Pediatric Hematology/Oncology Flashcards
1
Q
How is anemia defined physiologically?
A
Hemoglobin level too low to meet cellular oxygen demands
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%
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
4
Q
How is anemia defined practically?
A
Hemoglobin level at least 2 standard deviations below mean value for age, gender, and race
5
Q
What factors determine hemoglobin values in normal children?
A
Age, gender, race, degree of sexual maturation, altitude, heredity
6
Q
What is the recommended method for evaluating lead poisoning?
A
Venous blood lead level (BLL)
7
Q
What BLL is considered abnormal and warrants intervention?
A
≥ 5
8
Q
What are reticulocytes?
A
Young RBCs in circulation, still with residual RNA
9
Q
How are reticulocytes characterized?
A
Larger than normal RBCs, lack central pallor, bluish tint
10
Q
What additional screening should be done if BLL is elevated?
A
Screen for iron deficiency
11
Q
What is the normal percentage of reticulocytes in the RBC population?
A
0.5-1.5%
12
Q
What imaging might be considered if a patient is symptomatic for lead poisoning?
A
Plain abdominal x-ray
13
Q
How is the absolute reticulocyte count (ARC) calculated?
A
% retic x RBC count/L
14
Q
What should be done to manage lead poisoning?
A
End further exposure to lead
15
Q
What is the normal range for the absolute reticulocyte count (ARC)?
A
25,000 – 75,000
16
Q
Who else should be tested if one household member has elevated BLL?
A
Household members/children
17
Q
What public health measure can help manage lead exposure?
A
Lead abatement program
18
Q
What does an increased reticulocyte count indicate in the presence of anemia?
A
Shows bone marrow response
19
Q
What nutritional elements should be ensured in the diet to manage lead poisoning?
A
Calcium, vitamin C, iron
20
Q
What therapy is used for significantly elevated BLL?
A
Chelation therapy
21
Q
What does anemia with a low/normal ARC suggest?
A
Deficient production of RBCs
22
Q
What is a medical emergency in the context of lead poisoning?
A
Symptomatic lead intoxication
23
Q
What does anemia with a high reticulocyte count indicate?
A
Increased erythropoietic response
24
Q
What does stable anemia with a low reticulocyte count indicate?
A
Deficient production (reduced bone marrow response)
25
What does lead poisoning cause in erythrocytes?
Basophilic stippling
26
What is physiologic anemia of early infancy?
The smaller the premie, the earlier and lower the hemoglobin nadir
27
What is elevated in the blood due to lead poisoning?
Free erythrocyte protoporphyrin
28
What is the definition of neutropenia?
Decrease in the absolute neutrophil count below accepted norms for age.
29
How is the Absolute Neutrophil Count (ANC) calculated?
ANC = # total WBC * (% PMNs + % bands)
30
What is the ANC threshold for term newborns up to 1 week?
<3,000
31
What is the ANC threshold for infants aged 1 week to 2 years?
<1,100
32
What is the ANC threshold for children, adolescents, and adults?
<1,500
33
What is the hemoglobin nadir for a term infant at 12 weeks?
9.5 gm/dl
34
What condition is NOT caused by lead poisoning?
Microcytic anemia
35
What ANC range indicates mild neutropenia and its associated infectious risk?
1,000-1,500; none
36
What is a common cause of microcytic anemia in young children with lead poisoning?
Iron deficiency
37
What is the hemoglobin nadir for a premature infant at 6-8 weeks?
7.0 gm/dl
38
What dietary deficiency can lead to PICA?
Iron deficiency
39
What ANC range indicates moderate neutropenia and its associated infectious risk?
500-1,000; minimal
40
What is the response rate to splenectomy in managing chronic ITP?
60-80%
41
What can PICA lead to in terms of lead exposure?
Lead ingestion
42
What is a significant risk associated with post-splenectomy in chronic ITP management?
Sepsis
43
What ANC level indicates severe neutropenia and its associated infectious risk?
<500; moderate to severe
44
What are common causes of iron deficiency during childhood?
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
45
What is the most common cause of neutropenia?
