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Haematology/oncology Flashcards

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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
What is the presentation of G6PD?
- Neonatal jaundice - Anaemia - Intermittent jaundice, particularly in response to triggers - Gallstones - Splenomegaly
26
What are the causes of anaemia due to reduced RBC production?
- Bone marrow aplasia - Bone marrow replacement by tumour cells or granulomas - Def - Fe, folic acid, B12 - Thalassaemia = reduced Hb - ACD = reduced EPO
27
What are the causes of anaemia due to increased RBC destruction?
- Hereditary spherocytosis - G6PD - Haemolytic anaemia of newborn - Sickle cell and thalassaemia - Autoimmune haemolysis - DIC - Hypersplenism
28
What are the 2 types of Coombs +ve haemolytic anaemia?
1. Warm autoimmune haemolytic anaemia - IgG, spleen tags cells for splenic phagocytosis 2. Cold autoimmune haemolytic anaemia - IgM complement causing IV haemolysis
29
What are the causes of cold and warm AIHA?
Cold - idiopathic, post infections ~2-3 weeks post infection eg. EBV and mycoplasma Warm - idiopathic, lymphoproliferative neoplasms, drug induced, SLE
30
What are the Coombs -ve haemolytic anaemias?
- Microangiopathic haemolytic anaemia - Physical lysis of RBC = malaria - HUS - DIC
31
What do the results of Coombs test mean?
Coombs +ve = autoimmune haemolytic anaemia Coombs -ve = non autoimmune haemolytic anaemias and others ?
32
What is DIC?
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
33
What are the CF of DIC?
- Fever, confusion, coma - Excess bleeding - epistaxis, gingival bleeding, haematuria, bleeding from cannula sites - Petechiae, hypotension, bruising
34
What are the RF of DIC?
- Major trauma or burns - Multiple organ failure - Severe sepsis or infection - Severe obs complications - Malignancy, esp leukaemia - Incompatible blood transfusion - Transplant rejection
35
What are the blood test signs of DIC?
- 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
What is the management of DIC?
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
What is sickle cell disease?
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
What are the RF of sickle cell?
More common in places traditionally affected by malaria eg. black African or Caribbean
39
What is are the different types of sickle cell crisis?
1. Vaso occlusive - most common type 2. Aplastic crisis 3. Sequestration crisis 4. Acute chest syndrome 5. Hyperhaemolytic crisis - uncommon
40
What is a sequestration crisis?
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
What is acute chest syndrome?
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
What is a vaso occlusive crisis?
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
What is the presentation of sickle cell disease?
- 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
What is the screening for sickle cell?
- Pregnant women at risk of being carriers = tested - Newborn screening heel prick test at 5 days
45
What are the ix into sickle cell?
- FBC - Blood film - sickling of red cells - Sickle solubility test - Hb analysis by electrophoresis = +ve sickling test = Hb A and S
46
What should sickle cell be admitted to hospital?
- 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
What are the general management principles of sickle cell?
- 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
What are some triggers for sickle cell crises?
- Cold - Infection - Dehydration - Exertion - Ischaemia
49
What are the pros and cons of hydroxyurea?
Pros - reduce freq of crises and acute chest syndrome, reduce need for blood transfusions Cons - myelosuppressive, teratogenic, long term toxicity
50
What is the treatment for priapism?
Aspiration and irrigation of corpora cavernosa w adrenaline, needs to be sorted if >2 hours
51
What is the management of acute chest syndrome?
- Abx or antivirals - Blood transfusions - Incentive spirometry - encourages deep breathing - Artificial ventilation w NIV or intubation
52
What are some complications of sickle cell disease?
- Anaemia - Increased risk of infection - encapsulated bacteria - Stroke - AVN in large joints - Pulm HTN - CKD - Priapism - Sickle cell crises and acute chest
53
What are the different types of leukaemia? What ages do they affect?
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
Acute vs chronic and myeloid vs lymphocytic
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
