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US Pediatric Boards Edited > Heme/oncology > Flashcards

Flashcards in Heme/oncology Deck (165):
1

What diagnosis should you consider in a teenager with leg pain who is going through a growth spurt?

Osteogenic sarcoma

2

What is the treatment of osteogenic sarcoma ? (2)

Amputation or limb salvage
Chemotherapy

3

What is the most common site of metastasis from osteogenic sarcoma?

Lung

4

What type of bone sarcoma presents with a soft tissue component?

Ewing sarcoma

5

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

Brain tumors

6

What diagnosis presents with left leg pain that is worse at night but relieved with ibuprofen?

Osteoid osteoma

7

What is the diagnosis of a femur with central radiolucent area surrounded by thick sclerotic bone?

Osteoid osteoma

8

What are 3 poor prognostic signs of ALL?

Age <2
WBC > 50
T cell precursor

9

Which sanctuary sites of ALL are most resistant to therapy and common sites of relapse?

CNS and testes

10

What is the correct first step in diagnosis in a child with unexplained lymphadenopathy and cough?

Chest X-ray

11

What diagnosis should you consider in a teenager who presents with non tender cervical lymph node and high WBCs?

Hodgkin lymphoma

12

What cells would you expect to find on lymph node biopsy in a patient with Hodgkin lymphoma ?

Reed sternberg

13

What is the first course of action in a patient with fever who has had splenectomy?

Sepsis workup

14

What is the difference between lymphoblastic and nonlymphoblastic non Hodgkin lymphoma?

Lymphoblastic is t. Cell origin and non lymphoblastic is b. Cell origin

15

Where are the 3 most common sites of mass secondary to non Hodgkin lymphoma ?

Head, neck, abdomen

16

What 3 tumors should you consider in a child with non tender abdominal mass?

Wilms
Neuroblastoma
Non Hodgkin lymphoma

17

What is the difference in bone marrow findings between leukemia and lymphoma?

Leukemia has >25% blasts
Lymphomas has <25% blasts

18

How is langerhans cell histiocytosis diagnosed?

Skin biopsy and electron microscopy

19

How is langerhans cell histiocytosis treated ? (3)

Surgery, steroids or chemo

20

What diagnosis should you consider in a patient with a lytic skull lesion and seborrheic rash?

Langerhans cell histiocytosis

21

What urinary problems are associated with langerhans cell histiocytosis?

Diabetes insipidus and excessive urination

22

Where is the most common place for presentation of neuroblastoma?

Adrenal gland

23

What is the prognosis of neuroblastoma?

Survival 95% if occurs prior to 1 year - poor prognosis of older than 1

24

How is neuroblastoma diagnosed?

Tumor biopsy or high urine VMA/HMA plus bone marrow tumor

25

What is the best imaging study to find neuroblastoma?

CT with contrast

26

What are 3 common lab abnormalities associated with neuroblastoma ?

Pancytopenia
Elevated ferritin
Elevated LDH

27

What is the most Likely cause of HTN in a patient with neuroblastoma?

Renal artery compression

28

What diagnosis should you consider in a patient with non tender abdominal mass, HTN and diarrhea ?

Neuroblastoma

29

What syndrome can occur from mediastinal tumor in a patient with neuroblastoma ?

Horner syndrome (compression of recurrent laryngeal nerve)

30

What is the chance that a woman with retinoblastoma in both eyes will have a child with retinoblastoma?

50% chance of any of her children having retinoblastoma

31

What is the chance that a woman with retinoblastoma in one eye will have a child with retinoblastoma?

5% chance

32

What are the 3 treatment options for retinoblastoma?

Surgery, chemotherapy or radiation

33

A child with retinoblastoma is at greater risk for later development of what Cancer?

Osteosarcoma

34

What is the appropriate management in a patient with vomiting who has a full fontanelle on exam and develops posturing after being given IV fluids?

Steroids &
Measures to reduce intracranial pressure

35

A child presents with grape-like mass protruding from the vagina, what is the likely diagnosis ?

Rhabdomyosarcoma

36

What are two types of cancer that can get worse or present after a traumatic injury ?

Rhabdomyosarcoma and osteogenic sarcoma

37

A child presents with constipation and on rectal exam you palpate a mass, what Cancer should you consider ?

Rhabdomyosarcoma

38

What diagnosis should you consider in a patient with non tender abdominal mass, HTN and gross hematuria?

Wilms tumor

39

What ophthalmologic finding is associated with Wilms tumor?

Aniridia

40

What two diagnoses are associated with hemihypertrophy?

Wilms tumor
Beckwith wiedemann

41

What 3 lab abnormalities should be monitored for in a patient getting initiation of chemotherapy ?

Hyperphosphatemia
Hyperkalemia
Hyperuricemia

42

What is the treatment for tumor lysis? (3)

Hydration
Alkalinization
Allopurinol

43

What should be done in a patient on chemotherapy who is exposed to varicella ?

