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A TO REVISE - PHASE 3A > PAEDS ONCOLOGY/HAEMATOLOGY TO DO > Flashcards

PAEDS ONCOLOGY/HAEMATOLOGY TO DO Flashcards

1
Q

ALL
What is acute lymphoblastic leukaemia (ALL)?

A
  • Affects precursors to B + T cells
  • It leads to uncontrolled proliferation of immature blast cells affecting both the blood + bone marrow (lymphoid progenitor cells and lymphoblasts)
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2
Q

ALL
What are the broad categories of clinical presentation in ALL?

A
  • General = anorexia, fever, weight loss, night sweats
  • Bone marrow infiltration = pancytopenia
  • Reticuloendothelial infiltration = hepatmospenolmegaly, lymphadenopathy
  • Other organ infiltration (more common at relapse) = headache, testicular enlargement, bone pain
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3
Q

ALL
What are some good prognostic factors in ALL?

A
  • Age 2-10
  • Female
  • WCC <20
  • No CNS disease
  • Caucasian
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4
Q

ALL
What is the management for ALL?

A
  • Blood and platelet transfusion
  • Chemotherapy
  • Steroids
  • Allopurinol to prevent tumour lysis syndrome
  • Intrathecal drugs, e.g. methotrexate
  • Acute control of infections with IV antibiotics
  • Neutropenia makes this high risk
  • Stem cell transplant
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5
Q

HODGKINS LYMPHOMA
What is the clinical presentation of Hodgkin’s lymphoma?

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms, e.g. fever
Painful nodes on drinking alcohol
some patients (commonly young women) have disease localised to the mediastinum

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6
Q

HODGKINS LYMPHOMA
What is the management of Hodgkin’s lymphoma?

A
  • Combination chemo ± radiotherapy (overall 80% cured)
  • ABVD
    • Adriamycin
    • Bleomycin
    • Vinblastine
    • Decarbazine
  • autologous marrow transplant
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7
Q

NON-HODGKINS LYMPHOMA
What are the 3 broad presentations of Non-Hodgkin’s lymphoma?

A
  • T-cell malignancies
  • B-cell malignancies
  • Extra-nodal disease
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8
Q

NON-HODGKINS LYMPHOMA
How do T-cell malignancies present?

A
  • May present as ALL or non-Hodgkin lymphoma both being characterised by a mediastinal mass with bone marrow infiltration
  • Mediastinal mass may cause SVC obstruction
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9
Q

NON-HODGKINS LYMPHOMA
How do B-cell malignancies present?

A
  • Present as non-Hodgkin lymphoma with localised lymph node disease, usually in head + neck or abdomen
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10
Q

NON-HODGKINS LYMPHOMA
How does extra-nodal disease present?

A
  • Often GI > pain from obstruction, a palpable mass or even intussusception
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11
Q

NON-HODGKINS LYMPHOMA
What is the management of Non-Hodgkin’s lymphoma?

A

Steroids

R-CHOP
- Monoclonal antibodies to CD20 -> Rituximab
- CHOP regimen:
- Cyclophosphamide
- Hydroxy-daunorubicin
- Vincristine
- Prednisolone

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12
Q

BRAIN TUMOURS
What is a craniopharyngioma?
How does it present?

A
  • Developmental tumour arising from squamous remnant of Rathke pouch
  • Not truly malignant but locally invasive (bitemporal hemianopia often lower quadrant as superior chiasmal compression)
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13
Q

BRAIN TUMOURS
What are some signs of raised ICP?

A
  • Headache worse in morning
  • Papilloedema
  • Vomiting, esp. in the morning
  • Behaviour or personality change
  • Visual disturbance (squint secondary to 6th nerve palsy, nystagmus)
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14
Q

BRAIN TUMOURS
What are some focal neurological signs?

A
  • Spinal tumours = back pain, peripheral weakness of arms/legs or bladder + bowel dysfunction depending on level of lesion
  • Ataxia, seizures
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15
Q

NEUROBLASTOMA
What is a neuroblastoma? Epidemiology?

