Haematology and Oncology Flashcards
causes of Fe deficiency anaemia in a child?
inadequate dietary intake e.g. exclusively bottle fed milk
blood loss e.g. cow’s milk colitis-with non IgE mediated cows milk allergy*
malabsorption e.g. coeliac disease
what might immune (prev idiopathic) thrombocytopenic purpura (ITP) occur secondary to?
SLE antiphospholipid syndrome viral infections-CMV, HIV, Hep C, varicella zoster H pylori infection drugs lymphoproliferative disorders
how does ITP occur?
otherwise normal PLTs destroyed most often in response to unknown stimulus
AI disease in which Abs produced against platelet antigens and binding causes platelet destruction
may also be Ab inhibition of PLT production
likely genetic susceptibility as often FH
how can ITP be classified?
primary-PLT count less than 100 in absence of other causes or disorders assoc. with thrombocytopenia, or secondary-assoc. with other disease e.g. SLE
time scale: persistent=lasting 3-12mnths
chronic=lasted more than 1 yr
what does ITP most commonly occur in association with in children?
usually follows a viral infection, or occasionally following immunisation
common features of ITP in children?
follows viral infection
usually self-limiting following benign course with spontaneous recovery after 6-8wks
may be asymptomatic
most common presentation=petechiae (non-blanching) or bruising
may be epistaxis
haematuria and GI bleeds less common
may be menorrhagia in older girls
IC bleeds very rarely, but must be aware of
general measures in treating child with ITP?
if child asymptomatic, or skin petechiae or bleeding only, no matter their PLT count, can be managed by observation alone
further testing of FBC if child’s condition changes
advise to avoid NSAIDs and aspirin
avoid contact sports
educate about symptoms to look out for and report urgently
pharmacological tment of ITP in a child?
use in specialist setting if thought that clinical condition requires increase in PLT count
1st line: prednisolone, IV Ig, IV anti-D Ig-in rhesus +ve children
2nd line (if resistant to 1st line and signifiant bleeding): rituximab, high dose dexamethasone
tranexamic acid for menorrhagia, but CI if haematuria
emergency PLT transfusions
splenectomy
why should a BM aspirate be considered in the presentation of likely ITP?
to exclude leukaemia
what is acute lymphoblastic leukaemia?
a malignant clonal proliferation of lymphoid progenitor cells
most commonly of B cell origin
causes BM infiltration and other organs with leukaemic blast cells
most common Ca of childhood?
ALL-acute lymphoblastic leukaemia
causes of haemolytic anaemias in childhood?
genetic or acquired
genetic: red cell membrane defects-hereditary spherocytosis
red cell enzyme abnormalities-G6PDD, pyruvate kinase deficiency
haemoglobinopathies e.g. sickle cell disease, thalassaemia
acquired: DIC e.g. sepsis hypersplenism AI haemolysis isoimmune hamolysis e.g. haemolytic disease of the newborn, blood transfusion reactions infections-malaria, septicaemia drug and toxin induced
mode of inheritance of thalassaemia?
autosomal recessive
give 5 examples of conditions inherited in an autosomal recessive pattern?
CF CAH thalassaemia sickle cell disease glycogen storage diseases galactosaemia PKU oculocutaenous albinism werdnig-hoffmann disease (SMA I)
epidemiology of thalassaemias?
beta-usually from indian subcontinent, Mediterranean and middle east
alpha-south east asian
what is beta thalassaemia?
a condition inherited in an autosomal recessive pattern in which there is a severe reduction in beta-globin production and reduction in HbA production
types of beta thalassaemias?
major and intermedia
major=most severe form, HbA (alpha2beta2) cannot be produced due to abnormal beta globin gene
intermedia=beta globin gene mutations allow a small amount of HbA and/or a large amount of HbF (alpha2gamma2) to be produced.
clinical features of beta thalassaemias?
severe anaemia-transfusion dependent, from 3-6mnths of age, and JAUNDICE
failure to thrive/growth failure
EM haemopoiesis-in absence of regular blood transfusions develop hepatosplenomegaly and BM expansion; latter causing the classical facies with maxillary overgrowth and skull bossing
