bone marrow failure syndrome Flashcards
what is bone marrow failure ?
a reduction in the number of circulating blood cells - pancytopenia , which requires bone marrow examination
what would cause a hypocellular marrow ?
aplastic anemia
cytotoxic therapy
cytotoxic therapy
what would cause hypercellular bone marrow ?
myelodysplasia
haematological malignancies
secondary cancers
where is the best place to see signs of anemia ?
mucous membranes
what are the clinical problems associated with bone marrow failure ?
anemia
leucopenia
thrombocytopenia
what infections of the skin may be associated with bone marrow failure patients ?
at the site of IV cannula usually due to staph aureus or staph epidermidis
what is the protocol for management in neutropenia/leucopenia ?
administration of broad spectrum antibiotics after cultures
non absorbable anti fungals
give prophylactic acyclovir
what are some signs of thrombocytopenia bleeding ?
mucosal bleeding
purpura
retinal haemorrhages
what is the management in cases of thrombocytopenia ?
in absence of bleeding , prophylactic platelet transfusion
anti-HLA antibodies may develop so HLA matched platelets may be required
how to distinguish purpura ?
they dont blanch on pressure
in aplastic anemia what does the BM look like ?
hypocellular
what are the causes of aplastic anemia ?
congenital fanconi’s anemia
can be acquired such as with autoimmune or acquired aplastic anemia
what are the two major initial presentation of fanconi’s anemia ?
anemia
birth defects
birth defects
what are some of the congenital defects in fanconis anemia ?
radial ray defects
renal and urinary tract defects
skeletal defects
what do chromosomal breakages predispose the patient to ?
developing secondary leukemias
what is the management of aplastic anemia ?
remove the cause
supportive measures
supportive (RBC, platelets, antibiotics)
if the aplastic anemia is congenital what is the next step in management ?
bone marrow transplantation and supportive therapy
if the cause of the aplastic anemia is autoimmune what is the next step in management ?
anti-thomocyte globulin
along with immunosuppresants (cyclosporine)
other immunosuppressants such as cyclosporine
myelodysplastic syndromes ?
cytopenia with a hypercellular bone marrow
what can these myelodysplastic syndrome transform into ?
acute myeloid leukemia
what does the prognosis of myelodysplastic syndromes depend on ?
depends on :
number of cytopenia in the blood
number of blasts in the marrow
cytogenetic and chromosomal abnormalities
what is the managment for myelodysplastic syndromes ?
supportive treatment
cytotoxic chemotherapy
demethylating therapy (azacitidine)
allogenic stem cell transplantation
what are myelomas commonly preceded by ?
monoclonal gammopathy of undetermined significance
