Haematology Flashcards
Normal Hb levels in males + females
M = 130-180 F = 115 - 165
What are reticulocytes like? Why? what do they indicate?
larger than normal RBC with MCV >100, more purple (retention of RNA in cytoplasm – reticular network of RNA)
If they are raised they are the immature blood form of RBCs - indicates blood loss, an excess need for RBCs = tissue oxygenation not being met
What symptoms are specific to IDA?
- Stomatitis
- Koilonychia (spoon nails)
- Dysphagia (oesophageal epithelial integrity is damaged due to ID anaemia Plummer-Vinson syndrome)
- Pica
What symptoms are specific to MBA?
Mild jaundice
Bruising
Dementia
Neuropathy
What things cause IgM vs IgG mediated haemolytic anaemia?
IgG mediated (warm) = SLE, CLL IgM mediated (cold) = EBV, mycoplasma
Three major causes of dark urine
Cholestatic disease = conjugated bilirubin –> enter blood after cholestasis
Haemoglobinuria/haemosiderinuria in intravascular haemolysis (like G6PD def)
Or haematuria itself
What are the commonalities between sickle cell anaemia + thalassemia?
Both haemoglobinopathies.
Both investigated via Hb electrophoresis
Both produce “crew-cut appearance of skull
What are Heinz bodies? What conditions do they occur in?
Heinz bodies = precipitation of abnormal globins
Seen in:
- G6PD deficiency
- Thalassemia
5 features to know for G6Pd deficiency
Features to know
- Oxidative damage (old RBC)
- Heinz bodies (globins) – reticulin stains show small dots
- Bite + blister cells = dry RBCs [apple logo]
- Episodic haemolysis
- Splenomegaly, gall stones
hereditary spherocytosis features (5)
- Chronic haemolytic anaemia from birth (if severe) – pallor + jaundice
- Many spherocytes
- Massive splenomegaly
- Cholecystitis and black pigment cholelithiasis due to excess unconjugated bilirubin
- Aplastic, megaloblastic or haemolytic crisis
Name three inherited platelet function disorders, and which is the more common?
- VWD - more common
- Bernard-soulier syndrome
- Glanzman thrombasthenia
Rx for haemophilia
DDAVP (desmopressin)/F8 concentrate - desmopressin stimulates the release of F8 + VWF from the endothelium
How is PT done in the lab?
Blood put into citrate tube. Lab adds calcium + thromboplastin (TF) + heparin inhibtior
How is aPPT done in the lab?
Blood put into citrate tube. Lab adds calcium + phospholipid + intrinsic factor activator.
What do you suspect when both PT + APTT are raised?
Pathology of common pathway - F1,2,5,10
Pentad for TTP
Norty Fever Ruined My Tubes!
What are you thinking when you have a prolonged APTT that does not improve when you add normal plasma? Mechanism?
Normal plasma = replenish factors missing
Think antiphospholipid syndrome - its lupus anticoagulant prolongs APTT, but clinically is thrombophilic.
These autoantibodies activate coagulation in the body but bind to the phospholipid part of reagent in the laboratory to prolong PTT (so it looks like bleeding, but its actually hypercoagulability
What clinical picture does heparin-induced thrombocytopenia produce?
Pathogenesis
Clinical picture
Two clinical tests?
Prothrombotic picture
Path: prolonged Rx with unfractionated heparin –> antibodies developed against platelet factor 4 (PF4) and heparin
DIC-like: extensive thrombosis + DVT, petechiae
PF4-H assay = new
Serotonin release assay
Describe the process of determining compatibility for transfusion
Crossmatch
Major: donor RBC x pt plasma
Minor: donor plasma x pt RBC
Antibody screen
Mix pt’s plasma with known reagent RBCs - testing that the pt hasn’t developed alloAbs against known antigens
FAB classification of leukaemia:
- Most common AML?
- Most common ALL?
What about CML/CLL?
AML: M3 - promyelocytic AML most common
ALL: L1 - small monomorphic- most common ALL
CML+ CLL don’t get one, they are just most common clinically
Most common form of CLL?
