HemeOnc Flashcards
(499 cards)
1
Q
When is a physiological drop in H&H expected by?
A
2-3 month of life (8-10 weeks) in term infants
2
Q
How low of a Hgb can be normal for a term infant who is 8-10 weeks?
A
9
3
Q
What is done for asymptomatic physiologic anemia of infancy?
A
No further laboratory evaluation
4
Q
In a premature infant, when can the physiologic nadir of H&H happen?
A
1-2 months (7-8 weeks)
5
Q
What is the etiology of physiological anemia in newborns?
A
Low erythropoietin production
6
Q
What is the equation for normal MCV?
A
Roughly 70 + 2(age in years)
7
Q
What is the first thing you should do with a question concerning anemias?
A
Figure out whether it is micro-, macro-, or normocytic
8
Q
What is normal MCV for children?
A
70-90 (adults is 80-100)
9
Q
What is the MCV in a microcytic anemia?
A
Under 70
10
Q
What are the 3 main considerations for a microcytic anemia?
A
- Thalassemia
- Too little iron
- Too much lead
11
Q
What are the 3 usual hemoglobins?
A
- F (2 alpha/2 gamma)
- A (2 alpha/2 beta)
- A2 (2 alpha/2 delta)
12
Q
What are thalassemias caused by?
A
Defects in genes that code for hemoglobin chains- proper quantities aren’t produced
13
Q
What are hemoglobinopathies caused by?
A
Errors in sequencing of hemoglobin chains (quality)
14
Q
Which patients will eventually get iron overload?
A
Anyone undergoing regular transfusion therapy (thalassemia, sickle cell, aplastic anemia)
15
Q
What is done to remove excess iron in the body?
A
Chelation therapy
16
Q
What happens if iron builds up in the body?
A
Toxic buildup in liver (hemosiderosis) and hearts
17
Q
What is a significant cause of morbidity and mortality in chronically transfused patients?
A
Toxic buildup of iron in the liver and heart
18
Q
Where are the 2 alpha globin genes located?
A
Chromosome 16
4 alleles (aa/aa)
19
Q
What causes alpha thalassemia?
A
Mutations in one to all 4 of the alpha globin alleles
20
Q
What happens if only one or two alpha alleles (alpha globin gene) are defective?
A
Children are typically asymptomatic and it is picked up on routine screening
21
Q
What do labs show for kids with 1 or 2 defective alpha alleles (alpha globin gene)?
A
Microcytic hypochromic anemia with normal iron and lead studies
22
Q
What is it called when one alpha allele is defective?
A
“Silent trait”
23
Q
What is it called when two alpha alleles are defective?
A
“Alpha thalassemia trait”
24
Q
How is alpha thalassemia diagnosed?
A
Hemoglobin electrophoresis
25
If hemoglobin electrophoresis is done in the neonatal period, what does the electrophoresis show?
Hemoglobin Bart (gamma chains banding togther)
26
For alpha thalassemia with silent trait or alpha thalassemia trait, what is the treatment?
No treatment
27
What happens when there are 3 defective alpha globin genes?
Hemoglobin H disease
28
What does Hemoglobin H disease lead to?
Hemolysis and hepatomegaly
29
What can Hemoglobin H disease be treated with?
1. Transfusions
| 2. Usually a splenectomy
30
What does 4 defective alpha globin genes cause?
Hydrops fetalis
31
What does hydrops fetalis result in?
Still birth or death soon after birth
32
If a hydrops fetalis survives, what do they require?
Transfusions for life
33
How are thalassemias diagnosed?
Hemoglobin electrophoresis
34
What is elevated in thalassemias?
Hemoglobin F
35
What is caused by a defect in one of the beta globin gene alleles?
Thalassemia minor or thalassemia trait
36
True or False: Patients with thalassemia minor or thalassemia trait are asymptomatic?
True
37
How is thalassemia minor or thalassemia trait diagnosed?
When a mild microcytic anemia is noted on incidental testing
38
What results from severe deficiency of beta globin due to a mutation in both beta globin gene alleles?
Thalassemia Major/Cooley's anemia
-Can't make hemoglobin A1, so body makes A2 (delta) or F (gamma)
39
Small for age Greek child with anemia and an enlarged liver/spleen?
Thalassemia major
40
How do patients with thalassemia major present?
- 1st year of life
| - Profound microcytic, hypochromic anemia
41
What physical exam findings is seen in Thalassemia major?
Hepatosplenomegaly
42
What is seen on XR in Thalassemia major?
1. Thickened bone
| 2. Skull has "hair-on-end" appearance
43
What causes the "hair-on-end" appearance in skull XR for thalassemia major?
Extramedullary hematopoiesis
44
What is the treatment for Thalassemia major?
Chronic transfusion therapy
45
What are the 2 long term complications with beta thalassemia major?
1. Cholelithiasis
| 2. Hemosiderosis (iron deposition in heart, liver, pancreas)
46
What are the findings for sickle cell anemia on hemoglobin electrophoresis?
Hemoglobin F
| Hemoglobin S
47
What are the findings for beta thalassemia on hemoglobin electrophoresis?
Low/No Hemoglobin A1
Elevated hemoglobin A2
Hemoglobin F
48
10 week old infant of Mediterranean descent with hemoglobin of 9.0 and MCV of 89... diagnosis?
Physiologic anemia of infancy... this is a normocytic anemia, don't be fooled by ethnicity of Mediterranean
49
When does the risk for iron deficiency anemia in infants begin?
After 4 months
50
Who is the risk for iron deficiency anemia higher in, breast fed or formula fed infants?
Breast fed
51
When does the risk for iron deficiency anemia happen in preterm infants?
Earlier than 4 months (as in term infants) especially with no iron supplementation
52
Why are infants who are given cow's milk (versus formula or breast milk) before 12 months of age at increased risk for iron deficiency anemia?
Because of low amount of iron absorbed
53
True or False: Iron deficiency anemia is typically completely asymptomatic?
True
54
18 month old with pallor and tachycardia?
Iron deficiency anemia
55
Low hemoglobin (<6) and low MCV (<60)?
Iron deficiency anemia
56
Child with a history of drinking lots and lots and lots of cows milk (probably still on the bottle)?
Iron deficiency anemia
57
What else does low iron cause besides anemia?
Mild delays in cognitive development (even without frank anemia)
58
You find a microcytic anemia in a kid drinking too much milk, what do you do?
Trial of iron
59
If anemia improves after a one month trial of iron, what do you do?
Continue the iron treatment (it was probably iron deficiency anemia)
60
If anemia doesn't improve after a one month trial of iron, what do you do?
Investigate for other causes of microcytic anemia (lead toxicity or thalassemia) or of chronic iron deficiency (celiac)
61
How is iron deficiency anemia treated?