A
  • Arise from neural crest tissue in the adrenal medulla + sympathetic nervous system,
  • most common <5y,
  • NOT brain tumour
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16
Q

NEUROBLASTOMA
What is the clinical presentation of neuroblastoma?

A
  • Abdominal mass
  • Sx of metastatic = weight loss, hepatomegaly, pallor, bone pain + limp
  • Uncommon = paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules
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17
Q

NEUROBLASTOMA
How does the abdominal mass present?

A
  • Often crosses midline + envelopes major vessels + lymph nodes
  • Can grow very large
  • Classically abdo primary is of adrenal origin
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18
Q

NEUROBLASTOMA
What are the investigations for neuroblastoma?

A
  • Raised urinary catecholamine levels
  • CT/MRI + confirmatory biopsy
  • Evidence of metastatic disease = bone marrow sampling, MIBG scan ±bone scan
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19
Q

NEUROBLASTOMA
What is the management of neuroblastoma?

A
  • Localised primaries + no mets can often be cured with surgery
  • Metastatic = chemo, autologous stem cell rescue, surgery + radio
  • Immunotherapy may be used for long-term maintenance
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20
Q

BONE TUMOURS
What is the clinical presentation of bone tumours?

A
  • Limbs most common site (particularly femur, tibia + humerus)
  • Persistent localised bone pain often precedes mass, otherwise well
  • May be worse at night + cause disrupted sleep
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21
Q

BONE TUMOURS
What are some investigations for bone tumours?

A
  • Raised ALP on bloods
  • Plain XR followed by MRI + bone scan, ?PET scan + bone biopsy
  • CT chest for lung mets + bone marrow sampling to exclude involvement
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22
Q

BONE TUMOURS
How might bone tumours present on radiographs?

A
  • XR = destruction + variable periosteal new bone formation
  • Periosteal reaction leads to classic “sunburst” appearance
  • Ewing sarcoma often shows substantial soft tissue mass
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23
Q

RETINOBLASTOMA
What is a genetic cause of retinoblastoma?
How might it present?

A
  • Retinoblastoma susceptibility gene on chromosome 13 = AD but incomplete penetrance > offer genetic screening
  • All bilateral tumours are hereditary, 20% of unilateral are
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24
Q

RETINOBLASTOMA
What are some complications of retinoblastoma?

A
  • Significant risk of second malignancy (especially sarcoma) amongst survivors of hereditary retinoblastoma
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25
Q

LIVER TUMOURS
What are liver tumours?
In neonates?

A
  • Mostly hepatoblastoma or hepatocellular carcinoma
  • Primary liver tumours in neonates = haemangioma
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26
Q

LIVER TUMOURS
What are the investigations for liver tumours?

A
  • Elevated serum alpha fetoprotein in nearly all cases
  • USS/CT/MRI to visualise the tumour + extent of disease
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27
Q

FANCONI SYNDROME
What is fanconi syndrome?

A
  • Generalised reabsorptive disorder of renal tubular transport in the PCT resulting in…
    – Type 2 (proximal) renal tubular acidosis
    – Polydipsia, polyuria, aminoaciduria + glycosuria
    – Osteomalacia/rickets
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28
Q

FANCONI SYNDROME
What are some causes of fanconi syndrome?

A
  • Usually secondary to inborn errors of metabolism
    – Cystinosis (AR > intracellular accumulation of cysteine, most common)
    – Wilson’s disease, galactosaemia, glycogen storage disorders
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29
Q

FOETAL HAEMOGLOBIN
What is the main difference between HbF + HbA?

A
  • HbF has greater affinity to oxygen than adult so oxygen binds more easily + is more reluctant to let go = crucial for oxygen to transport from maternal to foetal Hb
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30
Q

FOETAL HAEMOGLOBIN
When is Hb concentration highest?
When does the shift from HbF to HbA occur?

A
  • At birth to compensate for low oxygen concentration in the foetus
  • By 6m of age very little HbF produced so HbA predominates
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31
Q

ANAEMIA OVERVIEW
What is anaemia?
How is it defined in paeds?

A
  • Hb level below the normal range
  • Neonate = <14g/dL
  • 1–12m = <10g/dL
  • 1–12y = <11g/dL
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32
Q

ANAEMIA OVERVIEW
What are some causes of decreased red cell production?
What are some clues?

