Haematology

Question 1

Microcytic anaemia causes

Answer 1

Low MCV <80 fl 
Defects in haem synthesis:
- iron deficiency
- anaemia of chronic disease
- sideroblastic anaemia
Defects in globin synthesis
- thalassaemia

Question 2

Iron deficiency anaemia

Answer 2

Blood loss: menorrhagia, upper/lower GI bleed, hookworms
Reduced absorption: coeliac disease, resections
Reduced intake: diet
Increased demands: pregnancy, growth

Question 3

Sideroblastic anaemia

Answer 3

Abnormality of haem synthesis (iron inclusion within the cell)
Inherited or secondary (alcohol/drugs/lead poisoning)

Question 4

α thalassaemia

Answer 4

Microcytic anaemia

2α = trait, 3α = HbH disease, 4α = Hb Barts (incompatible with life)

Question 5

ß thalassaemia

Answer 5

2β = transfusion dependent
Desferioxamine for chelation
Hepatosplenomegaly, jaw protrusion, big forehead, “hair on end” skull XR

Question 6

Management of sideroblastic anaemia

Answer 6

Treat the cause

Inherited: pyridoxine

Question 7

Management of lead poisoning

Answer 7

Remove source
Dimercaprol
D-penicillinamine

Question 8

Normocytic anaemia causes

Answer 8

MCV 80-100
Acute blood loss: peptic ulcer, varices
Failure of RBC production:
- bone marrow failure or suppression (aplastic anaemia, chemotherapy)
- bone marrow infiltration (malignancy)
- renal failure (reduced erythropoietin)
- early iron deficiency or anaemia of chronic disease
- hypothyroidism (+macrocytic)
- vitamin B2/6 deficiency
Haemolysis
Uncompensated fluid increase: pregnancy, fluid overload
Pooling of red cells in spleen: hypersplenism (portal cirrhosis)

Question 9

Macrocytic anaemia

Answer 9

MCV >100fl
Megaloblastic: vitamin B12 or folate acid deficiency
Drugs (methotrexate, hydroxyurea, azathioprine, zidovudine
Non-megaloblastic: 
Alcohol excess/liver disease (round macrocytes)
Myelodysplasia
Multiple myeloma
Hypothyroidism
Aplastic anaemia
Haemolysis (reticulocytosis)
Drugs
Pregnancy

Question 10

Blood film findings

• hypersegmented neutrophils
• schistocytes
• spherocytes
• Howell-Jolly bodies
• Heinz bodies
• dacrocytes
• codocytes
• burr cells

Answer 10

Hypersegmented neutrophils: megaloblastic anaemia, cytotoxic drugs, myelodysplastic syndrome
Schistocytes: MAHA, sickle cell disease
Spherocytes: hereditary spherocytosis, autoimmune haemolytic anaemia
Howell-Jolly bodies: reduced splenic function, severe haemolytic anaemia, myelodysplastic syndrome
Heinz bodies: G6PD
Dacrocytes: myelofibrosis
Codocytes: haemoglobinopathies, iron deficiency anaemia, hyposplenism, liver disease
Burr cells (echinocytes): uraemia, pyruvate kinase deficiency

Question 11

Haemolytic anaemia

Answer 11

Jaundice + anaemia
Hereditary: 
- membrane defects
- metabolic defects
- haemoglobinopathies
Acquired:
- autoimmune
- isoimmune
- drugs e.g. penicillin, quinine (RBC autoantibodies)
- trauma (MAHA)
- infection
- Paroxysmal nocturnal haemoglobinuria

Question 12

Sickle cell anaemia

Answer 12

African/Afro-caribbean
Symptomatic at altitude/hypoxia
Sickle cell crisis: acute chest syndrome, priaprism, joint pain

Question 13

Hereditary spherocytosis

Answer 13

Osmotic fragility test +ve
Spherocytes on blood film
Splenomegaly

Question 14

G6P deficiency

Answer 14

X-linked recessive
Triggers: moth balls/fava beans/nitrofurantoin/anti-malarials, infection
Heinz bodies