Infection-associated neutropenia
46
What preventive measures are needed before and after splenectomy for chronic ITP?
Vaccinations prior to surgery and prophylactic antibiotics following
47
What is the complete response rate to Rituximab in chronic ITP management?
30%
48
Why does excessive cow’s milk intake predispose to iron deficiency?
Contains minimal iron, iron poorly absorbed, leads to reduced intake of other foods, may cause GI bleeding
49
What are some mechanisms of infection-associated neutropenia?
Increased utilization, complement mediated margination, marrow suppression/failure, direct effect, cytokine/chemokine induced margination, antibody formation
50
Name two additional immunosuppressant agents used in chronic ITP management.
Mycophenolate mofetil, 6-mercaptopurine
51
What are the causes of iron deficiency during adolescence?
Rapid growth, blood loss, menstrual, gastrointestinal and renal in athletes, pregnancy, H. pylori infection
52
What does iron deficiency increase in the context of lead poisoning?
Lead absorption from the intestine
53
What do thrombopoietic agents do in chronic ITP management?
Stimulate bone marrow production of platelets
54
What is the most common cause of microcytic hypochromic anemia during childhood?
Iron deficiency
55
What are two broad categories of hemoglobin disorders?
Quantitative and qualitative disorders
56
What is an example of a quantitative hemoglobin disorder?
Thalassemia
57
What characterizes immune neutropenias of childhood?
Marrow production normal to increased, storage pool normal to decreased, increase in turnover of neutrophils, vascular compartment decreased
58
What is an example of a qualitative hemoglobin disorder?
Structural variants or hemoglobinopathies
59
What are the risk factors for lead poisoning?
Increased susceptibility in younger children, incomplete blood-brain barrier, higher prevalence of iron deficiency, exposure to lead
60
What is Diamond Blackfan Anemia (DBA)?
Congenital/inherited anemia
61
What are the categories of immune neutropenias of childhood?
Chronic benign neutropenia of childhood, alloimmune, autoimmune, drug-induced
62
List three thrombopoietic agents used in chronic ITP management.
Eltrombopag (Promacta), Romiplostim (NPlate), Avatrombopag (Doptelet)
63
What are common sources of lead exposure?
Peeling paint in housing built before 1970s, lead dust
64
What is Transient Erythroblastopenia of Childhood (TEC)?
Acquired anemia
65
What is the function of Fostamatinib in chronic ITP management?
Syk (spleen tyrosine kinase) inhibitor
66
What are the three major systems affected by lead poisoning?
Nervous system, heme biosynthetic pathway, renal system
67
What is the mechanism of chronic benign neutropenia of childhood?
Anti-neutrophil antibody
68
What is a common cause of anemia in children with viral illness?
TEC
69
What is the peak age for acute ITP in children?
2-4 years
70
What are the clinical presentations of lead poisoning?
Most are asymptomatic
71
What is the peak age for chronic ITP in adults?
15-40 years
72
What is the median age at diagnosis for chronic benign neutropenia of childhood?
8-11 months
73
What is the typical age range for diagnosis of TEC?
1-4 years
74
What is the age range for AML in children?
1 – 15 years
75
What are the effects of lead poisoning on the nervous system?
Decreased IQ, cognitive effects, seizures, encephalopathy
76
What is the male-to-female ratio for acute ITP?
1:1
77
What is the typical age for diagnosis of DBA?
<1 year
78
How many AML cases are diagnosed annually in the US?
750 cases
79
What is the male-to-female ratio for chronic ITP?
1:2-3
80
What percentage of leukemia cases in children does AML represent?
10%
81
What percentage of DBA patients have congenital anomalies?
30-50%
82
How does lead poisoning affect the heme biosynthetic pathway?
Interferes with several enzymatic steps, decreased hgb synthesis at BLLs of 40, hemolytic anemia at BLL >70
83
How common is antecedent infection in acute ITP?
Common 1-3 weeks prior
84
How common is antecedent infection in chronic ITP?
Unusual
85
What percentage of leukemia-related deaths in children is due to AML?
