haem Flashcards
which mutation is present in patients with polycythaemia
JAK2 mutation
what is the emperical antibiotic of choice for neutropenic sepsis
piperacillin with tazobactam
what treatment is is neutropenic sepsis generally a consequence of
chemotherapy
which bacteria generally causes neutropenic sepsis
gram positive - staph epidermis
definition of neutropenic sepsis
neutrophil count of < 0.5* 10^9 in patient having chemo and -
temp > 38
other signs + symptoms consistent with clinically significant sepsis
classical symptom of polycythaemia vera
intense itching when exposed to hot water / hot + humid weather
what are the features of Multiple Myeloma ?
CRABBI
Calcium : hypercalcaemia
Renal : renal damage presenting as dehydration and thirst
Anaemia
Bleeding
Bones
Infection
what is the gold standard investigation for Multiple Myeloma ?
bone marrow aspiration : confirms diagnosis if the number of plasma cells is raised
what sign is seen on peripheral blood film in multiple myeloma ?
Rouleaux formation
name 4 signs of transfusion associated circulatory overload
HTN
raised jugular venous pulse
afebrile
S3
give 3 signs of transfusion related acute lung injury
hypotension
pyrexia
normal // unchanged JVP
What is the management of DVT in pregnancy
subcutaneous low molecular weight heparin
What is the management of DVT in pregnancy
why do you treat vit b12 deficiency before starting treatment for folic acid ?
to avoid precipitate sub-acute combined degeneration of the cord.
An aplastic crisis may be triggered by ________ in persons with hereditary spherocytosis
parvovirus
how does a macrocytic anaemia present on blood film?
Hypersegmented neutrophil polymorphs
what is the bacteria that most commonly causes neutropenic sepsis ? How does it present under microscope ?
Staph. Epidermis, coagulase negative, gram positive bacteria
which cells of the body does myeloma affect
plasma cells
what are plasma cells ?
Plasma cells are b lymphocytes that produce antibodies
what is multiple myeloma
when myeloma affects multiple bone marrow areas in the body
what is monoclonal gammopathy of undetermined significance
it involves the production of a specific para protein ( abnormal antibody) without any other features of myeloma or cancer
what is smouldering myeloma
involves both abnormal plasma cells and paraproteins but no organ damage or symptoms
what is a plasmacytoma
tumour formed by cancerous plasma cells
what is the gold standard investigation for the diagnosis of sickle cell disease
haemoglobin electrophoresis
what is the most common cause of vitamin B12 deficiency ?
Pernicious anaemia
what is the typical picture of DIC on blood tests
low platelets
increased PT
increased APTT
bleeding time prolonged
what is the management of beta thalassaemia major
lifelong iron transfusion
when would you use prothrombin complex concentrate ?
emergency reversal of Anti-coagulation in patients with either severe bleeding or head injury with suspected intracerebral haemorrhage.
what is the first line investigation for patients with suspected AML
very urgent FBC within 48 hours
what is the management of folate deficiency
1 mg of IM hydroxocobalamin 3 times a week for 2 weeks then once every 3 months
how is acute chest syndrome in sickle cell disease managed ?
PATO
Pain relief
Abx
Transfusion
O2
what are the positive prognostic factors for ALL
age 3-7
female
what are the poor prognostic factors for ALL
age <1 or >10
wcc > 100 x 10^9
translocation of chromosomes 9:22 ( Philadelphia)
male
how does acute chest syndrome present and how is it managed?
Vaso-occlussion within pulmonary microvasculature causing lung parenchyma infarction.
presents with : dyspnoea, chest pain, pulmonary infiltrates on CXR and low pO2.
management includes pain relief , respiratory support and antibiotics
how does an aplastic crisis present ?
