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Hematology/Immunology Part 2 > Pediatric Hematology/Oncology > Flashcards

Pediatric 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 is an example of anemia despite a ‘high’ hemoglobin level?

A

A 2-year-old child with cyanotic congenital heart disease who has a Hgb of 14 gm/dL and Hct of 40%

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3
Q

What is an example of no anemia despite a ‘low’ hemoglobin level?

A

A 2-month-old thriving premature infant with Hgb 7.5 gm/dL

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4
Q

How is anemia defined practically?

A

Hemoglobin level at least 2 standard deviations below mean value for age, gender, and race

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5
Q

What factors determine hemoglobin values in normal children?

A

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

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6
Q

What is the recommended method for evaluating lead poisoning?

A

Venous blood lead level (BLL)

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7
Q

What BLL is considered abnormal and warrants intervention?

A

≥ 5

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8
Q

What are reticulocytes?

A

Young RBCs in circulation, still with residual RNA

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9
Q

How are reticulocytes characterized?

A

Larger than normal RBCs, lack central pallor, bluish tint

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10
Q

What additional screening should be done if BLL is elevated?

A

Screen for iron deficiency

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11
Q

What is the normal percentage of reticulocytes in the RBC population?

A

0.5-1.5%

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12
Q

What imaging might be considered if a patient is symptomatic for lead poisoning?

A

Plain abdominal x-ray

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13
Q

How is the absolute reticulocyte count (ARC) calculated?

A

% retic x RBC count/L

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14
Q

What should be done to manage lead poisoning?

A

End further exposure to lead

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15
Q

What is the normal range for the absolute reticulocyte count (ARC)?

A

25,000 – 75,000

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16
Q

Who else should be tested if one household member has elevated BLL?

A

Household members/children

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17
Q

What public health measure can help manage lead exposure?

A

Lead abatement program

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18
Q

What does an increased reticulocyte count indicate in the presence of anemia?

A

Shows bone marrow response

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19
Q

What nutritional elements should be ensured in the diet to manage lead poisoning?

A

Calcium, vitamin C, iron

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20
Q

What therapy is used for significantly elevated BLL?

A

Chelation therapy

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21
Q

What does anemia with a low/normal ARC suggest?

A

Deficient production of RBCs

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22
Q

What is a medical emergency in the context of lead poisoning?

A

Symptomatic lead intoxication

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23
Q

What does anemia with a high reticulocyte count indicate?

A

Increased erythropoietic response

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24
Q

What does stable anemia with a low reticulocyte count indicate?

A

Deficient production (reduced bone marrow response)

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25
Q

What does lead poisoning cause in erythrocytes?

A

Basophilic stippling

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26
Q

What is physiologic anemia of early infancy?

A

The smaller the premie, the earlier and lower the hemoglobin nadir

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27
Q

What is elevated in the blood due to lead poisoning?

A

Free erythrocyte protoporphyrin

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28
Q

What is the definition of neutropenia?

A

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

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29
Q

How is the Absolute Neutrophil Count (ANC) calculated?

A

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

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30
Q

What is the ANC threshold for term newborns up to 1 week?

A

<3,000

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31
Q

What is the ANC threshold for infants aged 1 week to 2 years?

A

<1,100

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32
Q

What is the ANC threshold for children, adolescents, and adults?

A

<1,500

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33
Q

What is the hemoglobin nadir for a term infant at 12 weeks?

A

9.5 gm/dl

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34
Q

What condition is NOT caused by lead poisoning?

A

Microcytic anemia

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35
Q

What ANC range indicates mild neutropenia and its associated infectious risk?

A

1,000-1,500; none

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36
Q

What is a common cause of microcytic anemia in young children with lead poisoning?

A

Iron deficiency

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37
Q

What is the hemoglobin nadir for a premature infant at 6-8 weeks?

A

7.0 gm/dl

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38
Q

What dietary deficiency can lead to PICA?

A

Iron deficiency

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39
Q

What ANC range indicates moderate neutropenia and its associated infectious risk?

A

500-1,000; minimal

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40
Q

What is the response rate to splenectomy in managing chronic ITP?

A

60-80%

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41
Q

What can PICA lead to in terms of lead exposure?

A

Lead ingestion

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42
Q

What is a significant risk associated with post-splenectomy in chronic ITP management?

A

Sepsis

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43
Q

What ANC level indicates severe neutropenia and its associated infectious risk?

A

<500; moderate to severe

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44
Q

What are common causes of iron deficiency during childhood?

