Haematological/Oncological Flashcards
why is paeds pt more prone to iron deficiency anaemia?
iron is required for growth and so early childhood will require a large amount of iron - children might not eat enough iron to cover the iron requirement
how can cow’s milk cause iron deficiency anaemia in children?
if whole cow’s milk is given too early - induce microscopic bleeding due to allergic reaction
aetiology of iron - deficiency in children
dietary iron deficiency
whole cow’s milk allergy - leads to microscopic bleeding
infections - malaria, TB, HIV and helmith infection
inherited conditions eg sickle cell, diamond-blackfan syndrome, beta thalassaemia
what is the range of HB in order to be considered to be anaemic in 1/2 - 5 yrs old
<11
what is the range of HB in order to be considered to be anaemic in 5- 11 yrs old
< 11.5
what is the range of HB in order to be considered to be anaemic in 12- 14 yrs old
< 12
symptoms and signs of iron-deficiency of anaemia
can be asymptomatic
pallor fatigue SOB/IWOB Tachycardia gallop rhythm systolic flow murmur cardiomegaly and hepatomegaly
failure to thrive
affect growth
things to consider asking during history taking ?
ethnic origin - african people - inc risk of sickle cell disease, malaria, diamond-blackfan syndrome, mediterranean people - inc risk of beta thalassemia
recent travel - infection and parasitic infection
investigation for iron-deficiency anaemia
FBC - shows microcytic, hypochromic and poikilocytosis
ferritin - reduced
folate, B12 - for macrocytic anaemia
coombs test - haemolytic anaemia
blood film - to exclude other types of anaemia eg sickle cells
TFT - hypothyroidism
management of iron deficiency anaemia
PO iron salts over 2-3 months - sytron (sodium feredetate)
iron transfusion
inc dietary intake
limit cow’s milk to 1 pint only
how would you prevent iron deficiency anaemia?
breast milk somewhat protective - low iron
no unmodified cow’s milk in first year of life
complications or IDA
cognitive impairment - cognitive and behavioural impairment, attention deficit
impaired muscle performance
developmental delay
high output heart failure
tran
what is the most common solid brain tumor in children?
70-80% infratentorial (glial tumors, medulloblastoma) or mid-line (Germ Cell tumor, craniopharyngioma - tumor of the pituitary gland
what are some clinical features for childhood brain tumour?
headache N+V visual distrubanec - double vision, papilloedema, squint hydorcephalus diabetes inspidius seizures gorwth restictions gait/co-ordination problems CN palsies
ix for childhood brain tumor
urgent referral 48 hours if suspected brian or CNS tumours
MRI and CT - MRI preferred, better imaging for soft tissue but takes longer and may require sedation, so CT is more often used first
biopsy
others ix –> hearing test and pituitary function, CSF analysis
what brain tumor could inc hCG and AFP in CSF suggest ?
pineal tumours
mx of childhood brain tumours
urgent referral surgical resection radio chem follow up - every 6 m for first 2 years
what is haemophilia A
it is factor 8 deficiency - can be congenital or acquired
what is haemophilia B
it is factor 9 deficiency - can be congenital or acquired
how is haemophilia pass down genetically
X-linked recessive
what is considered a mild hemophilia A or B
it is when factor 8/9 is between 5-40% of normal level
what is considered a moderate hemophilia A or B
it is when factor 8/9 is between 1-5% of normal level
what is considered a severe hemophilia A or B
it is when factor 8/9 is <1% of normal level
what are the symptoms of haemophilia
mild - bleeding after surgery eg circumcision
moderate - bleeding after minor trauma –> after hell stick test
severe - spontaneous bleeding into joints/muscle or intra-cranial bleeding
Investigation for haemophilia
• Activated partial Thromboplastin time (aPTT) – inc
o use to test intrinsic pathway
• Factor 8 assay or 9 – level dec
• normal prothrombin time
o use to test extrinsic and common pathway
• plasma von Willebrand factor assay – normal to exclude VWF deficiency
• other factors assay – to exclude problems elsewhere
• FBC – normal, low after bleeding
• LFT – factor deficit can be caused by liver disease
• FHx or carrier – test baby’s gender from 9 wks, if male: plan safe delivery
o can do amniocentesis or CVS to confirm diagnosis
management of haemophilia
o Avoid NSAIDs and IM injections
o Regular or prophylactic factor replacement therapy to those with severe haemophilia
Haemophilia A every 48hrs, for haemophilia B 2x week
Implantable port (from 2yrs) at home
o Minor bleeding – eg minor surgery/dential extraction
Pressure, elevation
IV desmopressin (0.3µg/kg/12hrs over 20min) to factor 8
o Major bleeding (haemarthrosis etc.)
