Aims and objectives Recap of haematology: anaemia, malignancies and - - PowerPoint PPT Presentation

▶
aims and objectives
SMART_READER_LITE
LIVE PREVIEW

Aims and objectives Recap of haematology: anaemia, malignancies and - - PowerPoint PPT Presentation

Nov 03, 2023 35 likes •558 views

Aims and objectives Recap of haematology: anaemia, malignancies and clotting Start with easy questions and gets progressively harder 35 seconds to answer Question 1: answer quickly Total number of questions: 12 Duration:

slide-1
SLIDE 1
slide-2
SLIDE 2

2

Aims and objectives

  • Recap of haematology: anaemia, malignancies and clotting
  • Start with easy questions and gets progressively harder
  • 35 seconds to answer
  • Question 1: answer quickly
  • Total number of questions: 12
  • Duration: 60 mins
  • Slides and recordings: app.bitemedicine.com
slide-3
SLIDE 3
slide-4
SLIDE 4
slide-5
SLIDE 5

5

Question 1

slide-6
SLIDE 6

6

Explanations

app.bitemedicine.com Serum iron In anaphylaxis, antihistamine should be given after adrenaline and fluids Ferritin NICE advises measuring this first-line. Low in iron deficiency Transferrin NICE advise measuring ferritin Total body iron This is not measured Urinary iron This is not measured A 25-year-old lady presents with lethargy. She complains of a craving for ice. Her Hb is 90g/L. What is your first-line investigation?

slide-7
SLIDE 7

Aetiology: Iron deficiency anaemia

Cause Reduced intake

  • Malnutrition
  • Breastfeeding
  • Malabsorption
  • Coeliac disease

Increased requirement

  • Pregnancy

Increased loss

  • Chronic bleeding
  • Colon cancer
  • Menorrhagia
  • Peptic ulcer disease
slide-8
SLIDE 8

Clinical features: Iron deficiency anaemia

Features

Glossitis Angular stomatitis/chelitis Koilonychia Pica

(1) (2)

slide-9
SLIDE 9

9

Investigations: Iron deficiency anaemia

Bloods

  • FBC: microcytic anaemia (MCV <80fL)
  • Blood film: hypochromic red cells, target cells
  • Iron studies
  • Ferritin: reduced
  • Serum iron: reduced
  • TIBC: increased
  • Transferrin saturation: decreased

Imaging

  • Endoscopy
  • Suspecting upper GI bleed
  • ≥60 years old with iron deficiency anaemia

Special tests

  • Coeliac serology
slide-10
SLIDE 10

10

Question 2

slide-11
SLIDE 11

11

Explanations

app.bitemedicine.com Thalassemia Microcytic anaemia (MCV < 80) G6PD deficiency X-linked recessive → seen in males Hereditary spherocytosis A hereditary cause of normocytic anaemia. Usually autosomal dominant Autoimmune haemolytic anaemia Rare in this age group. Associated with underlying conditions e.g. SLE or CLL, or with drugs Iron deficiency anaemia Microcytic anaemia (MCV < 80) A 4-year old girl presents with lethargy. Her Hb is 100 g/L and MCV 85fL. What is the likely cause?

slide-12
SLIDE 12

12

Anaemia

Anaemia

  • Men: Hb <130g/L
  • Women: Hb <120g/L
  • Classified based on mean corpuscular volume (MCV)

Microcytic (MCV < 80fL) Normocytic (MCV 80-95fL) Macrocytic (MCV >95fL) Iron deficiency Acute blood loss B12 deficiency Thalassaemia Haemolytic anaemia Folate deficiency Anaemia of chronic disease Anaemia of chronic disease Alcohol Sideroblastic anaemia Chronic kidney disease Liver disease Aplastic anaemia Hypothyroidism

slide-13
SLIDE 13

13

Question 3

slide-14
SLIDE 14

14

Explanations

app.bitemedicine.com Splenectomy Should be delayed Delay splenectomy till 6 years of age Delayed to reduce risk of post-splenectomy sepsis Delay splenectomy till 10 years of age Delayed till age 6 Stem cell transplant Not carried out Regular venesection (phlebotomy) Will exacerbate anaemia. This is a treatment for hereditary haemachromatosis Which of the following is the most appropriate treatment for this 4-year-old girl with hereditary spherocytosis?

