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If a patient is bleeding or bruising spontaneously what are the three process that could be disrupted?

Vascular defect
Congenital - Osler Weber Rendu syndrome, connective tissue disorders
Acquired - Infection, HSP, steriods, scurvy

Platelet disorder
Diseased marrow production - Aplastic anaemia, megaloblastic anaemia, malignancy, marrow suppression
Excess destruction - ITP, SLE, CLL, drugs, DIC, thrombotic thrombocytopenic purpura, sequestration
Poorly functioning - myeloproliferative disorders, NSAIDs, increased urea

Coagulation defects
Congenital - Haemophilia, von Willebrands
Acquired - anticoagulants, liver disease, DIC, vitamin K deficiency

1

When would you suspect a patient had ITP?

Immune thrombocytopenia causes low platelets and increased bleeding.
Most common in children (acutely 2 weeks after an infection) and women (chronically).
Causes bleeding and purpura (over dependent pressure areas) and increased epistaxis and menorrhagia.
Splenomegaly is not present

2

How do you treat ITP?

No treatment needed if mild
If symptomatic or platelets 30.

If relapsing splenectomy cures >80%

Can use immunosuppression if needed

3

What is Haemophillia A?

Factor VIII deficiency
X link recessive
Usually presents with pain after trauma or surgery as bleed into joints and muscles
Diagnosed by high APTT (INR will be normal) and low factor VIII assay.

4

How do you manage bleeding in a patient with Haemophilia A?

Factor VIII deficiency

Minor bleeding - pressure and elevation. Desmopressin raises factor VIII levels

Major bleeds (haemarthrosis) - increase factor VIII levels to over 50% of normal

Life threatening bleeds - need factor VII levels of over 100%, use recombinant factor VIII

5

What is Haemophilia B?

Christmas disease - factor IX deficiency
X link recessive
Behaves similar to Haemophilia A

6

What does von Willebrand factor do?

1. brings platelets into contact with exposed subendothelium
2. makes platelets bind to one another
3. bind factor VIII to stop destruction in circulation

7

What are the 4 most common forms of von Willebrand's disease?

Type I - autosomal dominant deficiency of vWF
Type II - abnormal vWF
Type III - undetectable vWF, autosomal recessive with gene deletions
Type Normandy - impaired vWF binding, causes an autosomal recessive mimic of haemophilia A

8

What signs and symptoms suggest von Willebrand disease?

Bruising, epistaxis, menorrhagia, - mild in type I and II
APTT increased, bleeding time increased, factor VIIIc decreased,
INR and platelets are normal

9

Which clotting factors are vitamin K dependent?

II, VII, IX, X

10

What are the differences between APPT, and PT?

Prothrombin time - tests extrinsic pathway
Tests for abnormalities in factor I, II, V, VII, X
Prolonged by warfarin, vitamin K deficiency, liver disease and DIC

Activated partial thromboplastin time - tests intrinsic pathway
Tests for abnormalities in I, II, V, VIII, IX, X, XI, XII
Prolonged by heparin treatment, haemophilia, DIC, liver disease

11

What are the differentials for a macrocytic anaemia?

B12 or Folate deficiency
Alcohol excess
Reticulocytosis
Renal failure
Myelodysblastic syndromes
Marrow infiltration
Hypothyroidism
Drugs - phenytoin, methotrexate,

12

What are the causes of B12 deficiency?

Dietary
Malabsorption:
Stomach (lack of intrinsic factor): pernicious anaemia, post gastrectomy
Terminal ileum: ileal resection, Crohn's, bacterial overgrowth, tropical sprue, tapeworms,
Congenital abnormalities in metabolism

13

What are the symptoms of vitamin B deficiency?

Symptoms of anaemia
Lemon tinge to skin (due to pallor and increased bilirubin)
Glossitis
Angular stomatitis
Irritability, depression, psychosis and dementia
Subacute combined degeneration of the spinal cord:
Joint position and vibration sensation lost
Extensor plantar responses
Absent knee and ankle jerks
Ataxia
Glove and stocking sensation loss

14

What is subacute combined degenernation of the spinal cord?

Symmetrical posterior dorsal column loss and symmetrical cortico-spinal tract loss - all caused by vit B deficiency

15

What is pernicious anaemia?

Autoimmune atrophic gastritis - leading to lack of intrinsic factor and therefore vit B12 deficiency

17

What are the different types of amyloidosis?

