zero to finals Flashcards
fibrinogen is a
clotting factor
when clotting factors are removed from the blood what is left
serum
reticulocytes originate from what stem cells
myeloid
how many months to red cell survive for
4
lifespan of platelets
around 10 days
target cells are mostly seen in
iron deficiency anaemia and post splenectomy
what refers to a variation in the size of the red blood cells
Anisocytosis
what are Heinz bodies
individuals blobs (inclusions) seen inside red blood cells
the inclusions inside Heinz bodies ar
denatured haemoglobin
Heinz bodies are mostly seen in
G6PD deficiency and alpha thalassaemia
Howell jolly bodies are blobs of what seen inside red blood cells
DNA material
why may see Howell jolly bodies
splenectomy, non functioning spleen (caused by sickle anaemia ) or in severe anaemia
Reticulocytes still have what material
RNA
RNA in reticulocytes have a what appearance
mesh like
normal to for about what % of red blood cells to be reticulocytes
1%
This percentage of reticulocytes goes up where there is a rapid turnover of red blood cells, such as with —, where the bone marrow is actively trying to replace lost cells.
haemolytic anaemia
what are fragments of red blood cells
Schistocytes
Schistocytes can also been seen in what replacements
metabolic heart valves
3 main causes of Microangiopathic haemolytic anaemia causing schistocytes
Haemolytic uraemia syndrome (HUS)
Disseminated intravascular coagulation (DIC)
Thrombotic thrombocytopenia purpura (TTP)
what are immature red blood cells with a nucleus surrounded by iron blobs
sideroblasts
sideroblastic anaemia occurs when
bone marrow cannot incorporate iron into the haemoglobin molecules
smudge cells are
ruptured white blood cells
smudge cells are particularly associated with what
CLL
what are spherocyets
sphere shaped red blood cells
spherocytes can indicate
autoimmune haemolytic anaemia or heredity spherocytosis
MCV in men and woman is
80-100 femtolitres
mnemonic for remembering the cause of microcytic anaemia is
TAILS
T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia
Treatment for anaemia of chronic disease
erythropoietin
what are the 3A’s and 2H’s for normocytic anaemia
A – Acute blood loss
A – Anaemia of chronic disease
A – Aplastic anaemia
H – Haemolytic anaemia
H – Hypothyroidism
macrocytic anaemia can be – or –
megaloblastic or normobalstic
megaloblastic macrocytic anaemia results from impaired
DNA synthesis
type of macrocytic megaloblastic anaemia
B12/ folate deficiency
Normoblastic macrocytic anaemia can be caused by
Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs, such as azathioprine
when might reticulocytosis occur
haemolytic anaemia or blood loss
symptoms specific too iron deficiency anaemia
Pica (dietary cravings for abnormal things, such as dirt or soil)
Hair loss
tachycardia or bradycardia is a sign of anaemia
tachycardia and raised red rate
what is atrophic glossitis
smooth tongue due to atrophy of the papillae
jaundice can indicate what kind of anaemia
haemolytic
bone deformities can indicate
thalassaemia
FBC measures
haemoglobin and MCV
LFTS are raised in
haemolysis
might look for what antibodies in pernicious anaemia
Intrinsic factor
what are indicated in unexplained iron defieicney anaemia to exclude GI cancer
colonoscopy and oesophagogastroduodenoscopy (OGD)
what is indicated for unexplained anaemia
bone marrow biopsy
iron deficiency causes what anaemia
microcytic hypochromic
other causes of iron deficiency anaemia
pregnancy due to increased requirements or peptic ulcer
most common cause of iron deficiency anaemia is
blood loss eg menorrhgia
what state of iron is soluble
Ferrous - Fe2+
what medications can interfere with iron absorption
PPIs eg omeprazole
what is also an acute phase protein released with inflammation, infection or cancer
ferritin
does normal ferritin exclude iron deficiency anaemia
no
what is not a very useful measure as it varies throughout the day
serum iron
what increases with iron deficiency anaemia
Total iron binding capacity and transferrin
what indicates the total iron in the body
transferrin saturation
what should do before taking a transferrin saturation
fast as it can temporarily increase after a meal
other cause of iron overload
acute liver damage ( liver contains lots of iron)
oral iron can be
ferrous sulphate or ferrous fumarate
when should iron infusions be avoided
during infections as there is a potential for it to feed the bacteria
3 key causes of low B12
pernicious anaemia, insufficient dietary B12, medications
medication that can cause low B12
PPIs and metformin
B12 is absorbed in the
distal ileum
3 symptoms of pernicious anaemia
Peripheral neuropathy, with numbness or paraesthesia (pins and needles)
Loss of vibration sense
Loss of proprioception
first line investigation for pernicious anaemia
intrinsic factor antibodies
what is initially given to patients with B12 deficiency
Intramuscular hydroxocobalamin
what is given for maintenance of vitamin B12 defieicny if its pernicious anaemia
2-3 monthly injections for life
what is given for maintenance of vitamin B12 defieicny if its diet related
oral cyanocobalamin or twice-yearly injections
other symptom of destruction of red blood cells
Splenomegaly (the spleen becomes filled with destroyed red blood cells)
blood film shows schistocytes (fragments of red cells ) in
haemolytic anaemia
most common inherited haemolytic anaemia in northern Europeans
hereditary spherocytosis
hereditary spherocytosis inheritance pattern
autosomal dominant
what presents with anaemia, jaundice, gallstones and splenomegaly
hereditary spherocytosis
what is a notable feature of hereditary spherocytsosi
aplastic crisis in the presence of the parvovirus
what is raised in terms of bloods in hereditary spherocytosis
MCHC ( mean corpuscular haemoglobin concentration) and reticulocyte count
what is treated with folate supplementation
hereditary spherocytosis
what enzyme is responsible for protecting the cells from oxidative damage
glucose 6 phosphate dehydrogenase
G6PD deficiency has what inheritance pattern
X linked recessive
what condition results in acute episodes of haemolytic anaemia triggered by infections , drugs or fava beans
G6PD deficiency
key medications that can be triggers for G6PD deficiency are
ciprofloxacin, sulfonylureas (e.g., gliclazide) and sulfasalazine.