slide-15
SLIDE 15

Hereditary spherocytosis

Definition: inherited defect in RBC membrane proteins leading

to a haemolytic anaemia

  • Usually autosomal dominant

Epidemiology:

  • Northern European and North American populations
  • 1 in 2000 people
slide-16
SLIDE 16

Pathophysiology: Hereditary spherocytosis

RBC membrane proteins:

  • Spectrin
  • Ankyrin
  • Band 3
  • Protein 4.2
slide-17
SLIDE 17

17

Investigations: Hereditary spherocytosis

(2)

slide-18
SLIDE 18

18

Management: Hereditary spherocytosis

Neonatal jaundice

  • Phototherapy or exchange transfusion

Blood transfusion

  • Hb <70g/L or
  • Hb <80g/L and cardiac co-morbidity

Folic acid

  • Daily until splenectomy

Splenectomy

  • Spleen removal reduces haemolysis
  • Delayed until patients are >6 years of age to reduce the risk of post-splenectomy sepsis
slide-19
SLIDE 19

19

Question 4

slide-20
SLIDE 20

20

Explanations

app.bitemedicine.com Raised HbH Associated with alpha thalassaemia Raised HbA Reduced in beta thalassaemia Raised HbA2 Raised along with HbF Raised HbS Associated with sickle cell anaemia Reduced HbF Should be raised A 9-month-old child is failing to thrive, and investigations reveal a diagnosis of beta thalassemia major. Which of the following would you expect?

slide-21
SLIDE 21

Introduction: Thalassaemia

Definition: autosomal recessive haemoglobinopathy

  • Impaired globin chain synthesis

Epidemiology:

  • Prevalent in areas of malaria
  • Alpha thalassaemia: Asian and African
  • Beta thalassaemia: Asian, Mediterranean and Middle Eastern
slide-22
SLIDE 22

Pathophysiology: Thalassaemia

Normal Hb Structure Proportion in adults HbA α2β2 90% HbA2 α2δ2 <2% HbF α2γ2 <2-5% Alpha thalassaemia Beta thalassaemia Reduced Reduced Reduced Increased Reduced Increased

slide-23
SLIDE 23

23

Question 5

slide-24
SLIDE 24

24

Explanations

app.bitemedicine.com Anti-GAD T1DM ANA Seen in connective tissue diseases e.g. SLE Anti-parietal cell Pernicious anaemia: most sensitive Anti-intrinsic factor Pernicious anaemia: most specific Anti-tTG Coeliac disease A 40-year-old type 1 diabetic has Hb 95 g/L and MCV 110 fL. Which of the following is the most sensitive investigation for the likely diagnosis?

slide-25
SLIDE 25

Pathophysiology: Pernicious anaemia

Definition: autoimmune process affecting vitamin B12

absorption

Epidemiology:

  • Most common cause of vitamin B12 deficiency

Risk factors:

  • Females > males
  • Autoimmunity: Addison’s disease, vitiligo, T1DM
slide-26
SLIDE 26

26

Question 6

slide-27
SLIDE 27

27

Explanations

app.bitemedicine.com t(15;17) APML t(9;22) CML t(8;14) Burkitt lymphoma t(11;14) Mantle cell lymphoma None of the above Gum hyperplasia suggests a diagnosis of AML (subtype monocytic) A 50-year-old presents with lethargy and weight loss. O/E there is hepatosplenomegaly and gum hyperplasia. What is the likely translocation involved?

