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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Results of vascular and platelet bleeding disorders

A

Prolonged bleeding from cuts
Bleeding into skin - bruising
Bleeding from mucous membranes

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2
Q

Result of coagulation disprders

A

Delayed bleeding into joints and muscles

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3
Q

Features of immune thrombocytopenic purpura - what is it? 2 types - acute and chronic

A

Antiplatelet autoantibodies
Acute (usually in children - 2 weeks after infection with sudden self-limiting purpura)
Or
Chronic - usually women - fluctuating course of bleeding, purpura, epistaxis and menorrhagia

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4
Q

Tests in ITP

A

Antiplatelet autoantibodies and megakaryocytes increased in bone marrow

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5
Q

Treatment of ITP

A

Often no treatment if mild as is self-limiting in children

If serious bleed can give steroids or immunoglobulins

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6
Q

What is Eltrombopag?

A

New oral thrombopoietin receptor agonist / stimulates thrombopoiesis

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7
Q

What is haemophilia a

A

Factor VIII deficiency

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8
Q

How are haemophilias inherited?

A

X linked recessive

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9
Q

Presentation of haemophilia a

A

Often early in life
Or after surgery
With bleeding into joints - crippling arthropathy
Bleeding into muscles - haematomas

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10
Q

Tests in haemophilia a

A

Increased aptt
Decreased factor VIII
Other bleeding time etc normal

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11
Q

Things to avoid in haemophilia

A

IM injections

NSAIDs

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12
Q

treatment of mild bleed and more severe bleed in haemophilia

A

Desmopression for mild

Recombinant factor VIII if life-threatening bleed

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13
Q

What is defect in haemophilia b?

A

Factor IX deficiency

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14
Q

Why get bleeding in liver disease?

A

Decreased synthesis of clotting factors and decreased absorption of vitamin k

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15
Q

If PT is long

A

Look for liver disease or anticoagulant use

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16
Q

If APTT long

A

Look for liver disease, Haemophilia or heparin use

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17
Q

If PT and APTt are very raised, low platelets and raised D-dimers

A

DIC

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18
Q

How does aplastic anaemia present?

A

Anaemia, infection and bleeding

All bone marrow products decreased

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19
Q

Treatment of aplastic anaemia

A

Support blood count
Red cell transfusion
Platelets
Neutrophils

Bone marrow transplant from HLA matched sibling may be curative
Otherwise immunosuppression with cyclosporin (most cases are autoimmune)

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20
Q

What are myeloproliferative disorders

A

Proliferation of a clone of haematopoietic myeloid stem cells in the marrow
Retain ability to differentiate into red blood cell, white blood cell or platelets

21
Q

What happens if RBC myeloproliferative disorder

A

Polycythaemia rubra vera

22
Q

If platelets proliferative

A

Essential thrombocythaemia

23
Q

WBc proliferative

A

Chronic myeloid leukaemia

24
Q

Causes of relative polycythaemia x2 acute and chronic

A

Acute - Dehydration

Chronic - obesity, hypertension, high alcohol and tobacco

25
Q

What is raised in polycythaemia rubra Vera

A

RBC, Hb, WCC, platelets, haematocrit

Marrow shows hypercellularity

26
Q

Treatment of PRV

A

Young and low risk / venesection
Older and higher risk - hydroxycarbamide
Also give aspirin - increased thrombosis risk

27
Q

What happens in myelofibrosis?

A

Hyperplasia of megakaryocytes - intense marrow fibrosis and myeloid metaplasia - harmopoiesis in spleen and liver

28
Q

Presentation of myelofibrosis

A

Hypermetabolic symptoms
Abdo discomfort due to have splenomegaly
Or signs of bone marrow failure

29
Q

What are plasma cell dyscrasias?

A

Abnormal proliferation of a single clone of plasma or lymphoplasmacytic cell - leading to increased secretion of immunoglobulin or a fragment of Ig
This causes dysfunction of many organs - esp kidney

30
Q

What is normally high and what is normally low in PCD

A

Usually IgG or IgA
This will be high
Others will be low - increased susceptibility to infection
ESR high

31
Q

What is seen in urine in PCD - myeloma

A

2/3 bence jones proteins - free Ig chains filtered by kidney

32
Q

Main symptom which is red flag for looking for myeloma in >50

A 
Back pain 
Caused by osteolytic bone lesions 
- backache
- pathological fractures 
- vertebral collapse
33
Q

Other effects of myeloma

A

Marrow infiltrate for plasma cells

Leads to anaemia, thrombocytopenia and neutropenia

34
Q

Effect of decreased other immunoglobulins in myeloma

A

Recurrent bacterial infections

35
Q

Treatment of effects of myeloma

A 
Analgesia for bone pain 
Bisphosphonate 
Correct anaemia 
Rehydrate for renal failure 
Antibiotics and immunoglobulin because of immunoparesis
36
Q

Chemotherapy for myeloma if fitter patient

A

VAD
Vincristine
Adriamycin
Dexamethasone

37
Q

Chemo for un fit myeloma patients (MPs are unfit)

A

Melphalan and prednisolone

Controls disease for about 1 year

38
Q

Cause of death in myeloma patients

A

Infection

Renal failure

39
Q

Congo red stain

A

Amyloid

40
Q

Signs of increased blood viscosity

A 
Lethargy
Confusion and decreased cognition 
CNS disturbance 
Chest pain
Abdominal pain
Faints
Visual disturbance
41
Q

Causes of hyperviscosity

A

High red cell count - PRV
High white cell count - leukaemia
High plasma components - immunoglobulins - myeloma
Drugs - OCP, IV Ig, erythropoietin, radio-contrast media

42
Q

What is thrombophilia?

A

Inherited or acquired coagulopathy predisposing to thrombosis
Usually venous thrombosis - PE or DVT

43
Q

What is the chief cause of inherited thrombophilia?

A

Activated protein C resistance/factor V Leiden

44
Q

What causes Apc resistance/Factor v Leiden

A

Single point mutation in factor V

Not broken down by APC

45
Q

Other inherited causes of thrombophilia

A

Prothrombin gene mutation - high levels of prothrombin
Protein c and s deficiency - neutralise factors V and VIII
Antithrombin deficiency

46
Q

3 processes involved in stopping bleeding

A

Vasoconstriction
Gap- plugging by platelets
Coagulation cascade

47
Q

Features of Von Willebrand disease

A

Common inherited bleeding disorder - mostly autosomal dominant
Mild and may only present as prolonged bleeding following injury eg. tooth extraction
Behaves like platelet disorder - menorrhagia and epistaxis rather than haemarthrosis

48
Q

Tests in VW disease

A

APTT may be prolonged
prolonged bleeding time
Factor VIII levels may be moderately reduced

49
Q

Management of VW disease

A

Tranexamic acid for mild bleeding

Desmopressin increases VW production

Haematology (6 decks)

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