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FOP/TAS > Haematology > Flashcards

Haematology Flashcards

1
Q

types of ALL

A
  1. B cell (80%)
  2. T cell
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2
Q

risk factors for ALL

A

trisomy 21
chemotherapy
immunodeficiency syndromes e.g. wiskott aldrich syndrome
ionising radiation
chromosomal breakage e.g. fanconi anaemia , ataxia telangiectasia

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3
Q

presentation of ALL

A

bleeding/ bruising/ petechiae - thrombocytopenia
weight loss
SOB, pallor, malaise - anaemia
increased infections - neutropenia
bone and joint pain
lymphadenopathy
splenomegaly/ hepatomegaly

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4
Q

investigations for ALL

A
  1. blood film* -> BLAST CELLS (single large nucleus, high nuclear to cytoplasmic ratio)
  2. lumbar puncture * -> send for cytogenetics and minimal residual disease
  3. bone marrow aspiration ** -> >20% blast cells, high nuclear to cytoplasmic ratio
  • CXR - mediastinal mass - important initial test before transfer
  • FBC
  • cytogenetics for associated translocations e.g. t (12,21)
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5
Q

factors indicating worse prognosis in ALL

A

boys
high WCC >50
minimal residual disease +ve
t(9,22), 11q23
hypodiploidy
< 2 y/o and >9 y/o
CNS disease
afro-caribbean

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6
Q

management of ALL

A
  1. initial IVF to prevent TLS
  2. chemotherapy - induction + consolidation (intrathecal methotrexate) + maintenance (oral dex)
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7
Q

cell cycle components

A
  1. G 1 - gap phase -> synthesis of components for DNA, under influenxe p53 gene, acts as checkpoints
  2. S - DNA synthesis
  3. G2 - 2nd gap phase
  4. M - mitotic stage
  5. G 0 - resting phase (cells most resistant to chemo drugs)
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8
Q

side effects of vincristine

A

neuropathy
constipation
jaw pain
foot drop

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9
Q

epidemiology of AML

A

highest rates < 1 y/o
15% of leukaemias

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10
Q

investigations for AML

A
  1. bone marrow aspiration and biopsy - immature myeloid lineage cells
  2. blood film - blast cells and auer rods
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11
Q

mutation in CML

A

philadelphia chromosome - translocation with chromosome 9 + 22 and BCR transcription factor with AB1 kinase

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12
Q

red flags for lymphadenopathy

A

supraclavicular lymph node
painless , enlarging, firm lymph node
B symptoms - night sweats, weight loss, fever
LN > 2cm
unexplained bruising
hepatosplenomegaly

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13
Q

what is tumour lysis syndrome

A

release of intracellular metabolites into blood after rapid lysis of malignant cells
crystallisation of uric acid or phosphate causes acute renal failure

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14
Q

blood tests for tumour lysis syndrome

A

hyperkalaemia
hyperphosphataemia
increased uric acid
increased urea
metabolic acidosis
hypocalcaemia

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15
Q

management of tumour lysis syndrome

A
  1. IV fluids !!!!!
  2. rasburicase - increases solubility of uric acid so it is secreted as allantoin
  3. allopurinolol - inhibits xanthine oxidase so blocks uric acid production
  4. may require haemofiltration if severe
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16
Q

presentation of superior vena cava obstruction

A

dyspnoea
distended chest and neck veins
horaseness of voice
facial swelling

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17
Q

risk factors for hodgkins lymphoma

A

SLE
rheumatoid arthritis
EBV
immunodeficiency disorders

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18
Q

types of hodgkins lymphoma

A
  1. nodular sclerosing *
  2. mixed cellularity
  3. lymphocyte rich
  4. lymphocyte depleted
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19
Q

presentation of hodgkins lymphoma

A

slow growing painless lymph node enlargement - cervical, supraclavicular, axilla
alcohol induced nodal pain
B symptoms
splenomegaly
local compression e.g. SVC obstruction, airway obstruction