30%
86
What is the hemoglobin level at diagnosis for DBA?
2-6 g/dL
87
What are the renal effects of lead poisoning?
Renal tubular dysfunction, chronic interstitial nephritis
88
Describe the onset of bleeding in acute ITP.
Abrupt
89
What is the overall survival rate for children with AML?
50%
90
What is the median length of illness for chronic benign neutropenia of childhood?
20 months
91
What are the gastrointestinal effects of lead poisoning?
Lead colic – vomiting, abdominal pain, constipation
92
What is the hemoglobin level at diagnosis for TEC?
3-9 g/dL
93
What is the white blood cell count in DBA?
Normal
94
Describe the onset of bleeding in chronic ITP.
Insidious
95
What is the platelet count in acute ITP?
<20,000/μl
96
Describe the therapy approach for AML in children.
Very short and very aggressive
97
What is the usual ANC level in chronic benign neutropenia of childhood?
<500
98
What are the endocrine effects of lead poisoning?
Decreased vitamin D metabolism with increased lead level
99
How is lead poisoning evaluated?
Venous blood lead level (BLL); routine screening recommended
100
What is considered an abnormal blood lead level (BLL)?
BLL ≥ 5
101
What additional screening is recommended if blood lead levels are elevated?
Screen for iron deficiency
102
What is the white blood cell count in TEC?
Normal to decreased
103
What is the platelet count in chronic ITP?
30,000 - 80,000/μl
104
What drugs are used during the induction phase of AML therapy?
High dose Ara-C, doxorubicin
105
What is the typical result of an antibody test in chronic benign neutropenia of childhood?
Positive
106
What percentage of acute ITP cases involve severe bleeding?
<10%
107
What is the platelet count in DBA?
Normal
108
What options are available during the consolidation phase of AML therapy?
More Ara-C or BMT if a match is available
109
What does the bone marrow show in chronic benign neutropenia of childhood?
Normal to increased myeloid series with a decrease at the band and/or seg level; some show earlier arrest
110
What is the platelet count in TEC?
Normal
111
Is there a maintenance phase in AML therapy for children?
No, it did not work
112
What percentage of chronic ITP cases involve severe bleeding?
Usually mild bleeding
113
Is the mean corpuscular volume (MCV) increased in DBA?
Yes
114
What is the incidence of cerebral hemorrhage in acute ITP?
<1%
115
What is the incidence of cerebral hemorrhage in chronic ITP?
3%
116
What imaging might be considered if symptomatic for lead poisoning?
Plain abdominal x-ray
117
What is the hemorrhagic death rate in acute ITP?
<1%
118
What is the mortality rate due to toxic death during AML chemotherapy?
7 – 9%
119
Is the mean corpuscular volume (MCV) increased in TEC?
No
120
Is hemoglobin F increased in DBA?
Yes
121
What is the hemorrhagic death rate in chronic ITP?
4%
122
What is the first step in managing lead poisoning?
End further exposure to lead
123
What is the second most common cancer in children?
Brain tumors
124
Is hemoglobin F increased in TEC?
No
125
What is the management approach for chronic benign neutropenia of childhood?
Supportive care; small risk for infection; G-CSF may be required in some patients with infections
126
What is the duration of acute ITP?
2-6 weeks
127
What nutritional measures can help manage lead poisoning?
Ensure adequate intake of calcium, vitamin C, iron and avoid fasting
128
What is the duration of chronic ITP?
Months to years
129
What is the most common type of solid neoplasm in children?
Brain tumors
130
What is the course of DBA?
Prolonged transfusion support or steroid therapy
131
What is the spontaneous remission rate in acute ITP?
83%
132
What therapy is used for significantly elevated lead levels?
Chelation therapy
133
What is the mechanism of cyclic neutropenia?
Apoptosis in precursors and cyclic hematopoiesis
134
What is the course of TEC?
Spontaneous recovery within weeks to months
135
What is the urgency of symptomatic lead intoxication?
It is a medical emergency
136
How many new brain tumor diagnoses are there annually in children?
3000-3500
137
What is the spontaneous remission rate in chronic ITP?