A

Blood loss, GI tract issues, cow’s milk, parasitic infection, esophageal varices, anatomic lesions, inflammatory bowel disease, epistaxis, menorrhagia, intrapulmonary or renal loss, idiopathic pulmonary hemosiderosis, chronic intravascular hemolysis

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45
Q

What is the most common cause of neutropenia?

A

Infection-associated neutropenia

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46
Q

What preventive measures are needed before and after splenectomy for chronic ITP?

A

Vaccinations prior to surgery and prophylactic antibiotics following

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47
Q

What is the complete response rate to Rituximab in chronic ITP management?

A

30%

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48
Q

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

A

Contains minimal iron, iron poorly absorbed, leads to reduced intake of other foods, may cause GI bleeding

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49
Q

What are some mechanisms of infection-associated neutropenia?

A

Increased utilization, complement mediated margination, marrow suppression/failure, direct effect, cytokine/chemokine induced margination, antibody formation

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50
Q

Name two additional immunosuppressant agents used in chronic ITP management.

A

Mycophenolate mofetil, 6-mercaptopurine

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51
Q

What are the causes of iron deficiency during adolescence?

A

Rapid growth, blood loss, menstrual, gastrointestinal and renal in athletes, pregnancy, H. pylori infection

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52
Q

What does iron deficiency increase in the context of lead poisoning?

A

Lead absorption from the intestine

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53
Q

What do thrombopoietic agents do in chronic ITP management?

A

Stimulate bone marrow production of platelets

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54
Q

What is the most common cause of microcytic hypochromic anemia during childhood?

A

Iron deficiency

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55
Q

What are two broad categories of hemoglobin disorders?

A

Quantitative and qualitative disorders

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56
Q

What is an example of a quantitative hemoglobin disorder?

A

Thalassemia

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57
Q

What characterizes immune neutropenias of childhood?

A

Marrow production normal to increased, storage pool normal to decreased, increase in turnover of neutrophils, vascular compartment decreased

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58
Q

What is an example of a qualitative hemoglobin disorder?

A

Structural variants or hemoglobinopathies

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59
Q

What are the risk factors for lead poisoning?

A

Increased susceptibility in younger children, incomplete blood-brain barrier, higher prevalence of iron deficiency, exposure to lead

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60
Q

What is Diamond Blackfan Anemia (DBA)?

A

Congenital/inherited anemia

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61
Q

What are the categories of immune neutropenias of childhood?

A

Chronic benign neutropenia of childhood, alloimmune, autoimmune, drug-induced

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62
Q

List three thrombopoietic agents used in chronic ITP management.

A

Eltrombopag (Promacta), Romiplostim (NPlate), Avatrombopag (Doptelet)

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63
Q

What are common sources of lead exposure?

A

Peeling paint in housing built before 1970s, lead dust

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64
Q

What is Transient Erythroblastopenia of Childhood (TEC)?

A

Acquired anemia

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65
Q

What is the function of Fostamatinib in chronic ITP management?

A

Syk (spleen tyrosine kinase) inhibitor

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66
Q

What are the three major systems affected by lead poisoning?

A

Nervous system, heme biosynthetic pathway, renal system

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67
Q

What is the mechanism of chronic benign neutropenia of childhood?

A

Anti-neutrophil antibody

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68
Q

What is a common cause of anemia in children with viral illness?

A

TEC

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69
Q

What is the peak age for acute ITP in children?

A

2-4 years

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70
Q

What are the clinical presentations of lead poisoning?

A

Most are asymptomatic

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71
Q

What is the peak age for chronic ITP in adults?

A

15-40 years

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72
Q

What is the median age at diagnosis for chronic benign neutropenia of childhood?

A

8-11 months

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73
Q

What is the typical age range for diagnosis of TEC?

A

1-4 years

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74
Q

What is the age range for AML in children?

A

1 – 15 years

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75
Q

What are the effects of lead poisoning on the nervous system?

A

Decreased IQ, cognitive effects, seizures, encephalopathy

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76
Q

What is the male-to-female ratio for acute ITP?

A

1:1

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77
Q

What is the typical age for diagnosis of DBA?

A

<1 year

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78
Q

How many AML cases are diagnosed annually in the US?

A

750 cases

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79
Q

What is the male-to-female ratio for chronic ITP?

A

1:2-3

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80
Q

What percentage of leukemia cases in children does AML represent?

A

10%

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81
Q

What percentage of DBA patients have congenital anomalies?

A

30-50%

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82
Q

How does lead poisoning affect the heme biosynthetic pathway?

A

Interferes with several enzymatic steps, decreased hgb synthesis at BLLs of 40, hemolytic anemia at BLL >70

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83
Q

How common is antecedent infection in acute ITP?

