Hematology & Oncology Flashcards
1
Q
In Sideroblastic anaemia
Which stain should be applied to a blood film to show ring sideroblasts ?
A
Perl’s stain
2
Q
Causes of Sideroblastic anaemia
A
Congenital cause:
-delta-aminolevulinate synthase-2 deficiency
Acquired causes
- MDS
- alcohol
- lead
- anti-TB medications
3
Q
Treatment of Sideroblastic anaemia?
A
Management
- supportive
- treat any underlying cause
- pyridoxine may help
4
Q
Vit B12 Absorbed in …….1…. After binding to …….2…. Which secreted from ……….3…….
A
- absorbed in the terminal ileum
- intrinsic factor
- secreted from parietal cells in the stomach
5
Q
Causes of vitamin B12 deficiency
A
- pernicious anaemia: most common cause
- post gastrectomy
- vegan diet or a poor diet
- disorders/surgery of terminal ileum (site of absorption)
Crohn’s: either diease activity or following ileocaecal resection - metformin (rare)
6
Q
Features of vitamin B12 deficiency?
A
- macrocytic anaemia
- sore tongue and mouth
- neurological symptoms
the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia - neuropsychiatric symptoms: e.g. mood disturbances
7
Q
Management of B12 deficiency
A
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
8
Q
Causes of Microcytic anaemia
A
- iron-deficiency anaemia
- thalassaemia*
- congenital sideroblastic anaemia
anaemia of chronic disease (more commonly a normocytic, normochromic picture) - lead poisoning
9
Q
In normal haemoglobin level associated with a microcytosis. In patients not at risk of thalassaemia, this should raise the possibility of ……
A
polycythaemia rubra vera which may cause an iron-deficiency secondary to bleeding.
10
Q
New onset microcytic anaemia in elderly patients should be urgently investigated to exclude……..
A
underlying malignancy
11
Q
Megaloblastic causes of macrocytic anaemia
A
- vitamin B12 deficiency
- folate deficiency
- secondary to methotrexate
12
Q
Normoblastic causes of macrocytic anaemia
A
- alcohol
- liver disease
- hypothyroidism
- pregnancy
- reticulocytosis
- myelodysplasia
- drugs: cytotoxics
13
Q
Causes of normocytic anaemia include
A
- anaemia of chronic disease
- chronic kidney disease
- aplastic anaemia
- haemolytic anaemia
- acute blood loss
14
Q
Vitamin B12 deficiency can be classified into the following three groups using serum vitamin B12 level:
Deficiency is likely: < ….
Deficiency is probable: ……
Deficiency is unlikely: >…..
A
- Deficiency is likely: <148 picomole/L
- Deficiency is probable: 148 to 258 picomole/L
- Deficiency is unlikely: >258 picomole/L
15
Q
What is the mechanism of anemia of chronic disease?
A
Decreased availability of iron , relatively decreased level of erythropoietin and mild decreased in the lifespan of RBC
16
Q
Increased in Hepcidin level leads to
A
Reduce release of iron from macrophages + dietary iron absorption
17
Q
Hepcidin synthesized in
A
Liver
18
Q
Hepcidin
1. High in ………
2. Low in …….
A
- Inflammation»_space;> decreased serum iron
- Hemochromatosis > low Hepcidin level»_space;> iron overload
19
Q
Hepcidin inhibits iron transport by binding to …….
A
the iron export channel ferroportin
20
Q
CKD anemia when GFR <
A
GFR < 35
21
Q
Metabolic acidosis inhibits conversion of ferric iron (FE 3+) to absorbable form …..
A
Ferrous iron ( Fe2+) in the duodenum»_space;> reduced iron absorption
22
Q
Patient on Erythropoietin or hemodialysis is require ? Which of the following
- Iv iron
Or - Oral iron
A
IV iron
23
Q
Target Hb in CKD ?