2%
138
What is the response rate to splenectomy in acute ITP?
71%
139
What is the 5-year survival rate for children with brain tumors?
60-70%
140
What are the two main types of leukocytes?
Granulocytes and mononuclear phagocytes
141
What is the response rate to splenectomy in chronic ITP?
66%
142
What are the inheritance patterns and genetic mutations associated with cyclic neutropenia?
Autosomal dominant and sporadic ELA-2 mutations
143
What are the clinical features of Horner's Syndrome in neuroblastoma?
Miosis, ptosis, anhidrosis
144
What are the three types of granulocytes?
Neutrophils, eosinophils, basophils
145
Where are brain tumors most commonly located in children?
Posterior fossa
146
What is the importance of National Cooperative Group Studies in pediatric oncology?
Higher cure rates for children on study
147
What was the cure rate for children with lymphoma on study in 1991?
76%
148
What are the clinical features of cyclic neutropenia?
Recurrent fever, pharyngitis, aphthous ulcers, periodontal disease
149
What is a common physical sign of neuroblastoma in children?
Racoon eyes and abdominal mass
150
What are the two types of mononuclear phagocytes?
Monocytes and lymphocytes
151
What is the ratio of infratentorial to supratentorial tumors in children?
2/3 infratentorial, 1/3 supratentorial
152
What was the cure rate for children with lymphoma not on study in 1991?
58%
153
What factors influence the prognosis of neuroblastoma?
Age, stage, n-myc amplification
154
What should you think of when considering infratentorial tumors?
Increased ICP
155
What is the typical cycle duration for cyclic neutropenia?
21 ± 3 days (range 14-28 days)
156
What was the cure rate for children with ALL on study in Delaware in 1983?
60%
157
What is the significance of spontaneous resolution in neuroblastoma?
It indicates a benign transformation.
158
What should be included in the history for neutrophil evaluation?
Symptoms, duration, number of infections, exposure to toxins/drugs, family history
159
What should you think of when considering supratentorial tumors?
Seizure
160
What are the common sites for Stage IVS neuroblastoma?
Skin, liver, bone marrow
161
What should be examined physically for neutrophil evaluation?
Infected sites, teeth and gums, lymph nodes, liver, spleen, other phenotypic findings
162
What was the cure rate for children with ALL not on study in Delaware in 1983?
19%
163
Name three common tumors of the posterior fossa in children.
Medulloblastoma, ependymoma, cerebellar astrocytoma
164
What is the cure rate for low-risk neuroblastoma?
95%
165
What is leukemia a disease of?
Bone marrow
166
How is cyclic neutropenia diagnosed?
Check CBC 2-3 times weekly for 6-8 weeks; ANC <200 for 3-5 days
167
What are general, non-localizing symptoms of brain tumors in children?
Headache, vomiting, behavioral changes, developmental delay, weight gain/loss
168
What percentage of leukemia patients present with hepatosplenomegaly?
60%
169
What percentage of leukemia patients present with fever?
55%
170
What is the cure rate for intermediate-risk neuroblastoma?
60%
171
What laboratory tests are important for neutrophil evaluation?
CBC + differential, peripheral smear, bone marrow aspirate, chemistries, anti-neutrophil antibodies
172
What percentage of leukemia patients present with malaise?
50%
173
What does the bone marrow show during neutropenia in cyclic neutropenia?
Myeloid hypoplasia, arrest at myelocyte level
174
What are the general classifications of neutropenia?
Marked decrease bone marrow reserve, normal marrow reserve
175
What is the cure rate for high-risk neuroblastoma?
15%
176
What percentage of leukemia patients present with bleeding?
42%
177
What is the management approach for cyclic neutropenia?
Aggressive antibiotic and supportive care for infections; G-CSF daily or alternate days if required
178
What are some localizing symptoms of brain tumors in children?
Cranial neuropathies, hemiparesis, hemisensory loss, ataxia, early handedness/change in handedness, seizures
179
What percentage of leukemia patients present with lymphadenopathy?
35%
180
How can spinal cord compression in neuroblastoma be treated?
