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Hematology/Immunology Part 2 > Pediatrics Hematology Oncology > Flashcards

Pediatrics Hematology Oncology Flashcards

1
Q

How is anemia defined physiologically?

A

Hemoglobin level too low to meet cellular oxygen demands.

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2
Q

What factors determine hemoglobin values in normal children?

A

Age, gender, race, degree of sexual maturation, altitude, and heredity.

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3
Q

What is the significance of reticulocytes in diagnosing anemia?

A

They help distinguish among different types of anemia by indicating bone marrow response.

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4
Q

Why does excessive cow’s milk intake predispose children to iron deficiency?

A

It contains minimal iron, poorly absorbed iron, reduces intake of other foods, and may cause GI bleeding.

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5
Q

What are the risk factors for lead poisoning in children?

A

Increased susceptibility in younger children, incomplete blood-brain barrier, higher prevalence of iron deficiency, and exposure to lead sources like old paint and dust.

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6
Q

What systems are affected by lead poisoning and how?

A

Nervous system (decreased IQ, cognitive effects, seizures, encephalopathy), heme biosynthetic pathway (anemia), renal system (renal tubular dysfunction, chronic interstitial nephritis).

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7
Q

What is the recommended evaluation for lead poisoning?

A

Venous blood lead level (BLL) testing and routine screening, with intervention for BLL ≥ 5.

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8
Q

What management steps are taken for lead poisoning?

A

End exposure, test household members, lead abatement, ensure adequate nutrition, and chelation therapy for high levels.

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9
Q

What is the recommended action when a venous blood lead level (BLL) is ≥ 5?

A

Intervention is warranted.

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10
Q

What additional screening is suggested if elevated BLL is detected?

A

Screen for iron deficiency.

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11
Q

What imaging technique is considered if symptomatic lead poisoning is suspected?

A

Plain abdominal x-ray.

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12
Q

What dietary recommendations are made to reduce lead absorption?

A

Ensure adequate intake of calcium, vitamin C, iron, and avoid fasting.

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13
Q

What is the efficacy of chelation therapy for lead poisoning?

A

Limited efficacy for significantly elevated levels.

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14
Q

What type of anemia is frequently seen in young children with lead poisoning?

A

Microcytic anemia due to concomitant iron deficiency.

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15
Q

What are the most common causes of microcytic hypochromic anemia during childhood?

A

Iron deficiency, thalassemia, anemia of inflammation.

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16
Q

What are the two broad categories of hemoglobin disorders?

A

Quantitative disorders and qualitative disorders.

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17
Q

What is the typical age range for diagnosis of Transient Erythroblastopenia of Childhood (TEC)?

A

1-4 years.

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18
Q

What is the primary difference in lab values between Diamond Blackfan Anemia (DBA) and TEC?

A

DBA shows increased MCV and elevated hemoglobin F, while TEC does not.

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19
Q

What is the definition of neutropenia?

A

Decrease in the absolute neutrophil count below accepted norms for age.

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20
Q

What is the calculation for Absolute Neutrophil Count (ANC)?

A

ANC = # total WBC * (% PMNs + % bands).

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21
Q

What is the infectious risk associated with severe neutropenia?

A

Moderate to severe.

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22
Q

What is neutropenia?

A

A decrease in the absolute neutrophil count below accepted norms for age.

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23
Q

How is the Absolute Neutrophil Count (ANC) calculated?

A

Total WBC count multiplied by the sum of the percentages of PMNs and bands.

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24
Q

What are the severity levels of neutropenia and their associated infectious risks?

A

Mild (1,000-1,500) - none, Moderate (500-1,000) - minimal, Severe (<500) - moderate to severe.

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25
Q

What is the most common cause of neutropenia?

A

Infection-associated neutropenia.

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26
Q

What are the categories of immune neutropenias of childhood?

A

Chronic benign neutropenia, alloimmune, autoimmune, drug-induced.

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27
Q

What is the mechanism behind chronic benign neutropenia of childhood?

A

Anti-neutrophil antibody.

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28
Q

What are the clinical features of cyclic neutropenia?

A

Recurrent fever, pharyngitis, aphthous ulcers, periodontal disease, cycles of 21 ± 3 days.

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29
Q

What is the primary treatment option for chronic ITP that has a 60-80% response rate?

A

Splenectomy

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30
Q

What are the two main types of platelet disorders?

A

Thrombocytopenia (quantitative) and thrombasthenia (qualitative).

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31
Q

What is a significant risk associated with splenectomy in ITP patients?

A

Post-splenectomy sepsis

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32
Q

Which medication used in chronic ITP has a 30% complete response rate?

A

Rituximab

33
Q

What is the typical presentation of immune thrombocytopenia (ITP) in children?

A

Acute onset of thrombocytopenia with skin findings and mucosal bleeding.

34
Q

What is the function of thrombopoietic agents in ITP treatment?

A

Stimulate bone marrow production of platelets

35
Q

What factors influence the choice of treatment for ITP?

