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Phase 2 AR > Haematology > Flashcards

Haematology Flashcards

1
Q

What is multiple myeloma?

A

Cancer of plasma cells

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

BM + renal failure

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2
Q

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

A

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

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3
Q

What causes the destruction of bone in multiple myeloma?

A

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

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4
Q

What does MGUS stand for in relation to multiple myelome?

A

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

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5
Q

What are the 3 aims of treatment for multiple myeloma?

A

Antimyeloma treatment - chemo

Prevent and treat bone damage - bisphosphates, surgery, dialysis

Improve QOL + survival - infection prophalaxis & anaemia treatment

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6
Q

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

A

Thalidomide-based

Velcade-based

Supportive (pain killers, bisphosphates, blood transfusions)

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7
Q

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

A

Same treatment if lenghty remmission

Second transplant if first transplant remission >2yrs

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8
Q

What is lymphoma?

A

Malignant growth of WBC

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9
Q

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

A

Immunodeficiency

Infections (EBV, Helicobacter pylori)

Autoimmune

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10
Q

Which haematological disease has the following presenting features?

Tiredness/malasie.

Bone/back pain

Infections

A

Multiple myeloma

Tiredness/malasie.

Bone/back pain

Infections

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11
Q

Which haematological disease has the followig presenting features?

Nodal disease

Compression syndromes

Systemic symptoms

A

Lymphoma

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

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12
Q

How would you diagnose lymphoma?

A

Blood film

BM biopsy

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

Cytogenetics

molecular techniques

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13
Q

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

A

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?

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14
Q

What are the two main subtypes of lymphoma?

A

Hodgkin’s

NHL

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15
Q

Give the 3 grades and an example of NHL

A

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)

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16
Q

Which haematological disease is being described?

Painless lymphadenopathy

B symptoms

Presence of Reed-Steinberg cells

A

Painless lymphadenopathy

B symptoms

Presence of Reed-Steinberg cells <– hallmark cell

Hodkin’s lymphoma

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17
Q

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

Children

Teenage and young adult

Middle age

Elderly

A

Teenage and young adult

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18
Q

In Hodgkin’s lymphoma… what is the difference between stage 2 and stage 3?

A

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
Q

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

a) Stage 1-2A
b) Stage 2B-4

A

a) Stage 1-2A = short course of combined chemo + radiotherapy
b) Stage 2B-4 = combined chemo

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20
Q

Give an example of a monoclonal Ab

A

Rituximab

Anti CD20 found on B cells

Chimeric mouse/human protein - chance of allergic reactions

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21
Q

— Which haematological disease is being described?

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

A

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

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22
Q

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

a) AML
b) CML
c) ALL
d) CLL

A

Chronic myeloid leakeamia

t(9,22)

Philaelphia gene

  • fusion of activated tyrosine kinade - ‘on’ for many cellular functions
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23
Q

What is leaukaemia?

A

Malignant proliferation of haemopoietic cells

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24
Q

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

A

Blood product support

Early diagnosis and treatment of infections

Recognition of atypical/unusual infections

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25
Q

For AML - is the following true of false?

Outcomes increase with increased age

A

False

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

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26
Q

Describe acute promyelocytic leukaemia

A

Subtype of AML

Accumulation of promyelocytes

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

Haematological emergency

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27
Q

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

Exaplain why.

A

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

Due to targetted ATRA therapy

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28
Q

CML is most common in which age group?

a) children
b) teenage and young adults
c) Middle age 40-60
d) elderly 60<

A

CML is most common from 40-60

29
Q

Explain the treatment for CML

A

Targeted molecular therapy

Tyrosine kinase inhibitors (uImatinib (Glivec)

uNilotinib, Dasatinib, Ponatinib)

Now a chronic condition

30
Q

Which of the following disease is being described?

Leucocytosis, basophilia, change in platelets

Normocytic anaemia

Abnormal leukocytes

Increase in B12

Slenomegaly

Philadelphia choromosome

A

Chronic myloid leukaemia

Leucocytosis, basophilia, change in platelets

Normocytic anaemia

Abnormal leukocytes

Increase in B12

Slenomegaly

Philadelphia choromosome

31
Q

Which is the most common leukaemia in children?

A

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

32
Q

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

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
Q

Explain the treatment for ALL

A

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
Q

Which is the most common leukaemia?

