Haem + onc Flashcards
Which organisms most commonly cause neutropenic sepsis?
Coagulase -ve gram +ve bacteria
particularly staph epidermis
What are auer rods? what conditions are they seen in?
Large, pink / red needle shaped structures in cytoplasm of myeloid cells
Seen in AML
Also in APML, high grade myelodysplastic syndromes and myeloproliferative disorders
What are some features of APML?
Presents younger than other forms of AML (avg 25yrs)
DIC / Thrombocytopaenia at presentation
Good prognosis
Genetic cause of APML
t(15;17) translocation - fusion of PML and RAR-alpha genes –> blocks the maturation of myeloid cells and leads to an accumulation of promyelocytes.
APML mx?
Treated with all-trans retinoic acid (ATRA) -> forces immature granulocytes into maturation to resolve blast criseses prior to definitive chemo
Bloods in DIC? Blood film?
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia
How can you distinguish between Waldenstrom’s macroglobulinaemia and multiple myeloma?
Waldenstrom’s macroglobulinaemia - Organomegaly with no bone lesions
Multiple myeloma - Bone lesions with no organomegaly
What are some features of Waldenstroms macroglobulinaemia?
Mx?
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance (the pentameric configuration of IgM increases serum viscosity)
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s
Mx - rituximab based combination chemo
Ix findings for waldenstrom’s macroglobulinaemia?
Monoclonal IgM paraproteinaemia (M - Macroglobulinaemia)
BM biopsy - diagnostic - infiltration of BM with lymphoplasmacytoid lymphoma cells
What genetic abnormality is associated with chronic myeloid leukaemia (CML)?
Philidelphia chromosome - t(9;22)
seen in 95% of cases
NB also seen in 25% of adults with ALL - poor prognostic feature in these cases
What genetic abnormality is seen in Burkitt’s lymphoma?
t (8;14) seen in Burkitt’s lymphoma
MYC oncogene is translocated to an immunoglobulin gene
What genetic abnormality is seen in Mantle cell lymphoma?
t(11;14) is seen in mantle cell lymphoma
deregulation of BCL-1 gene
What genetic abnormality is seen follicular lymphoma?
t(14;18) - increased BCL-2 transcription
What are some intravascular causes of haemolytic anaemia?
mismatched blood transfusion
G6PD deficiency*
red cell fragmentation: heart valves, TTP, DIC, HUS
paroxysmal nocturnal haemoglobinuria
cold autoimmune haemolytic anaemia
Extravascular causes of haemolysis
haemoglobinopathies: sickle cell, thalassaemia
hereditary spherocytosis
haemolytic disease of newborn
warm autoimmune haemolytic anaemia
What does haptoglobin do?
Binds to free Hb which is released in intravascular haemolysis - hence depleted in intravascular causes
What happens to free Hb once haptoglobin is saturated? how is this detected?
As haptoglobin becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by Schumm’s test).
Bloods in Warfarin?
PT - Prolonged
APTT - Normal
Bleeding time - Normal
Plts - Normal
Bloods in Aspirin use?
Bleeding time - prolonged
PT - Normal
APTT - Normal
Plts - Normal
Bloods in heparin use?
APTT - Prolonged
PT - Normal (usually) / prolonged
Bleeding time - normal
Plts - Normal
How does ITP present?
Commonly in older females - isolated thrombocytopaenia usually incidentally on bloods
If sx:
- petechiae, purpura, bleeding (epistaxis) -> catastrophic bleeding is not common
What is Evans syndrome?
When ITP is associated with AIHA
How can you distinguish between Ig in Cold and Warm AIHA?
Warm = Greece IgG
Cold = Moscow IgM
What can be seen in blood film in hereditary spherocytosis post surgery and why?
Howell-Jolly bodies - these are remnants of RBC nucleus normally removed by spleen
Hence visible post splenectomy
What causes raised MCHC?
Hereditary spherocytosis
AIHA - associated w/ spherocytosis
What causes reduced MCHC
Microcytic anaemia eg IDA
What is disproportionate microcytic anaemia? when is this seen?
This is when MCV is far more reduced than drop in Hb
Seen in B-thalassaemia trait
Blood film findings in hyposplenism? examples?
