Pediatrics Hematology Oncology Flashcards
How is anemia defined physiologically?
Hemoglobin level too low to meet cellular oxygen demands.
What factors determine hemoglobin values in normal children?
Age, gender, race, degree of sexual maturation, altitude, and heredity.
What is the significance of reticulocytes in diagnosing anemia?
They help distinguish among different types of anemia by indicating bone marrow response.
Why does excessive cow’s milk intake predispose children to iron deficiency?
It contains minimal iron, poorly absorbed iron, reduces intake of other foods, and may cause GI bleeding.
What are the risk factors for lead poisoning in children?
Increased susceptibility in younger children, incomplete blood-brain barrier, higher prevalence of iron deficiency, and exposure to lead sources like old paint and dust.
What systems are affected by lead poisoning and how?
Nervous system (decreased IQ, cognitive effects, seizures, encephalopathy), heme biosynthetic pathway (anemia), renal system (renal tubular dysfunction, chronic interstitial nephritis).
What is the recommended evaluation for lead poisoning?
Venous blood lead level (BLL) testing and routine screening, with intervention for BLL ≥ 5.
What management steps are taken for lead poisoning?
End exposure, test household members, lead abatement, ensure adequate nutrition, and chelation therapy for high levels.
What is the recommended action when a venous blood lead level (BLL) is ≥ 5?
Intervention is warranted.
What additional screening is suggested if elevated BLL is detected?
Screen for iron deficiency.
What imaging technique is considered if symptomatic lead poisoning is suspected?
Plain abdominal x-ray.
What dietary recommendations are made to reduce lead absorption?
Ensure adequate intake of calcium, vitamin C, iron, and avoid fasting.
What is the efficacy of chelation therapy for lead poisoning?
Limited efficacy for significantly elevated levels.
What type of anemia is frequently seen in young children with lead poisoning?
Microcytic anemia due to concomitant iron deficiency.
What are the most common causes of microcytic hypochromic anemia during childhood?
Iron deficiency, thalassemia, anemia of inflammation.
What are the two broad categories of hemoglobin disorders?
Quantitative disorders and qualitative disorders.
What is the typical age range for diagnosis of Transient Erythroblastopenia of Childhood (TEC)?
1-4 years.
What is the primary difference in lab values between Diamond Blackfan Anemia (DBA) and TEC?
DBA shows increased MCV and elevated hemoglobin F, while TEC does not.
What is the definition of neutropenia?
Decrease in the absolute neutrophil count below accepted norms for age.
What is the calculation for Absolute Neutrophil Count (ANC)?
ANC = # total WBC * (% PMNs + % bands).
What is the infectious risk associated with severe neutropenia?
Moderate to severe.
What is neutropenia?
A decrease in the absolute neutrophil count below accepted norms for age.
How is the Absolute Neutrophil Count (ANC) calculated?
Total WBC count multiplied by the sum of the percentages of PMNs and bands.
What are the severity levels of neutropenia and their associated infectious risks?
Mild (1,000-1,500) - none, Moderate (500-1,000) - minimal, Severe (<500) - moderate to severe.
What is the most common cause of neutropenia?
Infection-associated neutropenia.
What are the categories of immune neutropenias of childhood?
Chronic benign neutropenia, alloimmune, autoimmune, drug-induced.
What is the mechanism behind chronic benign neutropenia of childhood?
Anti-neutrophil antibody.
What are the clinical features of cyclic neutropenia?
Recurrent fever, pharyngitis, aphthous ulcers, periodontal disease, cycles of 21 ± 3 days.
What is the primary treatment option for chronic ITP that has a 60-80% response rate?
Splenectomy
What are the two main types of platelet disorders?
Thrombocytopenia (quantitative) and thrombasthenia (qualitative).
What is a significant risk associated with splenectomy in ITP patients?
Post-splenectomy sepsis
Which medication used in chronic ITP has a 30% complete response rate?
Rituximab
What is the typical presentation of immune thrombocytopenia (ITP) in children?
Acute onset of thrombocytopenia with skin findings and mucosal bleeding.
What is the function of thrombopoietic agents in ITP treatment?
Stimulate bone marrow production of platelets
What factors influence the choice of treatment for ITP?
