Haematology/oncology Flashcards

1
Q

What are some causes of infant anaemia?

A
  • Physiological anaemia of infancy
  • Blood loss
  • Haemolysis
  • Twin twin transfusion
  • Anaemia of prematurity
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2
Q

What are some causes of haemolysis in a neonate?

A
  • Haemolytic disease of the newborn
  • Hereditary spherocytosis
  • G6PD def
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3
Q

What is physiologic anaemia of infancy/neonates?

A

Normal dip in Hb ~6-9 weeks in healthy term babies. There is high Hb levels at birth = high O2 delivery = -ve feedback = reduction in EPO production = reduced Hb produced. Is not pathological.

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4
Q

What is anaemia of prematurity?

A

Premature neonates more likely to become anaemic than full term neonates:
- Less time in utero receiving Fe from mother
- RBC production can’t keep up with rapid growth in first few weeks
- Reduced EPO levels
- Blood tests remove a significant portion of circ vol

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5
Q

What is haemolytic disease of the newborn?

A

RBC rhesus antigens on mother are different to on fetus eg. mother rhesus D -ve and fetus rhesus D +ve = mother sensitised and produces Ab to rhesus D+ve, fine in first pregnancy but in second pregnancy w baby with rhesus D +ve = mothers Ab attach to RBC of fetus and fetus immune system attacks RBC = haemolysis = anaemia and high bilirubin.

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6
Q

How do you ix haemolytic disease of the newborn?

A

direct Coombs test = DCT, +ve in haemolytic anaemia

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7
Q

What are some causes of anaemia in older children?

A
  • Fe def anaemia
  • Blood loss - menstruation
  • Sickle cell
  • Thalassaemia
  • Leukaemia
  • Hereditary spherocytosis or eliptocytosis
  • Sideroblastic anaemia
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8
Q

What is a common cause of blood loss anaemia in developing countries? How is it treated?

A

Helminth infection eg. roundworms
Treat - albendazole Probs don’t need to know !

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9
Q

What are the causes of microcytic anaemia?

A

TAILS
Thalassaemia
ACD
Iron def anaemia
Lead poisoning
Sideroblastic anaemia

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10
Q

What are the causes of normocytic anaemia?

A

3As and 2Hs
Acute blood loss
ACD
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism

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11
Q

What are the causes of macrocytic anaemia?

A

Megaloblastic - B12 or folate def
Normoblastic macrocytic anaemia: alcohol, reticulocytosis, hypothyroidism, liver disease, drugs eg. azathioprine

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12
Q

What are the sx of anaemia?

A

Generic sx - tiredness, SOB, headaches, dizziness, palpitations, worsening of other conditions
Specific to Fe def anaemia - pica and hair loss

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13
Q

What are some signs of specific causes of anaemia?

A

Koilonychia - spoon shaped nails - Fe def
Angular chelitis - Fe def
Atrophic glossitis - Fe def
Brittle hair and nails - Fe def
Jaundice - haemolytic anaemia
Bone deformities - thalassaemia

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14
Q

What are the ix into anaemia?

A

FBC - Hb and MCV
Blood film
Reticulocyte count, high = haemolytic anaemia
Ferritin
B12 and folate
Bilirubin
Direct Coombs test
Hb electrophoresis - haemoglobinopathies

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15
Q

What is G6PD def?

A

Glucose 6 phosphate def - X linked recessive red cell enzyme disorder.
G6PD enzyme protects cells from reactive O2 species, if def = more vulnerable to ROS = haemolysis in RBC. Get acute haemolytic anaemia in periods of increased stress due to increased ROS.

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16
Q

What are some triggers of G6PD?

A
  • Intercurrent illness or infection
  • Fava/broad beans
  • Henna
  • Medications - primaquine (antimilarials), nitrofurantoin, dapsone, NSAIDs/aspirin
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17
Q

What are the ix into G6PD and what is the management?

A

Ix - blood film = Heinz bodies and bite cells
Treat - avoid triggers, some pt may require transfusion

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18
Q

What is extravascular haemolytic anaemia and what are some of the causes?

