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Paeds Written - HAEM Flashcards

1
Q

Epidemiology of ALL

A

Peak age 2-5 yo
Boys slightly > girls
Trisomy 21 increases risk!!

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2
Q

Presentation of ALL

A 
Bone pain
Splenomegaly
Hepatomegaly
Testicular swelling
Fever
Leukaemia cutis - petechial rash on face & trunk
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3
Q

Poor prognostic factors in ALL

A 
Age <2 or >10
WBC >20 at diagnosis
T or B cell surface markers
Not White
Male
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4
Q

Ix findings in ALL

A

BM biopsy >20% blasts

Film –> peripheral blasts

FBC, clotting –> anaemia, thrombocytopenia, neutropenia +/- DIC

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5
Q

Biochemical evidence of tumour lysis syndrome

A

High K+
High phosphate
High uric acid
High LDH

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6
Q

Pathophysiology of Idiopathic Thrombocytopenic Purpura

A

Immune-mediated (type 2 hypersensitivity)

Abs directed against glycoprotein IIb/IIIa or Ib-V-IX complex

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7
Q

Triggers for ITP

A

Viral infection

Live vaccine

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8
Q

Presentation of ITP

A

NO FEVER
Petechiae or purpuric rash
Bleeding e.g. epistaxis, gingival bleeding - less common

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9
Q

Management of ITP

A

Usually no tx needed
Advise to avoid activities that may cause trauma e.g. contact sports

If very low Plts (<10) or bleeding:

  • oral/IV corticosteroids
  • IV Ig
  • platelet transfusion? - only in emergency, will soon be destroyed
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10
Q

Signs & symptoms of Hodgkin’s lymphoma

A

Painless lymphadenopathy (neck)
Painful after EtOH
B symptoms - fever, night sweats, weight loss

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11
Q

Lymph node biopsy findings in Hodgkin’s lymphoma

A

Reed-Sternberg Cells - Owl’s eyes appearance

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12
Q

Staging for lymphoma

A

Ann Arbor

1 = one group of nodes
2 = >1 group of nodes, same side of diaphragm
3 = node above and below diaphragm
4 = extra nodal spread

A = no B Sx, B = B Sx

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13
Q

Diagnostic markers for Hodgkin’s lymphoma

A

CD30

CD15

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14
Q

Hodgkin’s lymphoma chemotherapy regime

A

ABVD

Adriamycin
Bleomycin
Vincristine
DTIC (Dacarbazine)

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15
Q

Specific types of Non Hodgkin’s lymphoma to know

A

H. Pylori associated gastric MALToma

Enteropathy associated T cell lymphoma - coeliac disease

HIV - adult T cell lymphoma

Transpant & EBV - post transplant lymphoproliferative disease

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16
Q

Histopathology of Burkitt’s lymphoma

A

Starry sky appearance

Germinal centre cells

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17
Q

Types of Burkitt lymphoma

A

Endemic - EBV infection, children in malaria endemic regions, involves jaw or fatal bones

Sporadic - EBV infection, ‘Western’ world

Immunodeficiency - HIV infection or post transplant

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18
Q

Presentation of Ewing’s sarcoma

A

Mass or swelling
Bone pain
Malaise, fever
Paralysis - may precipitate osteomyelitis

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19
Q

Where does Ewing’s sarcoma occur in the body?

A

Long bones of arms, legs

chest, skull & trunk

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20
Q

Radiological finding in Ewing’s sarcoma

A

Bone destruction

Onion skinning - overlying periosteal bone formation

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21
Q

Histology of Ewing’s sarcoma

A

Small round blue cells

22
Q

Most common primary bone malignancy of children?