slide-28
SLIDE 28

28

Introduction: Malignancy

slide-29
SLIDE 29

Pathophysiology: Acute myeloid leukaemia

9 subtypes of AML (FAB classification)

Acute promyelocytic leukaemia (M3)

  • t(15;17): fusion of retinoic acid receptor with

promyelocytic protein which blocks maturation

  • Younger patients ~ 45 years old
  • Associated with disseminated intravascular

coagulation

  • Good prognosis

Acute monocytic leukaemia (M5)

  • Monoblast accumulation
  • Gum infiltration

(1)

slide-30
SLIDE 30

30

Question 7

slide-31
SLIDE 31

31

Explanations

app.bitemedicine.com Erythrocyte Neoplastic cell in PCV Lymphocyte Neoplastic cell in certain leukaemias and all lymphomas Platelet Neoplastic cell in essential thrombocytosis Megakaryocyte Proliferation of the megakaryocyte leads to marrow fibrosis Granulocyte Neoplastic cell in AML/CML A 70-year-old presents with lethargy. O/E there is massive splenomegaly. Bone marrow biopsy shows fibrosis. What is the neoplastic cell?

slide-32
SLIDE 32

Introduction: Myelofibrosis

Definition: myeloproliferative condition. Neoplastic proliferation of

mature myeloid cells, particularly megakaryocytes

  • Leading to marrow fibrosis

Risk factors

  • Age: >65 years
  • Radiation
slide-33
SLIDE 33

Pathophysiology: Myelofibrosis

(1)

slide-34
SLIDE 34

34

Question 8

slide-35
SLIDE 35

35

Explanations

app.bitemedicine.com Rouleaux formation Stacked RBCs seen in myeloma Smudge cell CLL Auer rods AML Spherocytes Hereditary spherocytosis and autoimmune haemolytic anaemia Lymphoblasts ALL Which of the following is suggestive of myeloma?

slide-36
SLIDE 36

Pathophysiology: Myeloma

(3) (4)

slide-37
SLIDE 37

Investigations: Myeloma

(5)

slide-38
SLIDE 38

38

Question 9

slide-39
SLIDE 39

39

Explanations

app.bitemedicine.com Follicular lymphoma Not associated with Hashimoto’s thyroiditis Burkitt lymphoma Not associated with Hashimoto’s thyroiditis Marginal zone lymphoma Associated with chronic inflammatory states Mantle zone lymphoma Not associated with Hashimoto’s thyroiditis Hodgkin lymphoma Not associated with Hashimoto’s thyroiditis A 40-year old lady with a background of Hashimoto's thyroiditis presents with a nodule in the neck. What is the likely diagnosis?

slide-40
SLIDE 40

Marginal Zone Lymphoma

Germinal centre Mantle zone Marginal zone

slide-41
SLIDE 41

Marginal Zone Lymphoma

Definition: proliferation of small B cells that expand marginal zone Pathophysiology:

  • Can occur in lymph nodes or spleen
  • Extranodal sites:
  • Gastric MALToma (mucosa associated lymphatic tissue):

H.pylori infection

  • Thyroid gland: Hashimoto’s thyroiditis
  • Salivary gland: Sjögren's syndrome

Clinical:

  • Adults
  • Underlying condition
slide-42
SLIDE 42

42

Question 10

slide-43
SLIDE 43

43

Explanations

app.bitemedicine.com GpIIb/IIIa Idiopathic thrombocytopaenic purpura → IgG against GpIIb/IIIa Platelet factor 4 Associated with HIT Factor VIII Deficiency in Haemophilia A GpIb Deficiency in Bernard-Soulier disease Von Willebrand factor Deficency/impaired function in Von Willebrand disease A 10-year-old presents with petichae on his limbs. He had a viral URTI 2 weeks ago. Which of the following antigens is implicated in this disease?

slide-44
SLIDE 44

44

Idiopathic thrombocytopaenic purpura (ITP)

