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Clinical Haematology > Autoimmune Haemolytic Anaemia > Flashcards

Autoimmune Haemolytic Anaemia Flashcards

1
Q

True or False ?Autoimmune haemolytic anaemias (AIHAs) are characterized by a positive direct antiglobulin test (DAT), also known as the Coombs’ test .

A

TRUE!!

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2
Q

Fill in the blanks. “ In warm AIHA , red cells are coated by ________ antibody.”

A

IgG

” it is Warm in Georgia”

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3
Q

True or False? In warm AIHA , it can be coated with IgG alone or together with complement.

A

TRUE!!

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4
Q

What are the clinical features of Warm AIHA?

A
  • Enlarged spleen
  • Often associated with lymphoid malignancies infection and auto-immune diseases
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5
Q

Fill in the blanks. “ When Warm AIHA is associated with idiopathic thrombocytopenic purpura (ITP), it is called ___________.”

A

Evan’s syndrome

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6
Q

Fill in the blanks. “ When Warm AIHA is secondary to SLE , the cells are typically coated with _________ &_________.”

A

Immunoglobulin and Complement

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7
Q

At what temperature , is Warm AIHA detected at?

A

37 degrees celsius

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8
Q

True or False? In Warm AIHA, The DAT is positive as a result of IgG, IgG and complement or IgA on the cells and, in some cases, the autoantibody shows specificity within the Rh system.

A

TRUE!!

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9
Q

What is the most prominent feature in Warm AIHA?

A

Spherocytosis in peripheral blood ( typical extravascular haemolysis)

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10
Q

What are the treatment options for Warm AIHA?

A
  1. Treat underlying cause
  2. Corticosteroids - Prednisolone is the usual first-line treatment. - Antacid therapy & vitamin D and calcium supplements and possibly with oral bisphosphonate for patients on steroid therapy for over 3 months and over 40 years.
  3. Monoclonal antibody - CD20 ( Rituximab)

4.Splenectomy

5.Immunosuppression - Azathioprine, bortezomib, cyclophosphamide, chlorambucil, ciclosporin and mycophenolate mofetil have been tried with varying success.

  1. Folic acid

7.Blood Transfusions

  1. High dose Immunoglobulin
  2. Venous thrombosis prophylaxis
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11
Q

What is the autoantibody present in Cold AIHA?

A

IgM

” It is Cold in the Mountains”

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12
Q

What are the different types of autoantibodies that are presented in Cold AIHA?

A
  • Monoclonal - as in Primary Haemagglutinin syndrome or associated with lymphoproliferative diseases.
  • Polyclonal - after association with infections ex Infectious mononucleosis or Mycoplasma pneumonia
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13
Q

At temperature do the IgM antibodies is Cold AIHA bind to red cells?

A

4°C

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14
Q

True or False? Both complement & IgM factors can be detected on red cells in laboratory tests to determine Cold AIHA.

A

FALSE!! ONLY COMPLEMENT factors can be detected on red cells in laboratory tests, as the IgM antibody is eluted off as cells flow through warmer parts of the circulation.

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15
Q

Fill in the blanks. “ In nearly all these cold AIHA syndromes the antibody is directed against the _____________ on the red cell surface while in infectious mononucleosis it is _________.”

A

In nearly all these cold AIHA syndromes the antibody is directed against the ‘I’ ANTIGEN on the red cell surface. In infectious mononucleosis it is ANTI- I

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16
Q

True or False? In Primary cold agglutinin disease the patient has a chronic haemolytic anaemia aggravated by the cold and often associated with extravascular haemolysis.

A

FALSE!! The patient has a chronic haemolytic anaemia aggravated by the cold and often associated with INTRAVASCULAR HAEMOLYSIS.

17
Q

What are the clinical features of Primary Cold Agglutinin disease?

A
  • Mild jaundice
  • Splenomegaly
  • Acrocyanosis ( purplish skin discoloration) at the tip of the nose, ears, fingers and toes caused by the agglutination of red cells in small vessels.)
18
Q

True or False? In Primary Cold Agglutinin disease ,the DAT reveals COMPLEMENT (C3d) ONLY on the red cell surface.

A

TRUE!!

19
Q

What is the treatment for patients with Primary Cold Agglutinin disease?

A
  1. Keep the patient warm.
  2. Plasmapheresis may be needed initially to treat hyperviscosity.
  3. Rituximab is the best first-line therapy.
  4. Bortezomib-based therapy is also used
  5. Alkylating drugs
20
Q

True or False? In Paroxysmal Cold haemoglobinuria is a rare syndrome of acute INTRAVASCULAR haemolysis after exposure to the cold.

A

TRUE!!

21
Q

What is the Antibody associated with Paroxysmal Cold Haemoglobinuria?

A

Donath–Landsteiner antibody , an IgG antibody with specificity for the P blood group antigens.

22
Q

What are the three mechanisms associated with Drug- induced haemolytic anaemia?

A

1 Antibody directed against a drug–red cell membrane complex (e.g. penicillin, ampicillin); this only occurs with massive doses of the antibiotic.

2 Deposition of complement via a drug–protein (antigen)– antibody complex onto the red cell surface (e.g. quinidine, rifampicin).

3 A true autoimmune haemolytic anaemia in which the role of the drug is unclear (e.g., fludarabine).
In each case, the haemolytic anaemia gradually disappears when the drug is discontinued.

23
Q

What is March Haemoglobinuria?

A

This is caused by damage to red cells between the small bones of the feet, usually during prolonged marching or running. The blood film does not show fragments.

24
Q

What are the main causative agents of a microangiopathic haemolytic anaemia?

A

Meningococcal or pneumococcal septicaemia

25
Q

True or False? Malaria causes haemolysis by extravascular destruction of parasitized red cells as well as by direct intravascular lysis.

A

TRUE!!

26
Q

What is the causative agent of Blackwater fever?

A

Falciparum malaria

27
Q

True or False? Blackwater fever is an acute extravascular haemolysis accompanied by acute renal failure.

A

FALSE!! Blackwater fever is an acute INTRAVASCULAR haemolysis accompanied by acute renal failure

28
Q

True or False? Clostridium perfringens septicaemia can cause INTRAVASCULAR haemolysis with marked microspherocytosis

A

TRUE!!

29
Q

In which pathology can there be a case of acute haemolytic anaemia as a result of high levels of copper in the blood?

A

Wilson’s disease

30
Q
A

Clinical Haematology (26 decks)

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