what can diagnose G6PD defieicny
G6PD enzyme assay
presents with jaundice (often in the neonatal period), gallstones, anaemia, splenomegaly and Heinz bodies on a blood film.
G6PD deficiency
drug class that can cause G6PD deficiency is also
antimalarials
what autoimmune haemolytic anaemia is more common
warm
cold AIHA can be secondary to
lymphoma, leukaemia, systemic lupus erythematosus and infections (e.g., mycoplasma, EBV, CMV and HIV).
warm autoimmune haemolytic anaemia is usually
idiopathic
alloimmune haemolytic anaemia 2 scenarios are
transfusion reactions and haemolytic disease of the newborn.
paroxysmal nocturnal haemoglobinuria is caused by mutation in what
haemoatpoieic stem cells - mutations occur during the patients lifetime- mutation means activation of complement cascade
management for paroxysmal nocturnal haemoglobinuria
Eculizumab or bone marrow transplantation
what is monclonal antibody that targets C5
Eculizumab
the red urine in the morning in paroxysmal nocturnal haemoglobinuria contains
haemoglobin and haemosiderin
Microangiopathic haemolytic anaemia is usually secondary to something and what is a key finding seen on blood film
Schistocytes
what supplementation is given for prosthetic valve haemolyssi
iron and folic acid
the thalasaemias has what inheritance pattern
autosomal recessive
what is used to diagnose global abnormalities
haemoglobin electrophoresis
what may occur in thalassaemias
iron overload
what chromosome are the genes that code for alpha and beta thalassaemias on
alpha- 16
beta - 11
thalassaemia intermedia has how many abnormal copies of the beta global gene
2
how many functioning beta global genes do beta thalassaemia major have
none
what are features of thalassaemia major
prominent forehead, prominent cheekbones, flat nose, protruding upper teeth
sickle red blood cells are what shape
crescent
at birth how much haemoglobin is fetal and how much is adult
1/2 and 1/2
sickle cell anaemia has what inheritance pattern
autosomal recessive
sickle cell anaemia affects beta global on chromosome
11
difference between sickle cell trait and sickle cell disease
triat - one abnormal copy of the gene - usually asymptomatic
disease- two abnormal copies
those with sickle cell trait has a selective advantage against
malaria
when is sickle cell screened for in babies
day 5 - newborn blood spot
what can trigger sickle cell crises
dehydration, infection, stress, cold weather
what is the treatment for sickle cell crisis
supportive
what is the most common type of sickle cell crisis
vaso occlusive crisis
vaso oclusive crisis can cause what
distal ischaemia with pain and swelling in the hands or feet but can affect chest or back etc. it can be associated with fever and can cause priapism in men (painful and persistent erection)
how to treat priapism
urological emergency - aspirate blood from the penis
what sickle cell crisis causes an acutely enlarged and painful spleen
Splenic sequestration crisis
splenic sequestration crisis causes hyper or hypo splenism
HYPO
what describes the temporary absence of the creation of new red blood cells
aplastic crisis
aplastic crisis is usually triggered by infection with
parvovirus B19
cxr in acute chest syndrome will show
pulmonary infiltrates
antibiotic prophylaxis in sickle is with
penicillin V (phenoxymethylpenicillin)
what stimulates the production of Fetal haemoglobin
Hydroxycarbamide
monoclonal antibody that ca n be used for sickle
Crizanlizumab
what leukemias is associated with down syndrome
ALL
what leukemias is associated with warm haemolytic anaemia, Richters transformation and smudge cells
CLL
what leukemias has three phases, including a long chronic phase, and is associated with the Philidelphia chromosome
CML