A
  • Ineffective erythropoiesis (Fe, folate deficiency, CKD)
  • Red cell aplasia
  • Normal reticulocytes, abnormal MCV in nutrient deficiencies
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33
Q

ANAEMIA OVERVIEW
What are some causes of haemolysis?
What are some clues?

A
  • G6PD deficiency, haemoglobinopathies, hereditary spherocytosis
  • Raised reticulocytes, abnormal appearance on blood films, +ve direct antiglobulin test if immune cause
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34
Q

ANAEMIA OVERVIEW
What is haemolytic anaemia?
What is the normal lifespan of RBC?

A
  • Characterised by reduced red cell lifespan due to increased red cell destruction in the circulation (intravascular haemolysis) or liver/spleen (extravascular)
  • 120d
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35
Q

ANAEMIA OVERVIEW
How does haemolysis cause anaemia?
What is the difference in haemolytic anaemias in neonates + children?

A
  • Red cell survival reduced significantly but bone marrow production increases too, anaemia = bone marrow cannot compensate
  • Neonates = immune haemolytic anaemias, children = instrinsic abnormalities (G6PD)
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36
Q

ANAEMIA OVERVIEW
List 4 features of haemolytic anaemias

A
  • Anaemia
  • Hepatosplenomegaly
  • Unconjugated bilirubinaemia
  • Excess urinary urobilinogen
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37
Q

ANAEMIA OVERVIEW
What are some causes of anaemia in the neonate?

A
  • Reduced RBC production = congenital red cell aplasia + congenital parvovirus infection > red cell aplasia
  • Haemolytic anaemia = immune (haemolytic disease of newborn) or hereditary (G6PD etc)
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38
Q

ANAEMIA OVERVIEW
What are the main causes of anaemia of prematurity?

A
  • Inadequate erythropoietin production
  • Reduced red cell lifespan
  • Frequent blood sampling whilst in hospital
  • Iron + folic acid deficiency after 2-3m.
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39
Q

IRON DEF ANAEMIA
What are some causes of iron deficiency anaemia?

A
  • Inadequate intake = common as infants require additional iron for increasing blood volume
  • Malabsorption = Crohn’s + coeliac
  • Blood loss = common in menstruating females
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40
Q

IRON DEF ANAEMIA
What are some sources of iron?
What can affect iron absorption?

A
  • Breast milk, formula, cow’s milk or weaning (cereals)
  • Markedly increased when eaten with food rich in vitamin C + inhibited by tannin in tea
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41
Q

IRON DEF ANAEMIA
What are some signs of iron deficiency anaemia?

A
  • Generic = pallor (inc. conjunctival), tachycardia, tachypnoea
  • Pica = consumption of non-food materials
  • Koilonychia, angular cheilitis, brittle hair + nails
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42
Q

IRON DEF ANAEMIA
What are some investigations for iron deficiency anaemia and what would you see?

A
  • FBC = low Hb, microcytic (low MCV + MCH), normal reticulocytes
  • Blood film = hypochromic microcytic red cells
  • Iron studies:
    – Low = serum ferritin, iron + transferrin saturation
    – High = total iron binding capacity
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43
Q

IRON DEF ANAEMIA
What is…

i) transferrin saturation?
ii) total iron binding capacity?

A

i) Proportion of transferrin bound to iron
ii) Total space on transferrin for Fe to bind

44
Q

IRON DEF ANAEMIA
What are some side effects of treatment with oral iron supplementation?

A
  • Constipation
  • Black coloured stools
  • Nausea
45
Q

SICKLE CELL DISEASE
What is the genetics behind sickle cell disease?

A
  • Autosomal recessive
  • Abnormal gene for beta-globin on C11
  • Heterozygous = sickle-cell trait
  • Homozygous = sickle cell disease (HbSS)
46
Q

SICKLE CELL DISEASE
When does sickle cell disease present?
What do all sickle cell disease patients have?