AL amyloid (primary) - due to clonal proliferation of plasma cells, results in production of amyloidgenic monoclonal immunoglobulins.
Deposition is seen in:
Kidneys (nephrotic syndrome), heart (restrictive cardiomyopathy, arrhythmias, angina), nerves (peripheral and autonomic neuropathy), gut (macroglossia, malabsoprtion, perforation, haemorrhage, obstruction, hepatomegaly), vascular (periorbital purpura)

AA amyloid (secondary) - occurs in chronic inflammation. The amyloid is built up from serum amyloid A (acute phase protein)
Deposition in kidneys, liver, and spleen. Cardiac involvement is rare.

Familial amyloidosis - autosomal dominant disorder.

18

What stain would diagnosis amyloid?

Congo red stain with red-green birefringence

19

How do you treat vitamin B deficiency?

hydroxyocobalamin 1mg on alternate days for 2 weeks and then 1mg every 3 months for life - if due to malabsorption

20

What is special about Hodgkins lymphoma?

The Reed-Sternberg cells in them, they are cells with mirror image nuclei

It has more systemic features, patients get fevers, night sweats, puritis, lethargy

21

What is lymphoma?

Disorder charcterised by malignant proliferation of the lymphocytes, leading to lymphadenopathy - usually rubbery and non tender

22

How is lymphoma staged?

Ann Arbor staging - CXR, CT and bone marrow biopsy

I confined to lymph node region
II involvement of two or more nodal areas on he same side of the diaphragm
III involvement of nodes on both side of the diaphragm
IV spread beyond the lymph nodes (liver or bone marrow)

A - no systemic symptoms other than puritis
B - weight loss >10% in last 6 months, unexplained fever >38, drenching night sweats

23

What is the treatment for Hodgkin's lymphoma?

Adriamycin
Bleomycin
Vinblastine
Darcarbazine
+ radiotherapy

24

What is the treatment for high grade non hodgkin's lymphoma?

R - CHOP

Rituximab, Cyclophasphamide, Hydroxydaunorubin, Prednisolone

25

Explain the genetic basis of sickle cell:

Autosomal recessive resulting in the abnormal production of the B chains of haemoglobin.
There is an amino acid substitution at position 6 Glu - Val, so that HbS is produced rather than HbA.

The HbS polymerizes when deoxygenated causing the RBCs to deform, leading to sickle cells - fragile , haemolyse and block small vessels

HbSS = sickle cell aneamia
HbAS = sickle cell trait, no symptoms, protected from malaria, may have venoocculsive episodes in hypoxic enviroments ie pressurised aircraft
HbSC or HbS/B thal = can have symptomatic sickling

26

What would you see in blood tests of a sickle cell patient?

Hb 6-9
Increased reticulocytes (increased production)
Increased billirubin (due to increased haemolysis)

27

How do patients with sickle cell present acutely?

Vaso-occlusive crisis:
VV painful
Triggered by the cold, dehydration, infection or hypoxia
The occulsions can occur anyway but commonly in bone marrow, mesentery (presents with acute abdomen), CNS (leading to stroke, seizures)

Aplastic crisis:
Caused by parvovirus

Avascular necrosis of the femoral head

Priaprism

Sequestration crisis:
pooling of blood in the spleen leading to severe anaemia and shock.

Acute chest syndrome
pulmonary infiltrates (usually fat emboli from bone marrow)
pain, fever, tachypnoea, wheeze, cough

28

What are the long term complications of sickle cell?

Reduced growth and development
Leg ulcers
Cardiac problems
Neurological problems
Cholelithiasis
Renal problems
Eye problems
Pregnancy
Pulmonary hypertension due to repeated episodes of hypoxia leading to fibrosis
Reduced immunity due to splenic atrophy

29

How do you manage a sickle cell crisis?

ANALGESIA
Cross match blood if pyrexial or chest signs
Rehydrate
Antibiotics if fever, chest signs or unwell

30

Name the different types of myeloproliferative disorders?

Disorders caused by proliferation of a clone of haematopoietic myeloid stem cells in the marrow

Polycythaemia rubra vera - RBC
Chronic myeloid leukaemia - WBC
Essential thrombocytopenia - Platelets
Myelofibrosis - fibroblasts

There can be transformation from one to another and they all can transform into acute myeloid leukaemia