Ferrous sulfate (until 2 months after Hgb levels normalized- replenishes iron stores), improve diet (limit milk), get them off the bottle
62
When is parenteral iron administration given?
Rarely- only if cannot take PO iron or in extreme social circumstances
63
What is the most common environmental illness in kids?
Lead poisoning
64
What does lead poisoning affect?
Production of heme (interferes with a variety of enzyme systems)
65
Microcytic anemia with a high RDW?
1. Iron deficiency anemia
| 2. Lead toxicity
66
Microcytic anemia with a normal RDW?
Thalassemia
67
Microcytic anemia with a low RDW?
Nothing
68
Choice of 2 microcytic anemias, one with lower RDW likely due to?
Thalassemia (vs. Iron deficiency)
69
When is FEP (free erythrocyte protoporphyrin) elevated?
1. Lead poisoning
| 2. Iron deficiency anemia
70
When is FEP (free erythrocyte protoporphyrin) normal?
Thalassemia
71
What typically coexists with lead poisoning and enhances lead absorption?
Iron deficiency
72
What is the gold standard test for lead poisoning?
Whole blood lead level (not fingerstick)
73
What is an immature RBC with iron-bloated mitochondria surrounding the nucleus?
Ringed sideroblast
74
What is a ringed sideroblast evidence of?
1. Lead poisoning
| 2. Sideoblastic anemia
75
What is the MCV in macrocytic anemia?
>100 (often around 110)
76
What is B12 frequently due to?
Poor absorption of B12 (often from GI disorder like Crohn's disease)
77
Name 4 things B12 deficiency can be due to?
1. Intrinsic factor deficiency (pernicious anemia)
2. Bacterial overgrowth
3. Following a bowel resection
4. Vegetarian diet
78
Compromised gut + Heme question?
B12 deficiency
79
3 B's of B12 deficiency?
B12 Deficiency:
- Bacterial overgrowth
- Bowel resection
- HLS-B27 (Crohn's)
80
Goat's milk?
Macrocytic anemia from folic acid deficiency
81
What 2 deficiencies often coexist?
B12 and folate
82
What happens if you treat someone with a folate deficiency, but don't correct the B12?
CBC will look better, but this can lead to irreversible neurologic damage
83
What 3 things should you think of with a normocytic anemia?
1. Patient is bleeding
2. Patient has chronic disease
3. Hemolytic process
84
What are 3 reasons that a red cell might be destroyed?
1. Enzyme problems (G6PD)
2. Structural problem (HS)
3. Weird shape (Sickle Cell)
85
When a red cell breaks open, where do you find the remnants and in what form?
Urine, hemosiderin/bilirubin
86
Is serum haptoglobin low or high in hemolytic anemia?
Low- Used up in transporting released hemoglobin from RBCs
87
What results in an elecated retic count?
Hemolytic anemia
88
What is a normal retic count?
1%
89
What is the one exception of a hemolytic anemia not causing an elevated retic count?
Parvovirus (if it causes aplastic crisis in someone with a chronic hemolytic anemia- Sickle cell or Hereditary spherocytosis)
90
What is a sign that a hemolytic process in immune-mediated?
Positive Coombs test
91
What does the direct Coombs test identify?
Antibodies on the surface of the red cell
92
What protects a red cell from oxidative stress (which can lead to hemolysis)?
G6PD
93
What are small, purple granules in the red cell that form as a result of damage to the hemoglobin molecule?
Heinz bodies
94
What condition do you see Heinz bodies in?
G6PD
95
African American or Mediterranean boy with dark urine who is jaundiced and anemic
G6PD
96
Sudden onset of pallor and anemia in an otherwise healthy child?
G6PD
97
What are 5 things that can cause oxidative stress leading to issues in someone with G6PD?
1. Sulfa-containing antibiotic (Bactrim)
2. Nitrofurantoin
3. Moth Balls
4. Antimalarial meds
5. Fava beans
98
True or False: Hepatomegaly is seen in G6PD
False: There is NO hepatomegaly or splenomegaly in G6PD
99
How is G6PD inherited?
X-linked
100
When should testing for G6PD deficiency be done?
Several weeks after an episode
-Testing right after or during an episode isn't reliable because reticulocytes have a large amount of G6PD and can lead to false negative test
101
If you have a negative test for G6PD, does it rule this out?
Not necessarily. If the negative test was right after an episode, it could be a false negative test.
102
What is treatment for G6PD?
1. Hemodynamic support (Fluid +/- RBCs)
| 2. Remove the oxidant stressor
103
What might newborns with G6PD require?
Phototherapy and/or exchange transfusion (to help resolve the indirect hyperbilirubinemia)
104
What is the most common inherited cause of hemolytic anemia?
Hereditary Spherocytosis
105
What is caused by defects in the surface of red cells resulting in loss of erythrocyte surface area?
Hereditary Spherocytosis
106
How is hereditary spherocytosis inherited?
Autosomal dominant (most common... an autosomal recessive form can also occur)
107
What is the main feature of hereditary spherocytosis?
Chronic hemolysis
108
How does hereditary spherocytosis present?
1. Mild/Mod anemia
2. Splenomegaly
3. Intermittent jaundice
4. Gallstones
109
Is the mean corpuscular hemoglobin concentration (MCHC) decreased or increased in Hereditary Spherocytosis?
Increased- More hemoglobin per volume (smaller cell with same amount of Hgb)
110
How is hereditary spherocytosis diagnosed?
Osmotic fragility test- Cells break open more easily
111
What results from a spectrin deficiency or spectrin mutation?
Hereditary Spherocytosis
Ankyrin deficiency can also case HS, but spectrin is better choice
112
In hereditary spherocytosis when can you monitor until recovery?
If hemolysis isn't severe (Hgb >5-6)
113
What can be curative for Hereditary Spherocytosis?
Splenectomy
114
What is the most common cause of aplastic crisis in children with hereditary spherocytosis?
Parvovirus B19
115
What is the most common reason for children with hereditary spherocytosis to require a blood transfusion?
Aplastic crisis due to Parvovirus B19
116
Are defects of structure typically dominant or recessive?
Dominant
117
Are enzyme defects typically dominant or recessive?
Recessive
118
What is the mutation in Sickle Cell?
Substitution at amino acid 6 of the beta globin chain (valine is substituted for a glutamic acid)
119
Which type of anemia is a result of an amino acid substitution?
Sickle Cell
120
When does sickle cell anemia typically present clinically?
Not until after 6 months of age
121
When are children typically diagnosed with sickle cell disease?
When their newborn screens come back abnormal
122
How does vasoocclusive crisis present?
Acute pain secondary to ischemia and infarction
123
How is a vasoocculsive crisis treated?
Rehydration and pain control
124
What is dactylitis?