what leukemias results in a transformation from a myeloproliferative disorder and is associated with auger rods
AML
a key presenting feature of leukemias is bleeding under the skin due to
thrombocytopenia
suspected leukemia what should happen
FBC within 48hrs
children or young people with what symptoms should be sent for immediate specialist assessment
petechiae or hepatosplenomegaly
what establishes a definitive diagnosis of leukaemia
bone marrow biopsy
what bone marrow biopsy is better for seeing the cells
trephine
the Philadelphia chromosome is more associated with CML but can be seen in
ALL
ALL usually affects what lymphocytes
B
what other thing may be seen in ALL
pancytopenia
CLL usually affects what lymphocyte ( the same as ALL)
B
what may cause warm autoimmune haemolytic anaemia
CLL
what is righters transformation
CLL into high grade B cell lymphoma
what cells are particularly associated with CLL
smear or smudge cells
smear or smudge cells are what kind of cells
ruptured white blood cells
what phase of CML is often asymptotic and diagnosed after an incidental finding of a raised white cell count
chronic
what phase of CML the patient develops anaemia, thrombocytopenia and immunodeficiency
accelerated
what phase of CML the patient develops pancytopenia
blast
what has occurred on the Philadelphia chromosome in CML
reciprocal translocation
translocation of the Philadelphia chromosome creates a gene sequence called — which codes for a abnormal tyrosine kinase enzyme
BCR- ABL1
what myeloproliferative disorder might AML transform from
polycythaemia ruby vera or myelofibrosis
Auer rods
AML
bone marrow biopsy will show a high proportion of blasts in
AML
what is a tyrosine kinase inhibitor
ibrutinib
what is a monoclonal antibody that targets B cells
rituximab
what is low in tumour lysis syndrome
calcium
what reduces risk of tumour lysis syndrome
very good hydration and urine output before chemo
what may be used to suppress the uric acid levels in tumour lysis syndrome
allopurinol or rasburicase
what is the most common specific type of lymphoma
hodgkins
what has a bimodal age distribution with peaks around early 20s then at 80
hodgkins
4 rf for hodgkins
HIV, EBV, Autoimmune conditions and FH
NHL lymphomas include
diffuse large B cell , Burkitt, Malt
what typically presents as a rapidly growing painless mass in older patients
diffuse large B cell
what NHL is particularly associated with EBV and HIV
Burkitt
what lymphoma particularly affect the mucosa associated lymphoid tissue usually around the stomach
MALT
h. pylori is associated with what lymphoma
MALT
pesticides exposure increases your risk of
NHL
patients with what lymphoma may experience lymph node pain after drinking alcohol
hodgkins
what is a critical diagnostic investigation in lymphoma
lymph node biopsy
what are large cancerous B lymphocytes with two nuclei and prominent nucleoli, giving them a cartoonish appearance of an owl face with large eyes.
Reed sternerg cells - hodgkins lymphoma
what classification has replaced the Ann Arbor for Lymphoma
Lugano
critical treatment for hodgkins lymphoma is
chemo and radiotherapy
rituximab may be used for what lymphoma
NHL
plasma cells are what lymphocytes
B
Cancer in a specific type of plasma cell results in the production of large quantities of a specific
paraprotein (or M protein)
what is a paraprotein
abnormal antibody or part of an antibody
— involves the production of a specific paraprotein without other features of myeloma or cancer.
Monoclonal gammopathy of undetermined significance (MGUS)
what is the % risk of MGUS progressing to myeloma
1%
Smouldering myeloma involves abnormal plasma cells and paraproteins but no organ damage or symptoms. It has a greater risk of progression to myeloma (about — % per year).