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20
Q

diagnosis of hodgkins lymphoma

A

lymph node biopsy ** -> reed sternberg cells (multinucleated, giant lymphocyte cell)

CT - ann arbor classification
CXR - widened mediastinum

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21
Q

epidemiology of non hodgkins

A

5 x more common than hodgkins
originates from B or T lymphocytes

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22
Q

classification of non hodgkins lymphoma

A
  1. IMMATURE FORMS - T or B cell
  2. MATURE FORMS - Burkitts B cell (de novo mutation)
  3. LARGE CELL LYMPHOMAS - diffuse B cell, peripehral T cell, anaplastic large cell
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23
Q

presentation of non hodgkins lymphoma

A

painless slowly growing progressive peripheral lymphadenopathy
primary extranodal involvement and systemic symptoms e.g. B symptoms
burkitts can present with large abdo mass

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24
Q

diagnosis of non hodgkins lymphoma

A
  1. flow cytometry - identify absence of protein markers and differentiate between T and B cell types
  2. CXR
  3. lymph node excision
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25
Q

describe medulloblastoma

A

embryonic tumour of neuroendothelial tissue (usually in POSTERIOR FOSSA and CEREBELLUM -> cause hydrocephalus)

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26
Q

Presentation of medulloblastoma

A

headache - early morning
nausea and vomiting
ataxia, unsteady
papilloedema
obstructive hydrocephalus

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27
Q

investigations for brain tumour

A

MRI head and spine

Increased nuclear- cytoplasmic ratio = worse prognosis (feature of anaplastic cells)

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28
Q

management of medulloblastoma

A

chemo - cisplatin and vincristine
surgery
radiotherapy

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29
Q

most common brain tumour

A

astrocytoma / glioma - arises from glial precursor cells, AFFECTS MACROGLIA

  1. low grade - less agressive
  2. high grade - undifferentiated highly malignant, worse prognosis
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30
Q

how does a craniopharyngioma present

A
  1. headache
  2. visual disturbance
  3. hormonal imbalance e.g. diabetes insipidus
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31
Q

most common solid tumour in children

A

NEUROBLASTOMA

develops from neuronal ganglia of the peripheral sympathetic system
embryonal cancer and develops from VENTROLATERAL NEURAL CREST CELLS which migrate away from neural tube

60% arise from abdominal paraspinal ganglia + 30% arise from adrenal medulla

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32
Q

presentation of neuroblastoma

A

non tender mass across the midline
hypertension
abdominal pain, change in bowels
weight loss
Horners syndrome (if superior cervical gnaglion affected)
sweating

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33
Q

diagnostic tests for neuroblastoma

A

URINE CATECHOLAEMINES - Homovanillic acid and VMA elevated

CT abdo pelvis + biopsy ***

can resolve spontaneously

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34
Q

poorer prognosis factor in neuroblastoma

A

MYCN n-myc gene - proto oncogene -> requires more intensive therapy

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35
Q

risk factors for wilms tumour

A

family history
genetic defects e.g. WT1 gene
Beckwith wiedemann syndrome
trisomy 18
BRAC2 mutation in fanconi anaemia
Denys drash syndrome (ambiguous genitalia, b/l)
WAGR

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36
Q

presentation of wilms tumour

A

painless haematuria
abdominal mass - asymptomatic
hypertension
weight loss

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37
Q

diagnostic test for wilms tumour

A

CT abdomen or MRI

DIFFUSE ANAPLASIA = unfavourable diagnosis and needs more aggressive treatment

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38
Q

management of wilms tumour

A

radiotherapy + chemotherapy + surgical resection via radical nephrectomy with LN sampling

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39
Q

where does osteosarcomas affecr

A

metaphyseal region of long bones - usually distal femur or proximal fibula/ tibia