Pain and swelling of the hands and feet, typically seen in infants with sickle cell
125
How is dactylitis treated?
Aggressive pain control
126
How does sequestration crisis present?
Shock
127
What causes a sequestration crisis?
Pooling of blood in the liver and spleen- usually in response to an infection
128
True or False: Sequestration is considered a medical emergency?
True- Requires hospitalization and transfusion
129
When do you typically do a splenectomy in a sickle cell patient?
After the 2nd or 3rd sequestration episode
130
What typically causes aplastic crisis in sickle cell patients?
Parvovirus infection (people with sickle cell or HS need high retic counts to keep up with increased red cell destruction...if this isn't working, Hgb decreases)
131
How are aplastic crises treated?
Blood transfusions as needed
132
What should make you think of a hyperhemolytic crisis?
History of sickle cell with a recent infection
133
What often precedes an aplastic crisis or sequestration crisis?
Hyperhemolytic crisis
134
When do you see priapism?
Sickle cell
135
True or False: Antibiotic prophylaxis with penicillin is recommended for all children with sickle cell disease through age 5?
True
136
What extra vaccination should children with sickle cell disease receive?
Pneumococcal
137
Why do older children with sickle cell get antibiotics and a septic workup for fevers?
Because they are considered to be asplenic
138
What does the spleen remove from red cells?
Howell-Jolly bodies (you will see these nuclear particles once asplenic)
139
Howell-Jolly Bodies?
Sickle Cell
140
Heinz Bodies?
G6PD
141
True or False: Most states screen newborns for sickle cell disease?
True
Consider if child newly emigrated from developing country
142
By what age do children with sickle cell have functional asplenia?
5
143
What is typical of the abdominal exam for a child with sickle cell after age 5?
Small, hard, non-palpable spleen
144
If you have an older child with a palpable spleen, what should you think?
Either hemoglobin SC or sickle trait and thalassemia together (Hemoglobin-S, Beta-thal)
145
Children with sickle cell and thalassemia are resistant to what?
Malaria (thought how these two genes remained in gene pool)
146
What are the 3 features of acute chest?
1. Chest pain
2. Infiltrate on CXR
3. Hypoxia
147
Sickle cell patient with chest pain, infiltrate on CXR, and hypoxia, what do you do next?
Get an ABG to confirm the hypoxia (O2 sats usually not good enough for diagnosis)
148
What can resolve chronic hemolysis in patients with hemolytic anemias?
Partial or total splenectomy
149
What must you do a few weeks before a splenectomy in a kid with a hemolytic anemia?
Vaccinate against H. Flu, S. Pneumo, and N. Meningitidis
150
What 3 bacteria are post-splenectomy patients at risk for after surgery?
1. H. Flu
2. S. Pneumo
3. N. Meningitidis
151
True or False: Children with splenectomies should have antibiotic prophylaxis until adolescence?
True- this should be a consideration
152
If you have a post-splenectomy patient with fever, what do you do?
CBC, blood cultures, IV ceftriaxone
153
What is the treatment for ACS?
Transfusion
154
When transfusing someone for ACS, what do you have to consider?
Starting Hct... if it is low, simple transfusion is fine. If it is high, they may require an exchange transfusion
155
Which two problems is chest pain in a child with sickle cell difficult to distinguish between?
Pneumonia v. Pulmonary infarct
156
If you have a child with sickle cell disease who presents with fever, cough, and chest pain, what do you treat for?
Both pneumonia and pulmonary infarct (hard to tell difference between two)
157
African American child with stroke symptoms, what do you do?
Transfusion then MRI (treat before diagnose)
158
Child with symptoms of stroke, new to US, how do you diagnose the underlying disorder?
Hemoglobin electrophoresis (sickle cell is underlying disorder)
How to diagnose the problem would be MRI
159
What type of hematologic disease is cholelithiasis associated with?
Sickle cell disease
160
Child with sickle cell disease presents with right upper quadrant tenderness- what do you do?
Abdominal US (cholelithiasis)
161
What are anemias due to suppression of the red cell line in the bone marrow?
Hypoplastic and aplastic anemias
162
What can aplastic anemia present similarly to?
Acute leukemia
163
What are some features of acute leukemia that help to differentiate it from aplastic anemia?
1. Hepatosplenomegaly
2. Lymphadenopathy
3. Depression of WBC or Plt counts
4. High LDH
5. High uric acid
6. Fevers
7. Bone pain
8. Blasts
164
How is diagnosis for either aplastic anemia or acute leukemia confirmed?
Bone marrow biopsy
165
What drug is responsible for an aplastic crisis?
Chloramphenicol
166
What type of anemia is Fanconi's anemia?
Aplastic anemia
167
When does Fanconi's anemia present?
Later in childhood (usually after age 3)
168
Does Fanconi's anemia present with macrocytic or microcytic anemia?
Macrocytic
169
What type of hemoglobin is elevated in Fanconi's anemia?
Fetal hemoglobin
170
Name 4 physical characteristics associated with Fanconi's anemia?
1. Abnormal skin pigmentation
2. Growth retardation (short stature)
3. Renal abnormalities (urinary retention)
4. Absent or hypoplastic thumb
171
What type of anemias are Fanconi and Diamond-Blackfan?
Macrocytic
172
How is Fanconi's anemia inherited?
Recessive
173
Which type of anemia puts children at risk for transformation to AML or myelodyslpastic syndrome?
Fanconi's
174
What will patients with Fanconi's anemia require?
Transfusion of RBCs and platelets
175
What is the cure for Fanconi's anemia?
Bone marrow transplant
176
What can lead to a prolonged remission in Fanconi's anemia?
1. Immunosuppressants
| 2. Androgens (less common)
177
Which two conditions are profound isolated red cell anemias that present in infancy/toddlerhood?
Diamond-Blackfan Anemia
| Transient Erythroblastopenia of Childhood
178
What anemia is due to an arrest in the maturation of red cells?
Diamond-Blackfan Anemia
179
What anemia is a consequence of a suppression of erythroid production?
Transient Eryhtroblastopenia of Childhood
180
What are shared features of DBA and TEC?
1. Low Hgb and retic count
2. Gradual onsent
3. Relatively asymptomatic
181
Which often presents with anemia at birth, DBA or TEC?
DBA
182
Which 2 anemias affect the red cell line exclusively?
1. TEC
| 2. DBA
183
If you see a 2 or 3 year old with an anemia exclusively affecting the red cell line, what is it?
Probably TEC (will not be DBA because this affects infants)
184
What is the difference in time-course between DBA and TEC?
DBA is chronic
| TEC is transient
185
Kid with severe anemia who has thumb abnormalities, urogenital defects, or craniofacial problems?