A

Spleen and liver (RES) haemolysis abnormal RBC and those marked by Ab for splenic phagocytosis. There is normally splenomegaly and hepatomegaly.
- Sickle cell
- Hereditary spherocytosis

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19
Q

What is hereditary spherocytosis?

A

RBC are sphere shaped = spherocytes on blood film, easily haemolysed when passing through the spleen. Autosomal dominant.

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20
Q

What are the CF of hereditary spherocytosis?

A
  • Jaundice
  • Anaemia
  • Gallstones
  • Splenomegaly
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21
Q

What is a haemolytic crisis?

A

Haemolysis, anaemia and jaundice are more significant, triggered by infections

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22
Q

What is an aplastic crisis?

A

Often triggered by parvovirus infection.
Temp cessation of erythropoiesis = severe anaemia w/o reticulocyte response. Drop in Hb over ~1 week. Recovery may be spont but normally need transfusion.
Pt can present w high output congestive HF.

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23
Q

What are the ix into hereditary spherocytosis?

A
  • Spherocytes on blood film
  • MCHC raised on FBC
  • Reticulocytes raised unless aplastic crisis
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24
Q

What is the treatment of hereditary spherocytosis?

A
  • Folate supplementation
  • Splenectomy
  • Cholecystectomy if gallstones are causing problems
  • Transfusion in acute crisis
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25
Q

What is the presentation of G6PD?

A
  • Neonatal jaundice
  • Anaemia
  • Intermittent jaundice, particularly in response to triggers
  • Gallstones
  • Splenomegaly
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26
Q

What are the causes of anaemia due to reduced RBC production?

A
  • Bone marrow aplasia
  • Bone marrow replacement by tumour cells or granulomas
  • Def - Fe, folic acid, B12
  • Thalassaemia = reduced Hb
  • ACD = reduced EPO
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27
Q

What are the causes of anaemia due to increased RBC destruction?

A
  • Hereditary spherocytosis
  • G6PD
  • Haemolytic anaemia of newborn
  • Sickle cell and thalassaemia
  • Autoimmune haemolysis
  • DIC
  • Hypersplenism
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28
Q

What are the 2 types of Coombs +ve haemolytic anaemia?

A
  1. Warm autoimmune haemolytic anaemia - IgG, spleen tags cells for splenic phagocytosis
  2. Cold autoimmune haemolytic anaemia - IgM complement causing IV haemolysis
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29
Q

What are the causes of cold and warm AIHA?

A

Cold - idiopathic, post infections ~2-3 weeks post infection eg. EBV and mycoplasma
Warm - idiopathic, lymphoproliferative neoplasms, drug induced, SLE

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30
Q

What are the Coombs -ve haemolytic anaemias?

A
  • Microangiopathic haemolytic anaemia
  • Physical lysis of RBC = malaria
  • HUS
  • DIC
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31
Q

What do the results of Coombs test mean?

A

Coombs +ve = autoimmune haemolytic anaemia
Coombs -ve = non autoimmune haemolytic anaemias and others ?

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32
Q

What is DIC?

A

Disseminated IV coag - inappropriate activation of clotting cascades = thrombus formation and depletion of CF and platelets:
1. Lots of little clots form everywhere, uses up all CF
2. Bleed loads because reduced CF

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33
Q

What are the CF of DIC?

A
  • Fever, confusion, coma
  • Excess bleeding - epistaxis, gingival bleeding, haematuria, bleeding from cannula sites
  • Petechiae, hypotension, bruising
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34
Q

What are the RF of DIC?

A
  • Major trauma or burns
  • Multiple organ failure
  • Severe sepsis or infection
  • Severe obs complications
  • Malignancy, esp leukaemia
  • Incompatible blood transfusion
  • Transplant rejection
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35
Q

What are the blood test signs of DIC?

A
  • Thrombocytopenia = low platelets
  • Increased prothrombin time - takes longer for blood to clot
  • Increased D dimers
  • Decreased fibrinogen - helps w blood clotting
    (CF used up in lots of little clots)
36
Q

What is the management of DIC?

