9. Pancytopenia: causes, pathogenesis, diagnosis, treatment.

Question 1

pancytopnea can occur in ?

Answer 1

Aplastic pancytopnea (anemia)

Question 2

what are the symptoms of aplastic anemia

Answer 2

anemia :
tired and pale
angina
murmur

bleeding

infections

if fanconi anemia
cafe au lait spots
and short stature

dyskeratosisi congenita
peculiar nails and leukoplakai

TERT gene mutation
early greying of the hair

Question 3

what is the diagnosis of aplastic anemia

Answer 3

MCV is high (normal 80-100)
reticulocytes are decreased
=====

bne marrow biopsy = HYPOCELLULAR 
hematopetic cell are less than 25 percent of bone marrow space 
fat and stroma replace the cell 
megakaryocytes are usually absent 
BUT MORPHOLOGICALLY good 

(MDS = PANCYOPENA WITH hypercellular marrow)

=====
flow cytometry = cd34 is decreased = the myeloid progenitors

Question 4

what are the causes of aplastc anemia ?

Answer 4

inherited 
fanconi anemia = failure of bone marrow stem cells 
autosomal recessive 
dyskeratosisi congenita 
TERT gene defect 

ACQUIRED
idiopathic
or secondary = radiation , drugs
autoimmune = t cells activation = cytokines reelaed destroy the bone marrow

virus parvob19 , hep B
INTENSIVE CHEMO

Question 5

treatment of aplastic anemia

Answer 5

heamotpoetic stem cell transplant

immunosupression if autoimmunity =
antithymocyte globulin
cyclosporine

symptomatic
epo
blood cell trasnfusion

infections =
antibiotics
pegfilgrastim