Emergent chemotherapy
181
What is the increased risk associated with retinoblastoma loss of tumor suppressor gene?
Osteosarcoma, especially in the radiated field.
182
What percentage of leukemia patients present with bone/joint pain?
27%
183
What percentage of leukemia patients present with anorexia?
20%
184
What are primary disorders causing neutropenia?
Kostmann syndrome, Shwachman-Diamond syndrome, cyclic neutropenia
185
What is a major problem associated with opsoclonus-myoclonus ataxia syndrome in neuroblastoma?
Neurocognitive effects of autoimmunity
186
What are symptoms of increased ICP in children with brain tumors?
Headache, irritability, lethargy, vomiting, bulging fontanelle, separation of sutures, papilledema, Parinaud syndrome
187
What percentage of leukemia patients present with a mediastinal mass?
13%
188
What are secondary causes of neutropenia?
Chemotherapy, drug-induced, nutritional, viral infection
189
What are the two main types of platelet disorders?
Thrombocytopenia (quantitative) and thrombasthenia (qualitative)
190
What type of cancer is Wilms' tumor?
Embryonal cancer of the kidney
191
What is Parinaud syndrome?
Impaired upward gaze, convergence nystagmus, abnormal pupil responses
192
What percentage of leukemia patients present with abdominal pain?
10%
193
What is the definition of neutropenia?
Decrease in the absolute neutrophil count below accepted norms for age
194
What is a specific finding for platelet disorders?
Petechiae
195
What is the prevalence of Wilms' tumor?
1:10,000
196
What percentage of leukemia patients present with CNS disease?
5%
197
What are the types of tumors included in the Ewing family of tumors?
Ewing sarcoma of bone, extraosseous Ewing sarcoma, primitive neuroectodermal tumor, Askin tumor.
198
What percentage of leukemia patients present with testicular involvement?
1%
199
What is the primary site of Ewing sarcoma?
Pain, +/- palpable mass.
200
What are some complications of brain tumors in children?
Abdominal masses, Wilms’ tumor, neuroblastoma
201
How is the absolute neutrophil count (ANC) calculated?
ANC = # total WBC * (% PMNs + % bands)
202
What percentage of childhood cancers are kidney cancers, and what is the majority type?
7%, majority are Wilms' tumor
203
What is the most common extracranial solid tumor in children?
Neuroblastoma
204
What is the importance of personal and family history in platelet disorders?
Careful personal and family history of abnormal bleeding is very important
205
What is a common presentation for children with leukemia?
Persistent limp with anemia and thrombocytopenia
206
What is the ANC threshold for term newborns?
<3,000
207
What is the male to female ratio for Wilms' tumor?
1:1.1
208
What is the prevalence of neuroblastoma in children?
1:7000 births
209
Which areas are more frequently involved in Ewing sarcoma compared to osteosarcoma?
Diaphyses and central axis.
210
What percentage of leukemia patients have a platelet count <10,000?
11%
211
What percentage of leukemia patients have a platelet count between 10,000 and 49,000?
38%
212
What is the ANC threshold for infants aged 1 week to 2 years?
<1,100
213
What is the median age at presentation for unilateral Wilms' tumors?
44 months
214
What percentage of leukemia patients have a platelet count between 50,000 and 100,000?
21%
215
What is the incidence of immune thrombocytopenia (ITP) in children?
4.8 ITP cases per 100,000 children per year
216
What percentage of all childhood cancers does neuroblastoma represent?
8-10%
217
What percentage of leukemia patients have a platelet count >100,000?
30%
218
What is the median age at presentation for bilateral Wilms' tumors?
31 months
219
What is the ANC threshold for children, adolescents, and adults?
<1,500
220
What percentage of leukemia patients have hemoglobin <7 g/dL?
40%
221
What are the most common primary sites for Ewing sarcoma?
Pelvis (23%), femur (18%), rib (13%).
222
What percentage of leukemia patients have hemoglobin between 7 and 9 g/dL?
28%
223
Is neuroblastoma more common in boys or girls?