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40
Q

x ray changes in osteosarcoma

A

osteolytic cortical lesions
cortical erosion
cortical thickening

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41
Q

where does Ewings sarcoma affect

A

flat bones or mid shaft of bone e.g. pelvis, chest wall, vertebra

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42
Q

x ray in ewings sarcoma

A

‘moth eaten’ appearance
cortical thickening

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43
Q

list causes of microcyctic anaemia

A
  1. iron deficiency
  2. thalassamia major or trait
  3. anaemia of chronic disease
44
Q

list causes of macrocytic anaemia

A

folate deficiency
vit B12 deficiency
diamond blackfan anaemia
liver disease
hypothyroidism

45
Q

list causes of red cell aplasia (absent or reduced red cell precursors)

A
  1. diamond blackfan anaemia
  2. parvovirus induced red cell aplasia
  3. transient erythroblastopenia of childhood
46
Q

presentation of diamond blackfan anaemia

A

autosomal dominant
1. craniofacial abnormalities - cleft palate
2. thumb abnormalities - hypoplastic, triphalangeal
3. growth restriction
4. red cell aplasia

47
Q

investigations for diamond blackfan anaemia

A
  1. macrocytic anaemia with low reticulocyte count
  2. normal WCC
  3. increased HbF
  4. raised eADA
  5. bone marrow biopsy * - reduced erythroid precursors
48
Q

cause of iron deficiency anaemia

A
  1. reduced dietary intake - found in red meat, green veg, if drink unfortified milk
  2. malabsorption - coeliac disease, post gastrectomy, h.pylori
  3. blood loss - worms, cancers, ulcers, menorrhagic
49
Q

where is iron absorbed

A

absorbed in duodenum and upper jejunum
regulated by hepcidin (made in liver)

50
Q

presentation of iron deficiency anaemia

A

fatigue, lethargy
SOB
headahce
palpitations
hair loss
angular stomatitis and atrophic glossitis
koilonychia ***

51
Q

investigations reveal in iron deficiency anaemia

A

microcytic hypochromic anaemia
low serum iron and high transferrin = iron deficiency
low serum iron and low transferrin = chronic disease

52
Q

blood test results for haemolysis

A

anaemia
high reticulocytes
high LDH
high unconjugated bilirubin
reduced haptoglobin

53
Q

indicators intravascular vs extravascular haemolysis

A

intravascular- increased fragmented RBC and high LDH

54
Q

inheritance of sickle cell disease

A

autosomal recessive

55
Q

cause of sickle cell disease

A

point mutation of position 6 on beta globin gene (gluatmine -> valine)
results in formation of HbS which is insoluble and rigid
HbS polymerises in low oxygen tension and forms sickle cells which can cause occlusion and thrombosis

56
Q

protective factors in sickle cell

A

alpha thalassamie trait
increased fetal Hb

57
Q

presentation of sickle cell disease

A
  1. chronic haemolytic disease
  2. increased risk of infections for encapsulated organisms e.g. salmonella, pneumococcus - most common in early childhood
  3. jaundice
58
Q

describe sickle cell crises

A
  1. VASO-OCCLUSIVE CRISES
    painful dactylitis
  2. PAINFUL CRISES - common presentation
    swelling of fingers or toes, precipitated by infection dehydration, stress
  3. ACUTE CHEST CRISES
    pleuritic chest pain, fever
  4. SPLENIC OR HEPATIC SEQUESTRATIONS
    splenic enlargement, abdo pain
  5. APLASTIC CRISES
  6. CEREBRAL INFARCTION
59
Q

risk factors for painful crises

A

increased haematocrit or reduced fetal haemoglobin

60
Q

management of painful crises

A

dexamethasone - shorten episodes of pain
PO opiates

61
Q

diagnosis of sickle cell

A

high performance liquid chromatography *
CXR
new born screening !!! Hb electrophoresis

