haematology Flashcards
1
Q
3 main groups of non-Hodgkins lymphoma
A
indolent lymphoma
aggressive lymphoma
very aggressive lymphoma
2
Q
typical iron studies seen in iron deficiency anaemia
A
reduced serum ferritin
reduced serum iron
increased TIBC
reduced transferrin saturation
increased soluble transferrin receptor
3
Q
3 stages of CML
A
chronic - generally asymptomatic
accelerated
blast
4
Q
how do you diagnose monoclonal gammopathy of uncrtain significance
A
have a monoclonal immunoglobulin band (
5
Q
monitoring and target of warfarin
A
INR (2.5-3.5)
6
Q
which “letters” in the staging system for lymphoma are prognostic indicators
A
B, E, X
7
Q
key findings for polycythaemia
A
increased RBC
increased haematocrit
8
Q
what do you see in a peripheral blood film of iron deficiency anaemia
A
hypochromic microcytic RBCs
target cells
pencil forms
9
Q
principles of treatment of diffuse large B cell lymphoma
A
immunochemotherapy - treat them regardless of whether they have symptoms or not CURABLE
10
Q
main constituent of cryoprecipitate
A
fibrinogen
11
Q
what mouth signs do you use in severe Vitamin B12
A
Hunter’s glossitis
12
Q
what is in the DIC screen and what will this and the FBE show if the patient has DIC
A
Screen - APTT, INR, fibrinogen, D-dimer
high APTT
high INR
low fibrinogen
high D-dimer
low platlets
red cell fragmentation
13
Q
how do you test heparin activity
A
unfractionated heparin - APTT
LMWH - Xa inhibition
14
Q
treatment of CML
A
imatinib
dasatinib
nolotinib
15
Q
which enzyme on biochemistry is a poor prognostic sign for lymphoma
A
high lactate dehydrogenase
16
Q
what causes the increased infections in those with symptomatic myeloma
A
the M protein causes a reduction in normal Igs –> immunodeficiency –> predisposed to infection
17
Q
iron studies in thalassemia
A
- normal serum iron
- normal TIBC
- normal serum ferritin
- increased transferrin receptor
18
Q
treatment of acute promyelocytic leukaemia
A
high dose vitamin A and arsenic (causes cells to mature fast)
19
Q
explain the interpretatin of a correction study of an abnormal APTT or PT
A
if factor deficiency = APTT/PT will normalize
if ‘inhibitor’ present = persistent abnormality
20
Q
what are the end organ damage outcomes to diagnose symptomatic multiple myeloma
A
CRAB
- Calcium = hypercalcaemia –> confusion, constipation, dehydration, coma
- renal dysfunction/failure - from light chain deposition
- anaemia - myeloma produces inflammatory cytokines (IL-6) –> anaemia of chronic disease
- bone lesions/disease + hyperviscosity, amyloidosis and recurrent infections
21
Q
acute adverse reactions to transfusion
A
- allergy/anaphylaxia
- nebrile non-haemolytic transfusion reactions
- intravascular haemolysis
- infection
- transfusion related lung injury
22
Q
key result on blood test suggesting polycythaemia rubra vera over secondary polycythaemia
A
EPO is low
23
Q
6 causes of prolonged prothrombin time
A
- warfarin (II, VII, IX, X)
- liver disease
- vitamin K def
- DIC
- factor VII def
- rivaroxaban
24
Q
commonest ways to present with myeloma
A
problems with bony disease
renal impairment
25
what are some indications for fresh frozen plasma
coagulopathy and bleeding
massive transfusion
bipass
liver disease
acute DIC
warfarin overdose
TTP
plasma exchange
26
treatment of Haemophilia A
- purified or recombinant Factor VIII therapy
- Tranexamic acid
- Topical thrombin
27
what are the 2 most common lymphomas in the community
diffuse large B cell lymphoma
follicular lymphoma
28
treatment of DIC
- treat underlying cause
- give fresh frozen plasma (aim for APTT to be more than 1.5)
- replace fibrinogen (give cryoprecipitate) aiming for a fibrinogen level over 1.5
- give the patient platelets - aiming for above 50
29
what is "bulky" lymphoma
any lymph node mass that is more than 10cm in diameter or more than 1/3 of the diameter of the chest if the node is mediastinal
30
what is the staging system for lymphoma
Ann-Arbor staging system
- by CT scan
31
which factor is also known as prothrombin
factor 2
32
what does onycholysis (distal nail separation) suggest
trauma
psoriasis
thyrotoxicosis
33
How do you differentiate between Hodgkins and non-Hodgkins lymphoma
Hodgkins lymphoma has Reed-Sternberg Cells - don't express the CD20 antigen
34
CLL is the same disease as
small lymphocytic lymphoma
