haem Flashcards
sickle cell crisis mx?
analgesia oxygen may admit (low threshold for admission - esp if chest symptoms present) increase fluid intake!!! avoid other triggers
if Hb v low, blood transfusion
Triggers of crises in sickle cell??
dehydration
cold weather
excessive exercise
viral illness
Sickle cell anaemia complications?
dactylitis
splenic sequestration
gall stones
chronic pain
sickle cell anaemia prophylaxis?
immunisations
abx prophylaxis due to splenic dysfunction
folate supplementation due to rapid cell turnover
SCA medication to prevent crises?
hydroxycarbamide
immune thrombocytopenia cause?
often 1-2 wks after viral infection
- anti platelet igG autoantibodies
autoantibodies in immune thrombocytopenia?
anti platelet igG
immune thrombocytopenia presentation?
purpura, superficial bruising
petechiae
epistaxis or other mucosal bleeding
immune thrombocytopenia mx?
in 80% - self limiting and will self resolve
oral prednisolone or IVIG
platelet transfusion if severe
what is considered thrombocytopenia in children?
platelets <150 x 10^9/L
causes of low pl due to increased pl destruction / consumption?
ITP, SLE HUS TTP DIC hypersplenism
haemophilia presentation?
most frequently bleeding into joints or muscles
bleeding post-circumcision?
haemophilia
what to avoid in all pts with haemophilia?
IM injections
NSAIDS
aspirin
haemophilia mx?
replace missing factor
MDT- specialist centres, specialist nurse, physiotherapist, psychosocial support
self help / support groups
B thal major features?
severe anaemia - transfusion dependent from 3-6 months of age
jaundice
FTT
extramedullary haemopoiesis - heaptosplenomegaly
classical facies - maxillary overgrowth and skull bossing
B thal major mx?
regular blood transfusions
to reduce growth failure and bone deformities
or bone marrow transplant
+ iron chelation (desferrioxamine) to prevent chronic iron overload
cardiac failure, liver cirrhosis, diabetes, infertility -?
chronic iron overload
B thal trait presentation?
usually asymptomatic mild anaemia (hypo chromic, microcytic)
Raised HbA2 and HbF
haemolytic disease of newborn tx?
IVIG + phototherapy/ exchange transfusion
+ oral folate for 6 months
+ supportive e.g. if coagulopathy
pica - eating non food materials
IDA
cows milk in excess
IDA
inheritance of G6PD deficiency?
x linked
what pathway is implicated in G6PD deficiency?
pentose phosphate pathway
some triggers of acute haemolysis in G6PD deficiency
fava beans
moth balls
acute infection
certain drugs e.g. antimalarials, antibiotics
G6PD deficiency ix?
FBC, WCC, CRP
G6PD activity in RBC
Sickle cell anaemia susceptible to which infections?
encapsulated organisms strep pneumoniae h influenza meningococcus osteomyelitis from salmonella
acute chest syndrome in SCA mx?
severe hypoxia- need for mechanical ventilation
emergency transfusion
complications of SCA
avascular necrosis of femoral head hand - foot syndrome in late infancy sickle cell crises aplastic crises sequestration crises (sudden splenic enlargement, abdo pain and shock)
parvovirus B19 in SCA?
aplastic crisis
splenic sequestration in SCA presentation?
sudden splenic and hepatic enlargement, abdo pain and shock
general malaise, pallor lethargy, recurrent infections, easy brusing/ petechiae, bone pain
+ * hepatosplenomegaly, possible lymphadenopathy + testicular enlargement
ALL likely
immunophenotype to further subclassify into T cell or B cell
Ix in suspected ALL?
FBC, WCC,
Blood film
Bone marrow biopsy
CXR to identify mediastinal mass characteristic of T cell ALL.
what are the high risk features of ALL
Age <1 or >10
Tumour load > 50 x 10^9/L
Cytogenetic/ molecular abnormalities in tumour cells,
persistence of leukaemic blasts in BM despite chemo,
DDx of pancytopenia in child
Inherited - e,g, fanconi’s anaemia.
Acquired - e.g. viral (EBV/ parvovirus/ herpes), drugs e.g chemo, chloramphenicol, carbamazepine. >80% are idiopathic aplastic anaemia.
BM infiltration- leukaemia, neuroblastoma, myelofibrosis, myelodysplasia.
Pancytopenia Ix?
Blood film. MCV. FBC. Reticulocytes. Viral titres. BM aspirate and trephine.
Aplastic anaemia mx?
mx pending a diagnosis is supportive.
Platelet infusions if platelet count <5 x10^9 or actively bleeding.
Regular monitoring of temperature.
Decision to embark on definitive tx depends upon severity.
Bone marrow transplant offers best chance to those w matched sibling donor.
Otherwise, immunosuppressive tx w antithymocyte globulin and cyclosporin.
purpura in child.
ddx?
infections. -e.g. meningococcal septicaemia.
thrombocytopenia - ITP, ALL.
