A 31-year-old woman who is 25-weeks pregnant is brought to the Emergency Department by her husband. Over the past two days she has become increasingly confused. Her temperature is 37.8ºC and blood pressure is 104/62 mmHg. Blood tests show:
What is the most appropriate treatment?
Correct Answer E: TTP - plasma exchange is first-line.
This patient has thrombotic thrombocytopenic purpura, a condition associated with pregnancy.
Thrombotic thrombocytopenic purpura: management: Pathogenesis of thrombotic thrombocytopenic purpura (TTP):
Management:
A 54-year-old woman presents to the Emergency Department with a five day history of back pain. Her past medical history includes breast cancer and osteoarthritis. The back pain is located in the lower thoracic region and is made worse by coughing and sneezing. There has been no change in bowel habit or urinary symptoms.
On examination there is diffuse tenderness in the lower thoracic region. Peri-anal sensation is normal and lower limb reflexes are brisk.
Which one of the following is the most appropriate management plan?
Correct Answer E: This woman has spinal cord compression until proven otherwise and should have urgent assessment.
Recent NICE guidelines suggest contacting the local metastatic spinal cord compression coordinator in this situation. This should hopefully prevent delays in treatment by ensuring the patient is admitted to the most appropriate place.
Spinal cord compression: Spinal cord compression is an oncological emergency and affects up to 5% of cancer patients. Extradural compression accounts for the majority of cases, usually due to vertebral body metastases. It is more common in patients with lung, breast and prostate cancer.
Features:
What is the most common inherited bleeding disorder?
Correct Answer E:
Von Willebrand's disease: Von Willebrand's disease is the most common inherited bleeding disorder. The majority of cases are inherited in an autosomal dominant fashion* and characteristically behaves like a platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare.
Role of von Willebrand factor:
Types:
Investigation:
*Type 3 von Willebrand's disease (most severe form) is inherited as an autosomal recessive trait. Around 80% of patients have type 1 disease.
Which one of the following is not an indication for treatment in chronic lymphocytic leukaemia?
Correct Answer D: The absolute lymphocyte count is not an indication for treatment, rather than the rate of increase .
Chronic lymphocytic leukaemia: management:
Indications for treatment:
A 45-year-old man known to have haemochromatosis attends for blood test to assess when he next needs venesection. Of the options given, which one of the following blood tests should be used to assess the adequacy of venesection?
Correct Answer A: The British Committee for Standards in Haematology recommend 'transferrin saturation should be kept below 50% and the serum ferritin concentration below 50 ug/l'.
Haemochromatosis: investigation: Haemochromatosis is an autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation. It is caused by inheritance of mutations in the HFE gene on both copies of chromosome 6*. The British Committee for Standards in Haematology (BCSH) published guidelines for the investigation and management of haemochromatosis in 2000 There is continued debate about the best investigation to screen for haemochromatosis.
The 2000 BCSH guidelines suggest:
Diagnostic tests:
Typical iron study profile in patient with haemochromatosis:
Monitoring adequacy of venesection:
Joint x-rays characteristically show chondrocalcinosis.
*There are rare cases of families with classic features of genetic haemochromatosis but no mutation in the HFE gene.
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