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Clinical Practice > Haematology > Flashcards

Haematology Flashcards

1
Q

what are the symptoms and signs of haematological malignancy?

A 
tiredness
weight loss
severe, recent night sweats
bone pain
bleeding
bruising
petechiae (non-blanching)
lymphadenopathy
gum swelling
infiltration
organomegaly
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2
Q

what is the genetic abnormality that is responsible for the driving and production of leukaemia cells?

A

PML/RARA fusion gene

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3
Q

what type of malignancy is a PML/RARA fusion gene normally seen in?

A

acute promyelocytic leukaemia

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4
Q

describe the full diagnostic pathway of haematological malignancy

A

morphological examination of bone marrow aspirate
immunophenotyping with flow cytometry
cytogenetics
molecular diagnosis

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5
Q

describe leukaemia

A

acquired series of events
expansion of an abnormal clone of cells
which accumulate in the bone marrow or blood

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6
Q

what is the management of acute leukaemia?

A 
resuscitation and supportive therapy
central venous catheter
blood product support
coagulation factor support
antibiotics, antivirals, antifungals
antiemetics
fluids and allopurinol or rasburicase (prevention of tumour lysis)
specific combination chemotherapy
CNS directed therapy in lymphoblastic
bone marrow or stem cell transplant
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7
Q

describe the stem cell transplant process

A

they must be a tissue type or a HLA match (related or unrelated)
should have a transplant if the rate of dying from readmission is greater than the rate of dying from transplant (Philadelphia positive acute lymphoblastic leukaemia)

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8
Q

describe the process of obtaining a stem cell donation

A

collected from the bone marrow
collected from the peripheral blood after stimulation with G-CSF
they are infused into the patient via a peripheral vein

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9
Q

what are the side effects of chemotherapy?

A 
nausea and vomiting
hair loss
mouth ulcers
pancytopenic period
risk of infections and bleeding
infertility
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10
Q

what are the findings in CML?

A

high WCC
high neutrophils, eosinophils, basophils and myeloid precursor cells
Philadelphia chromosome (BCR/ABL fusion)
high LDH and Urate

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11
Q

what is the function of the BCR/ABL protein?

A

drives CML resulting in cell proliferation and apoptosis failure

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12
Q

what is the management of CML?

A 
TKI inhibitors block BCR/ABL (TKI) function and lead to cell death
imatinib
nilotinib
dasatinib
bosutinib
ponatinib
treated long-term with BCR/ABL monitoring
bone marrow transplant
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13
Q

what are the symptoms of bone marrow failure?

A

anaemia
bleeding
infection

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14
Q

what is the relapse rate of Ph positive ALL treated with chemotherapy?

A

100%

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15
Q

what are the risk factors for AML?

A 
myelodysplastic syndrome
pre-existing haematological disorders
down's syndrome
bloom syndrome
prior chemotherapy
radiation
tobacco smoke
benzene
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16
Q

what are the symptoms of AML?

A

anaemia (weakness, fatigue, pallor, malaise, palpitations, dizziness, dyspnoea, tachycardia)
thrombocytopenia (mucosal bleeding, easy bruising, petechiae/purpura, epistaxis)
neutropenia (infection susceptibility, fever, recurrent infections)
abdominal discomfort/fullness (hepatosplenomegaly)
weight loss
respiratory distress, altered mental status (leukostasis)

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17
Q

what are the findings in AML?

A 
deranged WCC
low RBC
low PLT
high LDH and uric acid (increased cell turnover)
deranged coagulation profile
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18
Q

what is the management of AML?

A 
chemotherapy - induction period, consolidation period, maintenance therapy
bone marrow transplant
blood cell transfusions
analgesia and good oral hygiene
antimicrobial prophylaxis
allopurinol
antiemetics
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19
Q

what are the complications of AML?

A

leukostasis
tumour lysis syndrome
disseminated intravascular coagulation

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20
Q

how is a diagnosis of AML made?

A

> 20% myeloid blasts in the bone marrow or peripheral blood

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21
Q

what is the pathophysiology of the Philadelphia chromosome?

A

add phosphates to tyrosine residues (tyrosine kinase)

inhibition of DNA repair

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22
Q

what are the symptoms of CML?

A 
asymptomatic (chronic phase)
upper abdominal pain (hepatosplenomegaly)
poor appetite
night sweats
gout
increased susceptibility of infection
dyspnoea
fatigue
easy bruising/petechiae/bleeding
neurological deficits
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23
Q

what are the features of a blast crisis?

