Haematology seminar 1

Question 1

What are some examination findings of anaemia?

Answer 1

Pallor
Jaundice
Splenomegaly
Lymphadenopathy
Koilonychia

Question 2

What is microcytic hypochromic anaemia?

Answer 2

When the red blood cells are small and pale in colour
Caused by iron deficiency and it’s a thalassaemia trait

Question 3

How can anaemia arise?

Answer 3

Problems of synthesis
Over-consumption
Bleeding

Question 4

What is thalassaemia?

Answer 4

When the body doesn’t make enough haemoglobin

Question 5

What causes normocytic anaemia?

Answer 5

Its anaemia of chronic disease caused by bone marrow failure or bleeding

Question 6

What causes macrocytic anaemia?

Answer 6

B12/folate deficiency
Myelodysplasia Haemolysis (reticulocytosis)
-Pregnancy
-Hypothyroidism
-Alcoholism

Question 7

What is macrocytic anaemia?

Answer 7

The red blood cells are larger than normal

Question 8

What is included in the haemolysis screen?

Answer 8

• Reticulocytes
• Direct anti-globulin test (DAT = Coomb’s test)
• Bilirubin
• LDH
• Blood film
  Eg. Spherocytes, Sickle cells, RBC fragments

Question 9

What are reticulocytes?

Answer 9

Immature red blood cells

Question 10

What is Haemoglobinopathy?

Answer 10

Inherited disorders of globin, the protein component of haemoglobin

Question 11

What are the different problems of synthesis causing anaemia?

Answer 11

Haematinic deficiency
- Iron deficiency (low ferritin)
- B12 (pernicious anaemia, gastrectomy or Crohn’s)
- Folate deficiency (diet, pregnancy or malabsorption e.g. coeliac)

Bone marrow failure
- Serum protein electrophoresis (myeloma?)
- Pancytopenia (bone marrow biopsy needed)
- Blasts (leukaemia)
- High MCV (myelodysplasia)

Renal failure
- Raised creatinine
- Low Epo levels

Question 12

What is the treatment for iron deficiency?

Answer 12

Iron sulphate tablets 200mg TDS for 3 months

Question 13

How is folate deficiency treated?

Answer 13

5mg oral OD for 3 months plus dietary advice

Question 14

How is B12 deficiency treated?

Answer 14

Hydroxycobalamin 1mg IM injections for 5 alternating days then every 3 months

Question 15

What is Hydroxycobalamin used for?

Answer 15

It’s a form of injectable B12

Question 16

How does Erythropoeitin work?

Answer 16

It’s naturally produced by the peritubular cells of the kidney

It stimulates red blood cell production

Question 17

What is hereditary spherocytosis?

Answer 17

Instead of being shaped like a disk, the cells are round like a sphere

These red blood cells (called spherocytes) are more fragile than disk-shaped RBCs

Question 18

How does G6PD cause haemolytic disease?

Answer 18

Without Glucose-6-phosphate dehydrogenase to protect the RBC’s, they break apart

Question 19

What is autoimmune haemolysis?

Answer 19

Premature destruction of healthy red blood cells

Warm (IgG-mediated): happens at room temperature
Cold (IgM): triggered by cold

Question 20

What is valve haemolysis?

Answer 20

A complication of prosthetic heart valves

Question 21

What are the 2 types of bone marrow biopsy?

Answer 21

Aspirate (marrow smears – haematologists)
Trephine (tissue sections – pathologists)

Question 22

What is aplastic anaemia?

Answer 22

When the body doesn’t make enough RBC’s because the immune system attacking the stem cells in your bone marrow

Question 23

Name some haematological marrow diseases

Answer 23

Leukaemia
Lymphoma
Myeloma
Myelodysplasia
Myelofibrosis

Question 24

What is acute myeloid leukaemia?

Answer 24

• Mostly older adults (>60 yrs)
  -25% of childhood cancers
• Cure rate <50%, prognosis depends on cytogenetics and gene mutations
• Treatment with intensive chemotherapy (DA 3+10 regime)
• Allogeneic stem cell transplants is potentially curative

Question 25

What is acute lymphoid leukaemia?

Answer 25

Commonest childhood cancer (mostly <10yrs) -Cure rate >90% - Treatment with chemotherapy for 2 years

Question 26

What is chronic myeloid leukaemia?

Answer 26

High WCC (leucocytosis) Massive splenomegaly

Question 27

What is the Philadelphia chromosome?

Answer 27

Abnormal fusion protein = BCR-ABL tyrosine kinase Causes CML

Question 28

What is the treatment for CML?

Answer 28

Imatinib It inhibits the ATP pocket of BCR-ABL

Question 29

What is Myelodysplasia?