Varicella zoster IG

44

What 4 things cause symptoms associated with anterior mediastinal masses?

Thymoma
Teratoma
Thyroid
T cell lymphoma

45

When should you intubate a patient suspected of having anterior mediastinal mass?

Never - airway compression Is below the vocal cords and it is dangerous to anesthetize these patients !!!

46

What diagnosis should you consider in a patient with a red face that is swollen as well as arm swelling and distended neck veins?

Superior vena cava syndrome

47

What is a potential side effect with use of cyclophosphamide? What can be done to prevent this side effect?

Hemorrhagic Cystitis - aggressive hydration and serial urinalysis

48

What is the potential side effect with use of bleomycin ?

Pulmonary fibrosis

49

What is a potential side effect with use of anthracycline (doxorubicin)?

Cardiac toxicity

50

What is a potential side effect of vincristine?

Neurotoxicity and SIADH

51

What side effect is associated with use of asparaginase?

Pancreatitis

52

What side effect is associated with use of procarbazine?

CNS toxicity

53

What hormone should be monitored in children having cranial radiation therapy?

Growth hormone

54

What potential side effect is associated with use of methotrexate?

Oral and GI ulcers

55

What is the cause of physiologic anemia of infancy?

Low erythropoietin production

56

What is the normal MCV for babies and toddlers?

70-90

57

What are 4 major causes of microcytic anemia in children ?

Iron deficiency
Thalassemia
Anemia of chronic illness
Lead poisoning

58

What diagnosis should you consider in a small for age Greek boy with anemia and large spleen?

Thalassemia major

59

What X-ray findings are compatible with thalassemia and why?

Thickened bone on X-ray
Skull with "hair on end" appearance - secondary to extramedullary hematopoiesis

60

What hemoglobin electrophoresis results are consistent with a diagnosis of beta thalassemia?

Low hemoglobin A1
High hemoglobin A2 (alpha delta)
High hemoglobin F

61

What are 2 long term complications of beta thalassemia and chronic transfusion therapy?

Cholelithiasis
Hemosiderosis

62

What are the results of hemoglobin electrophoresis in a patient with sickle cell anemia?

High hemoglobin F
High hemoglobin S

63

How long should iron deficiency anemia by treated?

Until 2 months after hgb levels are normal

64

What is the underlying cause of anemia in an 18 month old child with pallor whose diet consists mainly of milk?

Nutritional - low amount of iron is absorbed from cows milk

65

When is IV iron the correct treatment for anemia?

Only if child is Unable to take PO

66

What's the difference in the RDW when comparing iron deficiency anemia and thalassemia?

RDW is high in iron deficiency
RDW is low in thalassemia

67

What are the ferritin and TIBC in anemia of chronic disease ?

Ferritin is high, TIBC is low

68

A patient presents with microcytic anemia and has a history of sickle cell disease ...what is the likely cause of the anemia?

Iron deficiency

69

What is the appropriate treatment for anemia of chronic disease ?

Treatment of the underlying disease
(Remember that patient will already have adequate iron stores)

70

What is the gold standard for diagnosis of lead poisoning?

Whole blood lead level

71

What is this treatment for lead poisoning?

Chelation - penicillamine, dimercaprol and EDTA

72

When is the FEP (free erythrocyte protoporphyrin) elevated?

In lead poisoning and iron deficiency

73

What is the classic red blood cell appearance in lead poisoning?

Ringed sideroblasts

74

What type of anemia is caused by drinking goats milk?

Macrocytic anemia secondary to folate deficiency

75

What is the test that determines B12 absorption ?

Schilling test

76

What are 4 causes of B12 deficiency ?

Intrinsic factor
Bacterial overgrowth
Bowel resection
Infant of vegetarian mother

77

What diagnosis should you consider in a child with weakness, paresthesias and sore tongue whose lab findings reveal anemia?

Pernicious anemia

78

What is the cause of pernicious anemia?

Lack of intrinsic factor leads to impaired b12 absorption

79

What is the treatment of pernicious anemia?

IM cobalamin (aka B12)

80

You are presented with a patient who you suspect has folate deficiency, what is the most important step in diagnosis of the patient ?

Erythrocyte folic acid concentration is preferred over serum folate levels

*b12 deficiency must be ruled out before treatment!

81

What diagnosis should you suspect with red blood cells have "Heinz bodies"?

G6PD deficiency

82

What diagnosis should you consider in a black child with dark urine, jaundice and anemia shortly after treatment with Bactrim?

G6PD deficiency

83

What are the 2 products of red cell breakdown that are found to be elevated during hemolysis?
What level will be low during hemolysis?

Hemosiderin and bilirubin in urine

Low serum haptoglobin

84

When should G6PD testing be performed and why?