A
  • 6m as HbF unaffected so manifests as HbF reduces
  • All have moderate anaemia with detectable jaundice from chronic haemolysis
  • All have marked increase in infection susceptibility, esp. pneumococci + H. influenzae due to hyposplenism secondary to chronic sickling + microinfarction of the spleen
47
Q

SICKLE CELL DISEASE
What is a severe, classic feature of sickle cell disease?
Common location?
Presentation?
Most severe?

A
  • Vaso-occlusive (painful) crises
  • Bones of limbs + spine common (may lead to avascular necrosis e.g. femoral heads)
  • Pain, fever + often those of triggering infection
  • Acute chest syndrome
48
Q

SICKLE CELL DISEASE
What is acute chest syndrome?
What can cause it?
Management?

A
  • Fever or resp Sx (CP, tachypnoea) with new infiltrates on CXR
  • Can be due to infection (pneumonia, bronchiolitis) or non-infective (pulmonary vaso-occlusion or fat emboli)
  • Emergency > Abx or antivirals, blood transfusions for anaemia, may need NIV or intubation
49
Q

SICKLE CELL DISEASE
Name 2 other vaso-occlusive crises

A
  • ‘Hand-foot syndrome’ common leading to dactylitis
  • Priapism in men > urological emergency, aspiration
50
Q

SICKLE CELL DISEASE
Sickle cell disease may present with acute anaemia (sudden drop in Hb).
What can cause this?

A
  • Haemolytic crises (sometimes with associated infection)
  • Aplastic crises (parvovirus causes cessation of RBC production)
  • Sequestration crises
51
Q

SICKLE CELL DISEASE
What is a sequestration crisis?
What is the management?

A
  • Sudden hepatic or splenic enlargement, abdo pain + circulatory collapse from accumulation of sickled cells blocking blood flow
  • Supportive = blood transfusions, fluid resus, splenectomy can prevent this + used in recurrent crises as can lead to splenic infarction > increased infection susceptibility
52
Q

SICKLE CELL DISEASE
What are some investigations for sickle cell disease?

A
  • Prenatal Dx via CVS
  • Detection via Guthrie test
  • FBC = low Hb, high reticulocytes
  • Blood film = sickled RBCs
  • Dx with Hb electrophoresis showing high amounts of HbSS + absent HbA
53
Q

SICKLE CELL DISEASE
What are some complications of sickle cell disease?

A
  • Short stature + delayed puberty
  • Stroke + cognitive issues
  • Pulmonary HTN
  • Chronic renal failure
  • Psychosocial issues
54
Q

SICKLE CELL DISEASE
What is the general management for sickle cell disease?

A
  • Fully immunised (PCV, HiB, meningococcus)
  • Avoid vaso-occlusive crisis triggers
  • PO phenoxymethylpenicillin prophylaxis
  • PO folic acid as increased demands due to haemolysis
  • Hydroxycarbamide + hydroxyurea can stimulate HbF production to prevent painful crises
  • Bone marrow transplant curative + offered if failed response
55
Q

SICKLE CELL DISEASE
What are some potential triggers of vaso-occlusive crises?
How might these be prevented?

A
  • Cold, dehydration, excessive exercise, stress + hypoxia
  • Dress warmly, plenty of drinks
56
Q

SICKLE CELL DISEASE
What is the management of an acute crisis?

A
  • PO or IV analgesia according to need (?opiates)
  • IV fluids, oxygen
  • Infection treated with Abx, blood transfusion for severe anaemia
  • Exchange transfusion if severe (e.g. neuro complications)
57
Q

THALASSAEMIA
What is thalassaemia?
Consequence?
What are the 2 types?

A
  • AR disorder arising from ≥1 gene defects, resulting in a reduced rate of production of ≥1 globin chains
  • RBCs more fragile + breakdown easily
  • Alpha = defect in alpha globin chains
  • Beta = defect in beta globin chains
58
Q

THALASSAEMIA
What happens if there is deletion of 1 or 2 alpha globin chains?

A
  • Alpha thalassaemia trait
  • Often asymptomatic with mild or absent anaemia
  • Red cells hypochromic + microcytic
59
Q

THALASSAEMIA
What happens if there is deletion of 3 alpha globin chains?

