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
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? ## Footnote 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? ## Footnote Leucocytosis, basophilia, change in platelets Normocytic anaemia Abnormal leukocytes Increase in B12 Slenomegaly Philadelphia choromosome
**Chronic myloid leukaemia** ## Footnote 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. ## Footnote 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
47
The followin are targets for antiplatelet drugs. What are their normal actions and the drugs which target them? ## Footnote a) P2Y12 b) COX c) GPIIBIIIA
The followin are targets for antiplatelet drugs. Their normal are actions... ## Footnote 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
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. ## Footnote 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: ## Footnote 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 ## Footnote Platelets: 60x109/l WBC: 6x109 g/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 ## Footnote 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
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)** ## Footnote 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)** ## Footnote 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: ## Footnote High volume plasma exchange Rituximab - monoclonal Ab
57
Describe disseminated intravascular coagulation (DIC)
**Disseminated intravascular coagulation (DIC)** ## Footnote 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? ## Footnote 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: ## Footnote 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 x109 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 ​** ## Footnote Hydrate - aggrresive IV before and after chemo Watch kidneys Prevent hyperuricaemia - Lower uric acid
69