How to remember a salient feature of CLL?
Most common form of CLL = B cell (CD5 +ve)
CLL is very similar to SLL small lymphocytic lymphoma clinically - it has S, for massive SPLENOMEGALY
CLL pathogenesis
High incidence of BCL2 gene expressed (in BM blast cells) –> monoclonal expansion of non-functional but mature B lymphocytes –> hypogammaglobulinemia features despite high monoclonal Ab levels
Hence why BM biopsy shows = hypercellularity + high proportion monomorphic lymphocytes, crowding out of myeloid cells
CLL clinical features
The presentation of CLL is usually asymptomatic + has a highly variable clinical course. Slowly progressive = insidious, over many years
B symptoms = unexplained weight loss, drenching night sweats, fever.
Typical leukaemia: anaemia, fever + recurrent infections (i.e. candidiasis), bleeding
Massive splenomegaly, usually non-tender – accumulating cancerous lymphocytes
Mild hepatomegaly
Painless lymphadenopathy – freely mobile, rounded
Derm:
- Leukaemia cutis
- Chronic pruritus
Investigations in CLL (6)
Which are the most important 3?
Other 3?
FBC
Blood film
Flow cytometry - Detect the immunophenotype
Coombs test positive – pts with leukaemia have higher rates of autoimmune haemolytic anaemia
Serum protein electrophoresis - detect increased monoclonal Ab expansion
BM biopsy - shows ↑ cellularity with high proportion of monomorphic lymphocytes
Most important 3: FBC, blood film, flow cytometry
Not so: Coombs, serum protein electrophoresis, BM biopsy
What immunophenotype does follicular lymphoma have?
BCL2 positivity
What marker do B cells express? T cells? What do all lymphocytes express?
B cell = CD20
T cell = CD3
All lymphocytes= CD45
Burkitts lymphoma - what is it? Pathophys?
Burkitts = large B cell lymphoma
Pathophys = EBV infection –> T(8:14): MYC oncogene on 8 translocates to IgM gene on 14 –> overactivation of c-myc proto oncogene (which is antiapoptotic) –> activation of transcription –> lymphoma.
What conditions cause a Pel-Ebstein fever?
Malaria
Hodgkins lymphoma
Most common genetic abnormality in myelodysplasia
5q deletion
What is more common transformation to AML: MPD or MDS?
Answer = MDS
What is more typically producing pancytopenia: MPD OR MDS?
Answer = MDS
What do MDS + MPD have in common?
Both have hypercellular bone marrow
What is the classification system for myelodysplastic syndromes? Can you name the 4 aspects?
FAB classification
- RA: refractory anaemia (BM blasts <1%) – affects only RBCs, mildly
- RARS: RA with ring sideroblasts «1%) – more abnormal
- RAEB: RA with excess blasts (<5% are blasts)
- RAEB-T: RAEB in transformation (5-30% blasts)
Post-splenectomy management considerations + WHY
Post-splenectomy = increased risk of infection by encapsulated pathogens possibly –> overwhelming infection.
Protect with vaccinations against pneumococci, meningococci and Haemophilus influenzae type B.
o Although not encapsulated, yearly influenza vaccine is recommended
What are the 5 categories of symptoms/complications of multiple myeloma?
1) Cancer B sx
2) XS immunoglobulins
3) Osteoclastic action
4) Malignant plasma cell infiltration of BM
5) Immunodeficiency
Rx of hyperviscosity in multiple myeloma?
Plasmapharesis alleviates symptoms
Best initial test for multiple myeloma? Best confirmatory - and what are the findings?
Best initial = serum protein electrophoresis - determines what kind of gammopathy (IgG, IgA, kappa/delta)
Confirmative = BM biopsy
BM clonal cells equal to 10%
OR bony plasmacytoma OR extramedullary plasmacytoma. Plasmacytoma = mass with ≥10% clonal plsama cells
What will cause positive urine dipstick for blood?
The things that will cause positive blood on dipstick: haematuria (RBCs), myoglobin + haemoglobin.