A
  • Mild-moderate hypochromic microcytic anaemia + splenomegaly
  • Few patients are transfusion dependent
60
Q

THALASSAEMIA
What happens if there is deletion of all 4 alpha globin chains?

A
  • Alpha thalassaemia major
  • Death in utero with foetal hydrops from foetal anaemia
  • Occurs in families of South-East Asian origin, homozygotes
61
Q

THALASSAEMIA
What is the epidemiology of beta thalassaemia?
What are the three types?

A
  • Indian subcontinent, Mediterranean + Middle East
  • Beta thalassaemia minor (1 abnormal + 1 normal gene)
  • Beta thalassaemia intermedia (2 defective or 1 defective + 1 deletion genes)
  • Beta thalassaemia major (homozygous for deletion genes)
62
Q

THALASSAEMIA
What is beta thalassaemia minor?
How does it present?
Differentiate?

A
  • Carriers of abnormally functioning beta-globin gene
  • Mild microcytic + hypochromic anaemia (monitor)
  • Differentiate from Fe deficiency by measuring serum ferritin (normal)
63
Q

THALASSAEMIA
What is beta thalassaemia intermedia?
Management?

A
  • More severe microcytic anaemia, beta-globin mutation allow a small amount of HbA and/or a large amount of HbF to be produced
  • Monitor + occasional blood transfusion
64
Q

THALASSAEMIA
What is beta thalassaemia major?
How does it present?

A
  • Most severe form with no HbA as abnormal beta globin gene
    • Severe transfusion-dependent anaemia from 3-6m, jaundice, failure to thrive
65
Q

THALASSAEMIA
What is a complication of beta-thalassaemia major which isn’t common in developed countries?

A
  • Extramedullary haematopoiesis can occur if no regular blood transfusions
  • Leads to hepatosplenomegaly + bone marrow expansion leading to maxillary overgrowth + skull bossing
66
Q

THALASSAEMIA
What are some investigations for beta thalassaemia?

A
  • FBC + blood film = hypochromic microcytic anaemia
  • HbA2 raised in beta-thalassaemia trait, HbA2 + HbF raised in major
  • Serum ferritin to differ between Fe anaemia + check iron overload
  • Hb electrophoresis for Dx
  • DNA testing via CVS before birth
67
Q

THALASSAEMIA
What is the main complication of thalassaemia?
How might this present?

A
  • Repeated + Regular blood transfusions can cause chronic iron overload
  • Heart (cardiomyopathy, heart failure)
  • Liver (cirrhosis)
  • Pancreas (diabetes)
  • Pituitary (delayed growth + sexual maturation)
  • Skin (hyperpigmentation)
  • Arthritis + joint pain
68
Q

THALASSAEMIA
What is the management of thalassaemia?

A
  • Lifelong monthly blood transfusions for the most severe cases
  • Desferrioxamine for iron chelation to prevent overload
  • Bone marrow transplant can be curative, reserved for beta thalassaemia major
69
Q

HAEMOPHILIA
What are the 2 types of haemophilia?
What causes it?

A
  • Haemophilia A = factor VIII deficiency
  • Haemophilia B = factor IX deficiency
  • X-linked recessive (M>F), A>B, girls with Turner’s increased risk as 1 X
70
Q

HAEMOPHILIA
When does haemophilia typically present?
Important differential?

A
  • Around 1y as children become more mobile
  • NAI
71
Q

HAEMOPHILIA
What are some investigations for haemophilia?

A
  • FBC + blood film
  • Prothrombin time (factors 2, 5, 7, 10, extrinsic) normal
  • Activated partial thromboplastin time (intrinsic) = greatly increased
  • Severity dependent on amount of FVIII:C or FIX:C levels
  • Prenatal Dx with CVS
72
Q

HAEMOPHILIA
What is the management of haemophilia?

A
  • IV infusion of recombinant FVIII or FIX concentrate if active bleeding (or prophylactic to reduce arthropathy risk)
  • Desmopressin stimulates vWF release for bleeding/prevention, TXA
  • AVOID aspirin, NSAIDs + IM injections (can worsen bleeding)
73
Q

HAEMOPHILIA
What is a complication of the treatment for haemophilia?