Parvovirus B19 Infection
sudden fall in haemoglobin
bone marrow suppression leading to reduced reticulocyte count
how does a sequestration crisis present
sickling within spleen or lung causes pooling, worsening the anaemia.
associated with increased reticulocyte count
presenting with abdominal pain, haemodynamic instability and hepato/ splenomegaly
how does a vaso-occlusive crisis present ?
painful / vasooculsive crisis is precipitated by infection, dehydration, deoxygenation
clinical diagnosis presenting with acute pain,
what is the key presentation of chronic myeloid leukaemia and how is it managed?
increase in granulocytes at different stages of maturation.
managed first line with tyrosine kinase inhibitors such as imanitib
when is a V/Q scan more appropriate to use than CTPA
renal impairment
what reversal agent can be used for rivaroxaban and apixaban ?
andexanet alfa
what is a non-haemolytic febrile reaction and how does it present ?
fevers + chills - manage with slow, stop the transfusion and paracetamol.
how does a minor allergic reaction to blood transfusion present and how is it managed ?
pruritis and urticaria
manage by temporarily stopping and giving anti-histamine and monitoring
why can anaphylaxis due to blood transfusion occur?
in patients with IgA deficiency who have anti IgA antibodies
why and how does an acute haemolytic reaction present and how it managed ?
ABO-incompatible blood e.g. secondary to human error
presenting with -
Fever, abdominal pain, hypotension
management -
stop transfusion
confirm diagnosis and patient
supportive
why and how does TACO present ? how is it managed?
excessive transfusion rate causing pulmonary oedema and HTN, managed with stopping transfusion and considering IV Diuretics
why and how does TRALI present ? how is it managed?
hypoxia, pulmonary infiltrates on CXR , hypotension, managed by stopping transfusion, O2 and supportive care
what are the risk factors for Burkitt’s lymphoma ? how does it present on microscopy ? how is it managed? what is a risk factor of the management?
RF’s - HIV, EBV - v big rf
starry sky on microscopy
chemotherapy - can causes tumour lysis syndrome
what is the mechanism of action of unfractionated heparin ?
activates anti thrombin III
how are standard heparin and LMWH monitored
standard : APTT
LMWH : anti factor Xa- not recquired routinely
what is the reversal agent for heparin
protamine suphate
what is the pathophysiology of pernicious anaemia ?
antibodies to intrinsic factor and gastric parietal cells
what are the features of pernicious anaemia
anaemia - lethargy, pallor, dyspnoea
neurological features - peripheral neuropathy ( pins and needles)
jaundice- lemon tinge
glossitis
what is the most specific antibody for pernicious anaemia ?
anti- intrinsic factor antibodies
what is the management of pernicious anaemia
vitamin B12 replacement given IM , 3 injections per week for 2 weeks followed by 3 monthly treatment of vitamin B12 injections.
what is a key malignancy linked with pernicious anaemia
gastric cancer
what is the most common of lymphoma in the UK
diffuse large b cell
what is heparin induced thrombocytopenia ?
Prothrombotic state occurring due to antibodies forming against complexes of platelet 4 and heparin causing a drop in platelets.
managed with direct thrombin inhibitor such as argatroban
what are the key points in the investigation of myeloma?
Bloods: FBC and peripheral blood film - rouleaux
U+E showing renal failure
protein electrophoresis : showing raised IgA and IgG , known as Bence Jones proteins in the urine.
bone marrow biopsy - shows raised plasma cells
what imaging is used for mm
whole body MRI
what are some complications of blood transfusions
febrile reaction
ARDS
iron overload
clotting abnormalities
hyperkalaemia
how do you manage suspected DVT if D-dimer is positive but scan is negative ?
stop interim therapeutic anticoagulation and repeat proximal lung vein ultrasound scan 6-8 days later
what is myelofibrosis? what are it’s features? what lab findings are associated with it ?
myeloproliferative disorders due to hyperplasia of abnormal megakaryocytes.
elderly person with anaemia and fatigue
massive splenomegaly
lab findings include - high white cell count, tear drop poikilocytes
what anti-platelet regime is used in patients post stroke
aspirin 300 mg for 2 weeks then clopidogrel lifelong
what is the mechanism of action of aspirin
inhibits production of thromboxane A2
which haemophilia is most common? what is the nature of inheritance?
