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Clinical Haematology > CHAPTER 25: Bleeding disorders caused by vascular and platelet abnormalities > Flashcards

CHAPTER 25: Bleeding disorders caused by vascular and platelet abnormalities Flashcards

1
Q

Abnormal bleeding may result from?

A
  1. Vascular disorders
  2. Thrombocytopenia
  3. Defective platelet function
  4. Defective coagulation
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2
Q

Fill in the blanks. “ Vascular and platelet disorders tend to be associated with bleeding from _________ and into ___________.”

A

Vascular and platelet disorders tend to be associated with bleeding from MUCOUS MEMBRANES and into the SKIN.

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3
Q

True or False? In coagulation disorders ,the bleeding is often into joints or soft tissue.

A

TRUE!!

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4
Q

What are the general characteristics of Platelets/vessel wall diseases?

A
  1. Mucosal bleeding
  2. Petetchiae
  3. Persistent Bleeding from skin/cuts
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5
Q

What is a main characteristic feature of Coagulation disorders?

A

Deep Haematomas

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6
Q

True or False? Coagulation disorders are more common in Females than Males.

A

FALSE!! It is more common in MALES ( >80% male)

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7
Q

True or False? Hereditary haemorrhagic telangiectasia is an Autosomal recessive disease.

A

FALSE!! It is Autosomal DOMINANT.

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8
Q

What is the genetic mutation associated with Hereditary haemorrhagic telangiectasia?

A

Mutation of the endothelial protein, ENDOGLIN.

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9
Q

Which test is required for screening for all Hereditary haemorrhagic telangiectasia?

A

Pulmonary arteriovenous shunts

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10
Q

What are the clinical features of Hereditary haemorrhagic telangiectasia?

A
  1. There are dilated microvascular swellings which appear during childhood and become more numerous in adult life - that develop in the skin,mucous membrane and internal organs .
  2. Pulmonary, hepatic, splenic and cerebral arteriovenous shunts are seen in a minority of cases and may need local treatment.
  3. Recurrent expixtaxis
  4. Recurrent gastrointestinal tract haemorrhage may cause chronic iron deficiency anaemia.
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11
Q

What is the treatment for Hereditary haemorrhagic telangiectasia?

A
  1. Embolization
  2. Laser Treatment
  3. Oestrogens
  4. Tranexamic acid
  5. Iron supplementation
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12
Q

Which drugs can be used in to try to reduce gastrointestinal bleeding and bleeding at other sites in severe case of Hereditary haemorrhagic telangiectasia?

A
  1. Thalidomide
  2. Lenalidomide
  3. Danazole
  4. Epsilon-aminocaproic acid
  5. Bevacizumab

(anti-vascular endothelial growth factor)

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13
Q

What are the clinical features associated with Ehlers–Danlos syndromes ?

A
  1. Hereditary Collagen abnormalities
  2. Due to defective:
    - Platelet Adhesion
    - Hyperextensibility of joints
    - Hyperelastic friable skin
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14
Q

True or False? Pseudoxanthoma elasticum is associated with venous haemorrhage and thrombocytopenia.

A

FALSE!! It is associated with ARTERIAL haemorrhage and thrombosis.

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15
Q

What is a common benign disorder which occurs in otherwise healthy women, especially those of child-bearing age?

A

Simple easy bruising

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16
Q

What is Senile purpura?

A

This is caused by atrophy of the supporting tissues of cutaneous blood vessels is seen mainly on dorsal aspects of the forearms and hands.

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17
Q

Which Acquired Vascular disorder is usually seen in children and often follows an acute upper respiratory tract infection?

A

Henoch–Schönlein syndrome

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18
Q

True or False? Henoch–Schönlein syndrome is an IgG mediated vasculitis.

A

FALSE!! It is an immunoglobulin (Ig) A-mediated vasculitis.

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19
Q

True or False? Henoch–Schönlein syndrome is usually a self-limiting condition but occasional patients develop renal failure.

A

TRUE!!

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20
Q

What are the clinical features of Henoch–Schönlein syndrome ?

A
  • The characteristic purpuric rash accompanied by localized oedema and itching is usually most prominent on the buttocks and extensor surfaces of the lower legs and elbows can also present with BULLA formation
  • Painful joint swelling
  • Haematuria
  • Abdominal pain
  • Early urticarial lesions
  • Patients can develop renal failure
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21
Q

In which pathology is there an association with defective collagen causing perifollicular petechiae, bruising and mucosal haemorrhage?

A

Vitamin C deficiency ( Scurvy)

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22
Q

In which condition is the use of Tranexamic acid and aminocaproic acid ( Antifibrinolytic drugs) contraindicated because they might lead to clots obstructing the renal tract?