A
Hb 10-12
24
Q
In aplastic anemia bone marrow assessment is best made on …
A
Trephine biopsy, which often shows replacement of the normal cellular marrow by ( fatty marrow)
25
Hypocelullar bone marrow seen in …
Aplastic anemia
26
Causes of aplastic anemia
Idiopathic
Fanconi anemia
Dyskeratosis congenita
Drugs: cytotoxic, chloramphenicol, sulphonamides , phenytoin, GOLD
Infection: parvovirus, hepatitis
Radiation
27
Treatment of aplastic anemia
- Supportive: blood products, prevention and treatment of infection
- anti thymocyte and anti lymphocyte globulin
- immunosuppressive agents ( ciclosporin) may be given
- Allogeneic stem cell transplantation
28
anti thymocyte and anti lymphocyte globulin can cause
Serum sickness ( fever , rash , arthralgia )
29
Intravascular haemolysis: causes
1. mismatched blood transfusion
2. G6PD deficiency*
3. red cell fragmentation: heart valves, TTP, DIC, HUS
4. paroxysmal nocturnal haemoglobinuria
5. cold autoimmune haemolytic anaemia
30
Extravascular haemolysis: causes
1. haemoglobinopathies: sickle cell, thalassaemia
hereditary spherocytosis
haemolytic disease of newborn
warm autoimmune haemolytic anaemia
31
6 features of Fanconi anemia ?
- Autosomal recessive
- Aplastic anemia
- Risk of AML
- Neurological manifestation
- Skeletal abnormalities
- Skin pigmentation ( café au lait spots )
32
Normal types of Hb
1. Adult HbA ( 2 alpha & 2 Beta) normal > 95%
2. Adult HbA2 ( 2 alpha & 2 gamma ) normal < 3.5 %
3. Fetal Hb ( 2 alpha & 2 delta ) normal < 1.5 %
33
1. Alpha globulin gene are located on …..
2. Beta globulin gene are located on …..
I. Chromosome 16
2. Chromosome 11
34
Types of alpha thalassemia
1. Silent carrier: single alpha gene mutation ( patient is Healthy)
2. Alpha thalassemia trait ( minor ) 2 gene mutation ( Hb ~ 10 & microcytosis& normal Hb Electrophoresis )
3. HbH: 3 gene mutation: severe anemia
4. Alpha thalassemia major ( Hb barts or hydrops fetalis ) 4 gene mutation >>> death in utero.
35
Beta thalassemia trait
- Autosomal recessive
- Mild hypochromic , microcytic anemia
- Asymptomatic
- HbA2 raised > 3.5 %
36
Beta thalassemia features
1. Microcytic anemia
2. HbA2 & HbF raised
3. HbA absent
37
Beta thalassemia management
Repeated transfusion
- this leads to iron overload >> organ failure
- iron chelation therapy is therefore important
38
Norma serum gastrin level excludes …..
Pernicious anemia
39
Causes of warm AIHA
idiopathic
autoimmune disease: e.g. systemic lupus erythematosus*
neoplasia
lymphoma
chronic lymphocytic leukaemia
drugs: e.g. methyldopa
*MAN CIL
40
Management of warm AIHA
treatment of any underlying disorder
steroids (+/- rituximab) are generally used first-line
41
The antibody in cold AIHA is usually IgM and causes haemolysis best at ..... degree C.
at 4 degree Celsius.
42
In which type of autoimmune hemolytic anema,Patients respond less well to steroids ?
Cold AIHA
43
Causes of cold AIHA
neoplasia: e.g. lymphoma
infections: e.g. mycoplasma, EBV
44
FFP indications
1. PT ratio or activated partial thromboplastin time (APTT) ratio > 1.5
2. can be used prophylactically in patients undergoing invasive surgery where there is a risk of significant bleeding.
45
Cryoprecipitate contains ?
concentrated Factor VIII:C,
von Willebrand factor,
fibrinogen,
Factor XIII and
fibronectin, produced by further processing of Fresh FFP.
