haem/ onc Flashcards
WHat is the most common cause of inherited thrombophilia? ie clotting
Activated protein C resistance (Factor V Leiden) i
Length of tx for unprovoked vs provoked DVT? How do you classify each?
Unprovoked DVT = no RF OR RF cannot be easily removed eg cancer, thrombophilia. Treat for 6 months
provoked = removal factor eg post-op, HRT, COCP, flying, pregnant, immobile. Treat for 3 months
What is myelofibrosis? What is associated with this condition on blood film? Mx?
a myeloproliferative disorder
‘tear-drop’ poikilocytes on blood film
This occurs because they are ‘squeezed’ through the fibrotic tissue in bone marrow in myelofibrotic disorders.
mx with immunosuppression
What is the most common inherited bleeding disorder? What is found on ix for this?
Von Willebrand’s disease (low von willebrand factor which is needed for platelet aggregation)
AD inheritance
Ix:
prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin
What is tumour lysis syndrome? How is it prevented?
breakdown of tumour cells –> release of chemicals from the cell.
Causes high K + high PO4, and low Ca
patients are higher risk should receive either allopurinol or rasburicase
What is Polycythaemia vera? How does it present? Mx?
Excess RBC and often also excess WBC and plarelets.
Strong association (95%) with JAK2
Presents: itching after bath, spleenomegaly, hyperviscosity, HTN, haemorrhage (low platelets), low ESR
Mx: If asx treat with aspirin + venesection, if sx then immunosuppress
Describe SCLC? What features may it have? mx?
arise from APUD* cells
ADH → hyponatraemia
ACTH → Cushing’s syndrome
Lambert-Eaton syndrome: antibodies to voltage gated calcium channels causing myasthenic like syndrome
If VERY early disease (T1-2 with no nodal or mets) then surgery, otherwise poor prognosis, for chemo/ radiotherapy
What blood results would you see in IDA?
Total iron-binding capacity (TIBC) + transferrin levels are typically raised in iron-deficiency anaemia
Iron and ferritin are low
When would you do a d-dimer post wells score?
If scores 1 but DVT still seems likely - if negative ruled out, if positive USS needed
What would you see on FBC for SCD?
Sickle cell disease causes a normocytic anaemia with raised reticulocyte count - due to haemolysis
What do you see on bloods in DIC?
Often occurs with sepsis/ trauma
Low platelets and low coagulation factors (all coagulation factors are depleted), low fibrinogen (as microvascular clotting), raised d-dimer
schistocytes (sheared blood cells) - also see in TTP and HUS
When do you see elliptocytes?
These are rod/ pencil shaped erythrocytes seen in IDA, thalassaemia
When are heinz bodies seen?
Alpha thalassemia
G-6-PD deficiency
When are howell-jolly bodies seen?
characteristic of decreased splenic function, such as post-splenectomy/ severe haemolytic anaemia/ coeliacs disease
herediatry spherocytosis
What is Hereditary haemorrhagic telangiectasia?
ABnormal blood vessels form —> telangiectasia, epistaxis and chronic bleeding occur –> IDA
Adverse effects of following agents:
Cyclophosphamide
Bleomycin
Anthracyclines (e.g doxorubicin)
Fluorouracil (5-FU)
Vincristine, vinblastine
Cisplatin
methotrexte
Cyclophosphamide:Haemorrhagic cystitis/ tranitional cell carcinoma, myelosuppression
Bleomycin: lung fibrosis
Anthracyclines (e.g doxorubicin): cardiomyopathy
Fluorouracil (5-FU): Myelosuppression, mucositis, dermatitis
Vincristine: peripheral neuropathy
Cisplatin: Ototoxicity, peripheral neuropathy, nephropathy, low magnesium
Methotrexate: Myelosuppression, mucositis, liver fibrosis, lung fibrosis
What do clotting screens look like for liver failure?
In liver failure, all clotting factors are low except for factor VIII which is raised. Both PT and APTT can be prolonged.
What do clotting screens look like for Haemaphilia A and B?
APTT grossly elevated
PT and bleeding time normal
Haemophilia A would have low levels of factor VIII but the other clotting factors are not affected. Haemophilia B would have factor IX deficiency.
What does clotting screen look like in VWB?
Von Willebrand disease would have low levels of Von Willebrand factor but the other factors are not affected. Factor VIII may be low or normal.
What cancer is associated with acanthosis nigricans?
gastrointestinal adenocarcinoma
Which cancer is associated with pityriasis rotunda?
myeloma
Which cancer is assoiated with superficial thrombophlebitis?
pancreatic carcinoma
aplastic vs sequestrian vs thromotic crisis in SCD?
Aplastic crises
caused by infection with parvovirus
sudden fall in haemoglobin
bone marrow suppression causes a reduced reticulocyte count
Sequestration crises
sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
associated with an increased reticulocyte count
Thrombotic crises
precipitated by infection, dehydration, deoxygenation
painful vaso-occlusive crises should be diagnosed clinically
infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
When are aeur rods seen on blood film?