Boys (1.1:1.0)
224
What is the infectious risk for mild neutropenia?
None
225
What is the typical presentation of ITP in children?
Acute onset of thrombocytopenia in an otherwise healthy child; most present with skin findings only
226
What percentage of Wilms' tumor cases are familial?
1-2%
227
What percentage of leukemia patients have hemoglobin between 9 and 11 g/dL?
17%
228
What systemic symptoms are associated with Ewing sarcoma?
Fever, weight loss.
229
What is the median age at diagnosis for neuroblastoma?
19 months
230
What is the nature of bleeding in ITP?
Mucosal in nature
231
What percentage of leukemia patients have hemoglobin >11 g/dL?
15%
232
What is the infectious risk for moderate neutropenia?
Minimal
233
What is the most common presentation of Wilms' tumor?
Painless mass or abdominal swelling
234
What is the typical platelet count in ITP?
Usually <20,000/mm³
235
What percentage of leukemia patients have a WBC count <25,000?
64%
236
What percentage of neuroblastoma cases are familial?
1%
237
What is the infectious risk for severe neutropenia?
Moderate to severe
238
What percentage of leukemia patients have a WBC count between 25,000 and 50,000?
12%
239
What is the most common metastatic site for Wilms' tumor?
Lung
240
What is seen on a blood smear in ITP?
Large platelets
241
What percentage of leukemia patients have a WBC count between 50,000 and 99,000?
10%
242
What percentage of neuroblastoma cases are sporadic?
99%
243
What percentage of leukemia patients have a WBC count >100,000?
14%
244
What is the classic presentation of leukemia in terms of blood cell counts?
Pancytopenia
245
How are tumor genomics useful in neuroblastoma?
Prognostication (e.g., MYCN)
246
What defines non-high risk neuroblastoma cases?
Tumor biology
247
What is the overall survival rate for Wilms' tumor with surgical staging and multimodality treatment?
Exceeding 90%
248
What are the typical lab findings in ITP?
Normal hemoglobin, hematocrit, and MCV; may have anemia if significant bleeding; normal WBC count and differential
249
What is Tumor Lysis Syndrome (TLS)?
Oncologic emergency with increased uric acid, phosphorus, and potassium
250
What is the pattern of spread for Ewing sarcoma?
20% metastatic: isolated lung (35%), isolated bone (13%), isolated bone marrow (7%).
251
Is there any known association of Ewing sarcoma with radiation or syndromes?
No.
252
What imaging techniques are used in the staging workup for Ewing sarcoma?
Plain films, MRI or CT of primary site, CT chest/CXR, bone scan.
253
What causes Tumor Lysis Syndrome (TLS)?
Inability to handle byproducts of leukemia cell death
254
What is the most important prognostic factor for Wilms' tumor?
Anaplastic histology
255
When is a bone marrow biopsy indicated in ITP?
Atypical features (e.g., anemia, MCV, neutropenia, hepatosplenomegaly, etc.), constitutional symptoms
256
When can Tumor Lysis Syndrome (TLS) occur?
Spontaneously or after therapy is started
257
What is the treatment approach for non-high risk neuroblastoma cases?
Surgery +/- low dose chemotherapy
258
Which leukemias are most problematic for Tumor Lysis Syndrome (TLS)?
Leukemias with high growth fraction and/or high tumor burden
259
What is the diagnostic platelet count for ITP?
<100,000/mm³
260
What is the purpose of bone marrow aspirate/biopsy in Ewing sarcoma staging?
To check for bone marrow involvement.
261
What is the effectiveness of high-risk treatment for neuroblastoma?
Effective in only 50% with significant morbidity
262
What are the clinical presentations of Wilms' tumor?
Painless abdominal swelling or mass, palpable fixed non-tender flank mass, abdominal pain, gross hematuria, fever
263
What are the different durations of ITP?
Newly diagnosed: diagnosis-3 months; Persistent ITP: 3-12 months; Chronic ITP: >12 months
264
How is Ewing sarcoma stage assignment categorized?
Localized vs. metastatic.
265
What are the clinical presentations of neuroblastoma?
