specific peds heme/onc concerns

Question 1

When must RhoGAM be administered to a mom?

Answer 1

• give within 72 hrs of delivery of initial Rh+ fetus if mom is Rh-
• Also give whenever maternal and fetal blood may have mixed (SAB, TOP procedure)

Question 2

What is Fanconi anemia? What is the H/P?

Answer 2

• recessive disorder (DNA repair pathway is blocked); associated with BONE MARROW FAILURE, pancytopenia, incr. risk of leukemia
• pts will have fatigue, dyspnea, infections
• pts are often short, with abnormal skin pigmentation, horseshoe kidney and thumb abnormalities

Question 3

What are the key labs in fanconi anemia?

Answer 3

• low hct, WBCs, platelets
• increased AFP (alpha fetal protein)
• bone marrow biopsy- hypocellularity
• multi-strand breakage on chromosome analysis

Question 4

What is Diamond-Blackfan anemia?

Answer 4

• congenital PURE RBC anemia due to a defect in erythroid progenitor
• May be associated with craniofacial anomalies, thumb abnormalities, hear murmurs, hypogonadism, intellectual disability

Question 5

What is neuroblastoma?

Answer 5

tumors of neural crest origin that may arise in the adrenal glands or in sympathetic ganglia

Question 6

Who is at risk of neuroblastoma?

Answer 6

neurofibromatosis, tuberous sclerosis, pheochromocytoma, Beckwith-Wideman syndrome, Turner syndrome, poor maternal folate consumption

Question 7

What are labs of neuroblastoma?

Answer 7

possibly increased vanillylmandelic acid and homovanillic acid in 24 hr urine

Question 8

What are the complications and prognosis of neuroblastoma

Answer 8

good if diagnosed before 1 yr of age, but poor if diagnosed after 1. Neuroblastomas send mets to the bones and brain