Peds Blood Disorders Flashcards
Sx’s = life-threatening pyogenic infections in infancy w/ increased risk of AML
Severe Congenital Neutropenia (Kostmann Syndrome)
Sx’s = oral ulcers, fever, periodontal disease, recurrent bacterial infections
Cyclic Neutropenia
Sx’s = neutropenia, pancreas insufficiency, skeletal abnormalities (ribs/hands) w/ increased risk of MDS or Leukemia
Schwachman-Diamond Syndrome
Sx’s = GU and skeletal abnormalities in pts < 10 yo
Fanconi Anemia
Sx’s = delayed separation of umbilical cord, recurrent infections w/out pus formation
Leukocyte Adhesion Deficiency
Sx’s = recurrent purulent infections w/ catalase + bacteria that begin in infancy
Chronic Granulomatous Disease
Sx’s = Slapped cheek appearance 1-2 wks post-viral infection w/ Parvovirus B19
Fifths Disease
In pts w/ persistent, unexplained lymphadenopathy not associated w/ any obvious disease, what test should be ordered
CXR
Sx’s = XR recurrent sino-pulmonary and ear infections w/ atopic dermatitis
Wiscott-Aldrich Syndrome
Diseases more likely in pts < 3 yo w/ Trisomy 21 and > 3 yo w/ Trisomy 21
< 3 = AML
> 3 = ALL
Sx’s = macrocytic anemia w/ low reticulocyte count and pure red cell aplasia
Diamond-Blackfan Syndrome
Sx’s = pancytopenia that may not be seen until ~10 yo
Fanconi Anemia
Sx’s = Microcytic anemia w/ basophilic stippling
Pb-Toxicity
Sx’s = isolated thrombocytopenia (bruising/petechiae or mucocutaneous bleeding) w/ no sign of underlying cause in 2-10 yo
Acute ITP
Acute ITP most often occurs after….
Viral infection