Haemophilia A
X linked
what is the pathophysiology of an acute haemolytic reaction
Binding of IgM type antibodies to RBC’s causing haemolysis
what is the mechanism action of fondaparinaux
activates antithrombin 3
what is the best test to screen for haemachromatosis ? how are family members tested
transferrin solutions, genetic testing for HFE mutation
what is the diagnostic test for polycythaemia vera
JAK2 mutations screen
what are the laboratory features of beta-thalassaemia trait
mild hypochromic, microcytic anaemia ( disproportionate microcytosis to anaemia)
Hb2 raised
what other investigations are recommended in a patient with suspected PE - other than CTPA
chest xray to exclude other pathology
ECG which may show S1Q3R3 and sinus tachycardia
what combination of blood results are most likely to be seen in a patient with sickle cell disease
Low Hb
normal MCV
raised reticulocytes
what is the mechanism of action of aspirin ?
Non reversible COX 1 and 2 inhibitor
what will the ABG most likely show in PE
respiratory alkalosis due to hyperventilation
do anti-platelets need to be stopped before a dental procedure ?
take aspirin as normal
what is the diagnostic test for sickle cell disease ?
haemoglobin electrophoresis
which medication reduces risks of complications and acute crisis in sickle cell patients
Hydroxycarbamide
what is the vitamin B12 replacement regime?
vitamin B12 deficiency IM B12 1mg three times weekly then 1 mg IM every 3 months
which gene translocation is burkitt’s lymphoma associated with ?
C- myc gene translocation
what findings are seen on bone marrow aspirate in multiple myeloma?
increased number of plasma cells
how long before surgery is warfarin stopped
5 days
ileo-caecal valve resection can cause what type of anaemia
vitamin b12 deficiency
what would you be expected to see on blood film in DIC
schistocytes
what chromosome does beta-thalassaemia major affect ? what are it’s features and management ?
affects chromosome 11, presenting in 1st year of life with failure to thrive and hepatosplenomegaly
HbA2 and HbF are raised
HbA is absent
management is with repeated transfusions and iron chelation therapy with deferoxamine
what is essential thrombocytosis ? What are it’s features and management ?
myeloproliferative disorder, with overproduction of platelets, JAK2 mutation and characteristically a burning sensation in the hands.
managed with hydroxyurea ( hydroxycarbamide) and low dose aspirin
what clotting does warfarin cause
raised PT, normal APTT
what is seen on blood film in autoimmune haemolytic anaemia ?
spherocytes and reticulocytes
what test can be used to check for auto-immune haemolytic anaemia ?
positive direct anti-globulin test - Coomb’s test
what are the 2 types of AIHA? what are they caused by ?
Warm AIHA - IgG ( ur hot, a real G)
idiopathic, AI, lymphoma, CLL
Cold AIHA - IgM
neoplasia and infections
mx of warm is generally management of the underlying cause, steroids, rituximab
how often do patients with SCD require the pneumococcal vaccine ?
5 years
what is the mechanism of dabigatran
direct thrombin inhibitor
what biochemical pattern is seen in myeloma on blood film ?
High calcium , normal phosphate, normal ALP
what is the INR target post PE
3.5
what is low haptoglobin associated with
haemolysis
which organisms cause post splenectomy sepsis
streptococcus pneumoniae
haemophilus influenzae
meningococci
what is the mechanism of action of -
rivaroxaban
dabigatran
warfarin
heparin
direct factor Xa inhibitor - rivaroxaban
direct thrombin inhibitor - dabigatran
antithrombin III activator - heparin
inhibition of clotting factor II,VII, IX and X - warfarin
what are the guidelines regarding warfarin in patients undergoing emergency surgery ?
if surgery can wait 6-8 hours : 5 mg Vitamin K IV
surgery cannot wait : 25-50 units/ kg of four factor PT complex
give 4 complications of CLL
anaemia
hypogammaglobulinaemia
warm autoimmune haemolytic anaemia
transformation to high grade lymphoma - richter’s transformation
what are the features of Richter’s transformation
suddenly unwell patient
lymph node swelling
weight loss
night sweats
nausea
abdo pain
what type of infection is most likely to occur after platelet transfusion
bacterial
what are the enzyme inducers? what action do they have on warfarin?
SCRAP GP
smokers
carbamazepine
rifampicin
alcohol
phenytoin
griseofulvin
phenobarbital
reduce the action of warfarin
what are the enzyme inhibitors? what action do they have on warfarin?
