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Flashcards in Haematology Deck (69):
1

What is multiple myeloma?

Cancer of plasma cells 

Accumulation of malignant plasma cells that leads to destructive bone disease + hypercalceamia 

BM + renal failure 

2

Which of the following is associated with the accumulation of monoclonal immunoglobin or light chains (Bence Jones protein) in plasma?

a) ALL 

b) Hodgkin's lymphoma 

c) Multiple myeloma 

d) Non-Hodgkin's lymphoma 

Multiple myeloma is associated with the accumulation of monoclonal immunoglobin or light chains (Bence Jones protein) in plasma

3

What causes the destruction of bone in multiple myeloma?

Destruction of bone in MM is due to the malignant plasma cells stimulating the osteoclasts to erode the bone 

4

What does MGUS stand for in relation to multiple myelome?

Q image thumb

MGUS = Monoclonal gammopathy of undetermined significance. The first stage. 

<30g/dl, <10% plasma cells in BM, no ROTI, no evidence of amyloid or other LPD

5

What are the 3 aims of treatment for multiple myeloma?

Antimyeloma treatment - chemo

Prevent and treat bone damage - bisphosphates, surgery, dialysis 

Improve QOL + survival - infection prophalaxis & anaemia treatment 

 

6

Combination chemotherapies are used with multiple myeloma. Give an example of 3 types of combinations drugs. 

Thalidomide-based

Velcade-based

Supportive (pain killers, bisphosphates, blood transfusions) 

7

What are the treatment options after a first relapse in multiple myeloma?

Same treatment if lenghty remmission

Second transplant if first transplant remission >2yrs

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8

What is lymphoma?

Malignant growth of WBC 

9

Most cases of lymphoma are due to unknow causes. However give 3 possible causes (broad).

Immunodeficiency 

Infections (EBV, Helicobacter pylori) 

Autoimmune

10

Which haematological disease has the following presenting features?

Tiredness/malasie. 

Bone/back pain 

Infections 

Multiple myeloma 

Tiredness/malasie. 

Bone/back pain 

Infections 

11

Which haematological disease has the followig presenting features?

Nodal disease 

Compression syndromes

Systemic symptoms 

Lymphoma 

Of the WBC - commonly found in nodes, accumulation --> lumps 

12

How would you diagnose lymphoma?

Blood film 

BM biopsy 

Immunophenotyping (CD20 on B cells for monoclonal Ab treatment with rituximab) 

Cytogenetics 

molecular techniques 

13

What are the investigations that we use to stage a lymphoma case?

Blood tests

CT Scan chest/abdo/pelvis

Bone marrow biopsy 

PET Positron emiting - metabolically active cells take up the positrons 

Staging = how bad is it?

14

What are the two main subtypes of lymphoma?

Hodgkin's 

NHL 

15

Give the 3 grades and an example of NHL 

Low grade e.g. Follicular Lymphoma 

High grade e.g. Diffuse Large B Cell Lymphoma (most common) 

Very high grade e.g. Burkitt’s Lymphoma (most aggressive) 

16

Which haematological disease is being described?

Painless lymphadenopathy 

B symptoms 

Presence of Reed-Steinberg cells 

Painless lymphadenopathy 

B symptoms 

Presence of Reed-Steinberg cells 

Hodkin's lymphoma 

17

In which age group in Hodgin's lymphoma most common?

Children 

Teenage and young adult 

Middle age 

Elderly 

Teenage and young adult 

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18

In Hodgkin's lymphoma... what is the difference between stage 2 and stage 3?

Stage 2 - 2/< areas with cancer on the same side of the diaphram 

Stage 3 - 2/< areas of cancer on both sides of the diaphram 

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19

Which treatment would the following stages of Hodgkin's lymphoma receive?

a) Stage 1-2A 

b) Stage 2B-4

a) Stage 1-2A = short course of combined chemo + radiotherapy 

b) Stage 2B-4 = combined chemo 

20

Give an example of a monoclonal Ab

Rituximab 

Anti CD20 found on B cells 

Chimeric mouse/human protein - chance of allergic reactions 

21

— Which haematological disease is being described?