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
eg. coeliac / post-splenectomy
Bloods film features in IDA?
target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells
‘Tear drop’ poikilocytes are suggestive of which condition?
Myelofibrosis
What blood film feature is found in intravascular haemolysis?
Schistocytes
What are hypersegemented neutrophils suggestive of?
Megaloblastic anaemia
What are some examples of aromatase inhibitors? main adverse effects?
Anastrazole and Letrozole - used in ER +ve breast ca -> prevents androgen conversion into oestrogens (reducing peripheral oestrogen synthesis)
Adverse:
- Osteoporosis -> DEXA recommended when initiating
- Hot flushes
- Arthralgia + Myalgia
- Insomnia
Which medication should be used for chemo associated N+V?
Low risk - metoclopramide
High risk - 5HT3 antagonists eg ondansetron esp combined w dex
What genetic condtion is associated w young age tumours esp sarcomas + leukaemias? gene?
Li-Fraumeni Syndrome
p53 gene (sarcoma <45y & 1st degree relative any cancer + other family member any cancer <45y or sarcoma any age)
What is BRCA2 associated with in men?
Prostate Ca
Main symptoms of SVCO?
MOST COMMON - Dyspnoea
Swelling of face, neck and arms - ?oedema
Headaches - worse in AM
Visual disturbance
Pulseless JV distension
What is a leukaemoid reaction?
Presence of immature cells ef myeloblasts, promyelocytes and nucleated RBCs in peripheral blood
Due to infiltration of BM -> immature cells being pushed out
OR
Raised demand for new cells
What are some causes of leukaemoid reactions?
Severe infection
Severe haemolysis
Massive haemorrhage
Met Ca w/ BM infiltraion
How can you distinguish between leukaemoid reaction + myelofibrosis and CML?
Leucocyte Alkaline phosphatase (LAP) - raised in reactive response
CML - Low LAP
Leukaemoid reaction + Myelofibrosis - High LAP
What is HTLV1 and what ca is it associated with?
HTLV1 = human T cell lymphotropic virus
Associated w/ Adult T cell leukaemia
What ca is EBV associated with?
Hodgkins lymphoma
Burkitts lymphoma
Nasopharyngeal caricinoma
What ca is associated with HIV-1
High grade B cell lymphoma
What ca is associated w malaria?
Burkitts lymphoma
Starry sky appearance on microscopy suggests which ca? what does this describe?
Burkitts lymphoma
Starry sky = lymphocyte sheets interspersed w/ macrophages with dead apoptotic tumour cells
Electrolyte imbalances in Tumour Lysis Syndrome?
hyperkalaemia
hyperphosphataemia
hypocalcaemia
hyperuricaemia
acute renal failure
How can you distinguish between aplastic crises and sequestration crises in Sickle cells in blood test?
Aplastic - BM suppression -> reduced reticulocyte count
Sequestration - raised reticulocyte count
Inheritance pattern and mutation in hereditary angioedema?
Ix?
Autosomally dominant
Low plasma levels of C1-inhibitor (C1 inh, C1 esterase inhibitor)
Ix - Low C2 and C4 - even between attacks
What are the main adverse effects associated with Cisplatin?
Nephrotoxicity - main dose limiting issue
Ototoxicity
Peripheral neuropathy
Hypomagnesaemia
Features of TTP? Protein involved?
microangiopathic haemolytic anaemia, thrombocytopaenic purpura, neurological dysfunction, renal dysfunction and fever
Inhibition of ADAMTS13 - cleaves the large multimers of vWF
What are the features of transfusion associated graft v host disease? how can this risk be reduced?
GVHD - graft v host disease
G - Gut (enteritis)
V
H - Hepatitis
D - Dermatitis
Irradiated blood products -
Which drugs can trigger haemolysis in those with G6PD deficiency?
Anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
Mx of APML?
All-trans retinoic acid (ATRA) - forces immature granulocytes into maturation
This resolves a blast crisis before definitive chemo
What does anaemia with raised reticulocytes tell us?
Bleeding or haemolysis
low antibody levels, specifically in immunoglobulin types IgG, IgM and IgA. → recurrent chest and other infections
Is suggestive of which disorder?