Horner’s Syndrome, “Racoon Eyes,” abdominal mass
266
What percentage of Wilms' tumor patients experience hypertension?
25%
267
What indicates disease resolution in ITP?
Platelet count >100,000/mm³
268
What are the symptoms of Horner’s Syndrome?
Miosis, ptosis, anhidrosis
269
What can sub-capsular hemorrhage in Wilms' tumor lead to?
Rapid abdominal enlargement, anemia, hypertension, fever
270
What are the associated syndromes with Wilms' tumor?
Rare
271
What genetic translocation is pathognomonic for Ewing sarcoma?
t(11;22) leading to FLI-1/EWS fusion protein.
272
What does RFS stand for in Wilms' tumor outcomes?
Relapse free survival
273
Name two specific types of leukemia with high risk for Tumor Lysis Syndrome (TLS).
Burkitt’s, T-ALL
274
What are the main risks associated with Tumor Lysis Syndrome (TLS)?
Renal dysfunction/failure and metabolic complications
275
How can Tumor Lysis Syndrome (TLS) be prevented and treated?
Hydration, alkalinization of urine, Allopurinol or rasburicase
276
What are the two main types of leukemia in children?
ALL (Acute Lymphocytic Leukemia), AML (Acute Myelogenous Leukemia)
277
What percentage of childhood leukemia cases are ALL?
80%
278
What is the function of the FLI-1 protein in Ewing sarcoma?
DNA binding domain.
279
What percentage of childhood leukemia cases are AML?
15%
280
What percentage of childhood leukemia cases are CML and JMML?
5%
281
What is the most common pediatric malignancy?
ALL (Acute Lymphocytic Leukemia)
282
What is the natural history of ITP in terms of hemorrhage and platelet count?
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
What percentage of total pediatric malignancies does ALL represent?
25%
284
What does OS stand for in Wilms' tumor outcomes?
Overall survival
285
What is the function of the EWS protein in Ewing sarcoma?
Transcription activation.
286
How many cases of ALL are diagnosed per year in the US?
3,000 cases/year
287
What are the two main types of bone tumors mentioned?
Ewing's sarcoma, osteosarcoma
288
What percentage of Ewing sarcoma cases have the t(21;22) translocation?
5%.
289
What factors influence the choice of treatment for ITP?
Bleeding symptoms, family comfort level, assurance regarding follow-up, and less so on platelet count
290
What is the characteristic radiographic appearance of Ewing sarcoma?
Onion skin.
291
What are the cell types involved in Ewing's sarcoma and osteosarcoma?
Small round blue cells, spindle cells, new bone formation
292
What is the incidence rate of ALL in children?
3 cases/100,000 children
293
What is the usual time to response for observation and education in ITP management?
1-3 weeks
294
What is the usual time to response for corticosteroids in ITP management?
3-4 days
295
What is the peak age range for ALL?
2-5 years
296
Which racial group has a higher incidence of ALL?
White
297
What is the typical clinical presentation of osteosarcoma?
Pain +/- mass at primary site
298
Which gender has a higher incidence of ALL?
Boys
299
Where does osteosarcoma typically involve?
Metaphyses of long bones
300
What is the usual time to response for IVIG in ITP management?
24-48 hours
301
What are the most common primary sites for osteosarcoma?
Distal femur, proximal tibia, proximal humerus
302
How does socioeconomic status affect the incidence of ALL?
Higher SEC > Lower SEC
303
What are skip lesions in osteosarcoma?
Lesions occurring several cm from primary site
304
What is the usual time to response for Anti-D immunoglobulin in ITP management?
24-48 hours
305
What is a common cause of ALL?
Typically unknown
306
What is the hematogenous spread pattern in osteosarcoma?
Lung, bone
307
Name a genetic syndrome that increases the risk for ALL.
Down Syndrome (Trisomy 21)
308
What percentage of ALL cases are B Cell?
80%
309
What percentage of osteosarcoma cases are caused by radiation?
3%
310
What percentage of ALL cases are T Cell?
15%
311
What is the mechanism of action for corticosteroids in ITP management?
