Pediatric heme/onc

Question 1

Hemolytic disease of the newborn

Answer 1

Anti-Rh abs against fetal RBCs causing hemolysis

must have been sensitized during a previous pregnancy

events that warrant rhogam administration:
Maternal-fetal hemorrhage
Abortion
Amniocentesis
3rd trimester bleeding
Trauma/motor vehicle accident
Delivery of baby
Domestic abuse

Give immunoglobulin within 72 hours of the mixing event

Question 2

Fanconi anemia

Panconi anemia

Answer 2

Autosomal recessive disorder
Bone marrow failure
Pancytopenia:
-fatigue
-dyspnea on exertion
-infections

unique features:
short stature
abnormal skin pigmentation
-hyperpigmentation
-hypopigmented areas
-cafe-au-lait spots
-horseshoe kidney
-thumb abnormalities (absent or hypoplastic)

Labs:
Pancytopenia
serum alpha fetoprotein
hypocellularity

Treatment:
antibiotics (leukopenia)
transfusions (anemia)
bone marrow or hematopoietic stem cell transplant

Complications:
death from bone marrow failure

Question 3

Diamond- Blackfan anemia

Answer 3

Pure RBC anemia: fatigue, dyspnea, cyanosis, pallor

increased erythropoetin (body wants to make more cells but can’t)

Craniofacial abnormalities
Thumb abnormalities
Heart murmurs
Intellectual disability
Hypogonadism

Problems with 
pure RBC anemia
face
brain
thumbs
heart 
gonads

Question 4

Neuroblastoma

Answer 4

adrenal neuroblastoma
most common tumor of adrenal medulla in children, of neural crest cell origin
May arise in the adrenal glands OR sympathetic ganglion

H and P:
abdominal distension and pain
weight loss
malaise
bone pain
diarrhea
abdominal mass
HTN

(unlike Wilm’s tumor, no hematuria)

Horner syndrome if it occurs in the sympathetic ganglia of the chest
proptosis
movement disorders
hepatomegaly
fever
periorbital bruising

Labs: NE breakdown products:
increased VMA
increasd homovanilic acid (HVA) in the 24-hr urine collection

no episodic HTN, diaphoresis (this is not pheo)

Diagnostic testing:
CT scan shows tumor

Good prognosis if you can surgically resect this

Question 5

Rhabdomyosarcoma

Answer 5

most common soft tissue tumor in children

A tumor of striated muscle
Painful soft tissue mass with swelling
If large enough, can cause mass effect on nearby structures
Diagnose:
-biopsy
-CT or MRI to help with surgical planning