46
HbA1C higher then expected in
Due to increased RBC span
1. B12 deficiency
2. Folate deficiency
3. IDA
4. Splenectomy
47
neonatal jaundice is often seen
intravascular haemolysis
gallstones are common
splenomegaly may be present
Heinz bodies on blood films. Bite and blister cells may also be seen
G6PD deficiency
48
The diagnosis of G6pD deficiency is made by
G6PD enzyme assay
Should be checked 3 months after acute episode of hemolysis
49
Drugs causing hemolysis in G6pD deficiency
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
50
Drugs safe in G6pD deficiency
penicillins
cephalosporins
macrolides
tetracyclines
trimethoprim
51
Diagnosis of hereditary spherocytosis by
EMA binging test and the cryohaemolysis test
for atypical presentations electrophoresis analysis of erythrocyte membranes is the method of choice
52
Management of hereditary spherocytosis
acute haemolytic crisis:
- treatment is generally supportive
- transfusion if necessary
longer term treatment:
- Folate replacement
- splenectomy
53
PNH diagnosis by
Flow cytometry of blood to detect low levels of CD 59 & 55
54
treatment of PNH
blood product replacement
anticoagulation
eculizumab, a monoclonal antibody directed against terminal protein C5, is currently being trialled and is showing promise in reducing intravascular haemolysis
stem cell transplantation
55
Blood film
In Hyposplenism e.g. post-splenectomy, coeliac disease
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
56
Blood film in IDA
target cells
'pencil' poikilocytes
if combined with B12/folate deficiency a 'dimorphic' film occurs with mixed microcytic and macrocytic cells
57
Blood film in MF
'tear-drop' poikilocytes
58
Blood film in Intravascular haemolysis
Schistocytes
59
Blood film in megaloblastic anaemia
Hypersegmented neutrophils
60
Heinz bodies in
G6PD
Alpha thalassemia
61
Howell jolly bodies in
Hyposplenism
62
Burr cells ( echinocytes ) in
Uremia
Pyruvate kinase deficiency
63
Acanthocytes in
Abetalipoproteinemia
64
Basophilic stippling
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
65
Spherocytes seen in
Hereditary spherocytosis
Autoimmune hemolytic anaemia
66
Target cells in
Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease
67
Schistocytes ('helmet cells') in
Intravascular haemolysis
Mechanical heart valve
DIC
68
McArdle'S Disease
Muscle pain
Cramps
Autosomal recessive
Deficiency of myophosphorylase
Low lactate
Exercise related
69
Drug-induced pancytopaenia
cytotoxics
antibiotics: trimethoprim, chloramphenicol
anti-rheumatoid: gold, penicillamine
carbimazole*
anti-epileptics: carbamazepine
sulphonylureas: tolbutamide
*causes both agranulocytosis and pancytopaenia
70
Features of TTP
fever
fluctuating neuro signs (microemboli)
microangiopathic haemolytic anaemia
thrombocytopenia
renal failure
71
Causes of TTP
post-infection e.g. urinary, gastrointestinal
pregnancy
drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
tumours
SLE
HIV
72
TTP MANAGEMENT
no antibiotics - may worsen outcome
plasma exchange is the treatment of choice
steroids, immunosuppressants
vincristine
73
Features of Wiskott-Aldrich syndrome
recurrent bacterial infections (e.g. Chest)
eczema
thrombocytopaenia
low IgM levels
74
Wiskott-Aldrich syndrome causes primary immunodeficiency due to a combination..........
B and T cell dysfunction
75
ITP MANAGEMENT
- first-line treatment for ITP is oral prednisolone ( 80 % of patients respond )
- IVIG may also be used
it raises the platelet count quicker than steroids, therefore may be used if active bleeding or an urgent invasive procedure is required
- splenectomy if plt < 30 after 3 months
- immunosuppressive drugs : cyclophosphamide
76
What is Evan's syndrome
ITP in association with autoimmune haemolytic anaemia (AIHA)
77
In ITP
Antibodies are directed against................?