35
what are the lineage associated antigens on myeloid and lymphoid cells (when doing flow cytometry)
myeloid - myeloperoxidase
lymphoid - tdt
36
what is the normal number of plasma cells in the BM
less than 5%
37
3 groups of AML
- AML with recurrent cytogenetic features
- Secondary AML arising from MDS or arising from previous chemotherapy
- AML not otherwise classified
38
What is the special thing about apixaban compared to the other NOACs
Not as metabolized through the kidney as the others
39
most common cause of persistent APTT abnormality after correction study
lupus anticoagulant
40
classification of micro/normo/macrocytic
micro MCV less than 80
normo MCV 80-100
macro MCV more than 100
41
what are the extra "letters" in the staging system for lymphoma and what do they mean
A = no B symptoms
B = B symptoms present
S = spleen involved
E = extranodal disease
X = bulky disease
42
What is DIC and what is the pathobiology
Disseminated intravascular coagulation
trigger activates coagulation cascade --\> get little clots formed within BVs --\> as the coagulation cascade continues --\> depletion of the coagulation factors --\> bleed
43
what is the time frame for going from smouldering to symptomatic myeloma
18 months to 3 years
44
how many drinks per week does it take for you to be at risk of macrocytic anaemia
more than 14
45
principles of treatment for Hodgkins lymphoma
one of the most curable lymphomas - use combination ABVD chemotherapy or Esculated beacopp
46
clinical manifestations of haemophilia A
- haemarthrosis
- subcutaneous and intramuscular haematomas
- psoas and retroperitoneal haematomas
- Traumatic bleeding
47
explain the role of tissue factor
initiates thrombin generation
48
key blood results in haemolytic anaemia
normocytic (usually - may be macro if % reticulocytes are high)
high LDH
high unconjugated bilirubin
low haptoglobin
high reticulocytes
49
explain the staging system for lymphoma
Stage 1 = one focal area
Stage 2 = 2 focal areas but both on the same side of the diaphragm
Stage 3 = focal disease on each side of the diaphragm
Stage 4 = systemic disease
50
what is the difference between smouldering and symptomatic multiple myeloma
symptomatic multiple myeloma has evidence of end organ damage, while smouldering does not
51
what is the only indolent lymphoma that you can cure
stage 1 follicular lymphoma - treat by radiation
52
iron studiesin anaemia of chronic disease
- decreased serum iron
- decreased TIBC
- decreased transferrin saturation
- normal/increased serum ferritin
- normal soluble transferrin receptor
53
what is the commonest leukaemia
CLL
54
difference between the actions of unfractionated heparin compared to LMW heparin
unfractionated = inactivates Xa and IIa in approximately 1:1 efficiency
LMW = inactivates Xa and IIa in approximately 5:1 ratio
55
what causes polycythaemia rubra vera
mutationn in JAK2 leading to abnormality in RBC progenitors
56
Signs and Sx of polycythaemia rubra vera
hyperviscosity
hypertension
splenomegaly
57
5 major causes of macrocytic anaemia
ABCDEF
Alcohol
B12
compensatory reticulocytosis (myelodysplasia)
drugs
endocrine - hypothyroidism
Folate deficiency
58
what are the new anticoagulants (NOACs) prescribed for in general
- prevention of stroke/embolism in AF
- apixiban also used for VTE prevention
- rivaroxaban also used for VTE treatment
59
when should you suspect a patient has myeloma
- patient presents with a fracture or bone pain without any precipitating event and XR shows lytic lesions, severe OP or crush fractures
- unexplained anaemia and high ESR
- high total protein despite a normal or low albumin
- unexplained hypercalcaemia
- unexplained renal failure
60
what is the normal amount of blasts in the BM
less than 5%
61
what does nail thickening suggest
psoriasis
fungal infection
62
what are the stages of differentiation of myeloma
monoclonal gammopathy of uncertain significance --\> smouldering multiple myeloma --\> symptomatic multiple myeloma
63
what causes leukostasis in acute leukaemia
so many blasts in the blood --\> blood very thick
64
what is the definition of acute leukaemia
more than 20% blasts in the bone marroq
65
characteristics of acute leukaemia
blasts
acute time frame
die quickly
generally curable
lots of B symptoms
66