howell jolly bodies indicate hyposplenism

62
Q

management of sickle cell disease

A
  1. prophylactic penicillin
  2. folic acid
  3. vaccinations
  4. hydroxycarbamide/ hydroxyurea (inhibits ribonucleopeptide reductase) - increases HbF levels, prevents vaso occlusive crisis
  5. monthly transfusions
  6. monoclonal antibody (crizanlizumab)
63
Q

management of vaso-occlusive crises

A
  1. analegsia
  2. IV fluids
  3. oxygen
  4. exchange transfusion
  5. IV antibiotics
64
Q

inheritance of thalassaemia

A

autosomal recessive

65
Q

describe alpha thalassaemia

A

deletion of alpha globin on chromosome 16
alpha thalassaemia trait = deletion 12 genes -> asymptomatic

66
Q

describe beta thalassaemia

A

mutation in beta globin gene on chromosome 11
HbF compensates for absent beta globin gene until about 6 months old when fully transition to HbA

beta thalassaemia minor- asymptomatic - reassure
beta thalassaemia major - present 3-6 months, severe anaemia, jaundice, skull bossing

67
Q

diagnosis of beta thalassaemia

A

high performance liquid chromatography **

68
Q

blood results indicating beta thalassaemia

A

microcytic anaemia
high HbF
anaemia
normal ferritin
blood film - target cells, red cell fragments, microcytic, hypochromic

69
Q

management of beta thalassaemia major

A

regular blood transfusions every 3-6 weeks
deforoxamine (iron chelation)

CURE = STEM CELL TRANSPLANT

70
Q

inheritance of hereditary spherocytosis

A

autosomal dominant - common in northern europe

71
Q

pathophysiology in hereditary spherocytosis

A
  1. defect in gene for spectrin ** or ankyrin which make up erythrocyte cytoskeleton
    2.loss of membrane surface
  2. causes sphering of RBCS
  3. more prone to haemolysis and spherocytic red blood cells destroyed prematurely in the spleen (life span =28 days)
72
Q

presentation of hereditary spherocytosis

A

chronic haemolytic anaemia (parvovirus can precipitate aplastic crises)
jaundice + neonatal jaundice (unconjugated)
gallstones
splenomegaly

73
Q

investigations for hereditary spherocytosis

A

FBC - anaemia
increased reticulocyte count
blood film - spherocytes + reticulocytes
unconjugated bilirubinaeamia
negative DAT

74
Q

management of hereditary spherocytosis

A
  1. folate supplementation
  2. blood transfusions
  3. vaccinations
  4. splenectomy > 6 yo
75
Q

diagnosis of pyruvate kinase deficiency

A

blood film - prickle red cells
pyruvate kinase levels reduced

76
Q

function of VWF

A
  1. Carrier protein for factor VIII
  2. attaches platelets to sub endothelium following vessel injury

secreted by endothelial cells + stored in weibel palade bodies

77
Q

cause of VWF deficiency

A

AR or AD - defect in VWF gene on chromosome 12

78
Q

presentation of VWF disease

A

mucosal bleeding
menorrhagia
epistaxis
prolonged bleeding
easy bruising

79
Q

diagnsostic tests for VWF disease

A
  1. low platelets - in type 2b
  2. increased bleeding time - increased APTT
  3. VWF ristocetin cofactor assay
  4. factor vIII deficiency
80
Q

mechanism of action of tranexamic acid

A

inhibit conversion of plasminogen to plasmin and prevents breakdown of fibrin clots

81
Q

what is DIC

A

excessive upregulation and activation of clotting system leading to excess fibrin clots
caused by sepsis , trauma, malignancy

82
Q

blood results in DIC

A

increased PT
increased APTT
reduced platelets
increased fibrinogen
anaemia
reduced platelets

83
Q

cause of HUS

A

toxin related endothelial injury, thrombus formation and increased platelet consumption