Aim to factor 8 to 50% of normal
o Life-threatening bleeding (e.g., airway obstructing)
Need 100% levels factor 8 (recombinant factor 8)
IV desmopressin does not work for hemophilia B
what is Henoch-Schoenlein Purpura
small vessel vasculitis - characterized by tissue deposition of IgA containing immune complexes within affected organs
pathophysiology of Henoch-Schoenlein Purpura
small vessel leukocytoclastic (neurophil debris) vasculitis
- characterized by tissue deposition of IgA containing immune complexes within affected organs
- skin biopsy of lesions contains neutrophils and monocytes
- fluorescence microscopy of kidney –> IgA, C3, fibrin mainly
clinical features of Henoch-Scholenin Purpura
Tetrad = rash (palpable purpura and non-blanching, usually lower extremities), abdo pain, arthritis/arthralgia, glomerulonephritis
other signs of renal disease - asymptomatic haematuria +/- proteinurua, if severe –> rapid progressive nephritis, nephrotic syndrome and renal failure
scrotal pain or swelling
headache
seizures
pulmonary haemorrhage
investigation of HSP
urinalysis - assess renal involvment
24 hour urine for protein - raised due to renal involvement
U&Es + creatinine –> kidney function
serum IgA - maybe elevated
coag - should be normal in HSP, used to exclude other causes
skin biopsy - will show IgA involvement
Renal biopsy - will show IgA involvement
management. of HSP
mid + no renal involvment –> analgesia + supportive
if severe oedema/severe abdo pain/scortal swelling –> prednisolone
if kidney involvement –> prednisolone
if rapid progressive nephritis –> IV methyprednisolone + cyclophosphamide/azathioprine
if severe kidney damage –> renal transplant
definition of leukaemia
• malignant proliferation of white cell precursors in bone marrow
what is the most common childhood cancer
Acute lymophoblastic leukaemia
pathology of leukaemia and lymphoma?
• WBC (T, B etc), RBC and platelets are all made form bone marrows in long bones
• stem cells produce cell precursors which can either mature through lymphoid cell line makes T and B cells or myeloid cell line which matures the other WBCs, RBCs and platelets etc.
• in both leukaemia and lymphoma, genetics changes occurs in the precursors leading to malignant proliferations of cells.
o leukaemia 2 types as can affect both lymphoid and myeloid cell lines lymphoblastic or myelogenous
o lymphoma only affect lymphoid cell line T and B cells
clinical features of ALL?
- insidious onset
- anorexia, irritability and lethargy
- bone pain
- mucous membrane bleeding
- Petechiae
- as bone marrow fails due to malignant tumours replacing normal stem cells Pallor, fever, Petechiae
- lymphadenopathy
- splenomegaly
Ix for leukaemia?
- FBC with differentials inc WCC, dec Hb (anaemia), neurorpenia,
- peripheral blood smear thrombocytopenia +/- blast cells
- U&Es elevated Ca2+, K+, phosphorus, uric and lactic acid
- LFT liver can be affected
- diagnosis examination of bone marrow (bone marrow aspiration + trephine biopsy replaced by leukaemic lymphoblasts
mx for leukaemia
- chemo, prophylactic Radiotherapy to CNS and systemic radiotherapy for 2-3 yrs
- prophylactic ABx + antiviral
- prophylactic colony-stimulating factors (to stimulate neutrophiles)
- follow for relapse intensive retreatment
which cells are affected in lymphoma?
B cells
what is associated to inc risk for lymphoma?
- EBV infection associated to both
* NHL associated to other autoimmune disorders
clinical features for lymphoma?
