sickle cell disease Flashcards
sequestration crisis
Sequestration crisis occurs due to pooling of blood in the spleen as the spleen atrophies.
aneamia- Hb
shock
aplastic crisis
Aplastic crisis causes a sudden reduction in marrow production and so a drop in haemoglobin and red blood cell production.
parvovirus B19 infection
acute chest syndrome
Acute chest syndrome presents with pain in the chest, fever, tachypnoea, wheeze and cough. The child in the scenario only has pain and he does not appear to have any respiratory symptoms such as wheeze or cough, or have a fever. Acute chest syndrome is caused by pulmonary infiltrates from a fat embolism or an infection
vasocollusive crisis
acute onset of the pain and with no abnormalities on examination and no significant drop in haemoglobin make a vaso-occlusive or “painful” crisis the most likely explanation.
Common triggers include the cold dehydration, infection and hypoxia.
Vaso-occlusive crises may present with pain anywhere in the body and in toddlers under 3 years, hands and feet are commonly affected. Vaso-occlusive crises are caused by micro-occlusion of blood vessels by sickled red blood cells
A 2 year old boy is brought to the Accident and Emergency department by his parents in pain. A couple of hours ago, whilst out for a walk with his parents, he began crying excessively saying “it hurts!”. It is a particularly cold day outside. There was no history of trauma.
He was diagnosed with sickle cell disease at birth. He has otherwise been well recently, as has everyone else in his household.
Examination reveals no abnormalities. His observations are stable.
Investigations show that he has a haemoglobin (Hb of 90g/L), with measurements 3 months ago being recorded as 88g/L and 6 months ago as 93g/L.
What type of sickle cell “crisis” is this child most likely experiencing?
vaso-occlusive
This patient has features consistent with essential thrombocythaemia. The stroke, venous thrombosis and bleeding are due to dysfunctional platelets and erythromelalgia. tx?
The treatment for this is hydroxycarbamide to reduce the platelet count
Which of the following transfusable products has the shortest shelf-life?
platelets
room temp fro 7 days
lifespan of 10day
Red blood cells
Generally stored for 35 days at 4℃. In vivo, red blood cells typically have a lifespan of about 120 days, however it is important to note that transfused red cells last around 50-60 days.
Fresh frozen plasma (FFP)
Stored at -30℃ with a shelf life of a year. It is used in patients with coagulopathy (impaired blood coagulation) by replacing clotting factors. The type of FFP used depends on the patient’s blood group.
Platelets
Stored at room temperature for 7 days. They have a lifespan of 10 days before they are phagocytosed in the spleen and liver. 1 pool of platelets comes from 4 donors.
Cryoprecipitate
Cryoprecipitate is from FFP thawed overnight at 4-8℃. Shelf life is 1 year stored at -30 degrees. It contains fibrinogen, factor VIII and von Willebrand factor. It is generally used in patients with massive bleeding and low fibrinogen.
Which of the following transfusable products has the shortest shelf-life?
platelets
room temp fro 7 days
lifespan of 10day
Red blood cells
Generally stored for 35 days at 4℃. In vivo, red blood cells typically have a lifespan of about 120 days, however it is important to note that transfused red cells last around 50-60 days.
Fresh frozen plasma (FFP)
Stored at -30℃ with a shelf life of a year. It is used in patients with coagulopathy (impaired blood coagulation) by replacing clotting factors. The type of FFP used depends on the patient’s blood group.
Platelets
Stored at room temperature for 7 days. They have a lifespan of 10 days before they are phagocytosed in the spleen and liver. 1 pool of platelets comes from 4 donors.
Cryoprecipitate
Cryoprecipitate is from FFP thawed overnight at 4-8℃. Shelf life is 1 year stored at -30 degrees. It contains fibrinogen, factor VIII and von Willebrand factor. It is generally used in patients with massive bleeding and low fibrinogen.