A

> 20% lymphoblasts or myeloblasts in the blood or bone marrow
large clusters of blasts in the bone marrow on biopsy
chloroma development

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24
Q

define anaemia

A

Hb < 120g/L in females

Hb < 130g/L in males

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25
Q

what are the causes of microcytic hypochromic anaemia?

A

iron deficiency anaemia
thalassemias
sideroblastic anaemia

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26
Q

what are the causes of normocytic normochromic anaemia?

A 
combined iron and B12/folate deficiency
acute/subacute blood loss
renal failure
anaemia of chronic disease
lead poisoning
myelodysplasia
leukaemia
other primary bone marrow disorders
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27
Q

what are the causes of macrocytic anaemia?

A 
B12/folate deficiency
haemolytic anaemia
liver disease
hypothyroidism
alcohol
cytotoxic drugs
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28
Q

what factors are essential for erythropoiesis?

A 
erythropoietin
thyroxine
haematopoietic growth factors
vitamin B12
folic acid
iron
functioning globin genes
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29
Q

define microcytic hypochromic anaemia

A

decreased MCV

decreased MCH/MCHC

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30
Q

what are the features of haemolytic anaemia?

A

jaundice (haem breakdown product)
splenomegaly
mild-moderate red cell macrocytosis

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31
Q

what is the treatment of haemolytic anaemia?

A

symptomatic - blod transfusion

cause - prednisolone, oral folic acid, second-line immunosuppression, splenectomy

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32
Q

what tests should be completed in suspected macrocytic anaemia?

A

serum B12
serum folate
liver function

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33
Q

how do you treatment acute macrocytic anaemia?

A

oral folic acid
transfusion in CV compromise or acute bleeding
supplemental thiamine (Wernicke’s encephalopathy)

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34
Q

describe leucoerythroblastic reaction

A

immature erythrocytic and neutrophilic precursors in the blood

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35
Q

what are the causes of leucoerythroblastic anaemia?

A 
metastatic carcinoma
granuloma
CML
myelofibrosis
megaloblastic anaemia
lymphoma
acute leukaemias
recovery from BM insult
severe sepsis
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36
Q

what are the features of thalassemia?

A

microcytic hypochromic anaemia
normal RBC
low MCV, MCHC, MCH

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37
Q

what tests would you perform to confirmed thalassemia?

A

blood film
Hb electrophoresis (HbA2 elevated)
goblin gene sequencing

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38
Q

define myeloma

A

a cancer of the plasma cells

massive production of antibodies

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39
Q

describe the pathophysiology of myeloma

A

cancerous plasma cells migrate to the bone marrow and accumulate
surpasses the growth of the normal blood
secrete growth factors that destroy the overlying bone (lytic lesions)

40
Q

in what way can a myeloma present?

A

hypercalcaemia
renal failure
anaemia
bone lesions (fractures, pepper pot skull)

41
Q

describe MGUS

A

persistent paraprotein with no evidence of CRAB criteria

require follow up as can transform into myeloma

42
Q

what is the treatment of a myeloma?

A

combination of targeted biological agents
ultimately mutates until therapy is no longer effective and they die of complications
chemotherapy rarely used now

43
Q

what are the causes of bleeding disorders?

A

abnormal vascular endothelium
clotting factor deficiency
platelet abnormalities

44
Q

what are the causes of a factor VII deficiency?

A

oral warfarin therapy (first few days)
sepsis
congenital deficiency
early vitamin K deficiency

45
Q

what are the causes of a prolonged PT?

A

factor VII deficiency

46
Q

what are the causes of a prolonged PT and APTT?

A

vitamin K deficiency
oral dabigatran therapy
oral warfarin therapy
DIC

47
Q

what are the causes of DIC?

A

septicaemia
meningitis
malignancy

48
Q

what are the causes of prolonged APTT?

A 
DIC
liver disease
massive transfusion
unfractionated heparin therapy monitoring
heparin contamination from line locks
oral warfarin therapy
lupus anticoagulant
VIII, IX, XI, XII deficiency
49
Q

what is the cause of a prolonged APTT that ‘corrects’?

A

clotting factor deficiency

50
Q

what is the cause of a prolonged APTT that fails to ‘correct’?