Reticuloendothelial blockade and reduced synthesis of anti-platelet antibodies
312
What are the side effects of corticosteroids in ITP management?
Moodiness, weight gain, irritability, bone pain, hypertension, hyperglycemia
313
What is the median time for osteosarcoma to occur following radiation exposure?
9-11 years
314
What is the mechanism of action for IVIG in ITP management?
Reticuloendothelial blockade
315
What percentage of ALL cases are Mature B Cell (Burkitt’s-Type)?
2%
316
What imaging is used in the staging workup for osteosarcoma?
Plain films, MRI, CT chest/CXR, bone scan
317
What percentage of ALL cases are Indeterminant?
3%
318
How is ALL diagnosed?
Characteristic morphology and diagnostic immunophenotype of cells (lymphoblasts) from peripheral blood or bone marrow
319
What diagnostic techniques are used for ALL?
Flow cytometry, immunohistochemistry
320
How is osteosarcoma staged?
Localized vs. metastatic, resectable vs. unresectable
321
What are the side effects of IVIG in ITP management?
Aseptic meningitis, headache, infusion fever and chills
322
What is a notable radiographic feature of osteosarcoma?
Lytic or permeative, proximity to growth plates, starburst new bone formation
323
What is the mechanism of action for Anti-D immunoglobulin in ITP management?
Sensitized erythrocytes occupy the Fc receptors; use only in Rh+ non-splenectomized patients
324
What is the primary treatment for osteosarcoma?
Surgery
325
What is the role of chemotherapy in osteosarcoma treatment?
Used in combination with surgery, better outcomes
326
What are the main components of induction therapy for ALL?
Steroids, Vincristine, L-Asparaginase
327
How is treatment response evaluated in osteosarcoma?
Pre-op clinical response, pathologic response (necrosis assessment)
328
What additional drugs are used for higher risk ALL during induction?
Doxorubicin or Daunomycin
329
What are the side effects of Anti-D immunoglobulin in ITP management?
Hemolytic anemia (2 gm decline), DIC, fever and chills
330
What drugs complement induction therapy for ALL?
Ara-C, Cyclophosphamide
331
When is spinal/cranial irradiation used in ALL treatment?
If CNS (+)
332
What is the duration of maintenance therapy for ALL?
~2 years
333
What drugs are used in maintenance therapy for ALL?
6-MP, Methotrexate
334
What is the management approach for persistent/chronic ITP?
Repeat use of acute medications or observation; splenectomy; rituximab; additional immunosuppressant agents; thrombopoietic agents
335
What is a good WBC count at presentation for ALL prognosis?
<50,000
336
What is a good age range for ALL prognosis?
1-9 years
337
What type of chromosome abnormalities are considered good for ALL prognosis?
Good and Bad
338
What is a good response to treatment for ALL prognosis?
Remission by Day 14
339
What is a worse factor for ALL prognosis?
CNS Disease
340
What is the response rate to splenectomy in ITP management?
60-80%
341
Which cell type is better for ALL prognosis?
B cell
342
What is the survival rate for AML in children?
50%
343
What is the therapy approach for AML in children?
Very short and very aggressive
344
What is the risk associated with splenectomy in ITP management?
Post-splenectomy sepsis
345
What are the main components of induction therapy for AML?
High dose Ara-C, doxorubicin
346
What is the response rate to rituximab in ITP management?
30% complete response
347
What are some additional immunosuppressant agents used in ITP management?
Mycophenolate mofetil, 6-mercaptopurine
348
What are the main components of consolidation therapy for AML?
More Ara-C or BMT if match available
349
Is there maintenance therapy for AML?
None – did not work
350
What percentage of children with AML die a toxic death during chemotherapy?
7-9%
351
What do thrombopoietic agents do in ITP management?
Stimulate bone marrow production of platelets
352
What are some examples of thrombopoietic agents used in ITP management?
Eltrombopag (Promacta), Romiplostim (NPlate), Avatrombopag (Doptelet)
353
What is a Syk inhibitor used in adult ITP management?
Fostamatinib (Tavalisse)