Common variable immunodeficiency
can predispose to AI disorders + lymphoma
Most common sx of SVCO?
Dyspnoea
What does Rituximab target?
Mab targeting CD20 on B cells -> B cell depletion + immunosuppression
When is leucocyte alkaline phosphatase low?
chronic myeloid leukaemia
pernicious anaemia
paroxysmal nocturnal haemoglobinuria
infectious mononucleosis
When is leucocyte alkaline phosphatase raised?
myelofibrosis
leukaemoid reactions
polycythaemia rubra vera
infections
steroids, Cushing’s syndrome
pregnancy, oral contraceptive pill
What are the different translocations involved in blood cancers?
T8: 14 –> Burkitts (also has 8 letters)
T8: 21 –> Acute Myeloid L (before C)
T9: 22 –> Chronic Myeloid L
T11:14 –> Cell (Mantle) ‘Middle’ Lymphoma - 11 looks like mantlepiece
T14:18 –> Follicular Lymphoma
T15:17 –> Promyelocytic
+ Lymphomas always have a 14
Which ca shows raised levels of bombesin?
Small cell lung carcinoma
Gastric ca
Neuroblastoma
Which ca has raised levels of S-100?
Melanomas
Schwannomas
How can you distinguish between Waldenstroms macroglobulinaemia and MM?
Waldenstrom’s macroglobulinaemia (type of NHL) - Organomegaly with no bone lesions
Multiple myeloma - Bone lesions with no organomegaly
MoA of Irinotecan? adverse effect?
Inhibits topoisomerase I which prevents relaxation of supercoiled DNA
Can lead to myelosuppression
MoA of cyclophosphamide? Adverse effects?
Cyclophosphamide - alkylating agent causing crosslinking in DNA
Can cause haemorrhagic cystitis, myelosuppression and transitional cell ca
Which cytotoxic drugs act on microtubules?
Vincristine + vinblastine - inhibits formation of microtubules
Docetaxel - prevents microtubule depolymerisation + disassembly -> decreasing free tubulin
What is Meigs syndrome?
Ovarian fibroma associated w pleural effusion + ascites
Most common organism in neutropenic sepsis?
Coagulase+ve Gram +ve cocci usually staph epidermis then other staph and strep species
Activated protein C resistance is also known as?
Factor V leiden
Antiphospholipid syndrome in pregnancy mx?
Aspirin + LMWH
Anti-emetic that is a neurokinin1 receptor blocker (NK1)?
Aprepitant
How do Metoclopramide, domperidone, and haloperidol act as anti-emetics?
Dopamine blocking effecs
How are acute haemolytic crises managed in hereditary spherocytosis?
Generally supportive - IVF + high dose folic acid
Transfusion if necessary
What effect does the philipdelphia chromosome have on mortality in blood cancers?
Philadelphia translocation, t(9;22):
good prognosis in CML
poor prognosis in AML + ALL
recurrent bacterial infections (e.g. Chest)
eczema
thrombocytopenia
Is suggestive of which condition? blood also show what? gene?
Wiskott-Aldrich syndrome - primary immunodeficiency due to combined B+T cell dysfunction (mutated WASP gene)
Also see low IgM
What is the major determining factor in the use of cryoprecipitate in bleeding?
Fibrinogen levels <1.5g/L
Which type of hodgkins lymphoma has the best and worst prognosis?
Lymphocyte predominant - best
Lymphocyte depleted - worst
Mixed cellularity
Nodular sclerosing
Both offer good prognosis
Ix of choice for CLL?
Immunophenotyping - flow cytometry
Smudge / Smear cells are seen in which haem condition?
CLL
Mx of ITP?
Platelet count >30*109/L = Observation
Platelet count <30*109/L = Oral prednisolone
Emergency treatment: life-threatening or organ threatening bleeding = Platelet transfusion, IV methylprednisolone and intravenous immunoglobulin
What is Evans syndrome?
ITP associated with AIHA
What is given to prevent haemorhagic cystitis seen with cyclophosphamide?
Mesna
Raynauds is seen in which type of cryoglobulinaemia?
Type 1
What should be used instead of an osmotic fragility test for hereditary spherocytosis?
