notes from quesmed 2 Flashcards
sickle cell disease inheritance pattern
autosomal recessive
proteins found in multiple myeloma
M proteins in serum protein electrophoresis
Bence Jones detectable in urine via electrophoresis
bloods show what in DIC
low platelets
low clotting factors due to consumption of clotting factors
decreased fibrinogen
prolonged APTT
prolonged PT
increased fibrinogen degradation products (increased D dimer)
blood film may show shistocytes
fever 7-14 days after chemotherapy think
suspected neutropenic sepsis
IV piperacillin with tazobactam or specific empirical broad spectrum antibiotic choice on local guidelines
can also occur in patients with low neutrophil count eg haematological conditions
thrombocytopenia presentation
50% of patients are asymptomatic, with only an incidental FBC finding
Thrombosis (arterial or venous)
Bleeding (gastrointestinal or intracranial)
Hyperviscosity-related - dizziness/syncope, headache
Splenomegaly
Hyposplenism (caused by multiple splenic infarcts)
Erythromelalgia (a red/blue discolouration of the extremities, often accompanied by a burning pain)
Livedo reticularis (a net-like purple rash)
raised platelet count
JAK2 V617F in 50-60% patients
most common in women aged 50–70 years.
what kind of anaemia is caused by thalassemia
microcytic anaemia
what is methotrexate used for
ALL
types of vWF
Type 1 VWD: Partial quantitative deficiency in VWF
Type 2 VWD: Qualitative defects in VWF (e.g. decreased adhesion to platelets or factor VIII)
Type 3 VWD: Almost complete deficiency of VWF
common complication of polycythaemia and thrombocythaemia
Thrombosis
clinical signs of polycythaemia
Ruddy complexion (red face)
Conjunctival plethora (the opposite of conjunctival pallor)
Splenomegaly
Hypertension
management of primary myelofibrosis
nothing for mild
supportive for complications
chemo to help control (hydroxycarbamide)
JAK2 inhibitors
allogeneic stem cell transplant
essential thrombocythaemia
aspirin to reduce risk of thrombus formation
chemotherapy
anagrelide (specialist platelet lowering agent)
what are cabot rings in
megaloblastic anaemia
methylmanoic acid checks what
functional B12 status
tumour lysis syndrome
results from chemicals released when cells are destroyed by chemotherapy, resulting in:
High uric acid
High potassium (hyperkalaemia)
High phosphate
Low calcium (as a result of high phosphate)
can cause acute kidney injury due to uric acid forming in interstitial space and tubules of kidney
arrhythmias
systemic inflammation
CLL
one big selfish dictator making clones of himself usually B lymphocytes
often asymptomatic but can present with infections, anaemia, bleeding, weight loss
WARM haemolytic anaemia
richters transformation to high grade B cell lymphoma (non hodgkins)
smear or smudge cells ruptured white cells
CML
lots of different horrible zombies of diff types eg basophils, neutrophils
Cphiladelphia 9-22 creates abnormal gene sequence called BCR-ABL1
treat with -IBs
3 phases- chronic, accelerated, blast
how does G6PD deficiency cause haemolytic anaemia
enzyme is responsible for helping protect cells from damage by reactive oxygen species (ROS)
ROS are reactive molecules that contain oxygen, produced during normal cell metabolism and in higher quantities during stress on the cell.
The G6PD enzyme is particularly important in red blood cells. A deficiency in G6PD makes cells more vulnerable to ROS, leading to haemolysis in red blood cells. Periods of increased stress, with a higher production of ROS, can lead to acute haemolytic anaemia.
AML
can present at any age, normally middle aged onward
can be transformation from myeloproliferative disorder
blood film and bone marrow biopsy show high proportion of blast cells
auer rods in cytoplasm of blast cells
G6PD deficiency
defect in G6PD enzyme
more common in Mediterranean, Middle Eastern and African patients
x linked recessive
crises that are triggered by infections, medications or fava beans (broad beans)
neonatal jaundice
anaemia
intermittent jaundice
gallstones
splenomegaly
heinz bodies
medications that trigger haemolysis in G6PD deficiency
Primaquine (an antimalarial)
Ciprofloxacin
Nitrofurantoin
Trimethoprim
Sulfonylureas (e.g gliclazide)
Sulfasalazine and other sulphonamide drugs
Non-immune causes of intravascular haemolysis
microangiopathic haemolysis
G6PD deficiency
malaria
features of intravascular haemolytic anaemia
new onset anaemia,
jaundice,
haemoglobinuria,
absent organomegaly
warm autoimmune haemolytic anaemia
IgG mediated extravascular haemolytic disease
caused by SLE, idiopathic, lymphoproliferative neoplasms, drugs
managed with prednisolone or immunosuppression or transfusions if severe
cold Autoimmune haemolytic anaemia
IgM mediated haemolytic disease
caused by post infection (mycoplasma or EBV), idiopathic, lymphoproliferative disorders
treatment mostly supportive, warmed blood is transfused
normal adult haemoglobin is made of
2 alpha, 2 beta is around 97% of people
2 alpha, 2 delta is around 1.5-3% of people
δ2β2 and β2γ2 are not found in a healthy adult.The α chains are essential. alpha 0 is incompatible with life
what component of blood doesnt have to be cross matched
platelets
what is universal donor
O
can only receive from O
what is universal recipient
AB
can only give to AB
what can B receive blood from
B and O
antibodies against A
can give to B and AB
what can A receive from
A and O
antibodies against B
can give to A and AB
what does positive blood type mean
your blood cells have the Rh protein
HbS HbS
severe form of sickle cell
genotype for homozygous sickle cell trait
HbA HbA
represents homozygous adult (healthy) haemoglobin and would not produce sickle cell disease.
HbA HbS
group of disorders that affects haemoglobin to form abnormal haemoglobin molecules (HbS)
likely to have the less severe, heterozygous form of sickle cell disease
HbF HbA
HbF represents foetal haemoglobin, which is typically removed by six months of age