A

Common 1-3 weeks prior

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84
Q

How common is antecedent infection in chronic ITP?

A

Unusual

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85
Q

What percentage of leukemia-related deaths in children is due to AML?

A

30%

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86
Q

What is the hemoglobin level at diagnosis for DBA?

A

2-6 g/dL

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87
Q

What are the renal effects of lead poisoning?

A

Renal tubular dysfunction, chronic interstitial nephritis

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88
Q

Describe the onset of bleeding in acute ITP.

A

Abrupt

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89
Q

What is the overall survival rate for children with AML?

A

50%

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90
Q

What is the median length of illness for chronic benign neutropenia of childhood?

A

20 months

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91
Q

What are the gastrointestinal effects of lead poisoning?

A

Lead colic – vomiting, abdominal pain, constipation

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92
Q

What is the hemoglobin level at diagnosis for TEC?

A

3-9 g/dL

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93
Q

What is the white blood cell count in DBA?

A

Normal

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94
Q

Describe the onset of bleeding in chronic ITP.

A

Insidious

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95
Q

What is the platelet count in acute ITP?

A

<20,000/μl

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96
Q

Describe the therapy approach for AML in children.

A

Very short and very aggressive

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97
Q

What is the usual ANC level in chronic benign neutropenia of childhood?

A

<500

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98
Q

What are the endocrine effects of lead poisoning?

A

Decreased vitamin D metabolism with increased lead level

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99
Q

How is lead poisoning evaluated?

A

Venous blood lead level (BLL); routine screening recommended

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100
Q

What is considered an abnormal blood lead level (BLL)?

A

BLL ≥ 5

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101
Q

What additional screening is recommended if blood lead levels are elevated?

A

Screen for iron deficiency

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102
Q

What is the white blood cell count in TEC?

A

Normal to decreased

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103
Q

What is the platelet count in chronic ITP?

A

30,000 - 80,000/μl

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104
Q

What drugs are used during the induction phase of AML therapy?

A

High dose Ara-C, doxorubicin

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105
Q

What is the typical result of an antibody test in chronic benign neutropenia of childhood?

A

Positive

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106
Q

What percentage of acute ITP cases involve severe bleeding?

A

<10%

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107
Q

What is the platelet count in DBA?

A

Normal

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108
Q

What options are available during the consolidation phase of AML therapy?

A

More Ara-C or BMT if a match is available

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109
Q

What does the bone marrow show in chronic benign neutropenia of childhood?

A

Normal to increased myeloid series with a decrease at the band and/or seg level; some show earlier arrest

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110
Q

What is the platelet count in TEC?

A

Normal

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111
Q

Is there a maintenance phase in AML therapy for children?

A

No, it did not work

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112
Q

What percentage of chronic ITP cases involve severe bleeding?

A

Usually mild bleeding

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113
Q

Is the mean corpuscular volume (MCV) increased in DBA?

A

Yes

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114
Q

What is the incidence of cerebral hemorrhage in acute ITP?

A

<1%

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115
Q

What is the incidence of cerebral hemorrhage in chronic ITP?

A

3%

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116
Q

What imaging might be considered if symptomatic for lead poisoning?

A

Plain abdominal x-ray

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117
Q

What is the hemorrhagic death rate in acute ITP?

A

<1%

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118
Q

What is the mortality rate due to toxic death during AML chemotherapy?

A

7 – 9%

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119
Q

Is the mean corpuscular volume (MCV) increased in TEC?

A

No

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120
Q

Is hemoglobin F increased in DBA?

A

Yes

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121
Q

What is the hemorrhagic death rate in chronic ITP?

A

4%

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122
Q

What is the first step in managing lead poisoning?

A

End further exposure to lead

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123
Q

What is the second most common cancer in children?

A

Brain tumors

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124
Q

Is hemoglobin F increased in TEC?

A

No

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125
Q

What is the management approach for chronic benign neutropenia of childhood?

A

Supportive care; small risk for infection; G-CSF may be required in some patients with infections

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126
Q

What is the duration of acute ITP?

A

2-6 weeks

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127
Q

What nutritional measures can help manage lead poisoning?

A

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

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128
Q

What is the duration of chronic ITP?

A

Months to years

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129
Q

What is the most common type of solid neoplasm in children?

A

Brain tumors

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130
Q

What is the course of DBA?

A

Prolonged transfusion support or steroid therapy

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131
Q

What is the spontaneous remission rate in acute ITP?

A

83%

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132
Q

What therapy is used for significantly elevated lead levels?

A

Chelation therapy

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133
Q

What is the mechanism of cyclic neutropenia?

A

Apoptosis in precursors and cyclic hematopoiesis

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134
Q

What is the course of TEC?