'a clonal expansion of myeloid blasts in the bone marrow, blood or other tissues’ 

Acute myeloid leukemia = a clonal expansion of myeloid blasts in the bone marrow, blood or other tissues 

22

Which type of leukeamia is associated with the t(9,22) translocation?

a) AML 

b) CML 

c) ALL 

d) CLL 

Chronic myeloid leakeamia 

t(9,22)  

Philaelphia gene 

- fusion of activated tyrosine kinade - 'on' for many cellular functions 

23

What is leaukaemia?

Malignant proliferation of haemopoietic cells 

24

A patient with acute myeloid leukaemia needs supportive care. 
What does supportive care include here?

Blood product support 

Early diagnosis and treatment of infections 

Recognition of atypical/unusual infections 

25

For AML - is the following true of false?

Outcomes increase with increased age

 

False 

Outcomes decrease with increased age. Less able to tollerate the treatments 

26

Describe acute promyelocytic leukaemia

Subtype of AML 

Accumulation of promyelocytes 

t(15;17) Blocks differentation of promyelocytes --> granulocytes. Cannot swtich on the coagulation cascade = bleeding 

Haematological emergency 

27

Acute promyelocytic leakaemia is the most treatable with 90% remission rate. 

Exaplain why. 

Acute promyelocytic leakaemia is the most treatable with 90% remission rate. 

Due to targetted ATRA therapy 

28

CML is most common in which age group?

a) children 

b) teenage and young adults 

c) Middle age 40-60 

d) elderly 60<

CML is most common from 40-60 

29

Explain the treatment for CML 

Targeted molecular therapy 

Tyrosine kinase inhibitors (uImatinib (Glivec)

uNilotinib, Dasatinib, Ponatinib)

Now a chronic condition 

 

30

Which of the following disease is being described?

Leucocytosis, basophilia, change in platelets 

Normocytic anaemia 

Abnormal leukocytes 

Increase in B12 

Slenomegaly 

Philadelphia choromosome 

Chronic myloid leukaemia 

Leucocytosis, basophilia, change in platelets 

Normocytic anaemia 

Abnormal leukocytes 

Increase in B12 

Slenomegaly 

Philadelphia choromosome 

31

Which is the most common leukaemia in children?

Acute lymphoblastic leukaemia is the most common in childran 3-7 years 

32

A 5 year old girl presents with fever, chest infection, anaemia, tachycardia. She is pale, fatigued with a headache. 

What tests do you send for and what diagnosis would you heamatological disease do you suspect?

A 5 year old girl presents with fever, chest infection, anaemia, tachycardia. She is pale, fatigued with a headache. 

Tests: Blood film, BM biopsy, lymph node biopsy, immunophenotyping 

Diagnosis: Acute lymphoblastic leukaemia 

33

Explain the treatment for ALL 

Chemotherapy phases Remission induction, consolidaion, intensification, maintenance 

CNS therapy 

Stem cell transplant

Aim is to induce a long lasting remission. 

Adults with the philadelphia chromosome have a poorer prognosis 

34

Which is the most common leukaemia?

Chronic lymphocytic leukaemia is the most common leukaemia

35

Describe the clinical course of CLL 

Variable

Progressive lymphadenopathy / hepatosplenomegaly

Auto-immune - haemolysis, ITP

Bone marrow failure - due to marrow replacement

Hypogammaglobulinaemia & infection

36

Give the Hb values for anaemia

a) female 

b) male  

 

The Hb values for anaemia

a) female = <115g/l 

b) male  = <130g/l 

37

What are the pathological consequences of anaemia?

 Fatty change in myocardim and liver 

Aggravate angina 

Skin and nail atrophic changes

CNS cell death 

38

Give examples of problems of the failure of production which can lead to anaemia. 