SICKFACES.COM
sodium valproate
isoniazid
cimetidine
ketoconazole
fluconazole
alcohol
chloramphenicol
erythromycin
sulphonamides
ciprofloxacin
omeprazole
metronidazole
increase action of warfarin
the following cell types are seen in what condition ?
smear/ smudge cells
reed Sternberg cells
Auer rods
howell jolly bodies
heinz bodies
spherocytes
smear/ smudge cells - CLL
reed Sternberg cells - Hodgkins lymphoma
Auer rods- AML
howell jolly bodies - decreased spleen function
heinz bodies - G6PD, Alpha thalassaemia
spherocytes - hereditary spherocytosis
what medications cause aplastic anaemia ?
cytotoxic drugs
chloramphenicol
sulphonamides
phenytoin
gold
what are the secondary causes of polycythaemia?
COPD
Altitude
obstructive sleep apnoea
excessive erythropoietin
which condition can cause hypogonadotrophic hypogonadism
haemachromatosis
which patients can be managed as an outpatient if they are having a PE ?
PESI score class 1, 2
management of anti-phospholipid syndrome in pregnancy
Low dose aspirin
Low molecular weight heparin once a foetal heart is seen on ultrasound
which is the most common inherited bleeding disorder?
Von Willebrands disease
feature’s of von willebrands disease
prolonged bleeding time
APTT can be prolonged
haemophilia vs VW
von willebrands : increased PT, increased APTT and bleeding
haemophilia : increased APTT ( by a lot more), normal bleeding time
Tear drop poikilocytes are present in which condition
myelofibrosis
picture of CML on bloods
significantly raised WCC
predominance of neutrophils, eosinophils and basophils
picture of AML on blood film
thrombocytopenia - low platelets
neutropenia = decrease in neutrophils
general management of a sickle cell crisis
analgesia
rehydration
oxygen
antibiotics if infection
blood transfusion/ exchange transfusion
exchange transfusions rapidly reduce the number of HbS containing cells
what should be prescribed alongside red cells during transfusion
furosemide
why are packed red cells transfused with furosemide
chronic anaemia and large amounts of fluid will cause cardiovascular compromise
when are platelet rich plasma transfused
thrombocytopenia and bleeding
FFP
contains clotting, albumin and immunoglobulin - used in correcting clotting deficiencies
cryoprecipitate
factor 8 and fibrinogen
major indication for cryoprecipitate
low fibrinogen
indication for FPP
high PT - showing deficiencies in factor II, V, VII , X
when should the following be given
FFP
Cryoprecipitate
packed red cells
prothrombin complex
platelets
FFP : Raised PT indicating issue with clotting
cryoprecipitate : low fibrinogen
Packed red cells : low Hb
PTT : emergency reversal of anticoagulation
platelets : thrombocytopenia
first line management of ITP
oral prednisolone and pooled normal Human immunoglobulin
ITP
immune mediated reaction against platelet count with antibodies directed against glycoprotein IIB/IIa
hyper segmented neutrophils indicate :
megaloblastic anaemia
schistocytes are seen in
intravascular haemolysis
features of thrombotic thrombocytopenic purpura
fever
neurological signs
thrombocytopenia
haemolytic anaemia
renal failure
pathogenesis of TTP
clumping of von willebrands factor causing platelets to clump
causes of ttp
post infection
pregnancy
drugs - cocp, penicillin, clopidogrel, aciclovir
tumours
SLE
HIV
interpretation of well’s score for DVT
2 or more - DVT likely - carry out proximal leg vein ultrasound within 4 hours
cannot be carried out in 4 h - interim therapeutic anticoagulation within 4h and D dimer
dvt unlikely - do d dimer in 4 hours, if not intermediate anticoagulation
when is a platelet transfusion offered to a patient
platelet count < 30 and clinically significant bleeding
platelet count < 10 and no active bleeding / planned invasive procedure
ivg for ITP
antiplatelet autoantibodies ( IgG)
bone marrow aspiration shows megakaryocytes
Mx for ITP
oral prednisolone
IV immunoglobulins
G6PD inheritance pattern
X-linked
features of G6PD deficiency
neonatal jaundice
intravascular haemolysis
gallstones
Heinz bodies, bite and blister cells
drugs causing haemolysis
anti-malarials
ciprofloxacin
sulphonamides
sulphasalazine