A

lupus anticoagulant

51
Q

what are the symptoms and signs of haemophilia A and B?

A

major haemorrhage into joints
affected joint is tender, warm and distended
chronic haemorrhage causes degenerative joint changes and arthropathy
intracranial haemorrhage, intramuscular haematoma, GI haemorrhage, haematuria and haematospermia

52
Q

describe Von Willebrands Disease

A

epistaxis
menorrhagia
bleeding after surgery
no haemarthrosis, muscle or spontaneous bleeds

53
Q

name some acquired platelet disorders

A

anti platelet therapy
NSAIDs
SSRIs
herbal medication

very common

54
Q

name some congenital platelet disorders

A

Bernard Soullier syndrome
Glanzmanns thrombasthenia
storage pool disorders
Hermansky Pudlak syndrome

very rare

55
Q

where do lymphocytes develop from?

A

primary lymphoid tissue (bone marrow and thymus)

56
Q

what are the secondary lymphoid tissues?

A

lymph nodes
spleen
MALT

57
Q

what are the functions of lymphocytes?

A 
antibody production
antigen presentation
immune response coordination
immune response suppression
killing virally infected cells
tumour suppression
58
Q

define lymphoma

A

a group of diseases caused by accumulation of malignant lymphocytes with lymph nodes
non/hodgkin lymphoma

59
Q

what are the causes of lymphoma?

A 
viruses (EBV, HIV, HTLV-1, hep C)
bacteria (helicobacter pylori, chlamydia psittacosis)
immunosuppression
autoimmune disease
no cause
60
Q

what are the symptoms of lymphoma?

A 
lymphadenoapthy
fevers
night sweats
weight loss
fatigue
itch
61
Q

what are the causes of lymphadenopathy?

A 
non/hodgkins lymphoma
leukaemia
malignancy (draining)
CT disorders (RA, SLE, sarcoid)
infection
62
Q

describe a small lymphocytic lymphoma on histology

A

small uniform lymphocytes with clumped chromatin, no obvious nucleoli

63
Q

describe a diffuse large B cell lymphoma on histology

A

larger pleomorphic cells many with nucleoli

64
Q

describe a Hodgkin lymphoma on histology

A

inflammatory background with reed Sternberg cells

65
Q

describe immunohistochemistry

A

the most important method for immunophenotyping lymphocytes on fixed paraffin embedded material
allows visualisation of an antigen

66
Q

describe flow cytometry

A

requires fresh tissue

cells incubated with multiple flurochrome-labelled Abs and passed through laser light beam in a FACS machine

67
Q

describe FISH

A

fluorophore labelled DNA probes hybridise to specific DNA sequences
detect non-random chromosomal translocations in lymphoma

68
Q

describe the Ann Arbor staging of lymphoma

A

1 - single lymph nodes region
2 - 2+ sites, same side of diaphragm
3 - both sides of diaphragm
4 - diffuse involvement of extralymphatic sites

69
Q

describe classical Hodgkin lymphoma

A

can present at any age but has a peak incidence in young adults
m>f
reed Sternberg cells are abnormal cells of B lymphoid lineage

70
Q

what are the types of Hodgkin lymphoma?

A

classical Hodgkin lymphoma (nodular sclerosis, lymphocyte rich, lymphocyte deplete, mixed cellularity)
nodular lymphocyte predominant hodgkin lymphoma

80% of patients will be cured with standard 1st line therapy

71
Q

describe non-hodgkin lymphoma

A

large group of clonal lymphoid disorders
85% B cell
15% T or NK cell

72
Q

describe diffuse large B cell lymphoma

A 
1/3 NHL in western countries
high grade lymphoma
rapidly progressive
requires urgent treatment
RCHOP 1st chemotherapy
radiotherapy for sites of bulky disease
stem cell transplantation (refractory/relapse)
73
Q

what are the cells of origin of a diffuse large B cell lymphoma?

A

germinal centre phenotype

activated B cell phenotype (ABC, poorer prognosis)

74
Q

describe a double-hit DLBCL

A

concurrent MYC and BCL2 and/or BCL6 gene rearrangements
poor prognosis
RCHOP gives a poor outcome
requires FISH screening

75
Q

describe follicular lymphoma

A 
25% all NHL
low grade
median age of onset 60yrs
translocation t(14;18) = BCL2 gene overexpression
BCL2 has anti-apoptotic functions
76
Q

what is the treatment of follicular lymphoma?