A

Haematuria

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23
Q

In which condition can Steroid purpura be seen in?

A

Cushing syndrome

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24
Q

What is the most common cause of Thrombocytopenia?

A

Failure of Platelet production is the most common cause of thrombocytopenia and is usually part of a generalized bone marrow failure.

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25
Q

What is the Genetic mutation associated with Wiskott–Aldrich syndrome (WAS)?

A

WASP gene, the protein being a regulator of signalling in haemopoietic cells.

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26
Q

How is the diagnosis of Thrombocytopenia done?

A
  1. Clinical history
  2. Peripheral blood count
  3. The blood film and bone marrow examination.
27
Q

True or False? Chronic liver disease can also be associated with thrombocytopenia, either due to splenic sequestration of platelets if the liver disease is associated with splenomegaly, or due to decreased production of thrombopoeitin.

A

TRUE!!

28
Q

What is the most common cause of Thrombocytopenia WITHOUT anaemia or neutropenia?

A

Chronic idiopathic thrombocytopenic purpura (ITP)

29
Q

In ITP, what is the lifespan of platelets reduced to?

A

10 hours

30
Q

True or False? Chronic idiopathic thrombocytopenic purpura (ITP) has the highest incidence in women aged 15–50 years.

A

TRUE!!

31
Q

What is the Pathogenesis for Chronic idiopathic thrombocytopenic purpura (ITP)?

A

Platelet autoantibodies, usually IgG, result in the premature removal of platelets from the circulation by macrophages of the reticuloendothelial system, especially the spleen.

32
Q

Fill in the blanks. “ In Chronic idiopathic thrombocytopenic purpura (ITP) , the autoantibodies are usually directed against ______________.”

A

Glycoprotein (GP) IIb/IIIa or Ib complex.

33
Q

What are the clinical features of Chronic Idiopathic Thrombocytopenic purpura (ITP)?

A
  • Petechial Haemorrhage
  • Easy bruising
  • Menorrhagia
  • Intracranial haemorrhage is RARE
34
Q

What are the diseases that can be associated with Chronic Idiopathic Thrombocytopenic Purpura?

A

*Systemic lupus erythamatosus (SLE)
* Human immunodeficiency virus (HIV) infection
* Hepatitis C virus (HCV)
* Helicobacter pylori infection
* Chronic lymphocytic leukaemia (CLL)
* Hodgkin lymphoma
* Autoimmune haemolytic anaemia
* Aplastic anaemia
* Leukaemia
* Myelodysplastic syndromes
* Myelofibrosis
* Marrow infiltration (e.g. carcinoma, lymphoma, Gaucher’s disease)
* Multiple myeloma
* Megaloblastic anaemia

34
Q

How can one be diagnosed with Chronic Idiopathic Thrombocytopenic Pupura?

A

1.Platelet count is 10-100 × 109/L.

  1. Haemoglobin and white cell count are typically NORMAL unless there is iron deficiency anaemia because of blood loss.
  2. The bone marrow shows normal or increased numbers of Megakaryocytes.
  3. Sensitive tests are able to demonstrate specific anti- glycoprotein GPIIb/IIIa or GPIb antibodies on the platelet surface or in the serum in most patients.
35
Q

True or False? In Chronic Idiopathic Thrombocytopenic Porpura a platelet count above 20 × 109/L without symptoms does not require treatment.

A

TRUE!!

36
Q

What is the first line therapy for treatment of Chronic Idiopathic Thrombocytopenic Porpura?

A

High- Dose Corticosteroid therapy - Dexamethasone at 40 mg a day for 4 days and then reduced can be used, as can prednisolone 1mg/kg/day in adults, with the dosage gradually reduced after 10–14 days.

37
Q

What is the MOA for High-dose intravenous immunoglobulin therapy?

A

The mechanism of action may be blockage of Fc receptors on macrophages or modification of autoantibody production.

38
Q

What is the treatment that can be used for Chronic Idiopathic Thrombocytopenic Porpura patients in whom in whom steroids are contraindicated or who are refractory to steroids?

A

Thrombopoietin-receptor agonists - Romiplostim (sub- cutaneously), eltrombopag and avatrombopag (orally)

39
Q

What are examples of Thrombopoietin-receptor agonists?

A
  • Romiplostim (sub- cutaneously)
  • Eltrombopag and avatrombopag (orally)
40
Q

What is the name of the drug that inhibits the protein product of the SYK gene, which is involved in signal transduction in B and other cells of the immune system?

A

Fostamatinib

41
Q

In an attempt to treat Chronic Idiopathic Thrombocytopenic Porpura , a patient was given a drug which is considered to be an androgen which may cause virilization in women. Which drug could this possibly be?