DBA
186
Mnemonic for DBA
DBA:
Dysmorphic facies
Babies
Anemia
187
What is the median age of onset for TEC?
18-26 months (T for Toddler)
188
What is median age of onset for DBA?
2-3 months (BA for baby)
189
Which type of anemia, TEC or DBA, almost always has a spontaneous recovery?
TEC (DBA rarely has a spontaneous recovery)
190
Which type of anemia, TEC or DBA, is more likely to have dysmorphology?
DBA (uncommon in TEC)
191
What type of anemia, TEC or DBA is less likely to require transfusions?
TEC (Transfusions are common in DBA)
192
Which type of anemia, DBA or TEC, are steroids helpful?
DBA (Steroids aren't indicated for TEC)
193
Which has a higher incidence, DBA or TEC?
TEC is common (DBA is extremely rare)
194
Child with severe anemia (Hgb 3-5) who has a reticulocytosis (retic count 5-10%), recent viral illness... what is the cause?
Probably TEC (now in recovery phase)
195
What is the typical course for TEC?
Usually resolves spontaneously within several months...transfusion is rarely needed.
196
True or False: Steroids are used in TEC
False: Steroids are used with DBA
197
What 3 things are blood donations typically tested for?
1. Hep B
2. Hep C
3. HIV
198
What is the MCV in DBA versus TEC?
- DBA is macrocytic
| - TEC usually normal (unless patient is recovering with a reticulocytosis and MCV may be slightly elevated)
199
If you get a question with dark urine or a positive Coombs test (DAT), what type of anemia should you think?
Hemolytic anemia (look for signs of hemolysis)
200
What type of transfusion reaction presents as fever and chills?
Febrile Non-hemolytic reactions
201
How is the risk of febrile nonhemolytic reactions reduced?
By using leukocyte-filtered blood
202
True or False: Pre-medicating with anytipyretics prevents Febrile Non-hemolytic reactions?
False
203
What type of reaction occurs with transfused blood isn't properly cross-matched?
Hemolytic reactions
204
How do allergic reactions to blood products typically present?
Urticaria
205
What should you do if a kid has an allergic reaction to a blood transfusion?
Stop the transfusion and give an anti-histamine
206
True or False: When a patient has an allergic reaction to a blood transfusion, you can resume it if the urticaria goes away quickly after pausing and giving anti-histamine?
True
207
What is the definition of neutropenia?
Absolute neutrophil count (AND) of less than 1000 during first year of life and less than 1500 after 1st year of life
208
What is mild neutropenia?
ANC between 1000 and 1500
209
What is moderate neutropenia?
ANC between 500-1000
210
What is severe neutropenia?
ANC under 500
211
What are some classic presentations of neutropenia?
1. Recurrent mucosal ulceration (mouth to perirectal)
2. Gingivitis
3. Cellulitis
4. Abscess Formation
5. Pneumonia
6. Septicemia
212
What are some endogenous organisms that kids with neutropenia are most commonly infected with?
1. S. Aureus
2. S. Epi
3. Gram-negatives
4. Enterococci
213
What should recurrent mucosal ulcerations make you think of?
Neutropenia
214
Cyclic neutropenia, severe congenital neutropenia (Kostmann syndrome), chronic benign neutropenia, Shwachman-Diamond are all types of what?
Congenital neutropenias
215
What is the most common cause of acquired neutropenia?
Infection
216
What specific group of drugs are known to cause neutropenia?
Macrolides (but lots of drugs can do it)
217
What should you think of for a kid that is neutropenic after a cold?
Transient neutropenia (viral suppression)
218
How long does transient neutropenia typically last?
Couple of days
219
What treatment is needed for transient neutropenia?
None- No treatment or monitoring is needed
220
How is ANC calculated?
Multiplying the WBC by the % of segs plus bands
221
What needs to be done for a patient known to have neutropenia (from any cause) if they get a fever?
Blood culture and antibiotics
222
What should you do for a kid with an ANC <500 and a fever?
Admit to hospital and start IV antibiotics
223
Previously well child with febrile illness, no heptaosplenomegaly, no lymphadenopathy, WBC is 3.5, what is next step in management?
Repeat WBC in a few weeks- Transient neutropenia due to viral illness
224
How is cyclic neutropenia inherited?
Autosomal dominant
225
What is the timing for cyclic neutropenia?
Low WBC lasts around a week and reappears every month or so
226
What other findings are seen with cyclic neutropenia?
1. Oral lesions (common)
| 2. Enlarged lymph nodes
227
What age does cyclic neutropenia typically present in?
Younger than 10
228
How is diagnosis of cyclic neutropenia made?
Frequent CBCs (2x per week for 2 months)- Have to document the cycle
229
What 2 bacteria are responsible for most deaths in untreated patients with cyclic neutropenia?
1. Clostridia perfringens
| 2. Gram-negative organisms
230
What is the treatment for cyclic neutropenia?
1. Manage infections
| 2. Daily rhG-CSF (recombinant human granulocyte colony stimulating factor)
231
What does rhG-CSF do?
Reduces the number of neutropenic days
232
How is chronic benign neutropenia usually picked up?
Incidental finding
233
True or False: Children with chronic benign neutropenia rarely have oral ulcers or other signs of infection?
True
234
When kids with chronic benign neutropenia have oral ulcers or other signs of infection, what can be helpful?
G-CSF
235
How is chronic benign neutropenia differentiated from cyclic neutropenia?
Frequent CBCs
236
When to children with chronic benign neutropenia usually outgrow this?
By age 2
237
What is another name for severe congenital neutropenia?
Kostmann syndrome (Kostmann agranulocytosis)
238
How is Kostmann agranulocytosis inherited?
Autosomal recessive
239
What causes Kostmann agranulocytosis?
Arrest in the development of neutrophils
240
True or False: Kostmann agranulocytosis can cause a severe neutropenia and result in life-threatening infections?
True
241
How do you distinguish between Kostmann syndrome and cyclic neutropenia?
1. Cyclic neutropenia: ANC will be normal as cycle ends
| 2. Kostmann syndrome: ANC remains low
242
True or false: In Kostmann agranulocytosis the ANC will increase in response to G-CSF, but won't if it is cyclic neutropenia?
False: In both disorders ANC will rise in response to G-CSF
243
What is given to manage Kostman agranulocytosis?
rhG-CSF (this is life-saving)
244
What is helpful for patients with Kostmann agranulocytosis who don't respond to rhG-CSF?
Stem cell transplantation
245
What are the two main components of Shwachman-Diamond Syndrome?
1. Pancreatic exocrine insufficiency (leads to steatorrhea)
| 2. Pancytopenia
246
How do patients with Shwachman-Diamond Syndrome present?
Short stature, diarrhea/steatorrhea, recurrent infections, skeletal abnormalities
247
What types of infections do kids with Shwachman-Diamond Syndrome typically get?