These needle-shaped inclusions found within the cytoplasm of myeloid blasts indicate AML
When are Rouleaux formation seen on blood film?
multiple myeloma
What does a high PT vs high APTT show?
High PT - extrinsic pathway, factor 7, which is rarely deficinent in isolation - so raised if whole body issue eg DIC, liver disease, warfarin, vit K deficient
High APTT - intrinsic pathway, Factor 8,9 and 11. So haemophilias and VWB (binds to factor 8) have raised APTT
When does heparin- inducted thrombocytopenia occur?
5-14 days post-heparin exposure
What should you do if SCD + fever?
Admit - high risk of sepsis due to aspleenia
What is in cryoprecipitate?
Cryoprecipitate contains factor VIII, fibrinogen, von Willebrand factor and factor XIII
What is the monitoring for unfractionated heparin?
Unlike low molecular weight heparins that do not require monitoring unfractionated heparin does require monitoring, this is done by measuring the APTT.
What is post-thrombotic syndrome?
chronic venous insufficiency secondary to DVT
When are smear cells seen?
CLL
When do target cells look like?
eg iron-deficiency anaemia or hyposplenism eg thalassemia
They are red blood cells that have the appearance of a target with a bullseye (a dark centre, surrounded by a pale ring and a darker peripheral ring). They are caused either by an increase in red cell surface area or a decrease in intracellular haemoglobin.
What electroyte imbalance can occur from large volumes of RBC transfusion?
HIGH K
What is the reversal agent for dabigatran?
Idarucizumab
How do you differentiate spherocystotis from G6PD defciency?
Heritary spherocystosis:
AD inheritance
middle eastern, mediterannian + SE asia association
Both cause haemolytic anaemia, jaundice + gallstones, can be precipitated by infection
G6PD:
X linked recessive (just boys)
african-mediterranean
heinz bodies and bite cells
Sulph drugs, anti-malarials, fava beans and ciprofloxacin CI as can induce haemolysis
Mx of warfarin in following situations:
INR 5-8
INR 5-8 + minor bleed
INR >8
INR >8 + minor bleed
Major bleed
INR 5-8 - withold dose
INR 5-8 + minor bleed - stop warfarin, give vit K 1-3mg, restart when INR <5
INR >8 - stop warfarin, give bit K 1-5mg PO, restart when INR <5
INR >8 + minor bleed -Stop warfarin
Give IV vitamin K 1-3mg, restart when INR <5
Major bleed - IV vit K 5mg, prothrombin complex concentrate
Features of CML?
Philadephia chromosome associated
Age 60-70 yr
imatinib is now considered first-line treatment which is inhibitor of tyrosine kinase
splenomegaly may be marked
CAN progress to AML
Features of AML?
Avergae age 25 yrs
auer rods on blood film
Features of CLL?
Marked lymphadenopathy
SMudge cells on blood film (crushes little lymphocytes)
roughly 80 years of age - often asx
What cancers is alpha feto protein raised levels associated with?
liver ca and non seminoatous testiular ca
What blood results/ hx would you see for antiphospholipid syndrome? mx
young female patient with recurrent deep vein thrombosis, miscarriage and livedo reticularis
association with SLE
mild thrombocytopenia + prolonged APTT
anticardiolipin antibodies
–> the antiphospholipid antibodies which interfere with phospholipid-dependent coagulation tests, leading to a prolonged APTT.
mx - low-dose aspirin
Which blood products have the highest chance of causing infection
Platelet transfusions have the highest risk of bacterial contamination compared to other types of blood products as they are not kept refridgerated
What cancers is BRACA-2 associated with?
BRCA2 mutations substantially increase the risk of developing breast cancer in both men and women. It is also associated with ovarian cancer in women and prostate cancer in men.
What is Lynch Syndrome?
AD inheritance
Develop colonic cancer and endometrial cancer at young age
High risk individuals may be identified using the Amsterdam criteria, need to exclude FAP (Familial adenomatous polyposi)
When are target cells seen?
Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease
What is more common hodkins lymphoma or non-hodgkins lymphoma? Which lymphoma worsens with alcohol?
NHL is more common
Hodkins painful lympadenopathy with alcohol
When is FFP given?
PT > 1.5 x the normal
How does thalasssemia look on bloods? WHat are the different types?
microcytic anaemia
in beta get reduced beta bands, in alpha get reduced alpha bands
Thalassemia trait - asx - will have microcytic anaemia with normal iron studies
How would autoimmune haemolytic anaemia look on bloods?
Autoimmune haemolytic anaemia - anaemia, signs of haemolysis (raised reticulocytes + LDH, +ve coombs test)
Aplastic anaemia vs myelodysplastic syndrome
Aplastic anaemia = stops making stem cells, myelodysplastic syndromes = production abnormal cells