Glycoprotein IIb-Ib complex
78
Plt transfusion in active bleeding
1. Plt < 30 with clinical significant bleeding
2. Plt < 100 with severe bleeding or bleeding at critical sites , such as CNS
79
Plt transfusion in Pre-invasive procedure (prophylactic)
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
>100×109/L if surgery at critical site
80
What is the threshold of plt transfusion if no active bleeding or no planned invasive procedure
10
81
platelet transfusions have the highest risk of .................. compared to other types of blood product.
bacterial contamination
82
Features of hemophilia
haemoarthroses
haematomas
prolonged bleeding after surgery or trauma
83
PT and APTT and Bleeding time in hemophilia
Prolonged aPTT
Normal PT, Thrombin time and bleeding time
84
Von willebrand’s disease investigation
prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin
85
Von willebrand’s disease
Management
- tranexamic acid for mild bleeding
- desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
- factor VIII concentrate
86
desmopressin raises levels of vWF by inducing release of vWF from………?
Weibel-Palade bodies in endothelial cells
87
Treatment of DVT if the patient has active cancer
- previously LMWH was recommended
- the new guidelines now recommend using a DOAC, unless this is contraindicated
88
Treatment of DVT if renal impairment is severe (e.g. < 15/min)
LMWH, unfractionated heparin or LMWH followed by a VKA
89
Treatment of DVT if the patient has antiphospholipid syndrome (specifically 'triple positive' in the guidance)
LMWH followed by a VKA should be used
90
Pathophysiology of DVT/PE in pregnancy?
- increase in factors VII, VIII, X and fibrinogen
- decrease in protein S
- uterus presses on IVC causing venous stasis in legs
91
Management of DVT/PE in pregnancy?
- warfarin contraindicated
- S/C low-molecular weight heparin preferred to IV heparin (less bleeding and thrombocytopenia)
92
what is the most common cause of thrombophilia
- factor V Leiden (activated protein C resistance)
- prothrombin gene mutation: second most common cause
93
Features of protein C deficiency
- VTE
- skin necrosis following the commencement of warfarin: when warfarin is first started biosynthesis of protein C is reduced. This results in a temporary procoagulant state after initially starting warfarin, normally avoided by concurrent heparin administration. Thrombosis may occur in venules leading to skin necrosis
94
Antithrombin III inhibits …….
thrombin, factor X and IX.
It mediates the effects of heparin
95
Management of Antiphospholipid syndrome in pregnancy
- low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing
- low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually discontinued at 34 weeks gestation
- these interventions increase the live birth rate seven-fold
96
The best site for IM adrenaline injection is
the anterolateral aspect of the middle third of the thigh.
97
Refractory anaphylaxis defined as
respiratory and/or cardiovascular problems persist despite 2 doses of IM adrenaline
98
Serum tryptase levels are sometimes taken in such patients as they remain elevated for up to .............. following an acute episode of anaphylaxis
12 hours
99
Types of Cryoglobulinaemia
type I (25%):
monoclonal - IgG or IgM
associations: multiple myeloma, Waldenstrom macroglobulinaemia
type II (25%)
mixed monoclonal and polyclonal: usually with rheumatoid factor
associations: hepatitis C, rheumatoid arthritis, Sjogren's, lymphoma
type III (50%)
- polyclonal: usually with rheumatoid factor
-'associations: rheumatoid arthritis, Sjogren's
100
Features of Cryoglobulinaemia
- Raynaud's only seen in type I
- cutaneous ( vascular purpura, distal ulceration, ulceration)
- arthralgia
- renal involvement (diffuse glomerulonephritis)
101
Investigations of Cryoglobulinaemia
- High ESR
- Low complement ( especially C4)
102
Treatment of Cryoglobulinaemia
treatment of underlying condition e.g. hepatitis C
immunosuppression
plasmapheresis
103
Pulmonary causes of Eosinophilia
1. asthma
2. allergic bronchopulmonary aspergillosis
3. Churg-Strauss syndrome
4. Loffler's syndrome
5. tropical pulmonary eosinophilia
6. eosinophilic pneumonia
7. hypereosinophilic syndrome
104
Infective causes of Eosinophilia
1. schistosomiasis
2. nematodes: Toxocara, Ascaris, Strongyloides
3. cestodes: Echinococcus
105
Other causes of eosinophilia
1. drugs: sulfasalazine, nitrofurantoin
2. psoriasis/eczema
3. eosinophilic leukaemia (very rare)
106
Causes of Hyposplenism
1. splenectomy
2. sickle-cell
3. coeliac disease, dermatitis herpetiformis
4. Graves' disease
5. systemic lupus erythematosus
6. amyloid
107
Features of Hyposplenism
1. Howell Jolly bodies
2. Siderocytes
108
Causes of splenomegaly
myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher's syndrome
portal hypertension e.g. secondary to cirrhosis
lymphoproliferative disease e.g. CLL, Hodgkin's
haemolytic anaemia
infection: hepatitis, glandular fever
infective endocarditis
sickle-cell*, thalassaemia
rheumatoid arthritis (Felty's syndrome)
109
Causes of Massive splenomegaly
1. MF
2. CML
3. MALARIA
4. visceral leishmaniasis
5. Gaucher's syndrome
110
Methaemoglobinaemia describes haemoglobin which has been oxidised from ........
