Paeds Haem Flashcards
What is the pathophysio of Leukaemia?
A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell.
The excessive production of a single type of cell can lead to suppression of the other cell lines, causing underproduction of other cell types. This results in a pancytopenia, which is a combination of low:
Red blood cells (anaemia),
White blood cells (leukopenia)
Platelets (thrombocytopenia)
Normally cells follow a myeloid or lymphoid cell path and differentiate into blood cells but this does not happen in Leukaemia. You therefore get pancytopenia and a large build up of Myeloid and Lymphoid cells
Epidemiology of ALL?
Peak incidence is 2-5 years of age
ALL features and px?
anaemia: lethargy and pallor
neutropaenia: frequent or severe infections
thrombocytopenia: easy bruising, petechiae
bone pain (secondary to bone marrow infiltration)
splenomegaly
hepatomegaly - masses in RUQ and LUQ
fever is present in up to 50% of new cases (representing infection or constitutional symptom)
testicular swelling
AML px?
anaemia: lethargy and pallor
neutropaenia: frequent or severe infections
thrombocytopenia: easy bruising, petechiae
bone pain (secondary to bone marrow infiltration)
splenomegaly
hepatomegaly - masses in RUQ and LUQ
fever is present in up to 50% of new cases (representing infection or constitutional symptom)
testicular swelling
What is the NICE recommendation for children with sus leukaemia?
NICE recommend referring any child with unexplained petechiae or hepatomegaly for immediate specialist assessment.
If leukaemia is suspected based on the non-specific signs above, NICE recommend a very urgent full blood count within 48 hours.
Investigations to establish the diagnosis:
Full blood count, which can show anaemia, leukopenia, thrombocytopenia and high numbers of the abnormal WBCs
Blood film, which can show blast cells
Bone marrow biopsy
Lymph node biopsy
Tx for Leukaemia?
Chemo
What conditions is Leukaemia more associated with?
Down’s syndrome
Kleinfelter syndrome
Noonan syndrome
Fanconi’s anaemia
What is ITP?
ITP is caused by a type II hypersensitivity reaction. It is caused by the production of antibodies that target and destroy platelets. This can happen spontaneously, or it can be triggered by something, such as a viral infection.
TOM TIP: ITP is worth remembering as it is a key differential diagnosis of a non-blanching rash.
What are the charac features of ITP?
Idiopathic thrombocytopenic purpura (ITP) is a condition characterised by idiopathic (spontaneous) thrombocytopenia (low platelet count) causing a purpuric rash (non-blanching rash).
How does ITP present in children?
Idiopathic thrombocytopenic purpura usually present in children under 10 years old. Often there is a history of a recent viral illness. The onset of symptoms occurs over 24 – 48 hours:
Bleeding, for example from the gums, epistaxis or menorrhagia
Bruising
Petechial or purpuric rash, caused by bleeding under the skin
What is the difference between Petechiae and Purpura?
Petechiae are pin-prick spots (around 1mm) of bleeding under the skin. Purpura are larger (3 – 10mm) spots of bleeding under the skin. When a large area of blood is collected (more than 10 mm), this is called ecchymoses. These are all non-blanching lesions.
Mx of ITP?
The severity and management depends on how low the platelet count falls. Usually no treatment is required and patients are monitored until the platelets return to normal. Around 70% of patients will remit spontaneously within 3 months.
Treatment may be required if the patient is actively bleeding or severe thrombocytopenia (platelets below 10):
Prednisolone
IV immunoglobulins
Blood transfusions if required
Platelet transfusions only work temporarily - ANTIBODIES just destroy infused ones quickly lol
What lifestyle advice would you give to kids with ITP?
Avoid contact sports
Avoid intramuscular injections and procedures such as lumbar punctures
Avoid NSAIDs, aspirin and blood thinning medications
Advice on managing nosebleeds
Seek help after any injury that may cause internal bleeding, for example car accidents or head injuries
What is hereditary spherocytosis? List everything you know
In the name - spherical RBC’s - destroyed when passing through spleen:
- Jaundice, anaemia, gallstones and splenomegaly seen
Dx: Fhx + spherocytes on blood film, reticulocytes raised
Mx:Folate supplementation to aid more RBC, splenectomy
What are the one or two things that you need to look out for in hereditary sphero?
Infection with parvovirus causing aplastic crisis is a classic exam features of hereditary spherocytosis. It is worth remembering this connection, as there are multiple ways examiners like to ask this. A patient with spherocytosis may present with anaemia and you could be asked to identify the causative infectious agent. Alternatively, someone affected by parvovirus could develop anaemia and jaundice and you may be asked the underlying diagnosis.
During aplastic crisis there is increased anaemia, haemolysis and jaundice, without the normal response from the bone marrow of creating new red blood cells. Usually the bone marrow will respond to haemolysis by producing red blood cells faster, demonstrated by extra reticulocytes (immature red blood cells) in the blood. In aplastic crisis there is no reticulocyte response. This is often triggered by infection with parvovirus.
