haem

Question 1

causes of haemolytic anaemia

Answer 1

hereditary: 
spherocytosis 
G6PD deficiency 
haemoglobinopathics: SCD, thalassaemia 
acquired: 
autoimmune (cold/warm) 
microvascular: HUS, TTP, DIC
drugs: penicillin
infection: malaria
blood mismatch

Question 2

management haemochromatosis

Answer 2

cons: MDT, genetic counselling, monitor for complications (cirrhosis, HCC) 
med / surg: 
1. venesection
2. iron chelation: desferrioxamine 
liver transplant

Question 3

types of polycythaemia

Answer 3

primary: polycythaemia rubra vera (JAK2 mutation)
secondary (increased erythropoietin): hypoxia, renal cell carcinoma, erythropoietin abuse in athletes
reactive: due to reduced plasma volume

Question 4

causes of thrombocytopenia

Answer 4

reduced production: infection, drugs (e.g. penicillamine), myelofibrosis, myelodysplasia
increased destruction:
heparin, hypersplenism, HUS, TTP, DIC, ITP

Question 5

causes of thrombocytosis

Answer 5

reactive to infection, surgery, IDA 
malignancy 
hyposplenism 
essential thrombocytosis
polycythaemia ruba vera

Question 6

causes of neutropenia

Answer 6

viral infection
chemo / radiotherapy
drugs: carbimazole, clozapine

Question 7

causes of lymphocytosis

Answer 7

viral infection
lymphoma
CLL

Question 8

management of warfarin dose w/o bleed

Answer 8

< 6: reduce warfarin dose
6-8: omit warfarin dose + start on reduced dose
> 8: oral vit K

Question 9

management of warfarin w bleed (incl dose)

Answer 9

stop warfarin
5-10mg IV vit K
+ prothrombin complex (e.g. beriplex) if major bleed

Question 10

signs of hyposplenism on blood film

Answer 10

howell-jolly bodies
target cells
pappenheimer bodies (post-splenectomy)

Question 11

complications + management of splenectomy

Answer 11

haemorrhage
pancreatic fistula
infection esp encapsulated bacteria: NHS (neisseria, haemophilus, strep)
management:
immunisations: Hib, men A+C, annual influenza, 5yrly pneumococcal
penicillin V

Question 12

features of HUS

Answer 12

triad:
typically following E.coli gastroenteritis
1. renal failure (N&V, fluid retention, oliguria, haematuria, proteinuria)
2. MAHA (anaemia, jaundice, schistocytes)
3. thrombocytopenia (bruising)

Question 13

rain-drop skull on XR

Answer 13

multiple myeloma

Question 14

investigations for multiple myeloma

Answer 14

urine sample → Bence-Jones protein 
bloods: normocytic anaemia, blood profile (normal ALP), raised Ca, deranged U&Es (renal failure), rouleax formation on blood film
skeletal X-ray 
serum electrophoresis → paraprotein 
bone marrow aspirate → plasma cells

Question 15

disseminated intravascular coagulation

FBC + clotting + blood film findings

Answer 15

anaemia (normocytic), thrombocytopenia
prolonged aPTT + PT 
reduced fibrinogen 
increased fibrin degradation products + D-dimer 
schistocytes on blood film

Question 16

investigations haemochromatosis

Answer 16

bloods: FBC, iron studies (↑iron, ↑ferritin, ↑transferrin, ↓TIBC)
genetic testing: HFE
MRI liver + biopsy → Perl’s / prussian blue stain

Question 17

Auer rods

Answer 17

AML

Question 18

myeloblast granules +ve for Sudan black staining

Answer 18

AML

Question 19

Philadelphia chromosome (t9:22), Abelson-BCR

Answer 19

CML

Question 20

smudge / smear cells

Answer 20

CLL

Question 21

causes of autoimmune haemolytic anaemia (AIHA)

Answer 21

cold (IgM): mycoplasma, mononucleosis, HIV, lymphoma

warm (IgG): CLL, SLE

Question 22

causes of B12 deficiency

Answer 22

reduced intake e.g. in vegetarians 
reduced absorption (terminal ileum):
IBD (esp Crohn's) 
pernicious anaemia: Ab against intrinsic factor (Schilling test) 
gastrectomy / ileal resection

Question 23

presentation B12 deficiency

Answer 23

signs of anaemia
glossitis
mixed UMN + LMN signs of LL: spastic weakness, areflexia, extensor plantars (Babkinski +ve)
peripheral neuropathy
cerebellar signs: ataxia, +ve Romberg's

Question 24

causes polycythaemia

Answer 24

primary: polycythaemia rubra vera (JAK2 kinase mutation)
secondary:
to hypoxia: COPD, chronic lung disease, high altitude
inappropriate production: renal cell carcinoma, erythropoietin abuse in athletes

Question 25

presentation polycythaemia

Answer 25

pruritus after hot bath
splenomegaly (massive) 
plethoric face 
erythromelalgia: tenderness/redness/painful burning of fingers, palms, toes, heels
choreiform movements 
thrombosis 
headache 
dizziness 
night sweats

Question 26

clinical features multiple myeloma

Answer 26

CRAB: 
hypercalcaemia (causing low PTH) 
renal impairment
anaemia 
bone lesions (osteolytic: resorption by activated osteoclasts) 
\+ immunodeficiency

Question 27

multiple myeloma vs MGUS

Answer 27

MM: presence of paraprotein in urine / serum (electrophoresis)
bone marrow monoclonal cells > 10%
end-organ damage (hypercalcaemia, raised Cr, anaemia, bone lesions, osteoporosis, pathological fractures)

MGUS:
paraprotein < 30g/L
bone marrow monoclonal cells < 10%
no end-organ damage

Question 28

definition multiple myeloma

Answer 28

malignancy disease of clonal B cells resulting in bone marrow failure / destruction + overproduction of monoclonal paraprotein (bence jones proteins in urine)