Fe2+ to Fe3+.
111
chocolate' cyanosis
dyspnoea, anxiety, headache
severe: acidosis, arrhythmias, seizures, coma
normal pO2 but decreased oxygen saturation
Features of.....?
Methaemoglobinaemia
112
Causes of Methaemoglobinaemia
Congenital causes
1. haemoglobin chain variants: HbM, HbH
2. NADH methaemoglobin reductase deficiency
Acquired causes
1. drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite 'poppers'), dapsone, sodium nitroprusside, primaquine
2. chemicals: aniline dyes
113
Treatment of Methaemoglobinaemia
- For NADH methaemoglobinaemia reductase deficiency: >>> ascorbic acid
- IV methylthioninium chloride (methylene blue) if acquired
114
Investigations of Hereditary angioedema
C1-INH level is low during an attack
low C2 and C4 levels are seen, even between attacks. Serum C4 is the most reliable and widely used screening tool
115
Management of Hereditary angioedema
acute
- HAE does not respond to adrenaline, antihistamines, or glucocorticoids
- IV C1-inhibitor concentrate, fresh frozen plasma (FFP) if this is not available
prophylaxis: anabolic steroid Danazol may help
116
Selective oEstrogen Receptor Modulators (SERM) is used in the management of ........
oestrogen receptor-positive breast cancer.
117
Selective oEstrogen Receptor Modulators (SERM)
Side effects
menstrual disturbance: vaginal bleeding, amenorrhoea
hot flushes - 3% of patients stop taking tamoxifen due to climacteric side-effects
venous thromboembolism
endometrial cancer
118
Anastrozole and letrozole are aromatase inhibitors that reduces peripheral oestrogen synthesis.
Side effects?
osteoporosis
NICE recommends a DEXA scan when initiating a patient on aromatase inhibitors for breast cancer
hot flushes
arthralgia, myalgia
insomnia
119
IgG4-related disease
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
120
Severity of neutropenia
Mild : 1.0 - 1.5
Moderate: 0.5 - 1.0
Severe: < 0.5
121
Causes of neutropenia
1. viral
- HIV
- Epstein-Barr virus
- hepatitis
2.drugs
- cytotoxics
- carbimazole
- clozapine
3. benign ethnic neutropaenia
- common in people of black African and Afro-Caribbean ethnicity
- requires no treatment
4. haematological malignancy
- MDS
- aplastic anemia
5. rheumatological conditions
- SLE, RA: in Felty's syndrome
6. severe sepsis
7. haemodialysis
122
Definition of neutropenic sepsis
Neutrophil coun < 0.5 plus one of the following
1. T > 38
2. Signs or symptoms consistent with clinically significant sepsis
123
Aetiology of neutropenic sepsis
coagulase-negative, Gram-positive bacteria are the most common cause, particularly Staphylococcus epidermidis
this is probably due to the use of indwelling lines in patients with cancer
124
Management of neutropenic sepsis
- antibiotics must be started immediately, do not wait for the WBC
- starting empirical antibiotic therapy with Tazocin immediately
- if patients are still febrile and unwell after 48 hours an alternative antibiotic such as meropenem is often prescribed +/- vancomycin
- if patients are not responding after 4-6 days the Christie guidelines suggest ordering investigations for fungal infections (e.g. HRCT), rather than just starting therapy antifungal therapy blindly
- there may be a role for G-CSF in selected patients
125
1. autosomal dominant
2. defect in porphobilinogen deaminase
3. female and 20-40 year olds more likely to be affected
4. typically present with abdominal symptoms, neuropsychiatric symptoms, HTN and tachycardia.
