheumatoid arthritis, SLE)
- HIV (common cause of isolated platelet destruction worldwide)
List some causes of low platelets with abnormal clotting.
- DIC
- Alcohol
- Drugs
- Leukaemia
List some investigations for suspected DIC.
- Blood film
- D-dimer
- Fibrinogen
- Septic screen
- LFTs
How is DIC managed?
- Antibiotics
- Blood products
- Regular blood tests to assess response
How you get spherocytes
- Hereditary
- Autoimmune haemolytic anaemia
Causes of aquired haemolytic anaemia
Non-immune
Microangiopathic haemolytic ureamic syndrome (MAHA)
Malaria
Prostetic heart valves
Drugs: Dapsone
Immune Mediated
Autoimmune: warm/cold antibody type
Alloimmune (post blood transfusion)
Drug: methyldopa, penicillin
Causes of inherited haemolytic anaemia
Defect in:
Hb
Sickle cell
Thalassaemia
Membrane
Hereditary spherocytosis
RBC Enzymes
G6PD deficiency - would protect from oxidative stress
Causes of inherited haemolytic anaemia
Defect in:
Hb
Sickle cell
Thalassaemia
Membrane
Hereditary spherocytosis
RBC Enzymes
G6PD deficiency - would protect from oxidative stress
Biochemistry in Fe deficiency
Ferritin - needs to be low (high doesnt rule out)
Transferrin = High
Haematological picture of Pernicious anaemia
Pernicious anaemia = b12 defieicny
B12 required for DNA synthesis
1st - RBC
2nd - WBC, Platelets = Pancytopenia
Leukoerythroblastic blood film
Amongst RBC - teardrop cells
+ premature RBC (top Left)
Myelocyte - premature - Top Right
Causes of Pancytopenia
Leukaemia - High WCC
Many myelocyte precursors???
Chronic Myeloid Leukaemia (CML)
Clinical picture of CML
+ Blood film
As a result of BCR-ABL fusion between 9 and 22
Detected on RT-PCR
At diagnosis how do you treat CML
Imatinib
inhibitor of tyrosine kinase associated with BCR-ABL
v high respionse rate in the chronic phase
Month 60 after CML, developing blasts
Blast crisis
(acquisition of new mutations)
Jak2V61F mutation
Polycythemia Vera
Immunophenotyping - CLL
CD19 and CD5 - Both positive
Leukocytosis with all lymphocytes
Not pan = chronic
Peripheral blood - mature lymphocytes + smear/smudge cells
Gravest cytogenic rognosis
p53 (Chromosome 17 deletion)
Management of CLL based on “Brutons agammaglobinaemia”
Ibrutinib - “nib” = tyrosine kinase inhibitors
inhibitors brutons tyrosine kinase
recreating phenoytpe of inherited Brutons X linked agammaglobinaemia???
Multiple Myeloma
1 immunoglobulin raised, others are reduced = immune paresis
No
Myeloma cannot be excluded
Havent excluded light chain only myeloma -
Need to check serum free light chains or urine for bence jones proteins
Cast nephropathy
NOT AL Amyloid
Cast nephropathy is statistically the most common cause of renal failure in myeloma
Super high SFLC Kappa - light chains pass through basement membrane and prescipitate in renal tubules
AL amyloid - would see congo red / apple green bifringent depositions in kidney biopsy or small bowel or heart
Bleeding from wound and venepuncture sites withou stopping
which blood product to use?
PT and APTT is normal
Fibrinogen low
Cryoprecipitate
(has higher fibrinogen than FFP)
Higher APTT etc - give FFP