A

Bleeding symptoms, family comfort level, assurance regarding follow-up, and less so on platelet count.

36
Q

What is the peak age range for chronic ITP in adults?

A

15-40 years

37
Q

What is the mechanism of action for corticosteroids in treating ITP?

A

Reticuloendothelial blockade and reduced synthesis of anti-platelet antibodies.

38
Q

What is the male to female ratio in chronic ITP?

A

1:2-3

39
Q

What is the typical platelet count range in chronic ITP?

A

30,000 - 80,000/μl

40
Q

What is the natural history of ITP in terms of platelet count normalization?

A

40% within 6 weeks, 50% within 3 months, 65% within 6 months, 80% within 12 months.

41
Q

What percentage of children with acute ITP experience spontaneous remission?

A

83%

42
Q

What is the management approach for persistent or chronic ITP?

A

Repeat use of acute medications, observation, splenectomy, rituximab, additional immunosuppressants, thrombopoietic agents.

43
Q

What is the cure rate for children with lymphoma who were on study in the 1991 COG study?

A

76%

44
Q

What is the most common clinical presentation of leukemia in children?

A

Hepatosplenomegaly

45
Q

What is the most common pediatric malignancy?

A

Acute Lymphocytic Leukemia (ALL)

46
Q

What is the peak age range for ALL in children?

A

2-5 years

47
Q

What is the main risk associated with Tumor Lysis Syndrome (TLS) in leukemia?

A

Renal dysfunction/failure and metabolic complications

48
Q

What is the primary treatment approach for ALL during the induction phase?

A

Steroids, Vincristine, L-Asparaginase

49
Q

What is the survival rate for childhood AML?

A

50%

50
Q

What is the survival rate for children with AML?

A

50%

51
Q

What is the most common location for brain tumors in children?

A

Posterior Fossa

52
Q

What percentage of leukemia cases in children does AML represent?

A

10%

53
Q

What is the second most common type of cancer in children?

A

Brain tumors

54
Q

What is the most common extracranial solid tumor in children?

A

Neuroblastoma

55
Q

What is the median age at diagnosis for neuroblastoma?

A

19 months

56
Q

What is the 5-year survival rate for children with brain tumors?

A

60-70%

57
Q

What is the common treatment approach for high-risk neuroblastoma cases?

A

High-risk treatment with significant morbidity

58
Q

What are common symptoms of increased intracranial pressure in children with brain tumors?

A

Headache, irritability, lethargy, vomiting

59
Q

What is the genetic predisposition percentage for familial neuroblastoma?

A

1%

60
Q

What are the key clinical features of neuroblastoma?

A

Horner’s Syndrome, ‘Racoon Eyes’, and abdominal mass

61
Q

What factors influence the prognosis of neuroblastoma?

A

Age, stage, and n-myc amplification

62
Q

What is the significance of spontaneous resolution in neuroblastoma?

A

It indicates a ‘benign’ transformation, particularly in Stage IVS

63
Q

What is the cure rate for low-risk neuroblastoma?

A

95%

64
Q

What is the primary concern with opsoclonus-myoclonus ataxia syndrome in neuroblastoma?

A

Neurocognitive effects of autoimmunity

65
Q

What type of cancer is Wilms’ tumor?

A

Embryonal cancer of the kidney

66
Q

What is the most common presentation of Wilms’ tumor?

A

Painless mass or abdominal swelling

67
Q

What is the most important prognostic factor for Wilms’ tumor?

A

Anaplastic histology

68
Q

What are the common clinical features of osteosarcoma?

A

Pain and/or mass at the primary site, typically involving the metaphyses of long bones

69
Q

What genetic factor significantly increases the risk of osteosarcoma, especially in radiated fields?

A

Loss of tumor suppressor gene in retinoblastoma

70
Q

What is the typical pattern of spread for osteosarcoma?

A

Skip lesions and hematogenous spread, primarily to the lungs

71
Q

What are the primary sites commonly affected by Ewing Sarcoma?

A

Pelvis, femur, and rib

72
Q

What is the primary treatment approach for osteosarcoma?

A

Surgery and chemotherapy

73
Q

What is the most common primary site for osteosarcoma?

A

Distal femur

74
Q

What systemic symptoms are associated with Ewing Sarcoma?

A

Fever and weight loss

75
Q

What is the characteristic radiographic appearance of Ewing Sarcoma?

A

Onion skin

76
Q

What is the most common pattern of metastatic spread in Ewing Sarcoma?

A

Isolated lung, isolated bone, and isolated bone marrow

77
Q

What is the pathognomonic translocation found in 90%-95% of Ewing Sarcoma cases?

A

t(11;22) leading to FLI-1/EWS fusion protein

78
Q

What diagnostic procedures are included in the staging workup for Ewing Sarcoma?

A

Plain films, MRI or CT of primary site, CT chest/CXR, bone scan, bone marrow aspirate/biopsy

79
Q

What are the two main categories for stage assignment in Ewing Sarcoma?

A

Localized vs. metastatic

Hematology/Immunology Part 2 (9 decks)

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