Haematology - INCOMPLETE

Question 1

Question

Answer 1

Answer

Question 2

Felty syndrome

Answer 2

Extra-articular manifestations of RhA = SANTA = autoimmune cytopenia + RhA
Splenomegaly
Arthritis
Neutropenia
Thrombopenia
Anaemia

Question 3

TTP

Answer 3

Deficiency of vWF cleaving protease (ADAMTS13)
Anti-ADAMTS13 antibody
↓protease –> ↑vWF multimers –> platelet thrombi form –> RBC fragmenttion
Platelets comsumed –> TTP
Tx: Plasma exchange

Question 4

causes of reticulocytosis

Answer 4

Haemolysis, Haemorrhage, Haemolytic replacement

Question 5

Causes of absent reticulosis

Answer 5

Inadequate haematoinics (iron, B12), BM failure, major haemorrhage

Question 6

Dacrocyte

Answer 6

Tear drop RBC

DDx: Leuco-erythroblastic anaemia, Myelofibrosis

Question 7

Echinocyte (= Burr cells)

Answer 7

Pyruvate kinase deficiency

Question 8

Leuco-erythroblastic anaemia

Answer 8

Nucleated RBCs + Immature myeloid cells (myelocytes)
Dx Bone marrow infiltration (so both immature red and white cells spill out)
- Cancer
- Infection
- Myelofibrosis

Question 9

Tx of Essential thrombocythaemia

Answer 9

Anagrelide

Question 10

Target cells = Codocytes

Answer 10

IDA, Liver disease, Hyposplenism, Thalassaemia

Question 11

Blood film of hyposplenism

Answer 11

Acanthocytes (many specules)
Howell-Jolly bodies
Target cells

Question 12

Plummer vinson syndrome

Answer 12

IDA
Oesophageal webs
Dysphagia

Question 13

Sideroblastic anaemia

Answer 13

Due to ineffective erythrooiesis - Alcohol excess, MDS
Ring sideroblasts (ring of iron around nucleus in erythroid precursors)

Question 14

Warm agglutinins

Answer 14

Autoimmune haemolytic anaemia (WAIHA) - more common
\+ve Coomb's test
Associated with : CLL, Lymphoma
> 37oC
IgG
Spherocytes
Tx: Steroids, Splenectomy

Question 15

Cold agglutinin

Answer 15

Autoimmune haemolytic anaemia
\+ve Coomb's test
- Ab causes RBC to agglutinate and obstruct vessels when cold
Mycoplasma infection, EBV infection
< 37 oC
IgM
Avoid the cold

Question 16

Hereditary spheocytosis

Answer 16

Spectrin deficiency
Autosomal dominant
↑osmotic fragility

Question 17

Fanconi anaemia

Answer 17

Most common cause of inherited aplastic anaemia«
Mutation in DNA repair
Chromosomal fragility
Sx: aplastic anaemia + short stature + café au lait spots

Question 18

βthalassaemia X-ray

Answer 18

Hairs on end of skull X-ray

Question 19

Paroxysmal Cold Haemoglobinuria

Answer 19

Viral infection –> Hb in urine
Donath-Landsteiner antibodies –> stick to RBCs in cold –> complement activated on re-warming (self-limiting as IgG so dissociate at higher temp than IgM)

Question 20

Paroxysmal Nocturnal haemoglobinuria

Answer 20

Loss of GPI on RBCs –> complement mediated lysis (esp at night)
Morning haemoglobinuria
Thrombosis
Ham’s test: in vitro acid induced lysis

Question 21

TTP anitbody

Answer 21

Anti-ADAMTS13 antibody –> ↑VWF –> ↓Plt

Question 22

Reverse Warfarin

Answer 22

Vitamin K
Prothrombin complex concentrate
Fresh frozen plasma
Stop warfarin

Question 23

Scoring system for DVT/PE

Answer 23

Wells score

Question 24

Unfractionated heparin - monitoring

Answer 24

Monitor APTT

Question 25

Antidote for heparin

Answer 25

Protamine sulphate

Question 26

S/E of Heparin

Answer 26

Bleeding | Heparin-induced thrombocytopaenia

Question 27

Target INR for 1st DVT or AF

Answer 27

2.0 - 2.5

Question 28

Target INR for recurrent DVT

Answer 28

2.5 - 3.0

Question 29

Target INR for AF

Answer 29

2..0 - 3.0

Question 30

Target INR for mechanical prosthetic valve

Answer 30

2.5 - 3.5

Question 31

CLL staging

Answer 31

Rai-Binet staging

Question 32

Lymphoma staging

Answer 32

Ann-Arbor staging

Question 33

MM staging

Answer 33

Durie-Salmon staging system

Question 34

Dyskeratosis congenita

Answer 34

Telomere shortening | Triad of skin pigmentation, nail dystrophy, oral leukoplakia + BM failure

Question 35

Schwachman-Diamond syndrome

Answer 35

Neutrophilia | Skeletal abnormalities

Question 36

Diamond-Blackfan syndrome

Answer 36

Pure red cell aplasia, normal WCC, normal Plt | Dysmorphic

Question 37

Acute transfusion reaction

Answer 37

``` ABO Febrile non-haemolytic (no change in BP) Anaphylaxis TRALI TACO Bacterial infection ```

Question 38

Delayed transfusion reaction

Answer 38

Delayed haemolytic transfusion - rhesus Post-transfusion purpura GVHD Viral infection

Question 39

Cut-off between MGUS and MM

Answer 39

Monoclonal protein < 30 | BM plasma cells < 10%

Question 40

Smouldering MM

Answer 40

> 30g/L M spike (paraprotein) > 10% clonal plasma cells No CRAB (i.e. no end organ damage)

Question 41

Non-secretory myeloma

Answer 41

MM without M protein in blood or urine

Question 42

Solitary plasmacytoma

Answer 42

Bone lytic lesions (B of CRAB) but no other findings of MM