Haem Flashcards
Q: How is APTT, PT, and Bleeding Time altered in Hemophilia?
A: Hemophilia: APTT is increased, PT is normal, and Bleeding Time is normal.
Q: What is the result of APTT, PT, and Bleeding Time in von Willebrand’s disease?
A: von Willebrand’s disease: APTT is increased, PT is normal, and Bleeding Time is increased.
Q: How does Vitamin K deficiency affect blood clotting tests?
A: Vitamin K deficiency: APTT is increased, PT is increased, and Bleeding Time is normal.
Q: What is the cause of Acute Intermittent Porphyria (AIP)?
A: AIP is caused by a defect in porphobilinogen deaminase, an enzyme involved in the biosynthesis of haem.
Q: What is the result of the defect in porphobilinogen deaminase in AIP?
A: The defect leads to the toxic accumulation of delta-aminolaevulinic acid and porphobilinogen.
Q: What are the classical symptoms of Acute Intermittent Porphyria (AIP)?
Abdominal symptoms: Abdominal pain, vomiting
Neurological symptoms: Motor neuropathy
Psychiatric symptoms: E.g., depression
Other common signs: Hypertension and tachycardia
Q: How can Acute Intermittent Porphyria (AIP) be diagnosed?
Urine turns deep red on standing
Raised urinary porphobilinogen (elevated between attacks and more during acute attacks)
Assay of red cells for porphobilinogen deaminase
Raised serum levels of delta-aminolaevulinic acid and porphobilinogen
Q: What is the management approach for Acute Intermittent Porphyria (AIP)?
Avoiding triggers
Acute attacks:
IV haematin or haem arginate
IV glucose (if haematin/haem arginate is not immediately available)
Q: What is the most common form of acute leukaemia in adults?
A: Acute Myeloid Leukaemia (AML) is the more common form of acute leukaemia in adults.
Q: What are the typical features of Acute Myeloid Leukaemia (AML)?
Anaemia: Pallor, lethargy, weakness
Neutropenia: High white cell count but low functioning neutrophil levels, leading to frequent infections
Thrombocytopenia: Bleeding
Splenomegaly
Bone pain
Q: What are the poor prognostic features of Acute Myeloid Leukaemia (AML)?
Age > 60 years
20% blasts after the first course of chemotherapy
Cytogenetic abnormalities: Deletions of chromosomes 5 or 7
Q: What is the specific association for Acute Promyelocytic Leukaemia (M3)?
A: Acute Promyelocytic Leukaemia (M3) is associated with the t(15;17) translocation and fusion of PML and RAR-alpha genes.
Q: At what age does Acute Promyelocytic Leukaemia (M3) typically present?
A: It typically presents in younger patients, with an average age of 25 years.
Q: What are the characteristic features of Acute Promyelocytic Leukaemia (M3)?
Auer rods (seen with myeloperoxidase stain)
DIC or thrombocytopenia often at presentation
Good prognosis
Q: What is Antiphospholipid Syndrome?
A: Antiphospholipid Syndrome is an acquired disorder characterized by a predisposition to both venous and arterial thromboses, recurrent fetal loss, and thrombocytopenia.
Q: What pregnancy complications are associated with Antiphospholipid Syndrome?
Recurrent miscarriage
Intrauterine growth restriction (IUGR)
Pre-eclampsia
Placental abruption
Pre-term delivery
Venous thromboembolism
Q: What is the management for Antiphospholipid Syndrome in pregnancy?
Low-dose aspirin should be started once pregnancy is confirmed on urine testing
Low molecular weight heparin should be started once a fetal heart is seen on ultrasound, typically discontinued at 34 weeks gestation
These interventions increase the live birth rate seven-fold
Q: What is Aplastic Anaemia characterized by?
A: Aplastic anaemia is characterized by pancytopenia and a hypoplastic bone marrow.
Q: What are the typical features of Aplastic Anaemia?
Normochromic, normocytic anaemia
Leukopenia (with lymphocytes relatively spared)
Thrombocytopenia
May present as acute lymphoblastic or myeloid leukaemia
A minority of patients may later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia
Q: What are the causes of Aplastic Anaemia?
Idiopathic
Congenital: Fanconi anaemia, dyskeratosis congenita
Drugs: Cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
Toxins: Benzene
Infections: Parvovirus, hepatitis
Radiation
Q: What are the two types of Autoimmune Haemolytic Anaemia (AIHA)?
A: AIHA can be divided into ‘warm’ and ‘cold’ types, based on the temperature at which the antibodies cause haemolysis.
Q: What is the most common cause of Autoimmune Haemolytic Anaemia (AIHA)?
A: AIHA is most commonly idiopathic but can also be secondary to a lymphoproliferative disorder, infection, or drugs.
Q: What are the general features of haemolytic anaemia?
Anaemia
Reticulocytosis
Low haptoglobin
Raised lactate dehydrogenase (LDH) and indirect bilirubin
Blood film showing spherocytes and reticulocytes
Q: What is a specific feature of Autoimmune Haemolytic Anaemia (AIHA) on investigations?
A: A positive direct antiglobulin test (Coombs’ test) is a specific feature of AIHA.