Vasculitis- e.g. HSP.
trauma.
clotting disorders - e.g vwd, haemophilia a/b.
drugs e.g. steroids.
purpura in child Ix?
FBC (Hb, WCC, Pl), CRP, U+Es.
Blood film.
urine dip.
clotting profile.
Immune thrombocytopenia complications
most severe is intracranial haemorrhage, severe epistaxis, or GI bleeding.
Immune thrombocytopenia mx?
dependent on symptoms.
if asymptomatic, most docs won’t treat. Lifestyle advice e.g. avoid activities w high risk of trauma.
90% self-resolve in 6 months.
if bleeding -> admit + prednisolone + IVIG.
platelet transfusions only indicated in the case of an intracranial haemorrhage or to tx life-threatening bleeding.
what is acute sickle chest syndrome?
thrombosis, infection and fat embolism to the lung produces a syndrome of pleuritic chest pain, SOB, fever. hypoxia is.a frequent feature.
acute sickle chest syndrome mx?
admit. adequate analgesia (IV morphine), supplemental oxygen and hyper hydration. Physiotherapy to help pt to breathe deeply enough to ventilate the affected areas of the lungs. empirical abx tx started e.g. coamoxiclav and clarithromycin.
Discuss mx w senior paediatrician and haemotologist.
If deteriorate-> ventilatory support (CPAP) or intubation.
exchange or top up blood transfusion may be needed to reduce the % of sickling RBCs.
complications of sickle cell disease in children.
painful crises. avascular necrosis of hips/ shoulders. chest syndrome, abdo syndrome. splenic sequestration, aplastic anaemia. stroke, retinal vein occlusion. priapism, haematuria, enuresis, chronic renal failure. pigment gallstones, cholecystitis, biliary colic. hyposlenism, osteomyelitis. delayed puberty.
Main differential for Wilms tumour/ nephroblastoma?
Neuroblastoma- an embryonal cancer of the peripheral SNS. most arise in the abdomen either in the adrenal gland or in the retroperitoneal sympathetic ganglia.
the mass usually causes discomfort, and usually present with symptoms of metastatic spread.
95% of NB patients have raised urine VMA (vanilylmandelic acid) and HVA (homovanilic acid).
Wilms tumour tx?
surgery with pre and post surgery chemo and radiotherapy depending on the stage.
prognosis favourable, stage 1-3 pts have an overall cure rate of 88-98%.
refer to specialist unit. care of MDT - oncologist, paediatrician, nephrology, specialist nurses.
Suspected Wilms tumour Ix?
BP - usually raised.
Urine dip/ MCS.
Abdo USS.
FBC, WCC, CRP, U+Es. Creatinine.
ataxia - broad based unsteady gait in a child who had previously normal gait?
disorder of the cerebellum and or sensory pathways in the posterior columns of the spinal cord.
- heel shin test, dysdiadochokinesia, dysmetria.
Diagnosis of ALL
FBC. Blood film. analysis of BM aspirate.
Mx of ALL
remission induction: 4 wks of combination chemo.
Consolidation and CNS protection: oral chemotherapy. a course of weekly intrathecal drugs given to prevent later CNS relapse.
Delayed intensification: intense chemo w blocks of maintenance chemo.
continuing maintenance: chemotherapy continues for a total of 2 yrs in girls and 3 yrs in boys. patients are immunosuppressed and at risk of opportunistic infections. co-trimoxazole given to prevent PCP infection.
poor prognostic factors of ALL
outside age range 2-10 yrs. male sex.
total WCC >100 x 10^9 at presentation.
Certain chromosome abnormalities, CNS disease at presentation, slow response to tx.
Ddx of structural scoliosis
idiopathic,
neuromuscular e.g. cerebral palsy, Duchenne muscular dystrophy,
congenital structural abnormalities of the vertebrae e.g. hemivertebrae - high risk of assoc spinal cord defects, esp genitourinary.
syndromic e.g. neurofibromatosis and Marfan syndrome.
Ix of scoliosis
bend forward to touch toes - if scoliosis is postural, this will disappear.
if structural scoliosis found, thorough neurological examination.
Mx of scoliosis
all should be monitored regularly. (esp premenarchal girls- most at risk of causing unacceptable deformities)
bracing and surgical correction are treatment options. surgery is recommended earlier in those w neuromuscular or congenital scoliosis.
Child just diagnosed with AML. what is your first mx priority?
Prevent tumour lysis syndrome.
As tx begins, a large no of cells break down -> hyperK, hyperPO4, gout, fluid overload/ dehydration, raised Ur/Cr.
what is part of the prophylactic mx of tumour lysis syndrome?
hyperhydration, allopurinol/ rasburicase and monitoring of electrolytes from time of diagnosis.
von willebrands disease. inheritance?
auto dominant