1. Upper respiratory
| 2. Skin
248
What skeletal abnormalities do kids with Shwachman-Diamond syndrome get?
1. Clinodactyly
| 2. Syndactyly
249
What is the mnemonic for Swachman-Diamond Syndrome?
SDS:
- Substandard neutrophils
- Dumb pancreas
- Steatorrhea/Skeletal abnormalities
250
What other chronic illness can Shwachman-Diamond Syndrome get confused with?
Cystic Fibrosis- Both present with pancreatic insufficiency
251
How can you distinguish between Shwachman-Diamond Syndrome and CF?
-Scwachman-Diamond Syndrome has decreased cell lines (neutropenia/anemia/thrombocytopenia), normal electrolytes, no history of chronic pulmonary problems, normal sweat test
252
What is the treatment of Shwachman-Diamond Syndrome?
1. Control/prevent infections
2. Give pancreatic enzyme supplementation
3. Prevent orthopedic abnormalities
4. Monitor for leukemic transformation
253
What is the definition of thrombocytopenia?
Platelet count lower than 150,000
254
What 2 things cause thrombocytopenia?
1. Decreased production of platelets
| 2. Increased destruction of platelets
255
How do kids with thrombocytopenia present?
History of easy bruising and bleeding (especially mucosal bleeding)
256
What part of the medical history is important for thrombocytopenia?
Recent meds
257
What medications can cause thrombocytopenia?
1. Sulfas
2. Seizure meds
3. Vanco
258
How long does it take platelet counts to return to normal after discontinuing a drug that is causing it?
About one week
259
True or False: ASA and ibuprofen cause thrombocytopenia?
False- ASA and ibuprofen result in abnormal platelet function, but platelet count will be normal
260
What problems with platelets can cause bruising, petechiae, epistaxis, or GI bleeding?
Both thrombocytopenia and functional platelet disorders
261
What is deep muscle or joint bleeding likely due to?
Coagulopathy (not a platelet problem)
262
What is the most common hematologic abnormality in neonates admitted to the ICU?
Thrombocytopenia
263
True or False: Thrombocytopenia may be present with neonatal sepsis?
True
264
What is the most common cause of true neonatal primary isolated thrombocytopenia?
Related to maternal factors: Allo- or Auto- antibodies
265
What is the most common reason for a low platelet count?
Clumping from improper collection
266
What is an isolated, transient, but severe thrombocytopenia in a neonate due to platelet destruction by maternal antibodies?
Neonatal alloimmune thrombocytopenia
267
How does neonatal alloimmune thrombocytopenia work?q
Occurs when fetal platelets display the antigen inherited by the father- triggers maternal antibodies to attack them (similar to Rh disease)
268
What symptoms does the mother have with neonatal alloimmune thrombocytopenia?
Mother is asymptomatic, her platelets are normal
269
What happens in infants with autoimmune thrombocytopenia?
Mom has antibodies to her own platelets and infant's plaetmets
270
True or False: In autoimmune thrombocytopenia, both Mom and child have low platelets?
True
271
Healthy 3 year old with recent viral illness, low platelet count, ecchymoses, and petechiae?
ITP
272
What % of cases of ITP resolve in 3 months?
50%
273
What % of cases of ITP resolve in 6 months?
75%
274
What should you think when you have a patient with ITP who has persistent or severe headaches?
Intracranial hemorrhage
275
Which ITP patients should be referred to hematology?
Those with persistent ITP, mucosal bleeding, atypical features (other cell line deficits)
276
When is prognosis for ITP worse?
If it presents in an older kid (over age 10)
277
What happens in kids who present with ITP that are over 10?
It is likely to become a chronic problem
278
When is ITP more likely to be a single occurrence?
Younger patients
279
What is treatment for mild ITP?
Nothing
280
What can be given for ITP with extremely low platelet levels?
IVIG
281
In chronic ITP, what is a treatment consideration?
Splenectomy (should be avoided in kids under 5)
282
What can be a cause of purpura?
Vasculitic disorders
283
Bruising or purpura in a child with normal or increased platelet count?
HSP or other form of vasculitis (may consider abuse)
284
True or False: Shin bruising is normal in a 2 year old?
True
285
What is another name for anaphylactoid purpura?
HSP
286
What is a good treatment for ITP?
Steroids
287
Why are steroids not a first line treatment for ITP?
Because if it is actually leukemia rather than ITP, you can kill the child
288
What causes thrombocytopenia in Kasabach-Merritt Syndrome?
Localized consumptive coagulopathy
289
What is it called when you have a hemangioma that serves as a "sand trap" for paltelets?
Kasaback-Merritt Syndrome
290
True or false: The bone marrow is normal is Kasabach-Merritt Syndrome?
True
291
What are patients with Kasabach-Merritt Syndrome at risk for?
DIC
292
What is the treatment for Kasabach-Merritt Syndrome?
Control the hemangioma, give transfusions as needed
293
What should you think when you have an XR without a radius?
TAR- Thrombocytopenia Absent Radius
294
Is the WBC elevated or decreased in TAR?
High
295
50% of patients with TAR are symptomatic by what age?
1st week of life
296
What % of patients with TAR have symptoms by 4 months?
90%
297
What 3 blood disorders are associated with upper limb abnormalities?
DBA, Fanconi, TAR
298
What two blood disorders have problems with abnormal thumbs?
DBA and TAR
299
What skeletal abnormality is present in TAR?
Absent radius
300
What are the platelet counts in DBA, Fanconi, and TAR?
Normal in DBA, Low in Fanconi and TAR
301
What is the Hgb in DBA, Fanconi, and TAR?
Low in DBA and Fanconi, normal in TAR
302
When does DBA present?
At 2-3 months
303
When does Fanconi present?
After age 3
304
When does TAR present?
First month
305
What are the vitamin K dependent factors?
2, 7, 9, 10
306
What should you consider in early onset Vitamin K dependent bleeding?
Maternal factors (medications Mom is on)
307
What are some medications that can interfere with a Mom's Vitamin K stores?
1. Anticonvulsants
2. Warfarin
3. Some antibiotics
308
What are common sources of bleeding in an infant with a coagulopathy (like Vitamin K deficiency)?
1. Venipuncture sites
2. Penis after circumcision
3. Mucous membranes
4. GI tract
309
Infant born at home who is exclusively breast fed presents in the first week of life with bleeding?
Vitamin K deficiency
310
What lab is abnormal in hemorrhagic disease of the newborn (deficiency in the Vitamin K factors)?
Elevated PT (extrinsic pathway)
311
Who is more likely to get vitamin K deficiency, breast fed or bottle fed infants?
Breast fed (vitamin K is in fortified formulas)
312
What are the 2 main types of hemophilia?
