General Haem Flashcards
Where is the cancer in leukaemia?
cancer cells blood cells mainly in blood and bone marrow
Where is the cancer in lymphoma?
cancer cells develop in lymphocytes and tumours found in lymph nodes and lymphatic tissue
What is leukaemia?
a group of blood cancer which usually begin in bone marrow and result in high numbers of abnormal blood cells, and the dysfunctional cells crowd out the bone marrow and prevent the formation of other important blood cells
What is tumour lysis syndrome?
Metabolic abnormalities that arise as result of cancer treatment – esp leukaemia and lymphoma
What key things are released from tumour lysis?
- Phosphate
- Uric acid
- Potassium
What happens to released phosphate in tumour lysis syndrome?
- forms calcium phosphate crystals
2. leads to kidney failure and hypocalcaemia
What happen to released uric acid in tumour lysis syndrome?
forms urate crystals so GOUT
What happens to released potassium in tumour lysis syndrome?
causes hyperkalaemia so arrythmia
What is myelodysplasia?
group of syndrome where the immature blood cells do not mature normally
What are the causes of myelodysplasia?
- Primary: intrinsic bone marrow problem
2. Secondary: previous chemotherapy/radiotherapy
What does myelodysplasia cause?
chronic pancytopenia (anaemia, neutropenia, thrombocytopenia)
What are different types of myelodysplasia?
- Refractory anaemia
- Refractory anaemia with ringed sideroblasts
- Refractory anaemia with excess blasts
- Refractory anaemia with excess blasts in transformation
- Chronic myelomonocytic leukaemia
Can myelodyplasia progress?
yes, it is a cancer but can become AML
What is primary haemostasis responsible for and what does it depend on?
- Platelet aggregation and plug formation
2. Depends on: platelets and VWF
What would disorders for primary haemostasis involve?
superficial bleeding
What is secondary haemostasis responsible for and what does it depend on?
- Fibrin formation to stabilise platelet plug
2. Depends on: clotting factors
What does disorders of secondary haemostasis involve?
deep bleeding and bruising
What factors are involved in the intrinsic pathway?
Factor 1,2,8, 9,10,11,12
What measures intrinsic factor?
aPTT
What factors are in the extrinsic pathway?
tissue factor and 7a
What measure the intrinsic factor pathway?
PT
What are the different roles of VWF?
- Platelet adhesion
- Platelet aggregation
- Factor 8 stabilisation
How does VWF do platelet adhesion?
forms bridge between damaged sub-endothelium and platelets via GP1b receptor
How does VWF do platelet aggregation?
facilitates platelets binding to each other
How does VWF do factor 8 stabilisation?
binds to factor 9 and prevent its degradation
What are levels of VWF and inheritance of Type 1 VWF syndrome?
- Reduced levels of normal vWF
2. Autosomal dominant
What are levels of VWF and inheritance pattern of Type 2 VW syndrome?
- Adequate levels of defective vWF
2. Autosomal dominant
What are levels of VWF and inheritance pattern of Type 3 VW syndrome?
- Complete lack of vWF and highly reduced Factor 8
2. Autosomal recessive
What is the presentation of VWF?
- Superficial bleeding
- Brusing, epistaxsis
- Prolonged gum bleeding after dental procedures
- Prolonged bleeding from minor wounds
What is presentation of Type 3 VW syndrome?
more severe with reduced factor 8 so deep bleeding into joints and soft tissues
What is the diagnosis for Von willebrand syndrome?
Investigation results depend on type of VWsyndrome
Which types of VWS have redcued VWF?
type 1 and 3
Which VWS may have prolonged APTT?
type 3
What investigations would you do for VWS?
- Prolonged bleeding time: impaired primary haemostasis
- Prolonged APTT and normal PT: factor 8 reduction
- Reduced vWF – except in Type 2
- Normal platelets
What does an aspirate bone marrow biopsy let you see?
- cytology
- flow cytometry
- cytogenetics
- molecular genetics
What does a trephine biopsy allow you to see?
- Architecture
- Cytology
- Immunochemistry
- FISH cytogenetics
What are different types of lymph node biopsy?
- Fine needle aspirate (one useful in diagnosis of infection and metastiatic cancers)
- Core biopsy
- Excision biopsy
How does core biopsy work?
- Per-cutaneous US guided
- Day case
- No excision wound
What are uses of core biopsy?
- can detect non lymphoid material)
- TB
- Metastatic cancer
What are limitations of core biopsy?
- (limited) lymphatic architecture
2. Limits use in lymphoma diagnosis
How is an excision biopsy carried out?
- GA & surgery
2. Excision scar
What are the uses for excision biopsy?
- Optimal for lymphoma diagnosis &classification
- TB +granulomas
- Metastatic cancer
What is a malignancy of precursor B cells?
B cell ALL
What is a malignancy of germinal center cells?
mantle cell lymphoma
What is a malignancy of plasma cell?
multiple myeloma