- most commonly present with painless cervical +/- supraclavicular lymphadenopathy in a young adult/ adolescence/late childhood
- B symptoms fever, night sweats, weight loss occurs in 30% of pts
- often less common presentation dyspnoea &cough (mediastina adenopathy), SVC obstruction, abdo pain
ix for lymphoma
- FBC with differentials low Hb and platelets, WCC can be high or low
- LFT can be affected
- U&Es + creatinine can be affected
- ESR elevated
- CXR if dyspnoea and maybe able to see mediastinum adenopathy
- PET-CT scan affected area will light up
- contrast CT neck, chest and abdo/pelvis may show enlarged lympho nodes and other sites of disease
- excisional lymph node biopsy Hodgkin’s cell (Reed-Sternberg cell)
- bone marrow biopsy can be consider
- for NHL skin biopsy shows cutaneous T cells
mx for lymphoma
- chemo +/- radiotherapy for both
* NHL R-CHOP-21 (rituximab + cyclophosphamide+ doxorubicin + vincristine + prednisolone for 21 days)
what is sickle cell anemia
a single autosomal recessive gene mutation in the beta chain of hemoglobin resulting in the production of sickle cell hemoglobin
pathophysiology of sickle cell anaemia
in a hypoxic and acidotic environment, HbS becomes rigid and change to a crescent shape and so unable to carry O2 properly
the deformed HbS can also occlude small vessels or stick to the endothelium of large vessels to slow blood flow
which race is most commonly carrier of the sickle cell gene?
black population - approx 8% of the entire black population
what are some clinical features of sickle cell anaemia
persistent pain in abdo, bone and chest dactylitis fever pneumonia-like symptoms chronic haemolytic anaemia visual floaters tachycardia, pallor, jaundice, tachypnoea, lethargy
can have acute sickle crisis
ix for sickle cell anaemia
- DNA-based assay replacement of both beta haemoglobin subunits with HbS = diagnostics
- cellulose acetate electrophoresis sickle cell anaemia 75-95% HbS
- high performance liquid chromatography
- FBC anaemia
- peripheral blood smear presence of nucleated RBC, sickle-shaped cells and Howell-Jolly bodies
Mx of sickle cell anaemia
• treatment of crisis/ acute chest syndrome analgesia, Abx, antihistamine, warmth, fluids + O2 if needed
o blood transfusion if life-threatening
• ongoing management management of pain (hydroxycarbamide to dec frequency of pain episode) , pneumococcal immunisation, prophylactic Penicillin,
what is neuroblastoma
tumour develop from nerve cells in foetus (neuroblast) which affects the sympathetic nervous system
which organ does neuroblastoma normally affect?
mostly in adrenal gland (due to sympathetic nervous system)
but can start/spread to chest, abdo, spine, spinal cord
clinical features of neuroblastoma
abdo mass, pain
FTT
bleeding
hard, painless neck mass
irritable, fever, dec appetite
leg weakness/numbness (spinal cord involvement)
if spread (50%) - bone pain, anaemia, weight loss, sweating, watery diarrhoea
mx of nueroblastoma
chemo/radio
surgery (unless at spinal core)
stem cell transplant
peak incidence of neuroblastoma
2 yrs old
pathophysiology of thalassaemia
• inherited microcytic anaemia caused by mutations of the beta-globin gene leading to dec or absent synthesis of beta-globin ineffective erythropoiesis.
which races are most susceptible to thalassaemia
• ß-thalassaemia most common; Southeast Asian, middle east, Mediterranean backgrounds.
clinical features of thalassaemia trait
• Heterozygous ß-thalassaemia = thalassaemia trait
o Mild anaemia or asymptomatic
clinical features of thalassaemia major
• Homozygous thalassaemia
o Severe haemolytic anaemia
o Compensatory BM hyperplasia characteristic overgrowth of facial + skull bones
o Haemosiderosis (iron overload) + cardiomyopathy, DM + skin pigmentation due to repeated blood transfusion.
o lethargy, abdo distension, FTT, low height and weight
o pallor
o spinal changes (iron overload osteopenia)
o hepatosplenomegaly
ix for thalassaemia
- Hypochromic, microcytic anaemia in thalassaemia trait may be confused with iron def.
- Homozygous: severe hypochromic microcytic anaemia, bizarre fragmented poikilocytes and target cells.
• Diagnosis of type: Hb electrophoresis.
o ß-thalassaemia major: absence of HbA1, HbA2 + HbF
mx of thalassaemia
• Thalassaemia trait requires no treatment.
• Thalassemia major: regular blood transfusions to maintain Hb levels.
o Haemosiderosis (iron overload) is inevitable consequence, can minimise by use of SC/IV infusions of chelating agent desferrioxamine.
o Or PO iron chelating agents
what is Wilm’s tumour
it is nephroblastoma, most common renal malignancy in children
peaks at 5 yrs
presentation of Wilm’s tumours
unilateral, painless, abdo mass, haematuria + lethargy, weight loss etc
ix for Wil’s tumour
FBC, U&E, LFT, LFT, coag, CT or MRO +/- biopsy
Mx for Wilm’s tumour
surgery +/- adjuvant chemo +/- radiotherapy