EMA binding test
What are the diagnostic criteria for MM?
The diagnostic criteria for multiple myeloma requires one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of multiple myeloma.
Major criteria
Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine
Minor criteria
10% to 30% plasma cells in a bone marrow sample.
Minor elevations in the level of M protein in the blood or urine.
Osteolytic lesions (as demonstrated on imaging studies).
Low levels of antibodies (not produced by the cancer cells) in the blood.
How can you distinguish between MM and MGUS?
Differentiating features from myeloma
> normal immune function
> normal beta-2 microglobulin levels
> lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
> stable level of paraproteinaemia
> no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)
the presence of HCV infection (PCR positive), positive rheumatoid factor, low complement levels, and purpuric rash
Suggests which condition?
hepatitis C virus (HCV) associated mixed cryoglobulinaemia - TYPE 2
What medications can be safe in G6PD deficiency?
hepatitis C virus (HCV) associated mixed cryoglobulinaemia
Mx of acute and prophylaxis against hereditary angioedema?
Acute - IV C1 inh concentrate or FFP if not available
Prophylaxis - Anabolic steroid danazol
What are the features of lead poisoning?
abdominal pain
peripheral neuropathy (mainly motor)
neuropsychiatric features
fatigue
constipation
blue lines on gum margin (only 20% of adult patients, very rare in children)
How can you distinguish between TTP and HUS?
TTP has neurological signs too
Most common presenting complaint in myelofibrosis?
lethargy
Where is B12 absorbed?
B12 is actively absorbed in terminal ileum
What can occur in 10-15% of those with Waldenstroms macroglobulinaemia?
Hyperviscosity syndrome
Why does CKD increase risk of VTE?
Loss of antithrombin III - most common cause of this deficiency
Mx of neoplastic spinal cord compression?
High dose oral dex whilst awaiting MRI
Difference between TACO and TRALI?
TACO - HTN
TRALI - Hypotension
Which drugs can cause methaemoglobinaemia?
sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine
B12 replacement regime?
if no neurological involvement 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months
if a patient is also deficient in folic acid then it is important to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord
Can you get aplastic anaemia with PNH?
Yes - though not necessarily directly
What are the main bloods in tumour lysis syndrome?
Raised urate
Raised K
Raised PO4
Low Ca
Laboratory TLS is defined by ≥2 of the following occurring 3 days prior or 7 days post initiation of treatment for cancer:
Uric acid ≥ 476 µmol/L or 25% increase from baseline
Potassium ≥ 6.0 mmol/L or 25% increase from baseline
Phosphate ≥ 1.45 mmol/L (adults) or ≥2.1 mmol/L (children) or 25% increase
Calcium ≤ 1.75 mmol/L or 25% decrease from baseline
Clinical TLS is defined by: laboratory TLS plus at least one of the following:
Creatinine ≥1.5 x the upper limit of normal
Cardiac arrhythmia
Seizure
Sudden death
How can you distinguish between higher spinal cord compression than L1 v cauda equina?
Presence of UMN signs
Mx of alcoholic hepatitis?
Glucocorticoids eg pred
Pentoxyphylline sometimes used -not shown to improve surivial rates whereas glucocorticoids do
Mx of hyatid cysts?
Surgery is mainstay - cyst walls must not be ruptured during removal and contents sterilised first
Different genotypes of sickle cell disease?
normal haemoglobin: HbAA
sickle cell trait: HbAS
homozygous sickle cell disease: HbSS - severe disease
milder sickle cell disease: HbSC - Some patients inherit one HbS and another abnormal haemoglobin (HbC)
in the deoxygenated state the HbS molecules polymerise and cause RBCs to sickle
HbAS patients sickle at p02 2.5 - 4 kPa
HbSS patients at p02 5 - 6 kPa
Drug causes of pancytopaenia?
If you give a patient bad news about him suffering from Panyctopenia- It will cause ACS.
Antibiotics: Trimethoprim, Chloramphenicol.
