sickle cell disease Flashcards
what would you find in the blood film of sickle cell disease ?
sickle cells
what would you find in the blood film in HbSc
target cells
which test will reveal the genotype of the sickle cell ?
Hb electrophoresis or HPLC
what are the clinical syndromes associated with sickle cell disease ?
vaso-occlusive crisis aplastic crisis sequestrian crisis hemolytic crisis infections
what are aplastic crisis commonly associated with ?
parvovirus B19
what is the most common acute presentation of sickle cell anemia ?
acute painful episodes
what are the precipitating factors of sickle pain ?
infection dehydration cold damp conditions unaccustomed exercise stress
what are the presentations of vaso-occlusive crises ?
dactylitis girdle syndrome chest syndrome avascular necrosis of the hip priapism
what is girdle syndrome ?
pain in the lower abdomen, localized ileus due to sickling in the bowel
what is the drug management of acute sickle pain ?
opioid analgesics such as paracetamol
diamorphine
morphine
oxycodone
what is acute chest syndrome ?
acute illness that may progress to ARDS and is characterized by : fever respiratory symptoms a new pulmonary infiltrate ( hypoxia not included )
what are the causes of ACS ?
pulmonary infection
fat embolism
pulmonary infarction
hypoventilation
what are the clinical features of ACS?
onset usually within 24-72 hours of severe crisis fever shortness of breath bilateral crackles pulmonary infiltrates ( alveolar consolidation of the lower lobe )
what are the complications of ACS?
neurological complications
multi organ failure
what is the treatment of ACS ?
emergency blood transfusion IV fluids IV antibiotics effective pain relief. respiratory support
what is priapism ?
painful resistant erection due to vaso-occlusive obstruction of the venous drainage of the penis
when does sequestrian crisis happen ?
young children under the age of 5
old people with HbSc
what is a sequestrian crisis ?
pooling of large volumes of blood in the spleen or the liver
what are the ned results of sequestrian crisis ?
hypotension and hypovolemic shock
what are infections in sickle cell disease a result of ?
hyposplenism
how do we manage acute complications ?
hawi 3al 3ayann
oxygen fluids analgesia
transfusion if very anemic
DVT prophylactic
why may a new born with sickle cell appear normal or well ?
dur to high levles of HbF
what is the pattern of inheritance associated with sickle cell disease ?
autosomal reecessive
what is the difference between HbSS and HbSC ?
HbSS - is the most severe from of the disease
HbSCC - less severe but retinopathy and avascular necrosis are common associated problems
what disease are patients with sickle cell resistant to ?
malaria
what is present in blood film of HbSC ?
target cells
what are thee investigations needed to make a diagnosis of sickle cell anemia ?
gel electrophoresis
high performance liquid chromatography
sickle cell solubility
what is the prognosis of aplastic crisis ?
resolves spontaneously
what type of infections are associated with hyposplenism ?
encapsulated organsims