Haematological deficiencies = B12, folate & iron 

Chronic disease - inflammation, infection, neoplasm 

Hypoplasia 

Dyserythroiesis 

Marrow infiltration 

39

Give examples of increased destruction that can lead to anaemia. 

Hypersplenism  

Acute blood loss

Red cell abnormality (membrane/enzyme defects) 

Abnormality outside of red cell (immune haemolytic anaemias, drugs, toxins) 

40

Give the 3 main causes of microcytic anaemia and the MCV range 

Microcytic anaemia - MCV <80 

Iron deficiency 

Thalassaemia 

Chronic disease 

41

Give the 3 main causes of normocytic anaemia and the MCV range 

Normocytic anaemia 

Acute blood loss 

Chronic disease 

Combined haematinic deficiency

MCV 80-100 

42

Give the 3 main causes of macrocytic anaemia and the MCV range 

Macrocytic anaemia 

B12/folate deficiency 

Alcohol/liver disease 

Hypothyroid 

Heamatological 

MCV >80 

43

How do you test for a B12 deficiency?

IF Ab 

Schilling test 

Coeliac Ab 

44

Explain the life course of platelets 

Produced: megakaryokes in BM 

Circulation: 7-10 days 

Destroyed: Spleen by splenic macrophages 

45

What is the production of platelets controlled by?

Platelet production is controlled by thrombopoietin (TPO) 

Produced by the liver 

Binds to plts & MK - feedback 

46

Give the clotting cascade 

Intrinsic - XII, XI, IX, VII 

Extrinsic - VII (TF)

Joined - V, X, prothrombin --> thrombin --> fibrinogen --> fibrin 

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47

The followin are targets for antiplatelet drugs. What are their normal actions and the drugs which target them?

a) P2Y12 

b) COX 

c) GPIIBIIIA

The followin are targets for antiplatelet drugs. Their normal are actions...

a) P2Y12 - amplifies activation of platelets - Clopidogrel

b) COX - produces thromboxane A2 which induces platelet aggregation and vasoconstriction - Aspirin 

c) GPIIBIIIA - Aids platelet aggregation and adherence -Tirofiban 

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48

What is the normal range for platelet numbers?

150-400 X109

49

Give the clinical features of platelet dysfunction 

Easy bruising 

Mucosal bleeding 

Petechiae, purpura 

Traumatic haematomas

50

For thrombocytopenia... Give the values. 

a) Normal platelet range 

b) No clinical defects with thrombocytopenia

c) Increased bleeding post trauma 

d) Spontaneous skin and mucosal bleeding 

For thrombocytopenia... the values are:

a) Normal platelet range 150-400x109

b) No clinical defects >80x109

c) Increased bleeding post trauma 40-50x109

d) Spontaneous skin and mucosal bleeding <20x109

51

A 32 year old male presents with the following blood results

Platelets: 60x109/l

WBC: 6x10g/l

Hb: 150x109/l

Is this likely to be due to decreased production or increase destruction?

A 32 year old male presents with the following blood results 

Platelets: 60x109/l range = 150-400x109 = low 

WBC: 6x10g/l range = 4-11x109 = normal 

Hb: 150x109/l range = 135-180x109 = normal 

Defect due to increased destruction 

52

What are the two most common causes of thrombocytopenia due to increased destruction?