A
  • Formation of antibodies against the clotting factor can render it ineffective
74
Q

VON WILLEBRAND DISEASE
What is the physiological role of von Willebrand factor?

A
  • Facilitates platelet adhesion to damaged endothelium
  • Acts as carrier protein for FVIII:C, protecting it from inactivation + clearance
75
Q

VON WILLEBRAND DISEASE
What is von Willebrand disease (vWD)?
What causes it?
Types?

A
  • Deficiency of vWF leading to defective platelet plug formation + deficient FVIII:C > most common inherited bleeding disorder
  • AD, type 1 most common + mildest
  • Severity increases with type 2, type 3 has very low or no vWF (AR)
76
Q

VON WILLEBRAND DISEASE
What is the clinical presentation of vWD?

A
  • Bruising, excessive + prolonged bleeding after surgery, mucosal bleeding (epistaxis, menorrhagia, bleeding gums)
  • In contrast to haemophilia = spontaneous soft tissue bleeding like large haematomas uncommon
77
Q

VON WILLEBRAND DISEASE
What are some investigations for vWD?

A
  • FBC (normal platelets) + blood film, biochemical screen including renal + liver function
  • Prolonged bleeding time
  • Prothrombin time normal
  • APTT = elevated or normal
  • vWF antigen decreased, vWF multimers variable
78
Q

VON WILLEBRAND DISEASE
What is the management of vWD?

A
  • Pressure applied if active bleeding
  • Minimise bleeding with desmopressin or TXA
  • Severe = plasma derived FVIII concentrate or vWF infusion
  • AVOID aspirin, NSAIDs + IM injections as can worsen bleeding
79
Q

VON WILLEBRAND DISEASE
How is desmopressin given?
What does it do?

A
  • Nasal or s/c
  • Release of vWF + FVIII concentrate
80
Q

COAGULATION DISORDERS
What are acquired disorders of coagulation?

A

Secondary to

  • Haemorrhagic disease of the newborn due to vitamin K deficiency
  • Liver disease as location of clotting factor production
  • ITP + DIC
81
Q

COAGULATION DISORDERS
What can cause vitamin K deficiency?

A
  • Inadequate intake = neonates, long-term chronic illness
  • Malabsorption = coeliac, cystic fibrosis
  • Vitamin K antagonists = warfarin
82
Q

ITP
What is immune thrombocytopenic purpura (ITP)?

A
  • Commonest cause of thrombocytopenia in childhood
  • T2 hypersensitivity reaction with destruction of circulating platelets by anti-platelet IgGs
83
Q

ITP
What are the investigations for ITP?

A
  • FBC shows marked thrombocytopenia
  • May have compensatory megakaryocyte increase in bone marrow
84
Q

ITP
What is the management of ITP?

A
  • Often acute + self-limiting
  • Severe bleeding may need prednisolone, IVIg, blood/platelet transfusions
85
Q

HAEMOLYTIC DISEASE OF THE NEWBORN
what is the clinical presentation?

A
  • anti-D antibodies in mother detected by Coombe’s test that all women have at 1st antenatal appointment
  • routine USS may detect hydrops fetalis or polyhydramnios
  • mild cases = jaundice, pallor + hepatosplenomegaly, hypoglycaemia
  • severe cases = oedema, petechiae + ascites
86
Q

HAEMOLYTIC DISEASE OF THE NEWBORN
what are the investigations?

A
  • indirect coombe’s test show antibodies
  • antenatal USS shows hydrops fetalis
  • fetal blood sample
87
Q

HAEMOLYTIC DISEASE OF THE NEWBORN
what is the management in utero?

A
  • transfusion of O negative packed cells cross-matched with maternal blood at 16-18 weeks
88
Q

HAEMOLYTIC DISEASE OF THE NEWBORN
what is the management after delivery?

A

50% = normal haemoglobin + bilirubin but should be monitored for anaemia for 6-8 weeks
25% = require transfusion + may require phototherapy to avoid kernicterus
25% = stillborn or have hydrops fetalis

89
Q

HAEMOLYTIC DISEASE OF THE NEWBORN
what are the complications?

