Haematology

Question 1

Myeloma prognostic markers

Answer 1

B2 microglobulin and Albumin

high and low respectively is bad prog

Question 2

febrile and jaundiced 7 days after blood transfusion, management

Answer 2

Supportive mx unless symp anaemia

delayed transfusion reaction, typically 7-10 days post-tran
2ndary to sensitised from previous transfusions/preg; alloantibodies against red cell antigens

Question 3

Patient w flu-like symptoms headache, GI symps, haemorrhagic fever

Been in bat cave in Uganda

Answer 3

Marbug virus

Question 4

Auer rods seen it…

Answer 4

pathognomonic of Acute promyelocytic leukaemia M3
Assx w t(15;17)

M3 subtype of AML

Question 5

Fever/chills after blood transfusion

Answer 5

As long as no hypotension, dyspnoea wheezing Angioedema, Abdo pain etc

Slow/stop transfusion, paracetamol and monitor
Likely non haemolytic febrile reaction

Question 6

Blood film: schistocytes
Low platelets
High APTT and PT

Answer 6

DIC

Schistocytesdue to microangiopathic haemolytic anaemia

Question 7

Commonest cause of chronic DIC in elderly

Answer 7

Prostate cancer

Question 8

acquired factor VIII deficiency Vs Von willebrands

Answer 8

Both cause long APTT but in Vwb APTT corrects with addition of plasma

Question 9

Most common cause of hyperviscocity syndrome

Answer 9

Waldenström’s macroglobulinaemia (most common cause).

Presents with neuro symps like headaches, visual disturbances, papilloedema, hypertension, bleeding.,.
Treat with plasmapharesis to rapidly reduce viscosity and reduced thromboembolic events

Question 10

Patient with Waldenström’s macroglobulinaemia presents with headaches, visual disturbances, papilloedema, hypertension, bleeding.,.

Answer 10

Hyperviscocity syndrome

Treat with plasmapheresis

Question 11

Management of heparin induced thrombocytopenia

Answer 11

Switch to direct thrombin inhibitors like Argatroban or danaparoid

remember: low platelets but is actually a prothrombotic condition
and HIT antibodies have a high false positive rate
Can use Warkentin probability scale

Question 12

Treatment of atypical HUS

Answer 12

Eculizumab (a C5 inhibitor)

Question 13

fever, anaemia, thrombocytopenia, renal failure and confusion post partum.
Fragmented red cells on blood film

Answer 13

Thrombotic thrombocytopenic purpura

overlaps with HUS, but HUS is classically after bloody diarrhoea (EColi0157:H7) and more severe AKI

Question 14

Management of Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Answer 14

Cyclophosphamide and steroids (90% response)

Question 15

Complications of plasma exchange

Answer 15

Hypocalcaemia (due to citrate as anticoag)
Metabolic all
Coagulation depletion so bleeding risk

Question 16

Reed-Sternberg cells

Answer 16

hodgkin’s

Question 17

which hodgkin’s has worst prognosis?

Answer 17

Lymphocyte depleted (also rarest)

Question 18

which hodgkin’s has best prognosis?

Answer 18

Lymphocyte predominant

Question 19

alcohol-induced pain?

Answer 19

hodgkin’s lymphoma

Question 20

raised reticulocyte and LDH

Answer 20

Haemolysis

high reticulocyte count (medically known as reticulocytosis) can be found after blood loss due to injury, ulcers, or surgery

LDH is found intracellularly, so indicates damage…

Question 21

raised red cell count, platelets and splenomegaly

Answer 21

polycythaemia vera

Would usually have raised Hb but could be normal if co-existing IDA

Question 22

The most common cause of an isolated lymphocytosis in an adult

Answer 22

Chronic lymphocytic leukaemia (CLL)

Tx if constitutional symptoms
Anti-CD20 monoclonal antibody (e.g. obinutuzumab), chlorambucil or bendamustine

Question 23

red cell fragmentation

Answer 23

heart valves
TTP
DIC
HUS

Question 24

thrombotic thrombocytopenic purpura vs haemolytic uraemic syndrome

Answer 24

Overlap, but HUS is classically after bloody diarrhoea (EColi0157:H7) and more severe AKI

both can have fragmentation of red cells on blood film

DIC would have low platelets, prolonged APTT, PT

Question 25

ADAMTS13 deficiency

Answer 25

TTP ADAMTS13 Deficiency (a metalloprotease enzyme) which cleaves large multimers of von Willebrand's factor

Question 26

What is the deficiency in TTP

Answer 26

ADAMTS13 Which breakdowns large multimers of von Willebrand's factor

Question 27

alternative description for Auer rods

Answer 27

needle-like elongated cytoplasmic inclusions pathognomonic of acute myeloid leukaemia

Question 28

who do you screen for thrombophilia?

Answer 28

younger pt w spontaneous clotting events don't test during acute event though or on anticoag

Question 29

commonest thrombophilia

Answer 29

Factor V Leiden (heterozygous) 5% x4risk

Question 30

most severe thrombophilia

Answer 30

Antithrombin III deficiency rare 0.02% x10-20 risk

Question 31

ITP vs TTP

Answer 31

ITP: autoimmune destruction of platelets and is managed with immunosuppression - the first line is usually prednisone, IgGlob ``` TTP platelets clump within vessels due to deficiency of ADAMTS13 Fluctuating neuro, AKI, Raised LDH Medical emergency Plasma exchange. ```

Question 32

when do you treat Chronic lymphocytic leukaemia (CLL)?

Answer 32

B symps worsening of anaemia and/or thrombocytopenia >10 cm or progressive lymphadenopathy >6 cm or progressive splenomegaly lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months autoimmune cytopaenias e.g. ITP

Question 33

commonest cause of amyloidosis in uk

Answer 33

plasma cell disorders (e.g. myeloma) are the commonest cause of amyloidosis in the UK and lead to primary (AL) amyloidosis

Question 34

old person w anaemia, neutropaenia, thrombocytopenia

Answer 34

myelodysplasia

Question 35

``` 25 yr old presents acutely unwell w recent weight loss, nosebleed, oozing from his cannula sites and non-blanching purpura over his arms and legs. INR 8.5 PT 89 s (9-12) APTT ratio 1.7 (0.8-1.2) Platelets 43 ```

Answer 35

DIC, in acute promyelocytic leukaemia (APML presents younger than other AML) Tx Tretinoin AKA all-trans retinoic acid HSP doesn't have pancytopenia/bleeding Aplastic anaemia wouldn't have weight loss

Question 36

how do you assess for ?heparin induced thrombocytopenia

Answer 36

HIT antibodies have a high false positive rate Can use Warkentin probability scale 4Ts Score - Thrombocytopenia (30-50, >50) - timing 5-10d, or <1d if 100d since exposure - Thrombosis (thrombosis or skin necrosis) - other cause for thrombocytopenia

Question 37

what is Berger disease aka

Answer 37

IgA nephropathy

Question 38

what is IgA nephropathy aka

Answer 38

Berger disease

Question 39

bloody diarrhoea then AKI and thrombocytopenia... what type of haemolytic anaemia?

Answer 39

microangiopathic haemolytic anaemia (MAHA) in the context of HUS-TTP

Question 40

immediate therapy for acute promyelocytic leukaemia

Answer 40

Tretinoin PO for 3/12 AKA all-trans retinoic acid (ATRA) If diagnosed, one of the most treatable leukaemias, however, 1/3 present w catastrophic haemorrhage

Question 41

Prognosis with Philadelphia translocation t(9;22)

Answer 41

GOOD in CML (most commonly found) | BAD in AML + ALL