What are the 5 causes of microcytic hypochromic anaemia and what are the main 3
TAILS
**Thalassaemia**
**Anaemia of chronic disease**
**Iron deficiency -** reduced iron stores
Lead poisoning
Sideroblastic anaemia
67
what is pseduo-von Willebrand disease
mutation in platelet integrin GPIb so that it has decreased binding with vWF
68
major management of B-major thalassaemia
blood transfusion
iron chelation therapy
folic acid supplementation
splenomegaly
allogenic BMT
69
what symptoms point to lymphoma
palpable non-tender, rubbery lymphadenopathy
dyspnoea and cough (with mediastinal mass)
PUO and night sweats
70
what is an alloantibody
an antibody that develops in a patient's plasma after exposure to red blood cell antigens that are not present on their own red blood cells
71
how do you monitor response to therapy in CML
by PCR - the amount of the abnormal bcr-abl transcript in the peripheral blood each 3 months
72
what are the medical emergencies associated with acute leukaemia
DIC
leukostasis
febrile neutropaenia
73
what is myelofibrosis
normal bone marrow tissue is gradually replaced with fibrous scar like tissue causing progressive BM failure
74
which enzyme breaks down a thrombus
plasmin
75
what is febrile neutropaenia
fever of over 38 degrees in a presence of neutorphils less than 1
76
what is the difference between cure and complete remission
cure = the disease goes away and never comes back for the rest of the life
complete remission = disappearance of the disease based on the most sensitive test we have
77
principles of treatment of someone with very aggressive lymphoma
treat IMMEDIATELY
78
which factors are involved in the intrinsic coagulation pathway
factors 12, 11, 9 --\> 10
79
presentation of leukostasis
white out of the lungs --\> SOB
blurry vision
stroke
confusion
80
treatment of leukostasis
plasma exchange, then give chemotherapy to bring WCC down very quickly
81
functions of vWF
- mediates platelet adhesion at site of injury
- stabilizes factor VIII in circulation
82
causes of NON-megaloblastic anaemia
alcohol
drug induced
liver disease
endocrine (hypo/hyperthyroidism)
pregnancy
83
what is Haemophilia B
X linked recessive disorder that causes factor IX deficiency (less common than A)
84
basic principles of treatment of acute leukaemia
chemotherapy!
85
what do you do if you get an abnormal APTT or PTT
go onto do a correction study/mixing study - mix half of the patients serum with half of a control and perform the APTT / PT again
86
common causes of secondary polycythaemia
Smoking
COPD
PAH
OSA
R-L shunt
EPO producing tumour
87
iron studies in iron deficiency anaemia
- reduced serum iron
- reduced transferrin saturation
- increased TIBC
**- decreased serum ferritin**
- increased soluble transferrin receptor
88
when is the platelet numer low enough to have a risk of spontaneous bleeding
10-20 x 10^9
89
what is contained in fresh frozen plasma
mostly coagulation factors
90
what causes tumour lysis syndrome
when large numbers of neoplastic cells are killed rapidly, leading to the release of IC ions and metabolic byproducts into the systemic circulation --\> hyperuricaemia, hyperkalaemia, hyperphosphataemia, hypocalcaemia and acute renal failure
91
why do you get hypercalcaemia with multiple myeloma
the myeloma produces cytokines that bind to RANKL --\> activating OC --\> Ca release into serum
92
presentation of someone with diffuse large B cell lymphoma
lymphadenopathy and B symptoms
93
typical characteristic look of cells in acute promyelocytic leukaemia
have LOTS of Auer Rods
94
common symptoms of acute leukaemia
anaemia --\> tachycardia, pallor, tachypnoeic, SOB, fatigue
bleeding, bruising, petichiae, thrombocytopaenia
infection - fever bone pain B symptoms
95
3 ways to diagnose acute leukaemia
BM biopsy
confirmatory tests: flow cytometry and cytogentic molecular studies
96
4 major causes of normocytic anaemia
ABCD
Acute blood loss
bone marrow failure
chronic disease
destruction (aplastic, autoimmune, haemolytic, Sickle cell)
97
what other than enzymes are proteins are essential for the coagulation pathway
phospholipids
calcium
98
causes of reticulocytosis with normocytic normochromic anaemia
haemolysis
blood loss
99
what is myeloma
cancer of the plasma cells
100
types of very aggressive lymphoma
Burkitts lymphoma
acute lymphoblastic lymphoma - like ALL