What is the pathophysiology of leukaemia?
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
What are the RF of leukaemia?
- Radiation exposure eg. AXR during pregnancy - Down's, Kleinfelter syndrome = increased risk
57
What are the sx of leukaemia in children?
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
What are the signs of leukaemia in children?
Pale = anaemia Petechiae, purpura, bruising Severe infection Lymphadenopathy = most common finding Hepatosplenomegaly Expiratory wheeze Cranial nerve lesions Testicular enlargement
59
What are the referral criteria for suspected leukaemia? What are the ix?
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
What does FBC and blood film show in leukaemia?
FBC - anaemia, leukopenia, thrombocytopenia, high no abnormal WBC Blood film - blast cells = immature abnormal WBC
61
What is the management of leukaemia in children?
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
What are some complications of chemo?
- Failure to treat - Stunted growth and development - Immunodef and infections - Neurotoxicity - Infertility - Secondary malignancy - Cardiotoxicity
63
What is the prognosis of leukaemia?
ALL - ~80% AML - not as good
64
What are some life threatening presentations of leukaemia?
- Neutropenia = overwhelming sepsis and DIC - Thrombocytopenia = haemorrhage or stroke - Electrolyte imbalance - AKI - Acute airway obstruction - Leukostasis - CNS involvement
65
Hodgkins vs non Hodgkins lymphoma
Hodgkins - Reed Sternberg cells - large abnormal lymphocytes, have multiple nuclei, young adults Non Hodgkins - no Reed Sternberg cells, older pts
66
What is Hodgkins lymphoma?
Malignancy of lymphocytes w Reed Sternberg cells.
67
What are the RF of Hodgkin's lymphoma?
- EBV - HIV - Immunosuppression - Cigarette smoking
68
What are the CF of Hodgkin's lymphoma?
- 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
What are the ix into lymphoma?
- 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
What staging system is used in Hodgkins?
Ann Arbor
71
What is the management of Hodgkin's lymphoma?
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
What are some complications of treating lymphoma?
- Leukaemia in pt treated w chemo - Secondary solid tumours in pt treated w radiotherapy - Male and female infertility
73
What is autosplenectomy?
Physiological loss of spleen function = hyposplenism. Associated w sickle cell anaemia, coeliac disease, dermatitis herpetiformis, UC
74
What are the indications for splenectomy?
- Trauma - Spot rupture, often in splenomegaly eg. glandular fever just minor trauma - Hypersplenism - Neoplasia eg. lymphoma or leukaemic infiltration - Abscess or cyst
75
What are some complications of having a splenectomy?
- Thrombocytosis = raised platelets, can have prophylactic aspirin - Overwhelming post splenectomy infection by encapsulated bacteria
76
What are some causes of hyposplenism?
- Operative splenectomy - Functional hyposplenism - sickle cell, thalassaemia, Hodgkin's, coeliac, IBD - Bone marrow transplant - Congenital asplenia
77
What are the ix into hyposplenism?
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
What are some complications of hyposplenism?
Same as splenectomy - severe infection by encapsulated bacteria. Vaccines for encapsulated bacteria and antimalarial prophylaxis and antibiotic prophylaxis eg. penicillin 5
79
What is pancytopenia and what are some causes?
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
What are the CF of pancytopenia?
- Weakness and fatigue - Rashes and easy bruising - Pale skin - Tachycardia and SOB - Bleeding probs - Infections
81
What is the management of pancytopenia?
Immunosuppressants Drugs that stim bone marrow Bone marrow transplant and transfusions Stem cell transplant
82
What are some differentials for lymphadenopathy in children?
- Kawasaki disease - Lymphoma - Leukaemia - Infections - EBV - TB - SLE - Juvenile idiopathic arthritis
83
What are some differentials for bruising in children?
- Leukaemia - Lymphoma - Non accidental injury - Anaemia - Thrombocytopenia - Clotting disorder
84
What are the features of immune thrombocytopenia?
- Purpuric/petechial rash - Low platelets - Bruising - Uncommonly = bleeding, epistaxis or from gums
85
What is the management of immune/idiopathic thrombocytopenia? IPC
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

Paediatrics (15 decks)

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