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660810
bone marrow failure syndromes
Descripción
(Haematological disorders) Paediatrics Mapa Mental sobre bone marrow failure syndromes, creado por v.djabatey el 21/03/2014.
Sin etiquetas
haematological disorders
paediatrics
paediatrics
haematological disorders
Mapa Mental por
v.djabatey
, actualizado hace más de 1 año
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Creado por
v.djabatey
hace más de 10 años
35
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Resumen del Recurso
bone marrow failure syndromes
bone marrow failure aka aplastic anaemia
rare
reduction/absence of all 3 main lineages in bone marrow
-> peripheral blood pancytopenia
inherited or acquired
acquired
viruses (esp hepatitis)
drugs
sulphonamides
chemotherapy
toxins
benzene
glue
idiopathic
partial or complete
may start as failure of a single lineage but progress to develop all 3 cell lines
clinical presentation
anaemia
due to reduced RBC nos
infection
due to reduced WBC nos (esp neutrophils)
bruising & bleeding
due to thrombocytopenia
inherited aplastic anaemia
all rare disorders
Fanconi anaemia
commonest inherited form of aplastic anaemia
autosomal recessive inheritance
most kids have congenital anomalies
short stature
abnormal radii & thumbs
renal malformations
microphthalmia
pigmented skin lesions
px
1 or more anomalies
signs of bone marrow failure
aren't usually obv til 5-6 years old
neonates w/ Fanconi anaemia
normal blood count
diag by showing increased chromosomal breakage of peripheral blood lymphocytes
this test can be used to ID affected family members or prenatal diag
affected kids @ high risk of death from
bone marrow failure
transformation to acute leukaemia
recommended Rx
bone marrow transplantation using normal donor marrow from an unaffected sibling or matched unrelated marrow donor
Shwachman-Diamond syndrome
rare
autosomal recessive inheritance
bone marrow failure +
signs of pancreatic exocrine failure
signs of skeletal abnormalities
caused by mutations in SBDS gene
can be used for IDing unusual cases or prenatal diag
commonest haematological problems
isolated neutropenia
mild pancytopenia
increased risk of transforming to acute leukaemia
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