A

Chronic lymphocytic leukaemia is the most common leukaemia

35
Q

Describe the clinical course of CLL

A

Variable

Progressive lymphadenopathy / hepatosplenomegaly

Auto-immune - haemolysis, ITP

Bone marrow failure - due to marrow replacement

Hypogammaglobulinaemia & infection

36
Q

Give the Hb values for anaemia

a) female
b) male

A

The Hb values for anaemia

a) female = <115g/l
b) male = <130g/l

37
Q

What are the pathological consequences of anaemia?

A

Fatty change in myocardim and liver

Aggravate angina

Skin and nail atrophic changes

CNS cell death

38
Q

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

A

Haematological deficiencies = B12, folate & iron

Chronic disease - inflammation, infection, neoplasm

Hypoplasia

Dyserythroiesis

Marrow infiltration

39
Q

Give examples of increased destruction that can lead to anaemia.

A

Hypersplenism

Acute blood loss

Red cell abnormality (membrane/enzyme defects)

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

40
Q

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

A

Microcytic anaemia - MCV <80

Iron deficiency

Thalassaemia

Chronic disease

41
Q

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

A

Normocytic anaemia

Acute blood loss

Chronic disease

Combined haematinic deficiency

MCV 80-100

42
Q

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

A

Macrocytic anaemia

B12/folate deficiency

Alcohol/liver disease

Hypothyroid

Heamatological

MCV >80

43
Q

How do you test for a B12 deficiency?

A

IF Ab

Schilling test

Coeliac Ab

44
Q

Explain the life course of platelets

A

Produced: megakaryokes in BM

Circulation: 7-10 days

Destroyed: Spleen by splenic macrophages

45
Q

What is the production of platelets controlled by?

A

Platelet production is controlled by thrombopoietin (TPO)

Produced by the liver

Binds to plts & MK - feedback

46
Q

Give the clotting cascade

A

Intrinsic - XII, XI, IX, VII

Extrinsic - VII (TF)

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

47
Q

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

a) P2Y12
b) COX
c) GPIIBIIIA

A

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

48
Q

What is the normal range for platelet numbers?

A

150-400 X109

49
Q

Give the clinical features of platelet dysfunction

A

Easy bruising

Mucosal bleeding

Petechiae, purpura

Traumatic haematomas

50
Q

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

A

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
Q

A 32 year old male presents with the following blood results

Platelets: 60x109/l

WBC: 6x109 g/l

Hb: 150x109/l

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

A

A 32 year old male presents with the following blood results

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

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

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

Defect due to increased destruction

52
Q

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

A

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

53
Q

Describe immune thrombocytopenia

Give examples of when a primary and secondary infection will occur

A

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
Q

What are the treatments for immune thrombocytopenia

A

Immunosuppression - steroids/IVIG

Treat the underlying cause

Platelets - if bleeding

Tranexamic acid - if mucosal bleeding

55
Q

Describe thrombic thrombocytopenic purpura (TTP)

A

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
Q

What is the treatment for thrombic thrombocytopenic purpura (TTP)

A

The treatment for thrombic thrombocytopenic purpura (TTP) is:

High volume plasma exchange

Rituximab - monoclonal Ab

57
Q

Describe disseminated intravascular coagulation (DIC)

A

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
Q

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

A

Diseminated Intravascular Coagulation (DIC)

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

59
Q

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

a) MCV 78-98 fl
b) MCH 26-33 pg
c) MCHV 30-35 g/dl

60
Q

Describe thalassaemia

A

Globin chain disorder with leads to a reduction in Hb

61
Q

How do you monitor haemoglobinopathies?

A

Ferratin

Cardia and liver MRI

Endocrine testing

DEXA scanning

62
Q

Explain the problems with iron over load

A

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
Q

Explain how enzymopathies cause damage to RBC and two main causes

A

Shortened cell life due to oxidative damage

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

64
Q

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

A

Glucose-6-phosphate deficiency

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

65
Q

Decribe febrile neuropenia and who is at risk?

A

Febrile = fever >38

Neutrophils = <1.0 x109

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

66
Q

How do you treat febrile neuropenia?

A

Start broad spectrum antibiotics without waiting for the results

Identify those at risk

67
Q

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

A

Hyperviscosity

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

68
Q

Describe the treatment and prevention measures used with tumour lysis syndrome

A

Tumour lysis syndrome ​

Hydrate - aggrresive IV before and after chemo

Watch kidneys

Prevent hyperuricaemia - Lower uric acid

69
Q
A

Phase 2 AR (6 decks)

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