A

Spontaneous recovery within weeks to months

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135
Q

What is the urgency of symptomatic lead intoxication?

A

It is a medical emergency

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136
Q

How many new brain tumor diagnoses are there annually in children?

A

3000-3500

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137
Q

What is the spontaneous remission rate in chronic ITP?

A

2%

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138
Q

What is the response rate to splenectomy in acute ITP?

A

71%

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139
Q

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

A

60-70%

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140
Q

What are the two main types of leukocytes?

A

Granulocytes and mononuclear phagocytes

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141
Q

What is the response rate to splenectomy in chronic ITP?

A

66%

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142
Q

What are the inheritance patterns and genetic mutations associated with cyclic neutropenia?

A

Autosomal dominant and sporadic ELA-2 mutations

143
Q

What are the clinical features of Horner’s Syndrome in neuroblastoma?

A

Miosis, ptosis, anhidrosis

144
Q

What are the three types of granulocytes?

A

Neutrophils, eosinophils, basophils

145
Q

Where are brain tumors most commonly located in children?

A

Posterior fossa

146
Q

What is the importance of National Cooperative Group Studies in pediatric oncology?

A

Higher cure rates for children on study

147
Q

What was the cure rate for children with lymphoma on study in 1991?

A

76%

148
Q

What are the clinical features of cyclic neutropenia?

A

Recurrent fever, pharyngitis, aphthous ulcers, periodontal disease

149
Q

What is a common physical sign of neuroblastoma in children?

A

Racoon eyes and abdominal mass

150
Q

What are the two types of mononuclear phagocytes?

A

Monocytes and lymphocytes

151
Q

What is the ratio of infratentorial to supratentorial tumors in children?

A

2/3 infratentorial, 1/3 supratentorial

152
Q

What was the cure rate for children with lymphoma not on study in 1991?

A

58%

153
Q

What factors influence the prognosis of neuroblastoma?

A

Age, stage, n-myc amplification

154
Q

What should you think of when considering infratentorial tumors?

A

Increased ICP

155
Q

What is the typical cycle duration for cyclic neutropenia?

A

21 ± 3 days (range 14-28 days)

156
Q

What was the cure rate for children with ALL on study in Delaware in 1983?

A

60%

157
Q

What is the significance of spontaneous resolution in neuroblastoma?

A

It indicates a benign transformation.

158
Q

What should be included in the history for neutrophil evaluation?

A

Symptoms, duration, number of infections, exposure to toxins/drugs, family history

159
Q

What should you think of when considering supratentorial tumors?

A

Seizure

160
Q

What are the common sites for Stage IVS neuroblastoma?

A

Skin, liver, bone marrow

161
Q

What should be examined physically for neutrophil evaluation?

A

Infected sites, teeth and gums, lymph nodes, liver, spleen, other phenotypic findings

162
Q

What was the cure rate for children with ALL not on study in Delaware in 1983?

A

19%

163
Q

Name three common tumors of the posterior fossa in children.

A

Medulloblastoma, ependymoma, cerebellar astrocytoma

164
Q

What is the cure rate for low-risk neuroblastoma?

A

95%

165
Q

What is leukemia a disease of?

A

Bone marrow

166
Q

How is cyclic neutropenia diagnosed?

A

Check CBC 2-3 times weekly for 6-8 weeks; ANC <200 for 3-5 days

167
Q

What are general, non-localizing symptoms of brain tumors in children?

A

Headache, vomiting, behavioral changes, developmental delay, weight gain/loss

168
Q

What percentage of leukemia patients present with hepatosplenomegaly?

A

60%

169
Q

What percentage of leukemia patients present with fever?

A

55%

170
Q

What is the cure rate for intermediate-risk neuroblastoma?

A

60%

171
Q

What laboratory tests are important for neutrophil evaluation?

A

CBC + differential, peripheral smear, bone marrow aspirate, chemistries, anti-neutrophil antibodies

172
Q

What percentage of leukemia patients present with malaise?

A

50%

173
Q

What does the bone marrow show during neutropenia in cyclic neutropenia?

A

Myeloid hypoplasia, arrest at myelocyte level

174
Q

What are the general classifications of neutropenia?

A

Marked decrease bone marrow reserve, normal marrow reserve

175
Q

What is the cure rate for high-risk neuroblastoma?

A

15%

176
Q

What percentage of leukemia patients present with bleeding?

A

42%

177
Q

What is the management approach for cyclic neutropenia?

A

Aggressive antibiotic and supportive care for infections; G-CSF daily or alternate days if required

178
Q

What are some localizing symptoms of brain tumors in children?

