Autoimmune Haemolytic Anaemia Flashcards
True or False ?Autoimmune haemolytic anaemias (AIHAs) are characterized by a positive direct antiglobulin test (DAT), also known as the Coombs’ test .
TRUE!!
Fill in the blanks. “ In warm AIHA , red cells are coated by ________ antibody.”
IgG
” it is Warm in Georgia”
True or False? In warm AIHA , it can be coated with IgG alone or together with complement.
TRUE!!
What are the clinical features of Warm AIHA?
- Enlarged spleen
- Often associated with lymphoid malignancies infection and auto-immune diseases
Fill in the blanks. “ When Warm AIHA is associated with idiopathic thrombocytopenic purpura (ITP), it is called ___________.”
Evan’s syndrome
Fill in the blanks. “ When Warm AIHA is secondary to SLE , the cells are typically coated with _________ &_________.”
Immunoglobulin and Complement
At what temperature , is Warm AIHA detected at?
37 degrees celsius
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.
TRUE!!
What is the most prominent feature in Warm AIHA?
Spherocytosis in peripheral blood ( typical extravascular haemolysis)
What are the treatment options for Warm AIHA?
- Treat underlying cause
- 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.
- Monoclonal antibody - CD20 ( Rituximab)
4.Splenectomy
5.Immunosuppression - Azathioprine, bortezomib, cyclophosphamide, chlorambucil, ciclosporin and mycophenolate mofetil have been tried with varying success.
- Folic acid
7.Blood Transfusions
- High dose Immunoglobulin
- Venous thrombosis prophylaxis
What is the autoantibody present in Cold AIHA?
IgM
” It is Cold in the Mountains”
What are the different types of autoantibodies that are presented in Cold AIHA?
- Monoclonal - as in Primary Haemagglutinin syndrome or associated with lymphoproliferative diseases.
- Polyclonal - after association with infections ex Infectious mononucleosis or Mycoplasma pneumonia
At temperature do the IgM antibodies is Cold AIHA bind to red cells?
4°C
True or False? Both complement & IgM factors can be detected on red cells in laboratory tests to determine Cold AIHA.
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.
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 _________.”
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
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.
FALSE!! The patient has a chronic haemolytic anaemia aggravated by the cold and often associated with INTRAVASCULAR HAEMOLYSIS.
What are the clinical features of Primary Cold Agglutinin disease?
- 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.)
True or False? In Primary Cold Agglutinin disease ,the DAT reveals COMPLEMENT (C3d) ONLY on the red cell surface.
TRUE!!
What is the treatment for patients with Primary Cold Agglutinin disease?
- Keep the patient warm.
- Plasmapheresis may be needed initially to treat hyperviscosity.
- Rituximab is the best first-line therapy.
- Bortezomib-based therapy is also used
- Alkylating drugs
True or False? In Paroxysmal Cold haemoglobinuria is a rare syndrome of acute INTRAVASCULAR haemolysis after exposure to the cold.
TRUE!!
What is the Antibody associated with Paroxysmal Cold Haemoglobinuria?
Donath–Landsteiner antibody , an IgG antibody with specificity for the P blood group antigens.
What are the three mechanisms associated with Drug- induced haemolytic anaemia?
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.
What is March Haemoglobinuria?
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.
What are the main causative agents of a microangiopathic haemolytic anaemia?
Meningococcal or pneumococcal septicaemia
True or False? Malaria causes haemolysis by extravascular destruction of parasitized red cells as well as by direct intravascular lysis.
TRUE!!
What is the causative agent of Blackwater fever?
Falciparum malaria
True or False? Blackwater fever is an acute extravascular haemolysis accompanied by acute renal failure.
FALSE!! Blackwater fever is an acute INTRAVASCULAR haemolysis accompanied by acute renal failure
True or False? Clostridium perfringens septicaemia can cause INTRAVASCULAR haemolysis with marked microspherocytosis
TRUE!!
In which pathology can there be a case of acute haemolytic anaemia as a result of high levels of copper in the blood?
Wilson’s disease