1. Hemophilia A (Factor 8 deficiency)
| 2. Hemophilia B (Factor 9 deficiency- Christmas disease)
313
How are Hemophilia A/B inherited?
X-linked recessive
314
If you have a patient with hemophilia who is bleeding in the antecubital area after a blood draw, is this concerning?
Yes- Can lead to nerve compression (this is a medical emergency)
315
True or False: Blood should not be drawn from the jugular or femoral veins of individuals with hemophilia who haven't received replacement therapy?
True- This can be fatal
316
What is another name for Hemopbilia B?
Christmas disease (X-mas disease = X-linked recessive)
317
What family history is likely for hemophilia?
Affected uncles and boy cousin's on Mom's side
318
What presents in the neonatal period with bruising, bleeding from their circumcision, or bleeding at the site of venipuncture?
Hemophilia
319
What lab is abnormal in hemophilia?
PTT- it is prolonged
320
How is hemophilia diagnosed?
By measuring factor levels
321
What are old kids with hemophilia subject to?
Deep joint bleeds
322
What can cause mortality in kids with hemophilia?
Intracranial bleeding
323
What is the treatment for hemophilia?
Factor supplementation
324
What should be done for a patient with hemophilia who suffers head trauma without any clinical signs?
Replacement therapy (even without clinical signs)
325
Can a female with bleeding symptoms have hemophilia (for boards)?
No- Not hemophilia, think von Willebrands
326
Child who develops a hemotoma from the vitamin K shot... what do you think?
Hemophilia
327
Child with excessive bleeding after a circumcision... workup?
Look for a congenital factor deficiency
328
Prolonged PT?
Vitamin K deficiency (Factors 2, 7, 9, 10)
329
Prolonged PTT?
Hemophilia (Factors 8, 9)
330
True or False: Hemophilia can present in absence of a family history?
True
331
What should you think if they mention that Mom has a family history of a bleeding disorder?
They are hinting at an X-linked recessive disease
332
What is the % chance of being born with an X-linked recessive disease if a male is born to a Mom who is a carrier?
50%
333
What causes Von Willebrand's Disease?
Abnormal von Willebrand factor
334
What is Von Willebrand Factor required for?
Normal Factor VII function as well as normal platelet aggregation
335
Kid with either excessive bleeding after a dental procedure or tonsillectomy, epistaxis, or girl with menorrhagia?
Von Willebrand
336
If a preoperative screening shows an abnormal PTT, what should you think of?
Von Willebrand
337
What happens to PT and PTT in Von Willebrands?
PT is normal
| PTT is usually normal, but can be slightly prolonged for some people
338
What happens to levels of Von Willebrand factor in Von Willebrands disease?
They are low
339
If you have a girl with bleeding and levels of Von Willebrand factor are low, what is your diagnosis?
Von Willebrands
340
If you have a boy with bleeding and levels of Von Willebrand factor are low, what is your diagnosis?
Not clear- Need to measure Factor VII and IX levels to establish a diagnosis
341
What happens to bleeding time in Von Willebrand's?
It is prolonged
342
What is the treatment for Von Willebrands?
Usually nothing
343
What can be given for minor bleeds in Von Willebrands?
Intranasal or IV DDAVP (desmopressin)
344
How does desmopressin help in Von Willebrands?
It causes an increase in plasma vWF and factor VIII
345
What should be given for Von Willebrands in the setting of major surgery or life-threatening bleeds?
Replacement with a factor VIII concentrate
346
For Von Willebrands with major bleeds, is Factor VIII concentrate or cryoprecipitate better?
Concentrate
347
What can help with mucosal bleeding in Von Willebrands by inhibiting fibrinolysis?
Amicar (aminocaprioic acid)
348
What is typically in the history for DIC?
Sepsis, malignancy, sever burns (major illness)
349
What happens to platelets, fibrinogen, D-dimer, PT, PTT, and thrombin time?
```
Platelets low
Fibrinogen low
D-Dimer elevated
PT/PTT unpredictable
Thombin time prolonged
```
350
What is the most important factor in management fo DIC?
Treating the underlying condition
351
What is needed in DIC if clotting factors are low, if fibrinogen is low, or if Hgb is low?
Clotting factors: Platelets or FFP
Fibrinogen: Cryoprecipitate
Hgb: PRBCs
352
Patient with unilateral pain in long bone, adolescent going through a growth spurt...?
Don't be tricked into picking growing pains. Growing pains typically bilateral and usually only at night. This may be a bone tumor.
353
Weeks of unilateral limb pain, what should you do?
XR to look for malignancy
354
What are 3 clues that limb pain in a child is "real"?
1. No longer participating in sport
2. Limping
3. Waking at night crying in pain
355
What i treatment for osteogenic sarcoma (osteosarcoma)?
Amputation or limb salvage + chemo to prevent spread
356
Where do mets from osteosarcoma go?
Lungs
357
What are 3 elevated labs you might see in osteosarcoma?
1. LDH
2. Alk Phos
3. Sed rate
358
What is the most common primary malignant bone tumor in children and adolescents?
Osteosrcoma
359
When is the peak incidence of osteosarcoma?
In the teens (coincides with the growth spurt)
360
If there is trauma, should this steer you from a diagnosis of osteosarcoma?
No... there may be trauma, but with any other signs of potential tumor, like preceding recurrent persistent pain consider osteogenic sarcoma
361
Which type of bone tumor has a soft tissue component/
Ewing Sarcoma
362
True or False: Ewing Sarcoma isn't usually seen in African Americans?
True
363
Which type of bone tumor is more common in African Americans?
Osteogenic Sarcoma
364
Which type of bone tumor typically presents in a teenager going through a growth spurt who has pain?
Osteogenic sarcoma
365
What are common sites for osteogenic sarcoma?
Proximal and distal long bones (femur and humerus)
366
True or false: Both osteogenic sarcoma and ewing sarcoma can occur in long bones?
True
367
What is the treatment for bone tumors?
Chemo, radiation, surgery
368
Where do bone tumors commonly metastasize?
Lungs
369
What is the main location for osteogenic sarcoma mets?
Lungs
370
Tibia or femur pain that is worse at night, but relived by ibuprofen?
Osteoid osteoma
371
Which bone lesion has a central radiolucent area surrounded by thick sclerotic bone?
Osteoid osteoma
372
What is the most common childhood malignancy?
Leukemia
373
What are 4 types of childhood leukemia?
1. ALL
2. ANLL
3. CML
4. CLL
374
What is the most common childhood leukemia?
ALL (80%)
375
When is the highest incidence of ALL?
2-5 years of age
376
True or False: Both genetic and environmental factors are believed to contribute to development of ALL?
True
377
Child with bone pain or joint pain/swelling with fevers. Labs have pancytopenia?