A

Cranial neuropathies, hemiparesis, hemisensory loss, ataxia, early handedness/change in handedness, seizures

179
Q

What percentage of leukemia patients present with lymphadenopathy?

A

35%

180
Q

How can spinal cord compression in neuroblastoma be treated?

A

Emergent chemotherapy

181
Q

What is the increased risk associated with retinoblastoma loss of tumor suppressor gene?

A

Osteosarcoma, especially in the radiated field.

182
Q

What percentage of leukemia patients present with bone/joint pain?

A

27%

183
Q

What percentage of leukemia patients present with anorexia?

A

20%

184
Q

What are primary disorders causing neutropenia?

A

Kostmann syndrome, Shwachman-Diamond syndrome, cyclic neutropenia

185
Q

What is a major problem associated with opsoclonus-myoclonus ataxia syndrome in neuroblastoma?

A

Neurocognitive effects of autoimmunity

186
Q

What are symptoms of increased ICP in children with brain tumors?

A

Headache, irritability, lethargy, vomiting, bulging fontanelle, separation of sutures, papilledema, Parinaud syndrome

187
Q

What percentage of leukemia patients present with a mediastinal mass?

A

13%

188
Q

What are secondary causes of neutropenia?

A

Chemotherapy, drug-induced, nutritional, viral infection

189
Q

What are the two main types of platelet disorders?

A

Thrombocytopenia (quantitative) and thrombasthenia (qualitative)

190
Q

What type of cancer is Wilms’ tumor?

A

Embryonal cancer of the kidney

191
Q

What is Parinaud syndrome?

A

Impaired upward gaze, convergence nystagmus, abnormal pupil responses

192
Q

What percentage of leukemia patients present with abdominal pain?

A

10%

193
Q

What is the definition of neutropenia?

A

Decrease in the absolute neutrophil count below accepted norms for age

194
Q

What is a specific finding for platelet disorders?

A

Petechiae

195
Q

What is the prevalence of Wilms’ tumor?

A

1:10,000

196
Q

What percentage of leukemia patients present with CNS disease?

A

5%

197
Q

What are the types of tumors included in the Ewing family of tumors?

A

Ewing sarcoma of bone, extraosseous Ewing sarcoma, primitive neuroectodermal tumor, Askin tumor.

198
Q

What percentage of leukemia patients present with testicular involvement?

A

1%

199
Q

What is the primary site of Ewing sarcoma?

A

Pain, +/- palpable mass.

200
Q

What are some complications of brain tumors in children?

A

Abdominal masses, Wilms’ tumor, neuroblastoma

201
Q

How is the absolute neutrophil count (ANC) calculated?

A

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

202
Q

What percentage of childhood cancers are kidney cancers, and what is the majority type?

A

7%, majority are Wilms’ tumor

203
Q

What is the most common extracranial solid tumor in children?

A

Neuroblastoma

204
Q

What is the importance of personal and family history in platelet disorders?

A

Careful personal and family history of abnormal bleeding is very important

205
Q

What is a common presentation for children with leukemia?

A

Persistent limp with anemia and thrombocytopenia

206
Q

What is the ANC threshold for term newborns?

A

<3,000

207
Q

What is the male to female ratio for Wilms’ tumor?

A

1:1.1

208
Q

What is the prevalence of neuroblastoma in children?

A

1:7000 births

209
Q

Which areas are more frequently involved in Ewing sarcoma compared to osteosarcoma?

A

Diaphyses and central axis.

210
Q

What percentage of leukemia patients have a platelet count <10,000?

A

11%

211
Q

What percentage of leukemia patients have a platelet count between 10,000 and 49,000?

A

38%

212
Q

What is the ANC threshold for infants aged 1 week to 2 years?

A

<1,100

213
Q

What is the median age at presentation for unilateral Wilms’ tumors?

A

44 months

214
Q

What percentage of leukemia patients have a platelet count between 50,000 and 100,000?

A

21%

215
Q

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

A

4.8 ITP cases per 100,000 children per year

216
Q

What percentage of all childhood cancers does neuroblastoma represent?

A

8-10%

217
Q

What percentage of leukemia patients have a platelet count >100,000?

A

30%

218
Q

What is the median age at presentation for bilateral Wilms’ tumors?

A

31 months

219
Q

What is the ANC threshold for children, adolescents, and adults?

A

<1,500

220
Q

What percentage of leukemia patients have hemoglobin <7 g/dL?

A

40%

221
Q

What are the most common primary sites for Ewing sarcoma?

A

Pelvis (23%), femur (18%), rib (13%).