Bleeding symptoms, family comfort level, assurance regarding follow-up, and less so on platelet count.
What is the peak age range for chronic ITP in adults?
15-40 years
What is the mechanism of action for corticosteroids in treating ITP?
Reticuloendothelial blockade and reduced synthesis of anti-platelet antibodies.
What is the male to female ratio in chronic ITP?
1:2-3
What is the typical platelet count range in chronic ITP?
30,000 - 80,000/μl
What is the natural history of ITP in terms of platelet count normalization?
40% within 6 weeks, 50% within 3 months, 65% within 6 months, 80% within 12 months.
What percentage of children with acute ITP experience spontaneous remission?
83%
What is the management approach for persistent or chronic ITP?
Repeat use of acute medications, observation, splenectomy, rituximab, additional immunosuppressants, thrombopoietic agents.
What is the cure rate for children with lymphoma who were on study in the 1991 COG study?
76%
What is the most common clinical presentation of leukemia in children?
Hepatosplenomegaly
What is the most common pediatric malignancy?
Acute Lymphocytic Leukemia (ALL)
What is the peak age range for ALL in children?
2-5 years
What is the main risk associated with Tumor Lysis Syndrome (TLS) in leukemia?
Renal dysfunction/failure and metabolic complications
What is the primary treatment approach for ALL during the induction phase?
Steroids, Vincristine, L-Asparaginase
What is the survival rate for childhood AML?
50%
What is the survival rate for children with AML?
50%
What is the most common location for brain tumors in children?
Posterior Fossa
What percentage of leukemia cases in children does AML represent?
10%
What is the second most common type of cancer in children?
Brain tumors
What is the most common extracranial solid tumor in children?
Neuroblastoma
What is the median age at diagnosis for neuroblastoma?
19 months
What is the 5-year survival rate for children with brain tumors?
60-70%
What is the common treatment approach for high-risk neuroblastoma cases?
High-risk treatment with significant morbidity
What are common symptoms of increased intracranial pressure in children with brain tumors?
Headache, irritability, lethargy, vomiting
What is the genetic predisposition percentage for familial neuroblastoma?
1%
What are the key clinical features of neuroblastoma?
Horner’s Syndrome, ‘Racoon Eyes’, and abdominal mass
What factors influence the prognosis of neuroblastoma?
Age, stage, and n-myc amplification
What is the significance of spontaneous resolution in neuroblastoma?
It indicates a ‘benign’ transformation, particularly in Stage IVS
What is the cure rate for low-risk neuroblastoma?
95%
What is the primary concern with opsoclonus-myoclonus ataxia syndrome in neuroblastoma?
Neurocognitive effects of autoimmunity
What type of cancer is Wilms’ tumor?
Embryonal cancer of the kidney
What is the most common presentation of Wilms’ tumor?
Painless mass or abdominal swelling
What is the most important prognostic factor for Wilms’ tumor?
Anaplastic histology
What are the common clinical features of osteosarcoma?
Pain and/or mass at the primary site, typically involving the metaphyses of long bones
What genetic factor significantly increases the risk of osteosarcoma, especially in radiated fields?
Loss of tumor suppressor gene in retinoblastoma
What is the typical pattern of spread for osteosarcoma?
Skip lesions and hematogenous spread, primarily to the lungs
What are the primary sites commonly affected by Ewing Sarcoma?
Pelvis, femur, and rib
What is the primary treatment approach for osteosarcoma?
Surgery and chemotherapy
What is the most common primary site for osteosarcoma?
Distal femur
What systemic symptoms are associated with Ewing Sarcoma?
Fever and weight loss
What is the characteristic radiographic appearance of Ewing Sarcoma?
Onion skin
What is the most common pattern of metastatic spread in Ewing Sarcoma?
Isolated lung, isolated bone, and isolated bone marrow
What is the pathognomonic translocation found in 90%-95% of Ewing Sarcoma cases?
t(11;22) leading to FLI-1/EWS fusion protein
What diagnostic procedures are included in the staging workup for Ewing Sarcoma?
Plain films, MRI or CT of primary site, CT chest/CXR, bone scan, bone marrow aspirate/biopsy
What are the two main categories for stage assignment in Ewing Sarcoma?
Localized vs. metastatic