Think ALL
378
True or False: The WBC count may be normal or elevated at presentation with ALL?
True
379
Name 2 exam findings that could help to differentiate ALL from aplastic anemia.
1. Enlarged liver
| 2. Enlarged spleen
380
What is needed to formally diagnose leukemia?
Bone marrow examination
381
What is the treatment goal in kids with ALL?
Induce remission with one course of chemo
382
What is the 5 year survival rate for children with newly diagnosed ALL?
80%
383
What is the probability of achieving complete remission in 4 weeks with current chemo regimens?
Over 95%
384
True or False: Even with treatment, kids with ALL are at risk of relapse?
True
385
What are 2 "sanctuary sites" of ALL that are more resistant to therapy?
1. CNS
| 2. Testes
386
What are 2 common sites of relapse in ALL?
CNS and testes
387
True or False: Each ALL relapse has same prognosis as initial presentation?
False- each relapse has a worse prognosis
388
What is the most common congenital condition associated with leukemia?
Downs
389
Name 6 conditions which have an increased risk of leukemia.
1. Down
2. Ataxia telangectasia (+ other immunodeficiency syndromes)
2. Fanconi anemia
3. Bloom Syndrome
4. Klinefleter syndrome
5. Neurofibromatosis
390
In the context of a clinical picture of ALL, what are atypical lymphocytes?
Lymphoblasts (they can get reported incorrectly as atypical lymphocytes- don't get confused with mono if clinical picture is leukemia)
391
What 2 things are survivors of ALL at increased risk of?
1. Neuropsychological abnormalities
| 2. Cognitive defects
392
What is a major cause of death in kids with leukemia?
Infection
393
What 2 things increase the risk for infection in kids with leukemia?
1. Indwelling catheters
2. Neutropenia
*These kids get broad-spectrum IV antibiotics
394
What are the 2 main types of lymphoma?
1. Hodgkin's
| 2. Non-Hodgkin's (everything else)
395
Patient with unexplained lymphadenopathy with other symptoms like cough, new-onset wheezing, trouble swallowing, face/neck swelling, chest pain, dyspnea?
Get a CXR (could be lymphoma)
396
What does unexplained pruritus make you think of?
Lymphoma
397
What presents in teens as non-tender enlarged cervical or supraclavicular lymph nodes?
Hodgkin's lymphoma
398
What are 3 symptoms commonly seen with Hodgkin's lymphoma?
1. Weight loss
2. Fevers
3. Night sweats
399
True or False: Hodgkin's lymphoma is a slow-growing malignancy
True
400
What is seen on CBC in Hodgkin's lymphoma?
Elevated WBC with relatively low lymphocyte count
401
Lymph node biopsy with Reed Sternberg cells?
Hodgkin's
402
What should you think of with enlarged lymph nodes in younger children versus adolescents?
Younger children: More likely to be an infection
| Adolescents: More likely to be lymphoma
403
What can present with an enlarged cervical lymph node?
Atypical mycobacteria (will have other signs like a positive PPD)
404
If you have an enlarged supraclavicular lymph node, is this likely to be atypical mycobacteria?
No... see this with cervical nodes, not supraclavicular ones
405
True or False: Supraclavicular adenopathy means lymphoma until proven otherwise?
True
406
What is the next step after findings supraclavicular adenopathy?
CXR
407
Which type of lymphoma is more likely in a small child versus a teen?
Non-hodgkin
408
How does Non-Hodgkin lymphoma present?
Rapidly, typically with airway compression versus painless cervical adenopathy.
409
What should a non-tender mass in the abdomen make you consider?
1. Wilms
2. Neuroblastoma
3. Non-Hodgkin lymphoma
410
What are 4 things a patient with Langerhans Cell Histiocytosis might present with?
1. Persistent scalp or diaper seborrheic rash
2. Chronic ear drainage or chronic mastoiditis
3. Lytic lesions in the skull or vertebral collapse
4. Excessive urination
411
What is responsible for excessive urination in Langerhans Cell Histiocytosis?
DI and pituitary involvement
412
How is Langerhans Cell Histiocytosis diagnosed?
Skin biopsy and electron microscopy
413
What is another name for Histiocytosis X?
Langerhans Cell Histiocytosis
414
What is the most common solid tumor of infancy?
Neuroblastoma
415
What is the second most common solid tumor of childhood?
Neuroblastoma
416
What is the most common solid tumor of childhood?
Brain tumors
417
Where does neuroblastoma initially present in 50% of cases?
Adrenal glands
418
What is the survival rate if neuroblastoma occurs before 12 months of age?
95%
419
What is the only known tumor to spontaneously regress?
Neuroblastoma
420
What are 8 examples of typical presentation of neuroblastoma?
1. Persistent bone or joint pain
2. Non-tender abdominal mass
3. Weight loss, anorexia, night sweats, fever
4. UTI (obstructing abdominal mass)
5. Raccoon eyes and proptosis (from mets)
6. Horner syndrome
7. Irritability, HTN, diarrhea (catecholamine production)
8. Opsoclonus-myoclonus (dancing eyes)
421
What causes Horner syndrom seen in neuroblastoma?
Mediastinal tumor compressing recurrent laryngeal nerve (this also affects speech)
422
If you have a kid with a neuroblastoma who has HTN, what is causing the HTN?
Renal artery compression (pick this over excess catecholamine production)
423
True or False: Labs can be relatively normal in neuroblastoma?
True
424
What are some lab abnormalities you can see in neuroblastoma?
- Pancytopenia (from bone marrow involvement)
- Elevated ferritin
- Elevated LDH
- Elevated Alk Phos
425
What are the 2 ways you can diagnose neuroblastoma?
1. Biopsy tumor
| 2. Elevated urine VMA/HMA with neuroblasts found in bone marrow (with this, biopsy not needed)
426
When do you order urine catecholamines when suspecting neuroblastoma?
Never... this measures epinephrine and norepinephrine... specifically order urinary VMA/HMA
427
What is the most important prognostic feature for neuroblastoma?
Age
428
When is the prognosis for neuroblastoma better?
Under 1, excellent prognosis
| Over 1, poor prognosis
429
What are 2 things you should think of with raccoon eyes?
1. Child abuse (basal skull fracture or basilar head bleeds)
| 2. Neuroblastoma
430
What is the MIBG scan used for?
Neuroendocrine tumors (pheochromocytomas or neuroblastomas)
431
True or False: Surgical staging for neuroblastoma is important in treatment?
False... don't do surgical staging
432
What testing is needed to locate a neuroblastoma?
CT with contrast
433
What testing is needed to locate mets from a neuroblastoma?
Bone scan
434
What test is necessary in neuroblastoma to determine the extent of disease?