101
types of aggressive lymphoma
diffuse large B cell lymphoma
diffuse large T cell lymphoma
102
what are the medical emergencies of multiple myeloma
hypercalcaemia
spinal cord compression
renal failure
hyperviscosity
infections
103
what types of bleeding do you get with thrombocytopaenia
- skin and mucous membranes (petechiae, ecchymosis, haemorrhagic vesicles, gingival bleeding and epistaxis)
- menorrhagia
- GI bleeding
- intracranial bleeding
104
what are the screening tests for haemostatic defects
platelet count (150-400)
APTT (24-32 sec)
PTT/INR (10-12 seconds)
Thrombin time
105
cause of CML
t(9;22) of BCR and ABL that causes constitutively activated tyrosine kinase --\> cell growth
106
what skin and nail signs do you see in someone with severe iron deficiency
dry skin
koilonychia
brittle hair and nails
hair loss
itching
paleness
107
APTT and INR test which pathways
APTT = intrinsic (factors VIII, IX, XI, XII)
INR/PTT = extrinsic (factor VII)
108
how can myeloma be found by urine sample
can see Bence Jones protein in the urine
109
treatment options for VWB disease
desmopressin
Replacement therapy
Tranexamic acid (antifibrinolytics)
fibrin glue
110
which factors are involved in the extrinsic coagulation pathway
factor 7 --\> 10
111
types of indolent lymphoma
follicular lymphoma
CLL/SLL
mantle cell lymphoma
marginal cell lymphoma
112
1 unit of red cells increases the Hb by
10g/L
113
what is the difference between a group and hold and a cross match
group and hold - match ABO and Rh
cross match - check alloantibodies
114
indication to treat CLL/SLL
B symptoms
progressive lymphadenopathy
pancytopaenia
doubling white cell count in less than 6 months
115
characteristics of chronic leukaemia
mature cells
occurs over years
die at end stage
generally not curable
B symptoms only at end stage
116
what causes the acute renal failure in symptomatic myeloma
light chains get deposited in the kidneys increased infections hypercalcaemia
117
name 5 acquired bleeding disorders
- liver disease
- Vit K deficiency
- DIC
- Excessive anticoagulation
- idiopathic thrombocytopaenia
118
what does the direct Coombs' test do
tests for immune haemolysis
119
what are the typical iron studies seen in anaemic of chronic disease
Fe low
TIBC low
% saturation normal
normal/increased ferritin
120
typical peripheral blood film of macrocytic anaemia
hypersegmented neutrophils
macrocytic RBCs
121
what does gingiva hypertrophy suggest
leukaemia
122
what is induction thearpy
causes an acute reduction of the burden of disease
123
7 causes of prolonged APTT
- liver disease
- heparin anticoagulation therapy/dabigatran
- lupus anticoagulant
- DIC
- VWBd
- Haemophilia
- Factor XII, XI def
124
what is important to remember about acute promyelocytic leukaemia?
extremely curable! but deadly if you dont act - die from DIC
125
difference in treatment between "cold" and "warm" haemolytic anaemia
cold (C3) - extremely difficult to treat
warm (IgG) - treat like ITP (prednisolone, splenectomy, rituximab, IS)
126
what is haemophilia A
X linked recessive disorder that causes factor VIII deficiency
127
cause of corrected correction study with prolonged APTT and PT
- isolated deficiency in common pathway: factors V, X, II and fibrinogen
- multiple factor deficiences: liver disease, Vit K def, warfarin, DIC
128
principles of treatment for myeloma
MGUS - monitor only
smouldering myeloma - observe only
symptomatic myeloma - treat it! - young - induction therapy + autologous SCT - between 65-75 - depends on how fit they are - over 75 - induction therapy only
129
treatment of Haemophilia B
- prothrombin complex
- purified or recombinant factor IX
130
action of dabigatran and Rivaroxaban
dabigatran - IIa antagonist
rivaroxaban - Xa antagonist
131
broad causes of lymphadenopathy
infection
malignant disorder
autoimmune disease
storage disease
endocrine disease
132
how do you diagnose multiple myeloma
BM shows \>10% of plasma cells
133
which particular drugs can cause macrocytic anaemia
antipurines
antipyrimidines
AZT
134
what are B symptoms
weight loss (more than 10% of baseline weight in 6 month period)
temperature (more than 38)
drenching sweats
135
typical look of blasts in AML and ALL
AML - large blasts with Auer rods
ALL - small blasts with no cytoplasmic granules