A
  • kernicterus which can cause extrapyramidal, auditory and visual abnormalities and cognitive deficit
  • late-onset anaemia
  • graft-versus-host disease
  • portal vein thrombosis + portal hypertension
90
Q

HAEMOLYTIC DISEASE OF THE NEWBORN
how can it be prevented?

A

identify all women who have been sensitised by coombe’s testing at first antenatal visit

anti-D immunoglobulin should be given to all rhesus negative women at 28 + 34 weeks

91
Q

ALL
What are the risk factors for acute lymphoblastic leukaemia (ALL)?

A
  • Trisomy 21,
  • immunocompromised (HIV, immunosuppressants)
92
Q

ALL
What do blood and bone marrow tests show in ALL?

A

FBC and blood film = WCC usually high
Blast cells on film and in bone marrow

93
Q

HODGKINS LYMPHOMA
What blood results may you see in someone with Hodgkin’s lymphoma?

A
  • high ESR
  • FBC = anaemia (normochromic normocytic)
  • reed sternberg cells
  • low Hb
  • high serum lactase dehydrogenase
94
Q

NON-HODGKINS LYMPHOMA
What are the signs and symptoms of non-hodgkins lymphoma?

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms, e.g. fever
GI and skin involvement

95
Q

AML
What is acute myeloid leukaemia (AML)?

A

Neoplastic proliferation of blast cells (immature blood cells)
affects myeloid progenitor cells and myeloblasts

96
Q

AML
What are the risk factors for AML?

A

Preceding haematological disorders
Prior chemotherapy
Exposure to ionising radiation
Down’s syndrome

97
Q

AML
what are the clinical features of AML?

A

Anaemia -> breathlessness, fatigue, pallor
Infection
Hepatosplenomegaly
Peripheral lymphadenopathy
Gum hypertrophy
Bone marrow failure and bone pain

98
Q

AML
What would you expect to see on an FBC and bone marrow biopsy in someone you suspect to have AML?

A

FBC = anaemia and thrombocytopenia and neutropenia

BM biopsy = leukaemic blast cells (with Auer rods)

99
Q

AML
Describe the treatment for AML

A

Blood and platelet transfusions
IV fluids
Allopurinol to prevent tumour lysis
Infection control with IV antibiotics
Chemotherapy
Steroids
Sibling matched allogenic bone marrow transplant

100
Q

CML
What is chronic myeloid leukaemia (CML)?

A

Uncontrolled clonal proliferation of myeloid cells (basophils, eosinophils and neutrophils)

101
Q

CML
what are the clinical features of CML?

A

Insidious onset

Symptomatic anaemia
Abdominal pain - splenomegaly
Weight loss, tiredness, palor
Gout - due to purine breakdown
Bleeding - due to platelet dysfunction

102
Q

CML
what are the investigations for CML?

A

FBC - anaemia, raised myeloid cells, high WCC (eosinophilia, basophilia, neutrophilia)
Increased B12
Blood film - left shirt, basophilia
Bone marrow biopsy - increased cellularity
Philadelphia chromosome seen in 80+% of cases  t(9;2) - Stimulates cell division

103
Q

CML
What is the treatment for CML?

A

Chemotherapy
Tyrosine kinase inhibitors, e.g. Imatinib - Given orally
Stem cell transplant

104
Q

CML
Why does the Philadelphia chromosome cause CML?

A

FORMS fusion gene BCR/ABL on chromosome 22 –> tyrosine kinase activity –> stimulates cell division

105
Q

CLL
What is Chronic lymphoblastic leukaemia (CLL)?

A

Proliferation of mature B lymphocytes leads to accumulation of mature B cells that have escaped apoptosis
Chronic malignant transformation of mature lymphoid cells

106
Q

CLL
what are the investigations for CLL?

A

● Normal or low Hb
● Raised WCC with very high lymphocytes
● Blood film – smudge cells may be seen in vitro

107
Q

CLL
What is the treatment for CLL?

A

Watch and wait
Chemotherapy
Monoclonal antibodies, e.g. rituximab
Targeted therapy, e.g. bruton kinase inhibitors (ibrutinib)

A TO REVISE - PHASE 3A (16 decks)

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