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
What is the first Tx for ITP in children
Prednisone
What is the only curative Tx for ITP in children
Splenectomy
Sx’s = petechiae, bloody diarrhea, fatigue, gingival bleeding, periorbital and LE edema
HUS
What is the Tx for HUS/TTP that causes aplastic anemia
Chloramphenicol