A

Supportive - blood components, treat underlying condition
- Platelet <50 = platelet transfusion
- Prolonged PT = fresh frozen plasma but
- Fibrinogen <1g/L = fibrinogen concentrate
- Thrombosis is predominating = therapeutic heparin
- Non bleeding = prophylaxis of VTE w prophylactic heparin or LMWH

37
Q

What is sickle cell disease?

A

Autosomal recessive mutation in B globin gene -> glutamic acid to valine = sickling of RBC = HbSS. Sickling causes vaso occlusion and chronic haemolysis.
x1 HbS allele = carriers and those w 2 = HbSS and have the disease.

38
Q

What are the RF of sickle cell?

A

More common in places traditionally affected by malaria eg. black African or Caribbean

39
Q

What is are the different types of sickle cell crisis?

A
  1. Vaso occlusive - most common type
  2. Aplastic crisis
  3. Sequestration crisis
  4. Acute chest syndrome
  5. Hyperhaemolytic crisis - uncommon
40
Q

What is a sequestration crisis?

A

Sudden splenic enlargement = reduced Hb conc, circ collapse and hypovolaemic shock.
Treated by transfusion, as soon as possible to reduce mortality.
Recurrent splenic sequestration = indication for splenectomy.

41
Q

What is acute chest syndrome?

A

Vaso occlusive crisis affecting the lungs - new pulmonary infiltrate on chest radiograph with one or more:
- Fever
- Cough
- Sputum
- Tachypnoea or dyspnoea
- New onset hypoxia
Children = lung infections
Adults = infarcts
Is a medical emergency

42
Q

What is a vaso occlusive crisis?

A

Vaso occlusive crisis = small vessels obstructed by sickle cells = ischaemia = pain, ranging from mild to severe:
- Swollen painful joints
- Tachypnoea
- Neuro signs
- Mesenteric sickling and bowel ischaemia = abdo distension and pain
- Renal necrosis = renal colic or severe haematuria
- Priapism
Most common type.

43
Q

What is the presentation of sickle cell disease?

A
  • Usually between 3 and 6m when HbF levels start to fall
  • Anaemia, jaundice, pallor, lethargy, growth restriction, weakness
  • Infections by encapsulated bacteria
  • Splenomegaly
  • Delayed puberty
44
Q

What is the screening for sickle cell?

A
  • Pregnant women at risk of being carriers = tested
  • Newborn screening heel prick test at 5 days
45
Q

What are the ix into sickle cell?

A
  • FBC
  • Blood film - sickling of red cells
  • Sickle solubility test
  • Hb analysis by electrophoresis = +ve sickling test = Hb A and S
46
Q

What should sickle cell be admitted to hospital?

A
  • Severe pain not controlled by simple analgesia or low dose opioids
  • Dehydration
  • Severe sepsis
  • Acute chest syndrome signs or sx
  • New neuro sx and signs - risk of stroke
  • Acute fall in Hb signs
  • Acute enlargement of spleen or liver over 24 hours
  • Marked increase in jaundice
  • Haematuria
  • Priapism >2 hours - emergency
47
Q

What are the general management principles of sickle cell?

A
  • Avoid triggers of crises
  • Vaccination
  • Abx prophylaxis - penicillin V/phenoxymethylpenicillin
  • Hydroxycarbamide/hydroxyurea to produce HbF (doesn’t sickle)
  • Blood transfusion
  • Bone marrow transplant can be curative
  • Regular palpation of splenic size
48
Q

What are some triggers for sickle cell crises?

A
  • Cold
  • Infection
  • Dehydration
  • Exertion
  • Ischaemia
49
Q

What are the pros and cons of hydroxyurea?

A

Pros - reduce freq of crises and acute chest syndrome, reduce need for blood transfusions
Cons - myelosuppressive, teratogenic, long term toxicity

50
Q

What is the treatment for priapism?

A

Aspiration and irrigation of corpora cavernosa w adrenaline, needs to be sorted if >2 hours

51
Q

What is the management of acute chest syndrome?

A
  • Abx or antivirals
  • Blood transfusions
  • Incentive spirometry - encourages deep breathing
  • Artificial ventilation w NIV or intubation
52
Q

What are some complications of sickle cell disease?