5. urine turns deep red on standing
What is the diagnosis?
Acute intermittent porphyria (AIP)
126
Acute intermittent porphyria (AIP), defect in
porphobilinogen deaminase
127
classically photosensitive rash with bullae, skin fragility on face and dorsal aspect of hands
urine: elevated uroporphyrinogen and pink fluorescence of urine under Wood's lamp
What is the diagnosis?
Porphyria cutanea tarda (PCT)
128
Treatment of Porphyria cutanea tarda (PCT)
Chloroquine
129
Porphyria cutanea tarda (PCT), the defect in ......
uroporphyrinogen decarboxylase
130
photosensitive blistering rash
abdominal and neurological symptoms
more common in South Africans
What is the diagnosis?
Variegate porphyria
131
Variegate porphyria, the defect in ......
protoporphyrinogen oxidase
132
Management of Acute intermittent porphyria
- avoiding triggers
- acute attacks
1. IV haematin/haem arginate
2. IV glucose should be used if haematin/haem arginate is not immediately available
133
In polycythaemia vera
ESR level is .....
Leukocyte alkaline phosphate.....
ESR LOW
Leukocyte alkaline phosphate high
134
Treatment of polycythemia Vera
- aspirin
reduces the risk of thrombotic events
- venesection
first-line treatment to keep the haemoglobin in the normal range
- chemotherapy
hydroxyurea - slight increased risk of secondary leukaemia
phosphorus-32 therapy
135
..........% of patients progress to acute leukaemia
5-15
136
Causes of thrombocytosis
1. reactive: platelets are an acute phase reactant - platelet count can increase in response to stress such as a severe infection, surgery. Iron deficiency anaemia can also cause a reactive thrombocytosis
2.malignancy
3.essential thrombocytosis, or myeloproliferative disorder
4. hyposplenism
137
Management of ET
- hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count
- interferon-α is also used in younger patients
- low-dose aspirin may be used to reduce the thrombotic risk
138
CA 125
Ovarian cancer
139
CA 19-9
Pancreatic cancer
140
CA 15-3
Breast cancer
141
CEA
Colorectal cancer
142
S-100
Melanoma, schwannomas
143
Bombesin
Small cell lung carcinoma, gastric cancer, neuroblastoma
144
Thymoma is Associated with
myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH
145
Causes of death in thymoma
compression of airway
cardiac tamponade
146
the most common tumour of the anterior mediastinum is .....
Thymoma
147
Prevention of TLS
IV fluids
patients are higher risk should receive either allopurinol or rasburicase
148
Laboratory tumor lysis syndrome
2 or more of the following
1. uric acid > 475umol/l or 25% increase
2. K > 6 mmol/l or 25% increase
3. phos > 1.125mmol/l or 25% increase
4. Ca < 1.75mmol/l or 25% decrease
149
Cairo-Bishop score
- Laboratory tumor lysis syndrome: abnormality in two or more of the following, occurring within three days before or seven days after chemotherapy.
1. uric acid > 475umol/l or 25% increase
2. K > 6 mmol/l or 25% increase
3. phos > 1.125mmol/l or 25% increase
4. Ca < 1.75mmol/l or 25% decrease
Clinical tumor lysis syndrome: laboratory tumour lysis syndrome plus one or more of the following:
1. increased serum creatinine (1.5 times upper limit of normal)
2. cardiac arrhythmia
3. sudden death
150
Differentiating features of MGUS 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 CRAB
151
X-rays: 'rain-drop skull seen in
MM
152
Diagnostic criteria of MM
1 major + 1 minor
Or
3 minor
Major
1. Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
2. 30% plasma cells in a bone marrow sample
3. Elevated levels of M protein in the blood or urine
Minor criteria
1. 10% to 30% plasma cells in a bone marrow sample.