84
Q

presentation of ITP

A

1-4 weeks after URTI or live vaccines
petechiae
epistaxis
gingival bleeding
menorrhagia

85
Q

cause of ITP

A

splenic destruction of antibody coated platelets

development of autoantibodies (IGA or IgG) to platelet membrane surface (GPiib/IIa)

86
Q

management of ITP

A
  1. diagnosis of exclusion - blood film to exclude
  2. avoid contact sports
  3. reassure and usually resolves spontaneously
  4. avoid aspririn and ibuprofen
  5. can use pred or transexamic acid or IV IG
  6. monoclonal antibody rituximab is resistant ITP
87
Q

list inherited platelet disorders

A
  1. bernard soulier syndrome
  2. may hegglin anomaly
  3. glanzmann thrombasthenia
  4. wiskott aldrich syndrome
88
Q

inheritance of haemophilia

A

X linked recessive

89
Q

difference between haemophilia A and B

A

haemophilia A - deficiency in factor vIII
haemophilia B - deficiency in factor IX (vit K dependent)

90
Q

Presentation of haemophilia

A

haematoma at delivery
intracranial bleeding at delivery
excessive bleeding
epistaxis, joint bleeding
excessive bruising

91
Q

diagnostic tests for haemophilia

A
  1. factor vIII or IX deficiency (SEVERE <1%)
  2. prolonged APTT
92
Q

management of haemophilia

A
  1. genetic screening
  2. desmopressin
  3. recombinant factor complex as prophyalxis (can develop inhibitors)
  4. s/C vaccinations - avoid IM due to risk of haematoma
93
Q

inheritance of G6PD deficiency

A

X linked recessive

94
Q

presentation of G6PD deficiecny

A

acute haemolytic crisis in response to certain medications and FAVISM (fava beans)
neonatal jaundice

95
Q

drugs causing haemolytic crisis in G6PD deficiency

A

quinines
sulphonamides (co-trimoxazole)
nitrofurantoin
chloramphenicol
vit K
aspirin

96
Q

investigations for G6PD deficiency

A

indirect bilirubin rise
LDH rise
Hb reduced
redd cell G6PD levels reduced
peripheral smear - heinz bodies, bite cells, polychromasia

97
Q

complication of neuroblastoma

A

paraneoplastic syndrome - antibdoies formed against neuroblastoma cells targets cerebellar neurones and causes ataxia

98
Q

describe bernard soulier syndrome

A

deficiency of VWF glycoprotein IB-IX-V receptor
autosomal recessive
thrombocytopenia + large plataletes + increased bleeding time

99
Q

what does ionising radiation do to DNA

A

electron displacement resulting in double stranded breaks in DNA and apoptosis

100
Q

describe mantoux test

A

type IV hypersensitiity test
intradermal

101
Q

function of macrophage

A

detection, phagocytosis and destruction of bacteria

102
Q

reasons for not being to donate blood

A

travel to endemic area in last 6 months
any blood transfusions
organ transplant (risk of creutz felt jacob disease)
deferred for 3 months after body piercing or tattoos
< 17 y/o
< 50kg

103
Q

pathophysiology of alpha thalassaemia

A

defective mRNA that causes reduced or absent synthesis of alpha globin genes resulting in loss 3 alpha globin genes

104
Q

describe neonatal alloimmune thrombocytopenia

A

platelet antigens inherited from mother and father
if mother exposed to fetal antigens from the father that she does not have, she will produce antibodies against them
these antibdoies cross the placenta and then attack the paternal antigens in the fetus which can cause low platelet count at birth

= maternal antibodies to paternal inherited HPA-1a in the baby

105
Q

describe evans syndrome

A

thrombocytopenia + autoimmune haemolytic anaemia

106
Q

cause of aplastic anaemia

A

medications - chloramphenicol
parvovirus

diagnosis = bone marrow aspiration = peripheral blood macrocytosis

FOP/TAS (12 decks)

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