A
  • Anaemia
  • Increased risk of infection - encapsulated bacteria
  • Stroke
  • AVN in large joints
  • Pulm HTN
  • CKD
  • Priapism
  • Sickle cell crises and acute chest
53
Q

What are the different types of leukaemia? What ages do they affect?

A

Acute lymphoblastic leukaemia - most common (in children) 2-3 years
Acute myeloid leukaemia - next most common, less than 2 years
Chronic myeloid leukaemia - rare

54
Q

Acute vs chronic and myeloid vs lymphocytic

A

Acute - due to impaired cell differentiation = lots of malignant precursor cells in bone marrow
Chronic - excessive proliferation of mature malignant cells but cell differentiation is unaffected
Myeloid - myeloid precursor cell eg. neutrophils
Lymphocytic - lymphoid precursor eg. B cell

55
Q

What is the pathophysiology of leukaemia?

A

Genetic mutation in one precursor cells in bone marrow = excessive production of a single type of abnormal WBC.
This suppresses production of other cell types = pancytopenia:
Anaemia - low RBC
Leukopenia - low WBC
Thrombocytopenia - low platelets

56
Q

What are the RF of leukaemia?

A
  • Radiation exposure eg. AXR during pregnancy
  • Down’s, Kleinfelter syndrome = increased risk
57
Q

What are the sx of leukaemia in children?

A

General malaise, fatigue
Prolonged/recurrent fever
Irritability
Failure to thrive
SOB and reduced exercise tolerance
Dizziness and palpitations
Bleeding - epistaxis, bleeding gums, easy bruising
Bone/joint pain esp legs
Constipation
Cough
N+V, esp if CNS infiltration
Night sweats

58
Q

What are the signs of leukaemia in children?

A

Pale = anaemia
Petechiae, purpura, bruising
Severe infection
Lymphadenopathy = most common finding
Hepatosplenomegaly
Expiratory wheeze
Cranial nerve lesions
Testicular enlargement

59
Q

What are the referral criteria for suspected leukaemia? What are the ix?

A

Any children w unexplained petechiae or hepatomegaly need specialist assessment.
If have non specific signs and suspect leukaemia = FBC within 48 hours.
Ix - FBC, blood film, bone marrow and lymph node biopsy
Staging - CXR, CT, LP

60
Q

What does FBC and blood film show in leukaemia?

A

FBC - anaemia, leukopenia, thrombocytopenia, high no abnormal WBC
Blood film - blast cells = immature abnormal WBC

61
Q

What is the management of leukaemia in children?

A

ALL - high intensity chemo, usually via Hickman line, and asparaginase, can have bone marrow transplant to eliminate residual leukaemic cells but has significant morbidity and mortality associated w it
AML - intensive chemo then needs to be supported through period of marrow suppression until haematopoeietic recovery occurs

62
Q

What are some complications of chemo?

A
  • Failure to treat
  • Stunted growth and development
  • Immunodef and infections
  • Neurotoxicity
  • Infertility
  • Secondary malignancy
  • Cardiotoxicity
63
Q

What is the prognosis of leukaemia?

A

ALL - ~80%
AML - not as good

64
Q

What are some life threatening presentations of leukaemia?

A
  • Neutropenia = overwhelming sepsis and DIC
  • Thrombocytopenia = haemorrhage or stroke
  • Electrolyte imbalance
  • AKI
  • Acute airway obstruction
  • Leukostasis
  • CNS involvement
65
Q

Hodgkins vs non Hodgkins lymphoma

A

Hodgkins - Reed Sternberg cells - large abnormal lymphocytes, have multiple nuclei, young adults
Non Hodgkins - no Reed Sternberg cells, older pts

66
Q

What is Hodgkins lymphoma?

A

Malignancy of lymphocytes w Reed Sternberg cells.

67
Q

What are the RF of Hodgkin’s lymphoma?

A
  • EBV
  • HIV
  • Immunosuppression
  • Cigarette smoking
68
Q

What are the CF of Hodgkin’s lymphoma?

