Haematology Flashcards
Classic signs of Myeloma
CRAB C = Calcium (elevated) R = Renal Failure A = Anaemia B = Bone lesions
what blood tests would you do if you are thinking hereditary thrombophilia
- Factor V leiden
- prothrombin gene variant
how do you distinguish between haemophilia A and B
assays of factor VIII and IX activity
haemophilia A and B inheritance pattern
X- linked recessive
what is a tumour marker for myeloma you can test for on serum
B2M - beta-2 Microglobulin
what is the use of SPEP (serum protein electrophoresis) or urine electrophoresis in suspected Myeloma
95% will have M proteins
when would you do a serum free light chain assay?
if suspecting myeloma but no M proteins on SPEP or UPEP.
what radiology tests do you do for multiple myeloma
xrays to look for osteopenia “punched out” lesions.
skull, ribs, spine, pelvis, shoulders and long bones
diagnostic criteria for plasma cell myeloma
> 10% plasma cells in bone marrow
M protein on SPEP/UPEP
1 or more of CRAB features of organ dysfunction
in staging myeloma, what criteria are used
Albumin levels
beta-2 microglobulin levels
tx for myeloma
high dose chemo + stem cell transplant
+ bisphosphonates/radiotherapy/analgesics/spinal surgery for lytic bone lesions.
what demographic is at higher risk of AML
infants and adults
what demographic is at higher risk of ALL
children
what is tumour lysis syndrome
when start chemo for rapidly proliferating leukaemia –> rapid cell death –> rise in serum urate, K+, and phosphate –> renal failure
get also high LDH, high phosphate, low calcium, and metabolic acidosis
how do you prevent tumour lysis syndrome
hydration and allopurinol 24hrs before starting chemo
allopurinol = uricolytic
what features on coags would suggest DIC
prolonged PT (increased INR)
prolonged APTT
markedly decreased D-dimer
decreased fibrinogen
Auer rods = … leukaemia
AML
signs of AML OE/
gum hypertrophy
hepatosplenomegaly
bruising
what are myelodysplastic syndromes
disorders of bone marrow function/bone marrow failure
what features of bone marrow biopsy suggest myelodysplastic syndromes
increased marrow cellularity,
abnormal looking cells
ring sideroblasts
what is a poor prognostic factor for ALL
philadelphia chromosome
t(9;22) - BCR-ABL
if someone has philadelphia positive ALL what extra treatment is available to them?
tyrosine kinase inhibitors
imatinib
dasatinib
myelodysplastic syndrome –> malignancy yes or no
yes malignant transformation if additional cytogenetic abnormalities can –> AML
tx for myelodysplastic syndrome
supportive peripheral transfusions, bone marrow stimulation, cytotoxic chemo.
little role for bone marrow transplant
intrinsic pathway factors
XII, XI, IX, VIII
extrinsic pathway factors
VII, III
common clotting pathway factors
X, V, II, XIII, I
what is Burkitt’s lymphoma
cancer of the lymphatic system caused by EBV. mostly in central Africa
what do you see on microscopy with Burkitt’s lymphoma
vacuoles
causes of isolated prolonged aPTT
tx with heparin, dabigatran SLE Haemophilia A, B Factor XI deficiency VWD
how does heparin and dabigatran work?
block IIa (thrombin) from converting I –> Ia (fibrinogen –> fibrin)
treatment for VWD
desmopressin --> stimulates release of endogenous VWF plasma derived concentrates --> VWF replacement (recombinant VWF) other antifibrinolytics (e.g. aminocaproic acid)
aPTT long = ….pathway of clotting affected
intrinsic and common
PT long = …. pathway of clotting affected
extrinsic and common
causes of long PT normal APTT
warfarin vit K deficiency liver disease rivaroxaban factor VII deficiency = v. uncommon
how to reverse warfarin fast
prothrombinex (FFP if not available)
how to reverse warfarin completely
vitamin K + FFP
what does FFP have that prothrombinex doesn’t
factor VII
significant bleeding history and family history, but APTT, PT and platelets all normal. Likely cause
VWD
Antiplatelet drugs
Platelet dysfunction disorders
factor XI and XIII deficiency (uncommon)
what are some inherited qualitative platelet disorders?
Disorders of adhesion = Bernard-Soulier syndrome
Disorders of aggregation = Glanzmann thrombasthenia
Platelet granule issues = Storage pool deficiency = αSPD (grey platelet syndrome)