Question 15

Autoimmune haemolytic anaemia

Answer 15

Coomb’s test: erythrocytes with antibodies
Cold-activated: mycoplasma
Warm-activated: CML/lymphoma/SLE

Question 16

Paroxysmal Nocturnal Haemoglobinuria

Answer 16

Red discolouration of urine (haemoglobin and haemosiderin)

Ham’s test: lysis of erythrocytes in acidic solution

Question 17

Haemolytic Uraemic Syndrome

Answer 17

Triad: acute renal failure, thrombocytopaenia, microangiopathic haemolytic anaemia
E.coli -> blood cell haemolysis

Question 18

Trhombotic Thrombocytopenic Purpura

Answer 18

Pentad: HUS +CNS signs +fever

Clots, low platelets, rash

Question 19

Disseminated Intravascular Coagulation

Answer 19

Septic/post-natal/vascular insult

Low fibrinogen + presence of clots

Question 20

Anaemia of chronic disease

Answer 20

Rheumatoid arthritis
Cancer
Chronic inflammation

Question 21

Aplastic anaemia

Answer 21

Pancytopaenia
Hypocellular bone marrow
Absent reticulocytes

Question 22

Polycythaemia rubra vera

Answer 22

Uncontrolled RBC proliferation
JAK2 kinase/V167F mutation
Headaches, dizziness and strokes (viscosity)
Increased mast cells = pruritis after hot showers
Gout, splenomegaly, plethora

Question 23

Secondary polycythaemia

Answer 23

Excessive RBC production due to other disease (COPD/EPO-secreting tumour)
Blood doping (athletes)
Sustained hypoxia

Question 24

Immune Thrombocytopenic Purpura

Answer 24

Often idiopathic
Menorrhagia, nosebleeds, purpura
Associated with:
- infections
- autoimmune diseases
- malignancy
- drugs (quinine)

Question 25

ALL

Answer 25

Children Lymphadenopathy Bone marrow failure: anaemia, neutropaenia, thrombocytopaenia Blast cells

Question 26

AML

Answer 26

``` Adults RARA-PML protein Auer Rods Sudan Black B stain (myeloblasts) Normocytic normochromic anaemia ```

Question 27

APML

Answer 27

Treat with vitamin A and arsenic tr(15,17) CNS involvement Skin rash, gum bleeding

Question 28

CLL

Answer 28

Incidental finding Raised WCC Smear cells

Question 29

CML

Answer 29

``` 25-40 years Blast cells Philadelphia chromosome (BCR-ABL gene tr9,22) Imantinib 3 phases: chronic > blast > accelerated ```

Question 30

Hodgkin's lymphoma

Answer 30

``` Continuous spread Painful after EtOH Lymphadenopathy Hepatosplenomegaly Bone marrow failure Reed-Sternberg cells ```

Question 31

Non-hodgkin's lymphoma

Answer 31

Non-continuous spread Burkitt's lymphoma: mass on jaw, African descent Marginal zone lymphoma: MALT Diffuse Large B Cell lymphoma: hepatitis C

Question 32

Multiple myeloma

Answer 32

CRAB Bence Jones urinary protein Plasma cells Pathological fractures and back pain

Question 33

Haemophilia

Answer 33

``` A: Factor 8 B: Factor 9 X-linked Deep bleeding: haemarthrosis APTT affected only ```

Question 34

Von Willebrand disease

Answer 34

Superficial bleeding Loss of vWF Prolonged APTT and bleeding time

Question 35

Vitamin K deficiency

Answer 35

Liver disease | Pancreatic insufficiency

Question 36

Factor V Leiden

Answer 36

5% Caucasians | DVT <45yrs

Question 37

Blood Group O

Answer 37

x4 increased clot risk

Question 38

Antiphospholipid syndrome

Answer 38

``` Anti-cardiolupin Obstetric complications (multiple miscarriages) Thromboembolism events Thrombocytopaenia Livedo reticularis ```