A

Danazol

42
Q

What are the overall treatment options for Chronic Idiopathic Thrombocytopenic Porpura?

A
  1. Corticosteroids
  2. High-dose intravenous immunoglobulin therapy
  3. Monoclonal antibody - Rituximab (anti-CD20)
  4. Thrombopoietin-receptor agonists
  5. Splenectomy
  6. Immunosuppressive drugs
  7. Other treatments
  8. Platelet transfusions
  9. Stem cell transplantation
43
Q

True or False? Acute Idiopathic Thrompocytopenic Porpura is mostly common in CHILDREN and in approximately 75% of patients the episode follows vaccination or an infection such as chickenpox or infectious mononucleosis.

A

TRUE!!

44
Q

True or False? In Chronic ITP , there is a risk of cerebral haemorrhage and it is usually rare while in Acute ITP the main risk is of a Intracranial haemorrhage and it is usually rare also.

A

FALSE!! In Chronic ITP there is a risk of INTRACRANIAL HAEMORRHAGE but in Acute ITP the main risk is of a CEREBRAL HAEMORRHAGE and is often rare also.

45
Q

What is the main cause associated with Acute ITP?

A

Most cases are caused by non-specific immune complex attachments to platelets.

46
Q

True or False? In Acute ITP , . Most children do not have any bleeding even with platelet counts <10 × 109/L, but need to avoid trauma such as contact sports.

A

TRUE!!

47
Q

True or False? In Acute IPT , if the platelet count is over 30 × 109/L no treatment is necessary unless the bleeding is severe. Indeed, many doctors do not treat even with platelet counts <10 × 109/L if there is no haemorrhage.

A

TRUE!!

48
Q

What is the treatment options for Acute ITP?

A
  • Steroids and/or intravenous (IV) immunoglobulin
49
Q

What are particularly common causes of Drug - Induced Thrombocytopenia?

A
  • Quinine (including that in TONIC WATER) - Animalarial drug
  • Quinidine - Anti-arrhythmic drug
  • Heparin - Anticoagulant
50
Q

What are the clinical features associated with Drug- Induced thrombocytopenia?

A
  • The platelet count is often less than 10×109/L
  • Bone marrow shows normal or increased numbers of megakaryocytes.
  • Drug-dependent antibodies against platelets may be demonstrated in the sera of some patients.
51
Q

What is the immediate treatment for Drug-Induced Thrombocytopenia?

A

Stop the drug causing it duh! But platelet concentrates should be given to patients with dangerous bleeding.

52
Q

True or False? For Post- Transfusion Porpura, it occurs approximately 15 days after blood transfusion .

A

FALSE!! It occurs 10 Days after transfusion

53
Q

What is the antibody associated with Post-transfusion porpura?

A

Human platelet antigen-1a (HPA-1a) on the transfused platelets.

54
Q

What is the WHO bleeding grades?

A

Grade 0 - None

Grade 1. - Petechiae, ecchymoses, occult blood loss, mild spotting.

Grade 2. - Gross bleeding, i.e. epistaxis, haematuria, haematemesis not requiring transfusion.

Grade 3 - Haemorrhage requiring transfusion.

Grade 4 - Haemorrhage with haemodynamic compromise, retinal haemorrhage with visual impairment, CNS haemorrhage, fatal at any location.

55
Q

What is the deficiency associated with Thrombotic Thrombocytopenic purpura (TTP)?

A

There is deficiency of the ADAMTS13 metalloprotease which breaks down ultra-large von Willebrand factor multimers (ULVWF)

56
Q

Where is Ultra-large von Willebrand factor multimers (ULVWF) secreted from?

A

Weibel–Palade bodies

57
Q

What is the “ Pentad” of TTP?

A

Microangiopathic Haemolytic Anaemia
Thrombocytopenia
Fever
Renal Failure
Neurological Symptoms

58
Q

What are the clinical features associated with TTP?

A
  • Tissue ischaemia
  • Thrombocytopenia
  • Schistocytes
  • LDH increase
  • ADAMTS13 is absent or severely reduced in plasma
  • An anti-ADAMTS13 antibody is present
59
Q

What is the treatment of TTP?

A
  • Fresh Frozen Plasm or Cryosupernatant
  • Rituximab ( CD- 20)
  • Caplacizumab, an anti-VWF antibody that inhibits binding of platelets to ultra-large VWF multimers, promotes faster resolution of acute TTP.
60
Q

What are the infections associated with Haemolytic Uraemia Syndrome (HUS) ?

A

*Escherichia coli infection with the verotoxin 0157 strain or with other organisms, especially Shigella

61
Q

What is the mainstay treatment for HUS?

A

Renal dialysis and Control of Hypertension

62
Q
A

Clinical Haematology (26 decks)

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