A 67-year-old woman is referred to the haematology clinic. Her GP has noted that her platelet count is persistently elevated and no reactive cause can be found. Bloods taken a week before clinic are as follows:
What is the treatment of choice?
Correct Answer C:
Thrombocytosis:
Thrombocytosis is an abnormally high platelet count, usually > 400 * 109/l.
Causes of thrombocytosis:
Essential thrombocytosis:
Essential thrombocytosis is one of the myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis. Megakaryocyte proliferation results in an overproduction of platelets.
Features:
Management:
A 74-year-old woman with a past history of chronic lymphocytic leukaemia presents with lethargy. The following blood results are obtained:
What complication has most likely occurred?
Correct Answer D: Warm autoimmune haemolytic anaemia occurs in around 10-15% of patients with chronic lymphocytic leukaemia.
Chronic lymphocytic leukaemia:
Chronic lymphocytic leukaemia (CLL) is caused by a monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells (99%).
Complications:
Investigations:
A 31-year-old man is referred to the acute medical unit with a painful swollen left leg. The patient reports that he has the 'Factor V Leiden mutation'.
Which one of the following best describes the pathophysiology of his condition?
Correct Answer D: Factor V Leiden mutation results in activated protein C resistance.
Activated protein C resistance: Activated protein C resistance is the most common inherited thrombophilia. It is due to a mutation in the Factor V Leiden mutation.
Heterozygotes have a 5-fold risk of venous thrombosis whilst homozygotes have a 50-fold increased risk.
Which of the following is a cause of intravascular haemolysis?
Haemolytic anaemias: by site: In intravascular haemolysis free haemoglobin is released which binds to haptoglobin. As haptoglobin becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by Schumm's test). Free haemoglobin is excreted in the urine as haemoglobinuria, haemosiderinuria.
Intravascular haemolysis: causes:
Extravascular haemolysis: causes:
*strictly speaking there is an element of extravascular haemolysis in G6PD as well, although it is usually classified as a intravascular cause.
A 27-year-old male is receiving cyclophosphamide as part of his chemotherapy for non-Hodgkin' lymphoma.
What is the most appropriate management to reduce the likelihood of haemorrhagic cystitis?
Correct Answer E: Cyclophosphamide - haemorrhagic cystitis - prevent with mesna.
Cyclophosphamide may be converted to urotoxic metabolites such as acrolein. Mesna binds to these metabolites through its sulfhydryl-moieties and reduces the incidence of haemorrhagic cystitis.
Cyclophosphamide: Cyclophosphamide is an alkylating agent used in the management of cancer and autoimmune conditions. It works by causing cross-linking of DNA.
Adverse effects:
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