10
paraprotein is often the
light chain
bence jones protein refers to
free light chains in the urine
there is paraproteinaemia ( abnormally high level of this paraprotein) in
myeloma
calcium in myeloma
elevated
most common complications of myeloma
anaemia
anaemia in myeloma is
normocytic and normochromic
hyperviscocity syndrome may occur in
myeloma
what is raised in myeloma
calcium , ESR and plasma viscocity
what detects paraproteinaemia
serum protein electrophoresis
what detects Bence Jones protein
urine protein electrophoresis
what is required to confirm the diagnosis in myeloma
bone marrow biopsy
what imaging is first lien for bone lesion in myeloma
whole body MRI
lytic lesions in myeloma may be described as looking
punched out
what refers to multiple lytic lesion seen in the skull on X-ray in myeloma
Raindrop skull, sometimes called pepper pot skull
treatment of myeloma usually involves a combination of chemo which may include
Bortezomib (a proteasome inhibitor)
Thalidomide
Dexamethasone
what is meant by a autologous stem cell transplant
using the persons own stem cells
what is meant by allogeneic transplant
using stem cells from a healthy donor
drug for myeloma bone disease
biphosphonates
Myeloproliferative disorders involves the uncontrolled proliferation of
single type of stem cell
—are considered a form of cancer occurring in the bone marrow, although they tend to develop and progress slowly
myeloproliferative disorders
myeloproliferative disorders has the potential to transform into
AML
Myeloproliferative disorders to remember are
Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia
polycthaemia vera has what proliferating cell line
erythroid cells
PV has what blood finding
high haemoglobin
essential thrombocythaemi has what blood finding
high platelet count
primary myelofibrosis has what blood finding
low haemoglobin
JAK2 associated with the
myeloproliferative disorders
ruxolitinib is a
JAK2 inhibitor
what cytokine can cause bone marrow fibrosis
fibroblast growth factor
what will a blood film in myelofibrosis show
Teardrop-shaped red blood cells
Anisocytosis (varying sizes of red blood cells)
Blasts (immature red and white cells)
raised haemoglobin can present as
itching headaches and a red face
what is a complication of polycythaemia
gout
signs of polycythamia
Ruddy complexion (red face)
Conjunctival plethora (the opposite of conjunctival pallor)
what is done to confirm the diagnosis of myeloproliferative disorders
bone marrow biopsy
what may the bone marrow aspiration be with myelofibrosis
dry
other mutation in what genes in myeloprolferative disorders except form JAK2
MPL, CALR
polycythamei vera mx may involves
venesection and aspirin
mx of essential thrombocythaemia
aspirin
hydroxycarbamide is a type of
chemo
example of a platelet lowering agent
Anagrelide
rf for myelodysplastic syndrome
chemo or radiotherapy
who may present with anaemia, neutropenia and thrombocytopenia
myelodysplastic syndrome
myelodysplastic syndrome has the potential to transform into
AML
medications that can increase platelet destruction causing thrombocytopenia
sodium valproate and methotrexate
mx for immune thrrombocytpenic purpura
prednisolone
rituximab targets what proteins on B cells
CD20
—- is a condition where tiny thrombi develop throughout the small vessels, using up platelets.
Thrombotic thrombocytopenia purpura
problem with what protein in thrombotic thrombocytopenic purpura
ADAMTS13
heparin induced thrombocytopenia presents how long after starting treatment with heparin
5-10 days
what alternative anticoagulant can be given in heparin induced thrombocytopenia
fondaparinux or argatoban
what is the most common inherited cause of abnormal and prolonged bleeding
von willebrands disease
most von willebrands disease has what inheritance pattern
autosomal dominant
diagnosis of von will brands disease is through what
history
mx for von willebrands
only given in response to significant bleeding or trauma or in preparation for operations - desmopressin, tranexamic acid
both haemophilia have what inheritance
X linked recessive
All X chromosomes need to have the abnormal gene to have –
haemophilia
bleeding into the muscles I haemophilia can cause
compartment syndrome
complication of clotting factor infusions in haemophilia is
foramtion of antibodies against the treatment
when a thrombus develops it can travel from the deep veins through which side of the heart and into the lungs
right
how is antiphospholipid syndrome diagnosed
blood test for antiphospholipid antibodies
prophylaxis for VTE usually involves LMWH such as
enoxaparin
contraindications to LMWH for VTW prophylaxis if
active bleeding or existing anticoagulation with warfarin or a DOAC
what is the main contraindication for compression stockings
peripheral arterial disease
Ultrasound of the leg is required to diagnose deep vein thrombosis. NICE recommends repeating negative ultrasound scans after — days if the patient has a positive D-dimer and the Wells score suggests a DVT is likely.
6-8
what is the usual first line imaging investigation for a pulmonary embolism
CT Pulmonary angiogram
initial anticoagulant for DVT /pE
apixaban/ rivaroxaban - can be started before scan if there is a delay in getting scan
The NICE guidelines (2020) recommend considering — in patients with a symptomatic iliofemoral DVT and symptoms lasting less than 14 days.
catheter-directed thrombolysis
The options for long-term anticoagulation in VTE are a
DOAC, warfarin or LMWH.
do DOACs require monitoring
no
DOACs not suitable if
severe renal impairment, pregnancy or antiphopsholipid syndrome
what is the first lien in patients with antiphosphilipid syndrome for DVT/PE
warfarin
what is the first line anticoagulant in pregnancy
LMWH
syndrome involves obstruction to the outflow of blood from the liver caused by thrombosis in the hepatic veins or inferior vena cava. It is associated with hypercoagulable states (e.g., myeloproliferative disorders). It presents with a classic triad of:
Abdominal pain
Hepatomegaly
Ascites
Budd-Chiari
usual imaging for Budd chiari syndrome
doppler ultrasonography
