Haematology

Question 1

Classic signs of Myeloma

Answer 1

CRAB
C = Calcium (elevated)
R = Renal Failure 
A = Anaemia 
B = Bone lesions

Question 2

what blood tests would you do if you are thinking hereditary thrombophilia

Answer 2

• Factor V leiden

- prothrombin gene variant

Question 3

how do you distinguish between haemophilia A and B

Answer 3

assays of factor VIII and IX activity

Question 4

haemophilia A and B inheritance pattern

Answer 4

X- linked recessive

Question 5

what is a tumour marker for myeloma you can test for on serum

Answer 5

B2M - beta-2 Microglobulin

Question 6

what is the use of SPEP (serum protein electrophoresis) or urine electrophoresis in suspected Myeloma

Answer 6

95% will have M proteins

Question 7

when would you do a serum free light chain assay?

Answer 7

if suspecting myeloma but no M proteins on SPEP or UPEP.

Question 8

what radiology tests do you do for multiple myeloma

Answer 8

xrays to look for osteopenia “punched out” lesions.

skull, ribs, spine, pelvis, shoulders and long bones

Question 9

diagnostic criteria for plasma cell myeloma

Answer 9

> 10% plasma cells in bone marrow
M protein on SPEP/UPEP
1 or more of CRAB features of organ dysfunction

Question 10

in staging myeloma, what criteria are used

Answer 10

Albumin levels

beta-2 microglobulin levels

Question 11

tx for myeloma

Answer 11

high dose chemo + stem cell transplant

+ bisphosphonates/radiotherapy/analgesics/spinal surgery for lytic bone lesions.

Question 12

what demographic is at higher risk of AML

Answer 12

infants and adults

Question 13

what demographic is at higher risk of ALL

Answer 13

children

Question 14

what is tumour lysis syndrome

Answer 14

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

Question 15

how do you prevent tumour lysis syndrome

Answer 15

hydration and allopurinol 24hrs before starting chemo

allopurinol = uricolytic

Question 16

what features on coags would suggest DIC

Answer 16

prolonged PT (increased INR)
prolonged APTT
markedly decreased D-dimer
decreased fibrinogen

Question 17

Auer rods = … leukaemia

Answer 17

AML

Question 18

signs of AML OE/

Answer 18

gum hypertrophy
hepatosplenomegaly
bruising

Question 19

what are myelodysplastic syndromes

Answer 19

disorders of bone marrow function/bone marrow failure

Question 20

what features of bone marrow biopsy suggest myelodysplastic syndromes

Answer 20

increased marrow cellularity,
abnormal looking cells
ring sideroblasts

Question 21

what is a poor prognostic factor for ALL

Answer 21

philadelphia chromosome

t(9;22) - BCR-ABL

Question 22

if someone has philadelphia positive ALL what extra treatment is available to them?

Answer 22

tyrosine kinase inhibitors
imatinib
dasatinib

Question 23

myelodysplastic syndrome –> malignancy yes or no

Answer 23

yes malignant transformation if additional cytogenetic abnormalities can –> AML

Question 24

tx for myelodysplastic syndrome

Answer 24

supportive peripheral transfusions, bone marrow stimulation, cytotoxic chemo.
little role for bone marrow transplant

Question 25

intrinsic pathway factors

Answer 25

XII, XI, IX, VIII

Question 26

extrinsic pathway factors

Answer 26

VII, III

Question 27

common clotting pathway factors

Answer 27

X, V, II, XIII, I

Question 28

what is Burkitt's lymphoma

Answer 28

cancer of the lymphatic system caused by EBV. mostly in central Africa

Question 29

what do you see on microscopy with Burkitt's lymphoma

Answer 29

vacuoles

Question 30

causes of isolated prolonged aPTT

Answer 30

``` tx with heparin, dabigatran SLE Haemophilia A, B Factor XI deficiency VWD ```

Question 31

how does heparin and dabigatran work?

Answer 31

block IIa (thrombin) from converting I --> Ia (fibrinogen --> fibrin)

Question 32

treatment for VWD

Answer 32

``` desmopressin --> stimulates release of endogenous VWF plasma derived concentrates --> VWF replacement (recombinant VWF) other antifibrinolytics (e.g. aminocaproic acid) ```

Question 33

aPTT long = ....pathway of clotting affected

Answer 33

intrinsic and common

Question 34

PT long = .... pathway of clotting affected

Answer 34

extrinsic and common

Question 35

causes of long PT normal APTT

Answer 35

``` warfarin vit K deficiency liver disease rivaroxaban factor VII deficiency = v. uncommon ```

Question 36

how to reverse warfarin fast

Answer 36

prothrombinex (FFP if not available)

Question 37

how to reverse warfarin completely

Answer 37

vitamin K + FFP

Question 38

what does FFP have that prothrombinex doesn't

Answer 38

factor VII

Question 39

significant bleeding history and family history, but APTT, PT and platelets all normal. Likely cause

Answer 39

VWD Antiplatelet drugs Platelet dysfunction disorders factor XI and XIII deficiency (uncommon)

Question 40

what are some inherited qualitative platelet disorders?

Answer 40

Disorders of adhesion = Bernard-Soulier syndrome Disorders of aggregation = Glanzmann thrombasthenia Platelet granule issues = Storage pool deficiency = αSPD (grey platelet syndrome)