A 
watch and wait
radiotherapy
chemotherapy
stem cell transplantation
median survival 12-18 years

grade 3b treated like high grade lymphoma

77
Q

describe chronic/small lymphocytic leukaemia

A

most common (4/100,000 per yr)
>age
asymptomatic - watch and wait
treatment - cytopenia, splenomegaly, lymphadenopathy, rapid rise in WCC
associated with genetic abnormalities (13q, 11q, 17p deletions, trisomy 12)
13q - excellent prognosis
17p or TP53 gene - chemotherapy resistance

78
Q

what are the most common treatments for lymphoma?

A 
corticosteroids
chemotherapy
radiotherapy
monoclonal antibodies
targeted therapies
stem cell transplantation
CAR T-cell therapy
79
Q

describe monoclonal antibodies

A

target particular proteins (CD20, CD22, CD19, CD52) on lymphocyte surfaces
rituximab for lymphoma
ofatumumuab and obinutuzumab for CLL

80
Q

describe the cell-signalling pathway target treatment in lymphoma

A

Bruton-tyrosine kinase (BTK) inhibitors ibrutinib)

P13K (idelalisib)

81
Q

describe autologous stem cell transplantation

A

rein fusion of the patients own stem cells
rarely used as part of initial therapy
side effects - mucositis, diarrhoea, organ toxicity, infection, bleeding, morality risk 5%

82
Q

describe allogeneic stem cell transplantation

A

requires HLA donor
cytotoxic and immune therapy
myeloblative or reduced intensity conditioning regimens
side effects - graft vs host disease, morbidity and mortality approaching 30%

83
Q

describe some lymphoma emergencies

A

superior vena caval obstruction (venous drainage obstruction from head, arms and upper chest wall, facial swelling and dyspnoea)
hypercalcaemia
spinal cord compression
tumour lysis syndrome (rapid breakdown of tumour cells, most often in high grade lymphomas and Burkitts lymphoma)

84
Q

what is the treatment of stage IV Hodgkin lymphoma?

A

ABVD adriamycin, dacarbazine, bleomycin and vinblastine
interim CT-PET post two cycles

minimum clinical response - ICE chemotherapy, 2-4 cycles followed by autologous stem cell transplantation if complete response achieved

85
Q

what is the differential diagnosis of a soft tissue mass in chest?

A

lung cancer
other solid malignancy
lymphoma
sarcoid

86
Q

what is the management of superior vena cava obstruction secondary to lymphoma?

A

urgent biopsy
high dose steroids with tumour lysis prophylaxis
radiotherapy
chemotherapy

87
Q

what are the causes of mucocutaneous bleeding and delayed post-surgical bleeding

A

VWD
dysfunctional platelet syndrome
FXIII deficiency

88
Q

what are the symptoms and signs of a factor deficiency in males?

A

atraumatic bruising
spontaneous joint bleeds
immediate surgical bleeding

89
Q

describe an extrinsic pathway problem

A

prolonged PT only

factor VII defiency

90
Q

describe a common pathway problem

A 
prolonged PT and APTT
vitamin K deficiency
oral warfarin therapy
oral dabigitran therapy
DIC
91
Q

describe an intrinsic pathway problem

A

prolonged APTT

92
Q

describe haemophilia A and B

A

A - factor VIII deficiency, males
B - factor IX deficiency, males
x-linked recessive

93
Q

which type of Hodgkins lymphoma has the worst prognosis?

A

lymphocyte depleted

94
Q

what type of Hodgkins lymphoma has the best prognosis?

A

lymphocyte predominant

95
Q

what symptoms of Hodgkins lymphoma imply a poor prognosis?

A

weight loss >10% in the last 6 months
fever >38 degrees
night sweats

96
Q

what do myeloma plasma cells do?

A

migrate towards the bone marrow and accumulate
suppresses the growth of normal blood causing anaemia
secrete growth factors that destroy the overlying bone
causing lytic lesions; fracture easily
produce light chain fragments of IgG molecules which gather in the renal tubules, causing cast nephropathy

97
Q

describe myeloma

A

malignancy of plasma cells;
10% plasma cells in marrow
and
high Ca, renal impairment, anaemia, or bone lesions due to this (CRAB)
or
NEW; >60% plasma cells in marrows, very high light chains, MRI findings (SLiM)

Clinical Practice (14 decks)

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