2. Minor elevations in the level of M protein in the blood or urine.
3. Osteolytic lesions (as demonstrated on imaging studies).
4. Low levels of antibodies (not produced by the cancer cells) in the blood.
153
Markers of poor prognosis in MM
1. High B2 microglobulin
2. Low Albumin
154
Management of Waldenstrom's macroglobulinaemia
typically rituximab-based combination chemotherapy
155
What is the most common symptom in SVC obstruction
Dyspnoea
156
Management of SVC obstruction
1. endovascular stenting is often the treatment of choice to provide symptom relief
2. certain malignancies such as lymphoma, small cell lung cancer may benefit from radical chemotherapy or chemo-radiotherapy rather than stenting
3. the evidence base supporting the use of glucocorticoids is weak but they are often given
157
Management of spinal cord compression
high-dose oral dexamethasone
urgent oncological assessment for consideration of radiotherapy or surgery
158
Good prognostic factors of ALL
French-American-British (FAB) L1 type
common ALL
pre-B phenotype
low initial WBC
del(9p)
159
Poor prognostic factors of ALL
FAB L3 type
T or B cell surface markers
Philadelphia translocation, t(9;22)
age < 2 years or > 10 years
male sex
CNS involvement
high initial WBC (e.g. > 100 * 109/l)
non-Caucasian
160
Poor prognostic features of AML
> 60 years
> 20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7
161
Acute promyelocytic leukaemia M3
associated with t(.......)
t(15;17)
162
APML is treated with
all-trans retinoic acid (ATRA)
163
Most common tumour causing bone metastases
1. prostate
2. breast
3. lung
(in descending order)
164
Breast cancer: risk factors
1. BRCA1, BRCA2 genes
2. p53 gene mutations
3. obesity
4. OCP
5. not breastfeeding
6. nulliparity, 1st pregnancy > 30 yrs
7. early menarche, late menopause
8. 1st degree relative premenopausal relative with breast cancer
9. previous surgery for benign disease (?more follow-up, scar hides lump)
165
Burkitt's lymphoma is associated with the ......... translocation, usually t(........)
the c-myc gene translocation, usually t(8:14)
166
Complications of CLL
1. Anemia
2. Hypogammaglobulinaemia
3. Warm autoimmune hemolytic anemia
4. transformation to high-grade lymphoma (Richter's transformation)
167
Ritcher's transformation is indicated by one of the following symptoms:
lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdominal pain
168
Indications for CLL treatment
1. progressive marrow failure
2. massive (>10 cm) or progressive lymphadenopathy
3. massive (>6 cm) or progressive splenomegaly
4. autoimmune cytopaenias e.g. ITP
5. B symptoms
6. progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months
169
Treatment of CLL
fludarabine, cyclophosphamide and rituximab (FCR)
170
del 13q in CLL associated with
good prognosis
171
chromosome 17 (del 17p) in CLL associated with
Poor prognosis
172
Cyclophosphamide can cause hemorrhagic...1......
incidence reduced by the use of ......2...
1. Hemorrhagic Cystitis
2. hydration and mesna
173
t(11;14) in
Mantle cell lymphoma
174
t(14;18) seen in
follicular lymphoma
175
Malaria can lead to ........ lymphoma
Malaria can lead to Burkitt's lymphoma
176
Management of Hairy cell leukaemia
chemotherapy is first-line: cladribine, pentostatin
immunotherapy is second-line: rituximab, interferon-alpha
177
Lead poisoning results in defective ....
ferrochelatase and ALA dehydratase function.
178
Leucocyte alkaline phosphatase
Low in
CML
INFECTIOUS MONONUCLEOSIS
PNH
PERNICIOUS ANAEMIA
*CIPP
179
Causes of leukaemoid reaction
severe infection
severe haemolysis
massive haemorrhage
metastatic cancer with bone marrow infiltration
180
Normal pO2 but decreased oxygen saturation is characteristic of
methaemoglobinaemia
181
Acquired autoantibodies to factor VIII can occur with drugs
Penicillin
Phenytoin
Sulfa drugs