A

Osteosarcoma

23
Q

Presentation of osteosarcoma

A

Relatively painless
Mass/swelling
Restricted movement
Rapid mets to lung

24
Q

Radiological findings in Osteosarcoma

A

Sunburst appearance - soft tissue calcification

Codman’s triangle - elevated periosteum

25
Origin of neuroblastoma
Neural crest tissue | anywhere in sympathetic chain - most commonly adrenal medulla
26
Presentation of neuroblastoma
Abdominal mass - depends on site of tumour Systemic - weight loss, pallor, hepatomegaly, bone pain, limp Spinal cord compression Sx
27
Ix findings in neuroblastoma
Raised urinary catecholamine metabolites - VMA / HVA + confirmatory biopsy from BM + MIBG radioisotope scan (highlights areas e.g. phaeo, neuroblastoma)
28
Management of neuroblastoma
May spontaneously regress - very young infant Localised primary = surgery Metastatic = chemotherapy + radiotherapy (with autologous stem cell rescue) + surgery
29
Pathophysiology of Sickle cell disease
Point mutation in codon 6 of beta globin gene on Chr11 Causes presence of structurally abnormal haemoglobin - HbS Low oxygen conditions (or acidosis) --> aggregation of HbS --> occlusion of vessels
30
Forms of sickle cell disease
Sickle cell anaemia = homozygous HbSS Sickle cell trait = heterozygous for HbAS - mild anaemia Hb C disease - milder sickling than HbSS
31
Presentation of sickle cell anaemia
chronic haemolysis - anaemia, splenomegaly, gallstones, aplastic crises (parvovirus B19) Vaso-occlusive painful crises - dactylitis, acute chest syndrome, stroke, priapism, splenic sequestration, retinopathy, renal dysfunction Infection - encapsulated organisms esp. Long term - short stature, delayed puberty, heart failure, psychosocial issues
32
Mechanisms of anaemia in sickle cell anaemia
Haemolysis BUT also HbS is lower affinity Hb so more readily releases O2 to tissues --> lower EPO drive
33
Ix for sickle cell disease
Family origins questionnaire Guthrie/heelprick testing (neonate) FBC & blood smear --> nucleated RBC, sickle cells, Howell-Jolly bodies Solubility testing Haemoglobin electrophoresis = gold standard
34
Electrophoresis results in sickle cell disease
Normal beta globin (glutamine) = very +ve charge Defective beta globin (HbS - valine) = neutral Defective beta globin (HbC - lysine) = very -ve charge
35
management of painful crises
Admit (unless only mid-mod pain + apyrexial) Analgesia within 30 mins - regular paracetamol + NSAIDs - opioid Oxygen if sats <95% Corticosteroids NOT usually given in uncomplicated crises
36
Long term management principles for sickle cell disease
Hydroxycarbamide - increases foetal Hb Prophylactic penicillin V Immunisations (encapsulated organisms - strep, pneumococcus, HiB) Folic acid Nutrition + fluid intake Avoid triggers - cold, dehydration, stress +/- Splenectomy +/- HSCT
37
Signs & Sx of beta thalassaemia
Major - extramedually haematopoeisis - bone expansion, hepatosplenomegaly, frontal bossing - aneamia - heart failure - growth retardation - iron overload --> HF, gonadal failure Trait - asymptomatic - microcytic anaemia
38
Ix in (beta) thalassaemia
Family origins questionnaire (FOQ) – Indian, Mediterranean, Middle East Guthrie testing after antenatal screening Blood smear --> microcytic red cells, tear drop cells, microspherocytes, target cells, shistocytes, nucleated RBCs Haemoglobin electrophoresis (gold-standard)
39
Management of beta thalassaemia major
Blood transfusion +/- iron chelation (dexferrioxamine, deferiprone) HSCT if HLA-identical sibling
40
Pathophysiology of Haemophilia
X linked recessive Deficiency of factor 8 (A) or 9 (B) Causes intrinsic pathway defect
41
When might a female have Haemophilia A/B?
Turner's syndrome - only have 1 X chromosome
42
Clinical features of haemophilia
Haemarthrosis May seem like NAI Mild (>5% factor levels) - bleeding after surgery Moderate (1-5%) - bleeding after minor trauma Severe (<1%) - spontaneous bleeding (joints, muscles)
43
Clotting studies in Haemophilia
APTT (intrinsic) prolonged | PT (extrinsic) normal
44
Management of Haemophilia
Mild Haemophilia A = desmopressin Severe haemophilia - prophylactic factor replacement - 2-3 x per week - can be done at home - target = factor level baseline >2% Active bleeding = IV infusion factor 8/9 concentrate AVOID IM injections, aspirin, NSAIDs
45
Presentation of iron deficiency anaemia in children
Asymptomatic - until ~60-70 Feed slowly Tire easily Pica - eating soil, ice etc.
46
tx of iron deficiency anaemia
Dietary advise - leafy green veg, red meat Oral ferrous sulphate 200mg TDS - until normal Hb - continue for at least 3 months after - reached 2-4wks after starting, Hb should ride 2g/100mL - SE = black stools, constipation
47
Triggers in G6PD deficiency
``` Antimalarials - quinine Abx - nitrofurantoin Naphthalene moth balls Analgesics - aspirin Fava beans ```
47
Triggers in G6PD deficiency
``` Antimalarials - quinine Abx - nitrofurantoin Naphthalene moth balls Analgesics - aspirin Fava beans ```
48
Presentation of G6PD deficiency
Neonate: jaundice (<3 days old) Acute intravascular haemolytic after trigger exposure - fever - abdo pain - malaise - dark urine
49
Ix findings in G6PD deficiency
During acute episode: Raised G6PD - higher enzyme concentration in reticulocytes Blood film - Heinz bodies, bite cells After episode: G6PD levels low (gold standard is to repeat after 1 month)

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