Bilateral bone marrow evaluation
435
What 3 tests are important to determine staging for neuroblastoma?
1. CT with contrast
2. Bone scan
3. Bilateral bone marrow biopsy
436
What tumor presents with leukocoria or strabismus?
Retinoblastoma
437
What age group does retinoblastoma usually occur in?
Children under 5
438
What is leukocoria?
White pupil
439
What % of retinoblastoma presents with leukocoria?
60%
440
What % of retinoblastoma presents with strabismus?
20%
441
What is the typical presentation for a retinoblastoma?
Mom brings in picture with normal red reflex in only one eye
442
What % of retinoblastoma have a family history?
5%
443
What are the 3 mainstays of treatment for retinoblastoma?
1. Surgical excision
2. Chemotherapy
3. Radiation
444
How does sporadic retinoblastoma usually present?
Unilateral
445
How does genetic retinoblastoma usually present?
Bilateral
446
What is the inheritance for retinoblastoma?
Autosomal dominant with incomplete penetrance
447
Where is the gene for retinoblastoma located?
Long arm of chromosome 13
448
True or False: Lab investigation is often recommended for prognosis and genetic planning with genetic retinoblastoma?
True
449
What is the % of a child getting retinoblastoma if a parent had it in 2 eyes?
50%
450
What is the % of a child getting retionblastoma if a parent had it in 1 eye?
5% (1/20)
451
What is the % of a child getting retinoblastoma if a sibiling had unilateral disease?
5% (1/20)
452
True or False: Kids with retinoblastoma in one eye need to be monitored for disease in the other eye
True
453
True or False: Retinoblastoma doesn't increase risk of development of other malignancies?
False: Risk for other malignancies (sarcoma or malignant melanoma) increases
454
What is the most common soft tissue sarcoma in children?
Rhabdomyosarcoma
455
Kid with constipation who has a visible or palpated mass on rectal exam?
Rhabdomyosarcoma
456
Where do younger kids (2-6) tend to get rhabdomyosarcoma?
Head and neck
457
Where do older kids (teenagers) tend to get rhabdomyosarcoma?
Truncal or extremity
458
How is diagnosis of rhabdomyosarcoma made?
Biopsy
459
How is rhabdomyosarcoma treated?
1. Chemo
2. Surgery
3. Radiation
460
Kid with trauma to an extremity and the pain is getting worse after a couple of weeks...?
Rhabdomyosarcoma (don't be fooled by history of trauma... atypical pain after trauma)
461
Grape-like mass protruding from the vagina?
Rhabdomyosarcoma
462
What are two considtions that can get worse after a trauma injury?
1. Rhabdomyosarcoma
| 2. Osteogenic sarcoma
463
What is the most common pediatric abdominal malignancy?
Wilms tumor (nephroblastoma)
464
What is the median age for diagnosis of Wilms tumor?
3.5 years
465
What is the most common presentation of a Wilms tumor?
Asymptomatic abdominal mass
466
What are 5 exam findings with Wilms tumor?
1. Asymptomatic abdominal mass
2. Hypertension
3. Gross hematuria
4. Aniridia
5. Hemihypertrophy
467
How is diagnosis of Wilms tumor made?
Histology
468
How is Wilms tumor recognized on an XR?
It doesn't calcify
469
If you see calcification on an abdominal mass XR is it neuroblastoma or nephroblastoma?
Neuroblastoma
470
What are 2 things assocaited with hemihypertrophy?
1. Wilms tumor
| 2. Beckwith Wiedemann Syndrome
471
What types of tumors are children with Beckwith Wiedemann syndrom at risk for developing?
1. Wilms
2. Hepatoblastoma
3. Other cancers
472
Half the body being larger than the other?
Hemihypertrophy
473
How is Wilms tumor managed?
1. Nephrectomy
| 2. Chemo/radiation according to stage
474
What % of kids with Wilms tumor stage 1 and 2 may be cured?
90%
475
What occurs with the rapid breakdown of a large number of tumor cells?
Tumor Lysis Syndrome
476
When is tumor lysis syndrome most common?
At the initiation of chemo for large tumors, lymphomas, or leukemia
477
What 3 things help prevent/treat Tumor Lysis Syndrome?
1. Hydration
2. Alkalinization
3. Allopurinol
478
What happens to phosphate, potassium, uric acid, calcium, and sodium in tumor lysis?
1. Phosphate elevated
2. Potassium elevated
3. Uric acid elevated
4. Calcium decreased
5. Sodium not affected
479
What should you be on the lookout for with any tumor in or around the spine?
Cord compression- this presents with neurological symptoms or bowel/bladder dysfunction
480
How is cord compression from a spinal tumor managed?
Steroids and/or radiation
481
What is the primary symptom of an anterior mediastinal mass?
Respiratory distress (especially when supine)
482
When do you intubate for an anterior mediastinal mass?
You don't- Airway compression will be below the vocal cords and if patient is anesthetized there will be no way to maintain oxygenation/ventilation
483
What are the 4 things that can result in symptoms from anterior mediastinal massess?
1. Thymoma
2. Teratoma
3. Thyroid carcinoma
4. Lymphoma
484
Which tumor resulting in an anterior mediastinal mass most commonly results in airway compromise?
1. Lymphoma
485
What is given for any chemo patient who is exposed to varicella?
VZ-IG
486
Why do you never give systemic steroids unless you know malignancy has been ruled out?
Tumor lysis syndrome
*Especially with things like ITP, JIA, new-onset wheezing, and mono where symptoms overlap
487
What is the treatment for an anterior mediastinal mass?
Treat underlying condition
| -Can use steroids/radiation in an emergency with airway compromise
488
What is the side effect of alkylating agents for chemo?
Gonadal dysfunction
489
What is the side effect of cyclophosphamide?
Hemorrhagic cystitis
490
How do you monitor kids getting cyclophosphamide?
Serial urinalysis (watch for cystitis and bleeding)
491
How do you manage hemorrhagic cystitis?
Hydration to increase urine output
492
What is the side effect of cisplatin?
Hearing loss and peripheral neuropathy
493
What is the side effect of bleomycin?
Pulmonary fibrosis
494
What results from extrinsic compression of the SVC by an anterior mediastinal tumor?
SVC syndrome
495
What type of anterior mediastinal tumor most commonly causes SVC syndrome?
Lymphoma
496
How does SVC syndrome present?
1. Red face (plethora)
2. Facial swelling
3. Upper extremity edema
4. Distended neck veins
5. Neurologic symptoms
497
What is the side effect of anthracycline (doxorubicin and daunomycin)?
Cardiac toxicity
498
What are the side effects of vincristine and vinblastine?
1. Neurotoxicity
| 2. SIADH
499
What is the side effect of methotrexate?
1. Oral/GI uclers
| 2. Bone loss