Autoimmune thrombocytopenic purpura & disseminated intravascular coagulation are the two most common causes of thrombocytopenia due to increased destruction

53

Describe immune thrombocytopenia

Give examples of when a primary and secondary infection will occur 

Immune thrombocytopenia purpura (ITP) 

IgG antibodies against platelets + MK glycoproteins 

Opsonization = removal by reticuloendothelial system in speen 

Primary infection: following a vaccination/infection 

Secondary infection: association with malignancies (CLL), infections (HIV/Hep C), drugs (heparin, quinine, sulphonamides)

54

What are the treatments for immune thrombocytopenia

Immunosuppression - steroids/IVIG 

Treat the underlying cause 

Platelets - if bleeding 

Tranexamic acid - if mucosal bleeding 

55

Describe thrombic thrombocytopenic purpura (TTP)

Thrombic thrombocytopenic purpura (TTP)

Microscopic clots in small blood vessels around the body 

Schistocytes 

Vascular lesions 

Sporodic episodes 

Associated with high moleular weight von Willebrand factor

Leads to organ dysfunction, renal failure & neurological abnormalities 

56

What is the treatment for thrombic thrombocytopenic purpura (TTP)

The treatment for thrombic thrombocytopenic purpura (TTP) is: 

High volume plasma exchange 

Rituximab - monoclonal Ab 

57

Describe disseminated intravascular coagulation (DIC)

Disseminated intravascular coagulation (DIC)

A complicating factor of an underlying condition 

Causes both clotting and bleeding 

Vascular occlusion --> organ dysfunction 

Consumption of platelets and clotting factors --> Haemorrhage 

58

Which disorder of coagulation is being described?

Patient who has already been on the ward for 6 months now presents with :

Haemorrhage, multi-organ failure, haemolytic anaemia, bleeding from mucosal membranes 

 

Diseminated Intravascular Coagulation (DIC)

Haemorrhage, multi-organ failure, haemolytic anaemia, bleeding from mucosal membranes 

59

Give the nomal values and units for the following: 

a) MCV (mean corpsular volume) 

b) MCH (mean corpsular Hb) 

c) MCHV (mean corpsular Hb volume)

a) MCV 78-98 fl 

b) MCH 26-33 pg 

c) MCHV 30-35 g/dl 

60

Describe thalassaemia 

Globin chain disorder with leads to a reduction in Hb

61

How do you monitor haemoglobinopathies?

Ferratin 

Cardia and liver MRI 

Endocrine testing 

DEXA scanning 

62

Explain the problems with iron over load 

No physiological mechanism to excrete iron. 

Excess iron deposits in the liver and the spleen causing fibrosis and cirrhosis. It can also deposit in the endocrine glands and in the heart. 

63

Explain how enzymopathies cause damage to RBC and two main causes 

Shortened cell life due to oxidative damage 

Mainly caused by glucose-6-phosphate deficiency and pyruvate kinase deficiency 

 

64

Which of the following is being described?

Due to a variety of mutations, most are asymptomatic, X-linked. In a crisis haemoloysis, jaundice & anaemia occurs 

a) G6PD

b) Pyruvate kinase deficiency 

c) Iron deficincy 

d) Thalassaemia

Glucose-6-phosphate deficiency 

Due to a variety of mutations, most are asymptomatic, X-linked. In a crisis haemoloysis, jaundice & anaemia occurs 

65

Decribe febrile neuropenia and who is at risk?

Febrile = fever >38 

Neutrophils = <1.0 x10

People at risk: Chemo, haematological malignancies, BM failure syndromes, cyclical neutropenia 

 

 

 

66

How do you treat febrile neuropenia?

Start broad spectrum antibiotics without waiting for the results 

Identify those at risk 

67

Which of the following could be a possible diagnosis if a patient presents with: 

Lethary, headaches, confusion, visual disturbances, cranial nerve defects, ataxia, retinal haemorrhage 

a) Febrile neuropenia 

b) Hyperviscosity 

c) Alpha thalassaemia 

d) Beta thalassaemia 

 

Hyperviscosity 

Lethary, headaches, confusion, visual disturbances, cranial nerve defects, ataxia, retinal haemorrhage

68

Describe the treatment and prevention measures used with tumour lysis syndrome 

Tumour lysis syndrome ​

Hydrate - aggrresive IV before and after chemo 

Watch kidneys  

Prevent hyperuricaemia - Lower uric acid 

69