sulfonylureas
difference between hereditary spherocytosis and G6PD
HS = extravascular haemolysis and spherocytosis
G6PD = heinz bodies, blister and bite cells, intravascular haemolysis
The most common cause of an isolated thrombocytopenia
ITP
benign ethnic neutropenia
common in black/ afro carribean
what are the pre surgery/ procedure platelet thresholds
> 50 - normal
50-75 - high risk
100 - surgery at critical sites
presentation of hereditary spherocytosis
failure to thrive
jaundice
gallstones
aplastic crisis
signs of lead poisoning
abdo pain
peripheral neuropathy
fatigue
constipation
blue lines on gum margin
what does FBC show in lead poisoning
basophilic stippling
mx of lead poisoning
dimercaptosuccinic acid
medications that increase the risk of VTE
COCP
HRT
Raloxifene , tamoxifen
antipsychotics - olanzapine
test to differentiate b/w hereditary spherocytosis and G6PD
EMA binding test
myelodysplastic syndromes
ineffective haematopoiesis
peripheral blood cytopenia’s
risk of progression to AML
radiation and chemo increase this risk
clinical features of myelodysplastic syndromes
fatigue
weakness
pallor due to anaemia
recurrent infections due to neutropenia, easy bruising or bleeding due to thrombocytopenia
myelofibrosis
anaemia, thrombocytopenia and leukopenia with b symptoms
how quickly are red blood cells transfused
90-120 mins in a non urgent scenario
why are irradiated blood products used
to avoid transfusion associated graft vs host disease caused by viable t lymphocytes
clinical tumour lysis syndrome
laboratory tumour lysis syndrome plus-
increased serum creatinine
cardiac arrythmias
seizures
graft vs host disease
acute
painful maculopapular rash
jaundice
water or bloody diarrhoea
nausea and vomiting
chronic
skin, eye, GI, and lung manifestations
acute vs chronic graft vs host disease
acute - within 100 days of transplantation , affecting skin > 80%
chronic - after 100 days
transfusion threshold for patients with ACS
80 g/l
sideroblastic anaemia
hypochromic microcytic anaemia, basophilic stippling
all REFERRALS
A 48 hour blood test is recommended for children and young people with general symptoms or signs of leukaemia, however, when either hepatosplenomegaly or unexplained petichiae are present, the patient should be referred for immediate assessment
which lymph nodes does cervical cancer first spread to
internal and external iliac lymph nodes
deep inguinal lymph node cancers
anal and vulval cancer
thymoma
most common tumour of the anterior mediastinum and associated with myasthenia gravis
how does factor V leiden deficiency increase the risk of VTE
activated much more slowly by activated protein C
blood film in alcoholics
macrocytosis and thrombocytopenia
platelet transfusion threshold for severe bleeding
100
most common inherited thrombophilia
factor v leiden
transfusion most at risk of infection
platelet transfusion
which drugs can cause a methemoglobinemia
sulphonamides, nitrates
management of VWF
tranexamic acid
desmopressin
factor VIII concentrate
what is post thrombotic syndrome
painful and heavy calves
pruritis
swelling
venous ulceration
varicose veins
- it is a complication of DVT
mx of prothrombotic state
compression stockings
how is tranexamic acid given in cases of major haemorrhage
IV bolus followed by an infusion
diagnosis of G6PD
G6PD enzyme assays should be repeated around 3 months after acute haemolytic episodes to avoid false negatives
universal donor of FFP
AB RhD
how to differentiate haemolytic anaemia and hereditary spherocytosis
only mild spherocytosis in haemolytic anaemia
causes of neutropenia
HIV, EBV
Autoimmune
severe sepsis
haemodialysis
prevention of VTE in high risk patients
Rasburicase
what anaemia can prosthetic heart valves cause
haemolytic anaemia
how to differentiate essential thrombocytopenia and polycythaemia
essential thrombocytopenia = isolated thrombocytosis
diagnosis of acute intermittent porphyria
raised urine porphobilinogen
urine turns deep red on standing
management of flares of acute intermittent porphyria
IV haem arginate
typical DIC picture on bloods
low platelets, low fibrinogen, raised APTT , PT and raised D dimer