Answer 29

A group of cancers in which immature blood cells in the bone marrow do not mature or become healthy blood cells Causes anaemia, neutropenia, thrombocytopenia It can transform into AML Treated with Azacytidine (elderly) or curative allografting (young)

Question 30

What is Myelofibrosis?

Answer 30

Fibrosis of the bone marrow causing pancytopenia - Typically JAK2+ve - Transforms eventually into AML Treated with hydroxycarbamide or JAK inhibitors or allografting

Question 31

What is excess myelopoiesis in bone marrow?

Answer 31

Myelopoiesis: the production of myeloid cells by bone marrow hematopoietic stem and progenitor cells Typically carry JAK2 V617F mutation Excessive production causes a risk of thrombotic events

Question 32

What is Polycythemia rubra vera?

Answer 32

High Hb and haematocrit Treat with venesection

Question 33

What is essential thrombocythemia?

Answer 33

High platelet count Treated with hydroxycarbamide

Question 34

How does hydroxycarbamide work?

Answer 34

It works by increasing foetal haemoglobin

Question 35

What is myeloma?

Answer 35

Malignancy of plasma cells, it's treatable but incurable Treatment: - Chemotherapy for 4-6 months (cyclophosphamide, Velcade/thalidomide, steroids) - Autologous stem cell transplant if young and fit

Question 36

When should myeloma be concidered?

Answer 36

Hypercalcaemia Renal failure Unexplained anaemia Bone pain or pathological fractures

Question 37

What is the CRAB acronym for end organ failure?

Answer 37

Hyperkalaemia Renal failure Anaemia Bone disease It most typical clinical manifestations of multiple myeloma

Question 38

What is the work up for myeloma?

Answer 38

Blood tests - Haematology: anaemia, rouleaux, viscosity, ESR – Biochemistry: U+Es, Ca, Albumin, total protein, LDH, CRP, β2-M Serum protein electrophoresis - Paraprotein (Monoclonal M band) - Immunoglobulin levels low (immune paresis) Serum free light chain assay - Abnormal κ/λ light chain ratio - Staging investigations

Question 39

What is ITP?

Answer 39

Immune thrombocytopenic purpura A rare autoimmune disorder where the blood doesn't clot properly because the immune system destroys the platelets

Question 40

What are the symptoms of Immune thrombocytopenic purpura?

Answer 40

- Bruising - Nose bleeds - GI bleeds - Major haemorrhage Usually self-limiting in children

Question 41

What is AIHA?

Answer 41

Autoimmune haemolytic anaemia

Question 42

What are the symptoms of autoimmune haemolytic anaemia?

Answer 42

Pallor Jaundice Dark urine

Question 43

How is autoimmune haemolytic anaemia treated?

Answer 43

Transfuse as required (but difficulty cross-matching) – Warm AIHA – treat with steroids, splenectomy, Rituximab – Cold CHAD – treat with immunosuppression

Question 44

What are the causes of splenomegaly?

Answer 44

Haematological causes - Myeloproliferative disorders - Lymphoproliferative disorders - Haemolytic states Infective causes - Viral illness – EBV, CMV, HIV - Tropical (malaria, schistosomiasis) Portal hypertension due to liver disease Rheumatoid arthritis (Felty’s syndrome)

Question 45

What is Haemophilia?

Answer 45

A = Factor VIII B = Factor IX (Christmas disease) Both cause prolonged APTT X-linked recessive (males only affected) Causes severe bleeding into joints and muscles Treat with recombinant factor product (or DDAVP if mild) Treat prophylatically if severe haemophilia (<1% factor levels)

Question 46

What is von Willebrand’s disease?

Answer 46

Deficiency of von Willebrand factor (vWF) Low factor VIII and platelet aggregation causing mild bleeding into mucous membranes Treat with DDAVP or Haemate P

Question 47

How does Warfarin work?

Answer 47

It prevents γ-carboxylation of factors II, VII, IX, X Monitored by INR (based on prothrombin time) but watch drug interactions Reversed by vitamin K or Octaplex (25-50 IU/kg) if major bleed Teratogenic in pregnancy; safe in renal failure

Question 48

How does Heparin work?

Answer 48

It blocks thrombin to prevent clotting LMWH - Dosed by weight, no monitoring required - Safe in pregnancy - Unsafe in renal failure Unfractionated Heparin - Safe in renal failure - Reversed by protamine sulphate

Question 49

What is anti-phospholipid syndrome?

Answer 49

An autoimmune disorder that causes abnormal blood clots to form Treatment: – Anticoagulation with warfarin – LMW heparin and aspirin in pregnancy