222
Q

What percentage of leukemia patients have hemoglobin between 7 and 9 g/dL?

A

28%

223
Q

Is neuroblastoma more common in boys or girls?

A

Boys (1.1:1.0)

224
Q

What is the infectious risk for mild neutropenia?

A

None

225
Q

What is the typical presentation of ITP in children?

A

Acute onset of thrombocytopenia in an otherwise healthy child; most present with skin findings only

226
Q

What percentage of Wilms’ tumor cases are familial?

A

1-2%

227
Q

What percentage of leukemia patients have hemoglobin between 9 and 11 g/dL?

A

17%

228
Q

What systemic symptoms are associated with Ewing sarcoma?

A

Fever, weight loss.

229
Q

What is the median age at diagnosis for neuroblastoma?

A

19 months

230
Q

What is the nature of bleeding in ITP?

A

Mucosal in nature

231
Q

What percentage of leukemia patients have hemoglobin >11 g/dL?

A

15%

232
Q

What is the infectious risk for moderate neutropenia?

A

Minimal

233
Q

What is the most common presentation of Wilms’ tumor?

A

Painless mass or abdominal swelling

234
Q

What is the typical platelet count in ITP?

A

Usually <20,000/mm³

235
Q

What percentage of leukemia patients have a WBC count <25,000?

A

64%

236
Q

What percentage of neuroblastoma cases are familial?

A

1%

237
Q

What is the infectious risk for severe neutropenia?

A

Moderate to severe

238
Q

What percentage of leukemia patients have a WBC count between 25,000 and 50,000?

A

12%

239
Q

What is the most common metastatic site for Wilms’ tumor?

A

Lung

240
Q

What is seen on a blood smear in ITP?

A

Large platelets

241
Q

What percentage of leukemia patients have a WBC count between 50,000 and 99,000?

A

10%

242
Q

What percentage of neuroblastoma cases are sporadic?

A

99%

243
Q

What percentage of leukemia patients have a WBC count >100,000?

A

14%

244
Q

What is the classic presentation of leukemia in terms of blood cell counts?

A

Pancytopenia

245
Q

How are tumor genomics useful in neuroblastoma?

A

Prognostication (e.g., MYCN)

246
Q

What defines non-high risk neuroblastoma cases?

A

Tumor biology

247
Q

What is the overall survival rate for Wilms’ tumor with surgical staging and multimodality treatment?

A

Exceeding 90%

248
Q

What are the typical lab findings in ITP?

A

Normal hemoglobin, hematocrit, and MCV; may have anemia if significant bleeding; normal WBC count and differential

249
Q

What is Tumor Lysis Syndrome (TLS)?

A

Oncologic emergency with increased uric acid, phosphorus, and potassium

250
Q

What is the pattern of spread for Ewing sarcoma?

A

20% metastatic: isolated lung (35%), isolated bone (13%), isolated bone marrow (7%).

251
Q

Is there any known association of Ewing sarcoma with radiation or syndromes?

A

No.

252
Q

What imaging techniques are used in the staging workup for Ewing sarcoma?

A

Plain films, MRI or CT of primary site, CT chest/CXR, bone scan.

253
Q

What causes Tumor Lysis Syndrome (TLS)?

A

Inability to handle byproducts of leukemia cell death

254
Q

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

A

Anaplastic histology

255
Q

When is a bone marrow biopsy indicated in ITP?

A

Atypical features (e.g., anemia, MCV, neutropenia, hepatosplenomegaly, etc.), constitutional symptoms

256
Q

When can Tumor Lysis Syndrome (TLS) occur?

A

Spontaneously or after therapy is started

257
Q

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

A

Surgery +/- low dose chemotherapy

258
Q

Which leukemias are most problematic for Tumor Lysis Syndrome (TLS)?

A

Leukemias with high growth fraction and/or high tumor burden

259
Q

What is the diagnostic platelet count for ITP?

A

<100,000/mm³

260
Q

What is the purpose of bone marrow aspirate/biopsy in Ewing sarcoma staging?

A

To check for bone marrow involvement.

261
Q

What is the effectiveness of high-risk treatment for neuroblastoma?

A

Effective in only 50% with significant morbidity

262
Q

What are the clinical presentations of Wilms’ tumor?

A

Painless abdominal swelling or mass, palpable fixed non-tender flank mass, abdominal pain, gross hematuria, fever

263
Q

What are the different durations of ITP?

A

Newly diagnosed: diagnosis-3 months; Persistent ITP: 3-12 months; Chronic ITP: >12 months

264
Q

How is Ewing sarcoma stage assignment categorized?

A

Localized vs. metastatic.

265
Q

What are the clinical presentations of neuroblastoma?