Pathophysiology

  • Type II hypersensitivity reaction: IgG directed against

PLT antigens e.g. GpIIb/IIIa causing splenic consumption

Clinical presentation

  • Acute ITP: most common cause of thrombocytopaenia in

children

  • Post-viral infection or immunisation
  • Self-limiting
  • Chronic ITP: seen in women of childbearing age
  • Relapsing-remitting course

Management

  • Corticosteroids
  • IVIG
  • Splenectomy

(6)

slide-45
SLIDE 45

45

Question 11

slide-46
SLIDE 46

46

Explanations

app.bitemedicine.com Screen for thrombophilia No screening required for a provoked PE Screen if he develops a second PE No screening required for a provoked PE Only screen if stopping anticoagulation No screening required for a provoked PE Only screen if on lifelong anticoagulation No screening required for a provoked PE No further tests required No screening required for a provoked PE A patient presents with a PE after a long-haul flight. There is a family history of PEs. Which option is true regarding thrombophilia screening?

slide-47
SLIDE 47

47

Investigation & Management: Hereditary thrombophilia

When to test patients?

  • Not typically tested for, even if patients have a positive family history

NICE guidance

  • Hereditary thrombophilia screen: unprovoked PE/DVT AND a first-degree relative who has

had PE/DVT AND there is a plan to stop anticoagulation

Management:

  • Anticoagulation: treatment commenced if patients develop a thrombus
  • Duration of treatment is a complex decision made by a haematologist
slide-48
SLIDE 48

48

Question 12

slide-49
SLIDE 49

49

Explanations

app.bitemedicine.com Reduced PT Usually normal Reduced APTT Prolonged APTT Increased PT Usually normal Increased APTT Lupus anticoagulant which causes paradoxical prolongation of the APTT in vitro. In vivo, it increases risk of thrombosis Normal clotting screen APTT prolonged A lady with recurrent miscarriages is found to have a anti-phospholipid syndrome. Which of the following is classically seen?

slide-50
SLIDE 50

50

Acquired thrombophilia

Acquired Mechanism Medications

  • COCP and HRT → increased coagulation factors and reduced protein S
  • Tranexamic acid → inhibition of plasminogen activation

Malignancy

  • Release of procoagulant factors e.g. tissue factor

Smoking

  • Endothelial damage

Obesity

  • Increased coagulation factors and inhibition of fibrinolysis

Antiphospholipid syndrome • Acquired antibodies which lead to coagulation cascade activation

  • Associated with SLE
  • Lupus anticoagulant
  • Anti-cardiolipin
  • Beta2-glycoprotein I

Nephrotic syndrome

  • Urinary loss of antithrombin III
slide-51
SLIDE 51

51

References

1. Matthew Ferguson 57 / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 2. CHeitz / CC BY (https://creativecommons.org/licenses/by/2.0) 3.

  • Modified. A.Rad and Mikael Häggström, M.D. - Author info - Reusing imagesExample citation (in caption or footnote):-

&quot;By A. Rad and M. Häggström. CC-BY-SA 3.0 license.&quot; / CC BY-SA (http://creativecommons.org/licenses/by- sa/3.0/) 4. Yohan at fr.wikipedia, translated to English and converted to .svg by Sbmehta / CC BY-SA (http://creativecommons.org/licenses/by-sa/3.0/) 5. Gabriel Caponetti / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 6. User:Hektor / CC BY-SA (http://creativecommons.org/licenses/by-sa/3.0/) All other images were made by BiteMedicine or under basic license from Shutterstock and not suitable for redistribution.

slide-52
SLIDE 52

52

Further information

Stay up-to-date!

  • Website: www.bitemedicine.com
  • Facebook: ‘BiteMedicine for Students’ for all our updates
  • Instagram: @bitemedicine
  • Email: admin@bitemedicine.com

Feedback form: please provide your feedback on how we can improve our webinar integration system