8 - Pediatric Hematology/Oncology Flashcards
Progressive decline in Hgb during the 1st week of life that persists for 6-8 weeks
Physiologic anemia of infancy
Minimal Hgb levels in physiologic anemia of prematurity
7-9g/dL
Microcytic anemia causes
Thalassemia
Anemia of chronic disease
Iron deficiency
Lead poisoning
Sideroblastic anemia
Normocytic anemia causes
Anemia of chronic disease
Uremia
Hypothyroidism
Aplastic anemia
Macrocytic anemia causes
Folate deficiency
Vitamin B12 deficiency
Drug and alcohol induced anemia
Response to iron therapy in IDA
12-24hrs: subjective improvements
26-28hrs: initial bone marrow response
28-72hrs: reticulocytosis
4-30 days: increasing Hgb level
1-3 mos: repletion of iron stores
First lab value to decline in IDA
Serum ferritin
Definitive diagnosis of Thalassemia
Hb electrophoresis
Hereditary spherocytosis confirmatory test
Osmotic fragility test
Suggested by an increased MCHC
Hereditary spherocytosis
Curative for hereditary spherocytosis
Splenectomy
Mutation in Sickle cell disease
Missense mutation
Definitive diagnosis of Sickle cell disease
Hb electrophoresis
PBS findings of Sickle cell disease
Sickle cells
Howell-Jolly bodies
Most common hereditary bleeding disorder
von Willebrand disease
Most common and most serious congenital coagulation factor deficiencies
Hemophilia A
Most common hereditary hypercoagulable disorder
Factor V Leiden
Hallmark of hemophilia
Prolonged bleeding
Where is the earliest joint hemorrhages in children located?
Ankles
Most common childhood malignancy
Acute lymphocytic leukemia
Single most important prognostic factor in ALL
Response to treatment
Poor prognostic factors in ALL
• <2 years or >10 years old
• Male
• WBC >100,000 u/L on presentation
• Presence of CNS leukemia
• Presenceofmediastinalmass
At the end of induction therapy, peripheral blast count or Minimal Residual Disease must be
<0.01%
Sites of relapse in ALL
Bone marrow
CNS
Testes
Most common malignant extracranial/abdominal tumor in childhood
Neuroblastoma
2nd most common malignant abdominal tumor in childhood
Wilms tumor
Most common solid tumor in childhood
Brain tumor
Most common soft tissue tumor
Rhabdomyosarcoma
Malignancy with highest mortality
Brain (PNET)
WAGR Syndrome
Wilms tumor Aniridia
GU malformation
Mental retardation
Beckwith-Wedemann syndrome
• Visceromegaly
• Macroglossia
• Omphalocele
• Hyperinsulinemic hypoglycemia
• Wilms tumor, hepatoblastoma
Denys-Drash syndrome
• Nephropathy
• Renal failure
• Male pseudohermaphrodism
• Wilms tumor
Malignancy that may present with raccoon eyes, subcutaneous tumor nodules, “dancing eyes, dancing feet”
Neuroblastoma
Treatment for neuroblastoma Stage 4S
Observation (nearly 100% survival)
Bone tumor usually found on metaphysis of long bones with sunburst pattern on x-ray
Osteosarcoma
Bone tumor usually found on diaphysis of long bones with onion-skinning or moth-eaten appearance on x-ray
Ewing sarcoma
Most common congenital cause of aplastic anemia
Fanconi anemia
Neonatal polycythemia
Hematocrit >65% in term infants