A

Horner’s Syndrome, “Racoon Eyes,” abdominal mass

266
Q

What percentage of Wilms’ tumor patients experience hypertension?

A

25%

267
Q

What indicates disease resolution in ITP?

A

Platelet count >100,000/mm³

268
Q

What are the symptoms of Horner’s Syndrome?

A

Miosis, ptosis, anhidrosis

269
Q

What can sub-capsular hemorrhage in Wilms’ tumor lead to?

A

Rapid abdominal enlargement, anemia, hypertension, fever

270
Q

What are the associated syndromes with Wilms’ tumor?

A

Rare

271
Q

What genetic translocation is pathognomonic for Ewing sarcoma?

A

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

272
Q

What does RFS stand for in Wilms’ tumor outcomes?

A

Relapse free survival

273
Q

Name two specific types of leukemia with high risk for Tumor Lysis Syndrome (TLS).

A

Burkitt’s, T-ALL

274
Q

What are the main risks associated with Tumor Lysis Syndrome (TLS)?

A

Renal dysfunction/failure and metabolic complications

275
Q

How can Tumor Lysis Syndrome (TLS) be prevented and treated?

A

Hydration, alkalinization of urine, Allopurinol or rasburicase

276
Q

What are the two main types of leukemia in children?

A

ALL (Acute Lymphocytic Leukemia), AML (Acute Myelogenous Leukemia)

277
Q

What percentage of childhood leukemia cases are ALL?

A

80%

278
Q

What is the function of the FLI-1 protein in Ewing sarcoma?

A

DNA binding domain.

279
Q

What percentage of childhood leukemia cases are AML?

A

15%

280
Q

What percentage of childhood leukemia cases are CML and JMML?

A

5%

281
Q

What is the most common pediatric malignancy?

A

ALL (Acute Lymphocytic Leukemia)

282
Q

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

A

Reduction or cessation of new hemorrhage within 3-10 days; rise in platelet count within 1-3 weeks; normalization of platelet count in 40% of cases within 6 weeks, 50% within 3 months, 65% within 6 months, 80% within 12 months

283
Q

What percentage of total pediatric malignancies does ALL represent?

A

25%

284
Q

What does OS stand for in Wilms’ tumor outcomes?

A

Overall survival

285
Q

What is the function of the EWS protein in Ewing sarcoma?

A

Transcription activation.

286
Q

How many cases of ALL are diagnosed per year in the US?

A

3,000 cases/year

287
Q

What are the two main types of bone tumors mentioned?

A

Ewing’s sarcoma, osteosarcoma

288
Q

What percentage of Ewing sarcoma cases have the t(21;22) translocation?

A

5%.

289
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

290
Q

What is the characteristic radiographic appearance of Ewing sarcoma?

A

Onion skin.

291
Q

What are the cell types involved in Ewing’s sarcoma and osteosarcoma?

A

Small round blue cells, spindle cells, new bone formation

292
Q

What is the incidence rate of ALL in children?

A

3 cases/100,000 children

293
Q

What is the usual time to response for observation and education in ITP management?

A

1-3 weeks

294
Q

What is the usual time to response for corticosteroids in ITP management?

A

3-4 days

295
Q

What is the peak age range for ALL?

A

2-5 years

296
Q

Which racial group has a higher incidence of ALL?

A

White

297
Q

What is the typical clinical presentation of osteosarcoma?

A

Pain +/- mass at primary site

298
Q

Which gender has a higher incidence of ALL?

A

Boys

299
Q

Where does osteosarcoma typically involve?

A

Metaphyses of long bones

300
Q

What is the usual time to response for IVIG in ITP management?

A

24-48 hours

301
Q

What are the most common primary sites for osteosarcoma?

A

Distal femur, proximal tibia, proximal humerus

302
Q

How does socioeconomic status affect the incidence of ALL?

A

Higher SEC > Lower SEC

303
Q

What are skip lesions in osteosarcoma?

A

Lesions occurring several cm from primary site

304
Q

What is the usual time to response for Anti-D immunoglobulin in ITP management?

A

24-48 hours

305
Q

What is a common cause of ALL?

A

Typically unknown

306
Q

What is the hematogenous spread pattern in osteosarcoma?

A

Lung, bone

307
Q

Name a genetic syndrome that increases the risk for ALL.

A

Down Syndrome (Trisomy 21)

308
Q

What percentage of ALL cases are B Cell?

A

80%

309
Q

What percentage of osteosarcoma cases are caused by radiation?

A

3%

310
Q

What percentage of ALL cases are T Cell?