AED: Carbamazepine (can also cause agranulocytosis)
anti-rheumatoid: Penicillamine, Gold
Carbimazole
Cytotoxics
Sulfonylureas: Tolbutamide
AAA CC S
Which lung ca can secrete HCG? prognosis
Large cell lung ca - anaplastic + poorly differentiated w/ poor prognosis
Which non small cell lung ca is found central v peripheral
central - SCC
peripheral - adeno + large cell
What are the different primary immunodeficiencies affecting T+B cells?
SCID,
Wiskott-Aldrich syndrome,
ataxic telangiectasia
Hyper IgM Syndromes
B-cell primary immunodeficiencies?
Common variable immunodeficiency
Bruton’s (x-linked) congenital agammaglobulinaemia
Selective immunoglobulin A deficiency
Neutrophil associated primary immunodeficiencies?
Chronic granulomatous disease
Chediak-Higashi syndrome
Leukocyte adhesion deficiency
What ethnicity is benign ethnic neutropaenia seen in?
black African and Afro-Caribbean ethnicity
CLL why may there be increased risk of infections?
hypogammaglobulinaemia
Features + mx of essential thrombocytosis?
platelet count > 600 * 109/l
both thrombosis (venous or arterial) and haemorrhage can be seen
a characteristic symptom is a burning sensation in the hands
a JAK2 mutation is found in around 50% of patients
Overlaps with CML, polycythaemia + myelofibrosis
Mx = hydroxyurea (hydroxycarbamide), Inf-a in younger pts + low dose aspirin to reduce thrombosis risk
Which cytotoxic agent - Degrades preformed DNA? adverse effect?
Bleomycin
Can lead to lung fibrosis
Which cytotoxic drug Causes cross-linking in DNA?
Cisplatin
Which cytotoxic drug Inhibits ribonucleotide reductase, decreasing DNA synthesis?
Hydroxyurea (hydroxycarbamide)
Which Ca is Warm AIHA associated with? Blood film findings?
CLL is associated w warm AIHA in 10-15% of cases
Blood film can be normal as haemolysis occurs extravascularly
What is the main advantage of using capecitabine instead of fluorouracil?
This is a pro drug for 5-FU means it can allow for oral administration hence OP mx
Possible mx in ITP?
oral prednisolone (80% of patients respond)
splenectomy if platelets < 30 after 3 months of steroid therapy
IV immunoglobulins
immunosuppressive drugs e.g. cyclophosphamide
Widespread lymphadenopathy + multi organ problems - what dx?
IgG4 disease
Examples include:
Riedel’s Thyroiditis
Autoimmune pancreatitis
Mediastinal and Retroperitoneal Fibrosis
Periaortitis/periarteritis/Inflammatory aortic aneurysm
Kuttner’s Tumour (submandibular glands) & Mikulicz Syndrome (salivary and lacrimal glands)
Possibly sjogren’s and primary biliary cirrhosis
Haemodilution in pregnancy - bloods show what?
Normocytic anaemia
Most common cause of SVCO?
Primary tumour - Most common is non-small cell lung cancer, small cell lung cancer and non-Hodgkin lymphoma.
CALR is associated with what?
JAK2 -ve essential thrombocytosis
Hypo / hyperdiploidy in ALL effect on prognosis
Hypo = poor prognosis
Hyper = good prognosis
SCID prognosis?
Patients are afflicted with significant infections from infancy and do not survive into childhood unless the condition is diagnosed early and treated with bone marrow transplantation.
Chromosome changes and prognosis in CLL? Other poor prognostic features?
Short arm of chromosome 17 (del 17p) - Poor prognosis
long arm of chromosome 13 (del 13q) - Good prognosis
Poor prognostic
male sex
age > 70 years
lymphocyte count > 50
prolymphocytes comprising more than 10% of blood lymphocytes
lymphocyte doubling time < 12 months
raised LDH
CD38 expression positive
TP53 mutation
Desmopressiin (DDAVP) use in VWD?
desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
Poor prognosis in HL?
‘B’ symptoms also imply a poor prognosis
weight loss > 10% in last 6 months
fever > 38ºC
night sweats
Other factors associated with a poor prognosis identified in a 1998 NEJM paper included:
age > 45 years
stage IV disease
haemoglobin < 10.5 g/dl
lymphocyte count < 600/µl or < 8%
male
albumin < 40 g/l
white blood count > 15,000/µl
Reed sternberg cells seen in?
