Peds Heme/Onc Flashcards
What are the clinical hallmarks of sickle cell disease?
vaso-occlusive phenomena and hemolysis
can lead to acute and chronic pain and tissue ischemia or infarction
What is the mutation involved in sickle cell disease?
point mutation in the beta globin gene
results in sickle hemoglobin= HbS
Acute complications of sickle cell dz
- infections
- severe anemia
- aplastic crisis-Parvo B19
- Vaso-occlusive phenomena
- Dactylitis
What are some of the recommendations for sickle cell dz?
- antibiotic prophylaxis (w/in first 3 mo until age 5)
- Folic acid w/o iron, Vit D and calcium
- Hydroxyurea
What should and should not be used in sickle cell crisis?
Should: IVF, hydration
NOT= demerol and toradol
What is Henoch-Schonlein purpura?
inflammatory vasculitis
Immunoglobulin A vasculitis
What is the tetra of sx in Henoch-Schonlein purpura?
- Distinct rash (purpuric non-blanching on legs/trunk)
- Arthritis
- GI- abd pain, N/V
- Renal- proteinuria, hematuria
Sudden appearance of petechial rash, bruising, and/or bleeding in an otherwise healthy child, what do you suspect?
Idiopathic Thrombocytopenic purpura
What is the normal platelet count in Idiopathic Thrombocytopenic purpura
<100,000 (LOW!)
need to watch and eval for spontaneous bleed
MC malignancy of childhood?
Acute lymphocytic leukemia
Who is at 14x risk of having Acute lymphocytic leukemia
Pt’s w/ Down Syndrome
Sx of Acute lymphocytic leukemia
- Supraclavicular-epitrochlear lymphadenopathy*
- fever, bleeding, bone pain
- hepatosplenomegaly
Dx and tx for Acute lymphocytic leukemia
dx= bone marrow biopsy
tx= chemo
What chromosome abnormality is involved in Chronic Myelocytic Leukemia?
Philadelphia chromosome (translocation of chromosomes 9 and 22)
What is the “Radiologist sign”
swollen lymph node in neck that is visible when the pt turns head?
MC childhood CA in 15-19 yos
Hodgkins Lymphoma
MC sign of Hodgkins Disease?
- Painless cervical or supraclavicular adenopathy*
- mediastinal mass causing cough/SOB
What are the 3 “B” sx that indicate bad prognosis
(In Hodgkins Disease slide?)
- Unexplained weight loss
- unexplained persistent fever
- recurrent drenching night sweats
What staging system is used for Hodgkins disease?
Ann Arbor staging system
What is used for dx in Hodgkins disease? (pathology)
tissue biopsy= Reed-Sternberg cells!!
Signs and sx of non-Hodgkins lymphoma
enlarging, non-tender lymphadenopathy
Burkitt lymphoma mimics…
acute appendicitis or intussusception
Sx of Neuroblastoma?
presenting sx reflect the location of the primary tumor
periorbital ecchymosis “Raccoon eyes
Average age of pt with Wilms tumor?
2-5 yo