A

15%

311
Q

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

A

Reticuloendothelial blockade and reduced synthesis of anti-platelet antibodies

312
Q

What are the side effects of corticosteroids in ITP management?

A

Moodiness, weight gain, irritability, bone pain, hypertension, hyperglycemia

313
Q

What is the median time for osteosarcoma to occur following radiation exposure?

A

9-11 years

314
Q

What is the mechanism of action for IVIG in ITP management?

A

Reticuloendothelial blockade

315
Q

What percentage of ALL cases are Mature B Cell (Burkitt’s-Type)?

A

2%

316
Q

What imaging is used in the staging workup for osteosarcoma?

A

Plain films, MRI, CT chest/CXR, bone scan

317
Q

What percentage of ALL cases are Indeterminant?

A

3%

318
Q

How is ALL diagnosed?

A

Characteristic morphology and diagnostic immunophenotype of cells (lymphoblasts) from peripheral blood or bone marrow

319
Q

What diagnostic techniques are used for ALL?

A

Flow cytometry, immunohistochemistry

320
Q

How is osteosarcoma staged?

A

Localized vs. metastatic, resectable vs. unresectable

321
Q

What are the side effects of IVIG in ITP management?

A

Aseptic meningitis, headache, infusion fever and chills

322
Q

What is a notable radiographic feature of osteosarcoma?

A

Lytic or permeative, proximity to growth plates, starburst new bone formation

323
Q

What is the mechanism of action for Anti-D immunoglobulin in ITP management?

A

Sensitized erythrocytes occupy the Fc receptors; use only in Rh+ non-splenectomized patients

324
Q

What is the primary treatment for osteosarcoma?

A

Surgery

325
Q

What is the role of chemotherapy in osteosarcoma treatment?

A

Used in combination with surgery, better outcomes

326
Q

What are the main components of induction therapy for ALL?

A

Steroids, Vincristine, L-Asparaginase

327
Q

How is treatment response evaluated in osteosarcoma?

A

Pre-op clinical response, pathologic response (necrosis assessment)

328
Q

What additional drugs are used for higher risk ALL during induction?

A

Doxorubicin or Daunomycin

329
Q

What are the side effects of Anti-D immunoglobulin in ITP management?

A

Hemolytic anemia (2 gm decline), DIC, fever and chills

330
Q

What drugs complement induction therapy for ALL?

A

Ara-C, Cyclophosphamide

331
Q

When is spinal/cranial irradiation used in ALL treatment?

A

If CNS (+)

332
Q

What is the duration of maintenance therapy for ALL?

A

~2 years

333
Q

What drugs are used in maintenance therapy for ALL?

A

6-MP, Methotrexate

334
Q

What is the management approach for persistent/chronic ITP?

A

Repeat use of acute medications or observation; splenectomy; rituximab; additional immunosuppressant agents; thrombopoietic agents

335
Q

What is a good WBC count at presentation for ALL prognosis?

A

<50,000

336
Q

What is a good age range for ALL prognosis?

A

1-9 years

337
Q

What type of chromosome abnormalities are considered good for ALL prognosis?

A

Good and Bad

338
Q

What is a good response to treatment for ALL prognosis?

A

Remission by Day 14

339
Q

What is a worse factor for ALL prognosis?

A

CNS Disease

340
Q

What is the response rate to splenectomy in ITP management?

A

60-80%

341
Q

Which cell type is better for ALL prognosis?

A

B cell

342
Q

What is the survival rate for AML in children?

A

50%

343
Q

What is the therapy approach for AML in children?

A

Very short and very aggressive

344
Q

What is the risk associated with splenectomy in ITP management?

A

Post-splenectomy sepsis

345
Q

What are the main components of induction therapy for AML?

A

High dose Ara-C, doxorubicin

346
Q

What is the response rate to rituximab in ITP management?

A

30% complete response

347
Q

What are some additional immunosuppressant agents used in ITP management?

A

Mycophenolate mofetil, 6-mercaptopurine

348
Q

What are the main components of consolidation therapy for AML?

A

More Ara-C or BMT if match available

349
Q

Is there maintenance therapy for AML?

A

None – did not work

350
Q

What percentage of children with AML die a toxic death during chemotherapy?

A

7-9%

351
Q

What do thrombopoietic agents do in ITP management?

A

Stimulate bone marrow production of platelets

352
Q

What are some examples of thrombopoietic agents used in ITP management?

A

Eltrombopag (Promacta), Romiplostim (NPlate), Avatrombopag (Doptelet)

353
Q

What is a Syk inhibitor used in adult ITP management?

A

Fostamatinib (Tavalisse)

Hematology/Immunology Part 2 (9 decks)

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