Hodgkins lymphoma
Mx of CLL, CML, Hodgkins and NHL?
FCR (Fludarabine, Cyclophosphamide and Rituximab) - CLL (Ibrutinib or Venetoclax instead of FCR if TP53)
AVBD (Doxorubicin, bleomycin, vinblastine, dacarbazine) - Hodgkins
R-CHOP - Non-Hodgkins
Imatinib - CML
Anterior mediastinal mass + symptoms of myasthenia
Suggestive of what dx?
Thymoma
Ix findings in VWD?
prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin
Ham’s test used for what?
diagnose paroxysmal nocturnal hemoglobinuria (PNH)
What to check when thinking of lead poisoning?
Coproporphyrin
He is initially treated with low-molecular weight heparin but is switched after three days to warfarin. He then develops necrotic skin lesions on his lower limbs and forearms.
Which one of the following conditions is characteristically associated with this complication?
development of necrotic skin lesions in this patient after being switched to warfarin suggests the presence of a condition known as warfarin-induced skin necrosis (WISN)
Associated with Protein C deficiency
What are thymomas associated with?
myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH
When to use skin patch v skin prick testing?
If it goes in you (food) > skin prick T1 HS 15-30 mins
If it goes on you (e.g. contact) > skin patch
MoA of HPV 16/18 mediated cervical ca?
HPV 16 & 18 produces the oncoproteins E6 and E7 genes respectively which inhibit TSGs
E6 inhibits p53 tumour suppressor gene
E7 inhibits RB tumour suppressor gene
Defect in which enzyme in AIP and PCT?
Acute intermitten porphyria - porphobilinogen deAminase
PCT - uroporphyrinogen deCarboxylase
Indications for tx in CLL?
progressive marrow failure: the development or worsening of anaemia and/or thrombocytopenia
massive (>10 cm) or progressive lymphadenopathy
massive (>6 cm) or progressive splenomegaly
progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months
systemic symptoms: weight loss > 10% in previous 6 months, fever >38ºC for > 2 weeks, extreme fatigue, night sweats
autoimmune cytopaenias e.g. ITP
What is the most common inherited bleeding disorder?
VWD
Prognostic marker in Myeloma?
B2 microglobulin - raised levels imply poor prognosis.
Low levels of albumin are also associated with a poor prognosis
Which haem malignancy shows:
there is an increase in granulocytes at different stages of maturation +/- thrombocytosis?
CML
AML -less time, less platelets,
CML - more time, more platelets
Band cells are an immature form of neutrophils
How are acute painful vaso occlusive crises diagnosed in sickle cell?
Diagnosed clinically
ESR in polycythaemia rubra vera?
Low - increased number of red blood cells that reduce the relative proportion of plasma and thus sedimentation
AML poor prognostic features?
AML poor prognostic features:
> 60 years
20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7
TTP mx?
Plasma exchange = 1st line
Steroids, immunosuppressants and Vincristine may also help
Rasburicase - use case and moa?
Used to reduce risk of tumour lysis syndrome in high risk pts (allopurinol given in lower risk - DO NOT GIVE TOGETHER AS REDUCES EFFECT OF RASBURICASE)
Recombinant urate oxidase -> conversion of urate to allantoin
Primary peritoneal ca - marker?
Ca125 + monitor disease progression / response to tx
The universal donor of fresh frozen plasma is what?
AB RhD -ve
Test for diagnosis and prognosis in AML?
Diagnosis - Immunophenotyping
Prognosis - Cytogenetics
What are the most common types of transformations seen in patients with polycythaemia vera?
5-15% progress to myelofibrosis or AML
When to use VKAs for DVT?
if renal impairment is severe (e.g. < 15/min) then LMWH, unfractionated heparin or LMWH followed by a VKA
if the patient has antiphospholipid syndrome (specifically ‘triple positive’ in the guidance) then LMWH followed by a VKA should be used
Is Histamine implicated in Hereditary angioedema (HAE)?
No
However Kallikrein + High molecular weight kininogen + CNHesterase inh + bradykinin are implicated
Other than High LAP what are other features of leukaemoid reactions?
toxic granulation (Dohle bodies) in the white cells
‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus
Which one of the following causes of thrombophilia is associated with resistance to heparin?