A
  • Young adults w cervical or supraclavicular non tender lymphadenopathy = typical
  • Alcohol induced pain = suggestive but rare
  • Compression of surrounding structures eg. SOB or abdo pain
  • B sx = fever night sweats weight loss in 30%
  • Hepatomegaly or splenomegaly
69
Q

What are the ix into lymphoma?

A
  • FBC - exclude leukaemia, mononucleosis and other causes of lymphadenopathy
  • FNA lymph node and biopsy w Reed Sternberg cells = diagnostic
  • Low haem and raised LDH indicate high red cell turnover = poor prognosis
  • Need HIV test
  • CXR and CT thorax abdo for staging
70
Q

What staging system is used in Hodgkins?

A

Ann Arbor

71
Q

What is the management of Hodgkin’s lymphoma?

A

Initial - chemoradiotherapy
High dose chemo followed by autologous stem cell transplant if don’t respond to initial therapy.
Vaccines - pneumococcal and influenza, meningococcal
Reproductive counselling
Can be cured in 80-90% of pt

72
Q

What are some complications of treating lymphoma?

A
  • Leukaemia in pt treated w chemo
  • Secondary solid tumours in pt treated w radiotherapy
  • Male and female infertility
73
Q

What is autosplenectomy?

A

Physiological loss of spleen function = hyposplenism.
Associated w sickle cell anaemia, coeliac disease, dermatitis herpetiformis, UC

74
Q

What are the indications for splenectomy?

A
  • Trauma
  • Spot rupture, often in splenomegaly eg. glandular fever just minor trauma
  • Hypersplenism
  • Neoplasia eg. lymphoma or leukaemic infiltration
  • Abscess or cyst
75
Q

What are some complications of having a splenectomy?

A
  • Thrombocytosis = raised platelets, can have prophylactic aspirin
  • Overwhelming post splenectomy infection by encapsulated bacteria
76
Q

What are some causes of hyposplenism?

A
  • Operative splenectomy
  • Functional hyposplenism - sickle cell, thalassaemia, Hodgkin’s, coeliac, IBD
  • Bone marrow transplant
  • Congenital asplenia
77
Q

What are the ix into hyposplenism?

A

Blood film - things that would normally be eliminated by spleen
- Howell Jolly bodies - remnants of nucleus in RBC
- Pappenheimer bodies - iron in RBC
- Target cells

78
Q

What are some complications of hyposplenism?

A

Same as splenectomy - severe infection by encapsulated bacteria.
Vaccines for encapsulated bacteria and antimalarial prophylaxis and antibiotic prophylaxis eg. penicillin 5

79
Q

What is pancytopenia and what are some causes?

A

Decrease in all blood cells due to problems w bone marrow.
- Cancer, lupus, bone marrow disorders
- Infections
- Drugs
- Environmental toxins eg. radiation
- Chemoradiotherapy
- Autoimmune disorders
- Idiopathic

80
Q

What are the CF of pancytopenia?

A
  • Weakness and fatigue
  • Rashes and easy bruising
  • Pale skin
  • Tachycardia and SOB
  • Bleeding probs
  • Infections
81
Q

What is the management of pancytopenia?

A

Immunosuppressants
Drugs that stim bone marrow
Bone marrow transplant and transfusions
Stem cell transplant

82
Q

What are some differentials for lymphadenopathy in children?

A
  • Kawasaki disease
  • Lymphoma
  • Leukaemia
  • Infections
  • EBV
  • TB
  • SLE
  • Juvenile idiopathic arthritis
83
Q

What are some differentials for bruising in children?

A
  • Leukaemia
  • Lymphoma
  • Non accidental injury
  • Anaemia
  • Thrombocytopenia
  • Clotting disorder
84
Q

What are the features of immune thrombocytopenia?

A
  • Purpuric/petechial rash
  • Low platelets
  • Bruising
  • Uncommonly = bleeding, epistaxis or from gums
85
Q

What is the management of immune/idiopathic thrombocytopenia? IPC

A

No bleeding = no treatment, resolves in most children in 6 months
<10 platelets or significant bleeding:
- 1st line = Oral/IV steroids
- IV immunoglobulin if life threatening severe bleed
- Platelet transfusion in emergency