Several weeks after an acute episode because testing during an episode will be false negative

85

What is the difference between type A and type B G6PD deficiency?

Type A affects Africans and hemolysis occurs only after exposure to oxidants
Type B affects mediterraneans and has chronic hemolysis

86

What is the etiology (enzyme deficiency) of hereditary spherocytosis?

Spectrin deficiency

87

What diagnosis should you consider in a patient who presents with mild anemia, splenomegaly and intermittent jaundice whose MCHC is elevated ?

Hereditary spherocytosis

88

Why does hereditary spherocytosis cause hemolysis?

Defective red cell surface with fragile cells that break easily

89

What is the diagnostic method for hereditary spherocytosis?

Osmotic fragility test

90

What is the inheritance pattern of hereditary spherocytosis?

Autosomal dominant

91

What is the treatment (3) for hereditary spherocytosis?

Folic acid
Transfusion PRN
Splenectomy

92

What is the most common cause of aplastic crisis in children with hereditary spherocytosis?

Parvovirus

93

What amino acid substitution occurs in sickle cell anemia ?

Valine in place of glutamic acid at amino acid 6 of the beta globin

94

What vaccine is administered early in patients with sickle cell anemia?

Pneumococcal vaccine at age 2

95

What type of crisis should you consider in a sickle cell patient who presents with signs of shock?

Sequestration crisis due to pooling of blood in liver and spleen

96

What diagnosis should you consider in a patient with Howell Jolly bodies?

Sickle cell anemia

97

What should you consider in a 7 y/o patient with sickle cell anemia who presents with palpable spleen?

Hemoglobin SC or
Sickle cell plus thalassemia

98

You are presented with a sickle cell patient with chest pain, low O2 sats and infiltrate on chest X-ray, what's the next best test?

ABG

99

What is the treatment for acute chest syndrome ?

Transfusion of pRBC or exchange transfusion

100

What is the best treatment for an African American child with symptoms of stroke?

Exchange transfusion followed by MRI

101

What imaging study should you obtain in a sickle cell patient with RUQ pain? Why?

Abdominal US to rule out gallstones which are associated with Sickle cell

102

How can u differentiate aplastic anemia from acute leukemia?

Aplastic anemia would have normal liver, no lymphadenopathy and normal LDH / uric acid

103

What side effect can occur w use of chloramphenicol ?

Aplastic anemia

104

A patient presents with renal abnormalities, abnormal skin pigmentation and macrocytic anemia, what diagnosis should you consider?

Fanconi anemia

105

What orthopedic problems are common in children with fanconi anemia?

Growth retardation
Absent/hypoplastic thumb

106

What future Cancer risk is likely in patients with fanconi anemia?

AML or myelodysplastic syndrome

107

What is the cure for aplastic anemia?

Bone marrow transplant

108

What are the two profound red cell anemias present in childhood? How do you differentiate the two?

Diamond Blackman anemia - presents in young infants and is chronic

Transient erythroblastopenia of childhood - presents in toddlers and self resolves

109

What type of anemia is due to an arrest in maturation of red blood cells?

Diamond blackfan

110

What anemia is due to suppression of erythroid production secondary to viral illness?

Transient erythroblastopenia of childhood

111

What diagnosis should you consider in a 2 month old with Abnormal facial features and severe anemia?

Diamond blackfan

112

Which type of severe childhood anemia is treated with steroids ?

Diamond blackfan

113

What type of reaction occurs when blood is not properly cross matched?

Hemolytic reaction

114

What type of transfusion reaction causes urticaria that typically resolves after antihistamine use?

Allergic reaction

115

How is risk for febrile nonhemolytic transfusion reaction reduced?

Use of leukocyte filtered blood

116

What diagnosis presents with WBCs full of lysosomal granules and abnormal chemotaxis?

Chediak Higashi

117

What diagnosis presents with frequent bad skin and lung infections in a child with fair skin, blond hair and blue eyes?

Chediak higashi

118

What are 3 common pathogens in patients with chediak Higashi?

Staph aureus
Strep pyogenes
Pneumococcus

119

How is chronic granulomatous disease diagnosed?

Nitroblue tetrazolium test measures oxidative burst

120

What types of infections are common in children with chronic granulomatous disease?

Recurrent bacterial (staph and gram negative) and fungal infections

121

What are 3 possible treatment options for chronic granulomatous disease?

Infection control
Interferon
Bone marrow transplant

122

What diagnosis presents with impaired wound healing, severe periodontal disease and a high WBC count in the absence of infection?

Leukocyte adhesion deficiency

123

What types of infections are common in patients with neutropenia?

Gran negative ulcers of the mucosa

124

What are 5 congenital neutropenias?

Cyclic neutropenia
Kostmann syndrome (severe congenital neutropenia)
Benign neutropenia
Shwachman diamond
Chediak Higashi

125

What are the two most common causes of acquired neutropenia?