Antithrombin III deficiency
anti-Xa levels should be monitored carefully to ensure adequate anticoagulation
Causes of warm v cold AIHA?
Causes of cold AIHA
- neoplasia: e.g. lymphoma
- infections: e.g. mycoplasma, EBV
Causes of Warm AIHA
- Idiopathic
- autoimmune disease: e.g. systemic lupus erythematosus*
- neoplasia (lymphoma, chronic lymphocytic leukaemia)
- drugs: e.g. methyldopa
Prevalence of Factor V leiden?
5%
When to discharge after anaphylaxis?
fast-track discharge (after 2 hours of symptom resolution):
- good response to a single dose of adrenaline
- complete resolution of symptoms
- has been given an adrenaline auto-injector and - trained how to use it
- adequate supervision following discharge
minimum 6 hours after symptom resolution
- 2 doses of IM adrenaline needed, or
- previous biphasic reaction
minimum 12 hours after symptom resolution
- severe reaction requiring > 2 doses of IM adrenaline
- patient has severe asthma
- possibility of an ongoing reaction (e.g. slow-release medication)
- patient presents late at night
- patient in areas where access to emergency access care may be difficult
- observation for at 12 hours following symptom resolution
What causes sequestration crises and what may be seen?
Sequestration crises occur due to sickling of cells within the splenic vasculature, and resultant trapping of significant blood volume within the spleen itself.
You can see splenomegaly and hypotension
Sideroblastic anaemia can be caused by what? what stain to use?
Congenital cause: delta-aminolevulinate synthase-2 deficiency
Acquired causes
myelodysplasia
alcohol
lead
anti-TB medications
Ix = Prussian blue / Perls staining will show ringed sideroblasts
Can INF-A inhibtors be used for Hairy cell leukaemia (rare B cell disorder)?
No
NHL is associated with which AIHA?
Warm AIHA + Cold AIHA - look at IgG v IgM
The patient’s symptoms of purplish discolouration of her peripheries and nose, along with fatigue and lethargy, combined with the laboratory results showing spherocytes on her blood film
? dx
Possible AIHA
vCJD risk in blood transfusion?
There had previously been a small risk of vCJD transmission but the risk has now been eliminated through screening
a + b thalassaemia gene located where?
alpha thalassemia = chromosome 16
beta thalassemia = chromosome 11
Mx of aplastic anaemia?
Blood products
Infection prevention
Anti-thymocyte globulin (ATG) and anti-lymphocyte globulin (ALG)
Stem cell transplants
Mx of hereditary spherocytosis?
Acute haemolytic crises - supportive + transfusion if req
Long term - Folate replacement + splenectomy
ECOG - performance status for future onc therapies scale
0 Fully active, able to carry on all pre-disease performance without restriction
1 Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature, e.g., light house work, office work
2 Ambulatory and capable of all selfcare but unable to carry out any work activities; up and about more than 50% of waking hours
3 Capable of only limited selfcare; confined to bed or chair more than 50% of waking hours
4 Completely disabled; cannot carry on any selfcare; totally confined to bed or chair
5 Dead
Causes of hyposplenism?
splenectomy
sickle-cell
coeliac disease, dermatitis herpetiformis
Graves’ disease
systemic lupus erythematosus
amyloid
Which BRCA causes prostate ca in men?
BRCA 2
Features and cause of myelofibrosis?
e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc
a myeloproliferative disorder
thought to be caused by hyperplasia of abnormal megakaryocytes
the resultant release of platelet derived growth factor is thought to stimulate fibroblasts
haematopoiesis develops in the liver and spleen
Latex fruit syndrome?
It is recognised that many people who are allergic to latex are also allergic to fruits, particularly banana, pineapple, avocado, chestnut, kiwi fruit, mango, passion fruit and strawberry.
experiencing intense pruritis especially after a hot shower and her skin appearing red and flushed over the past year.
Suggests what?
PML
Methaemoglobinaemia mx?
NADH methaemoglobinaemia reductase deficiency: ascorbic acid
IV methylthioninium chloride (methylene blue) if acquired