Infection and drugs (macrolides)

126

What is the inheritance pattern of cyclic neutropenia ?

Autosomal dominant

127

What diagnosis would you consider in an 8 y/o make with oral lesions that occur once a month associated with low WBC count that lasts approx 1 week?

Cyclic neutropenia

128

What is the typical pathogenic cause of oral lesions associated with cyclic neutropenia?

Clostridium perfringens

129

What disease presents as an incidental finding of low WBCs that are not associated with signs of infection and typically resolves by age 2?

Chronic benign neutropenia

130

What is the inheritance pattern of kostmann agranulocytosis ?

Autosomal recessive

131

What diagnosis presents as severe neutropenia that does NOT rise and fall in cycles?

Kostmann syndrome

132

What diagnosis presents as short stature, steatorrhea and recurrent skin and URI infections as well as syndactyly?

Shwachman Diamond

133

How do you distinguish cystic fibrosis from shwachman diamond syndrome (other than a sweat chloride test of course)?

Both have pancreatic insufficiency and frequent infections but ...SDS also presents with pancytopenia

134

What platelet problems are caused by use of aspirin and ibuprofen ?

Causes abnormal platelet function. Platelet count will be normal.

135

What is the first line treatment for ITP?

IVIG

136

When should platelet transfusion by indicated in patients with ITP?

Platelets lower then 20,000 or active bleeding

137

What is the prognosis of ITP in younger and older children?

50% resolve within 3 months in younger children
Can become chronic problem in children older than 10y/o

138

What should you be concerned about in a patient with ITP who presents with persistent headache?

Intracranial hemorrhage

139

What should you do in a 2 y/o patient with multiple bruises on the shins but normal platelets ?

Nothing - this is normal and typically not a sign of abuse

140

What diagnosis should you consider in a patient with purpura with normal platelet count?

Henoch schonlein purpura (or other vasculites)

141

What is anaphylaxtoid purpura?

Henoch Schonlein purpura

142

What diagnosis presents as a male child with diaper rash, bruising and chronic ear infections ?

Wiskott Aldrich Syndrome

143

How do you distinguish wiskott Aldrich syndrome from ITP?

ITP has large platelets
WAS has small platelets

144

What is the treatment of wiskott Aldrich ?

Bone marrow transplant

145

What is the inheritance pattern of wiskott Aldrich?

X linked (only in boys)

146

What syndrome presents with a large hemangioma and thrombocytopenia caused by consumptive coagulapathy? What are these patients at risk for developing?

Kasabach Merritt Syndrome - risk for DIC

147

What workup should be done for a child with excessive bleeding after circumcision ?

Congenital factor deficiency

148

What lab value is elevated when there is deficiency of vitamin K?

PT is elevated

149

Which are the vitamin K dependent factors in the coagulation cascade?

2, 7, 9 and 10

150

What lab value is abnormal in patients with hemophilia ?

Prolonged PTT

151

What should be done in a patient with hemophilia who suffers head trauma but is clinically doing well with no symptoms ?

Replacement of factors is indicated as soon as possible

152

What is the inheritance pattern of hemophilia ?

X linked recessive

153

What diagnosis should you consider in a patient with low platelets, high WBC count and abnormal X-ray of the radius ?

TAR

154

What's the similarities & the main difference between diamond blackfan anemia and fanconi anemia?

Both with thumb abnormalities and low hgb but only fanconi has low platelets

155

What type of hemophilia typically presents in females?

None - they are X linked and only in males

156

Which factor level is affected in patients with Von willebrand disease?

Factor 8

157

What's the usual hematologic cause of bleeding into a joint?

Hemophilia

158

What is the dangerous complication of bleeding into the antecubital region after a blood draw in a patient with hemophilia ?

Nerve compression - this is a medical emergency

159

What diagnosis should you consider in a female with menorrhagia who lab testing reveals normal PT and prolonged PTT? What is the next appropriate test?

Von willebrand - order Von willebrand factor activity

160

What should be the next step in a male with prolonged PTT and low Von willebrand factor activity?

Order factor 8 and 9 levels to rule out hemophilia

161

What is the management of Von willebrand in the case of mild bleeding?
What if there is major bleeding?

Intranasal DDAVP (stimate)
Factor 8 concentrate if major bleeding

162

What is the inheritance pattern of Von willebrand disease?

Autosomal dominant

163

What is the mechanism of action of aminocaproic acid (amicar) in patients with Von willebrand ?

Inhibits fibrinolytic to stop mucosal bleeding

164

What diagnosis should you consider in a septic patient with low platelets, prolonged thrombin time and low fibrinogen?

DIC

165

What population is most likely to be diagnosed with osteogenic sarcoma? How do they present ?

Black teenager who presents with bone pain