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pediatric Boards > Heme/oncology > Flashcards

Heme/oncology Flashcards

0
Q

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

A

Osteogenic sarcoma

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1
Q

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

A

Black teenager who presents with bone pain

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2
Q

What is the treatment of osteogenic sarcoma ? (2)

A

Amputation or limb salvage

Chemotherapy

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3
Q

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

A

Lung

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4
Q

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

A

Ewing sarcoma

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5
Q

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

A

Brain tumors

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6
Q

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

A

Osteoid osteoma

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7
Q

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

A

Osteoid osteoma

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8
Q

What are 3 poor prognostic signs of ALL?

A

Age <2
WBC > 50
T cell precursor

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9
Q

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

A

CNS and testes

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10
Q

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

A

Chest X-ray

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11
Q

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

A

Hodgkin lymphoma

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12
Q

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

A

Reed sternberg

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13
Q

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

A

Sepsis workup

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14
Q

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

A

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

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15
Q

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

A

Head, neck, abdomen

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16
Q

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

A

Wilms
Neuroblastoma
Non Hodgkin lymphoma

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17
Q

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

A

Leukemia has >25% blasts

Lymphomas has <25% blasts

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18
Q

How is langerhans cell histiocytosis diagnosed?

A

Skin biopsy and electron microscopy

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19
Q

How is langerhans cell histiocytosis treated ? (3)

A

Surgery, steroids or chemo

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20
Q

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

A

Langerhans cell histiocytosis

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21
Q

What urinary problems are associated with langerhans cell histiocytosis?

A

Diabetes insipidus and excessive urination

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22
Q

Where is the most common place for presentation of neuroblastoma?

A

Adrenal gland

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23
Q

What is the prognosis of neuroblastoma?

A

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

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24
Q

How is neuroblastoma diagnosed?

A

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

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25
Q

What is the best imaging study to find neuroblastoma?

A

CT with contrast

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26
Q

What are 3 common lab abnormalities associated with neuroblastoma ?

A

Pancytopenia
Elevated ferritin
Elevated LDH

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27
Q

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

A

Renal artery compression

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28
Q

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

A

Neuroblastoma

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29
Q

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

A

Horner syndrome (compression of recurrent laryngeal nerve)

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30
Q

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

A

50% chance of any of her children having retinoblastoma

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31
Q

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

A

5% chance

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32
Q

What are the 3 treatment options for retinoblastoma?

A

Surgery, chemotherapy or radiation

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33
Q

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

A

Osteosarcoma

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34
Q

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?

A

Steroids &

Measures to reduce intracranial pressure

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35
Q

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

A

Rhabdomyosarcoma

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36
Q

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

A

Rhabdomyosarcoma and osteogenic sarcoma

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37
Q

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

A

Rhabdomyosarcoma

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38
Q

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

A

Wilms tumor

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39
Q

What ophthalmologic finding is associated with Wilms tumor?

A

Aniridia

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40
Q

What two diagnoses are associated with hemihypertrophy?

A

Wilms tumor

Beckwith wiedemann

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41
Q

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

A

Hyperphosphatemia
Hyperkalemia
Hyperuricemia

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42
Q

What is the treatment for tumor lysis? (3)

A

Hydration
Alkalinization
Allopurinol

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43
Q

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

A

Varicella zoster IG

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44
Q

What 4 things cause symptoms associated with anterior mediastinal masses?

A

Thymoma
Teratoma
Thyroid
T cell lymphoma

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45
Q

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

A

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

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46
Q

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

A

Superior vena cava syndrome

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47
Q

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

A

Hemorrhagic Cystitis - aggressive hydration and serial urinalysis

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48
Q

What is the potential side effect with use of bleomycin ?

A

Pulmonary fibrosis

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49
Q

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

A

Cardiac toxicity

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50
Q

What is a potential side effect of vincristine?

A

Neurotoxicity and SIADH

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51
Q

What side effect is associated with use of asparaginase?

A

Pancreatitis

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52
Q

What side effect is associated with use of procarbazine?

A

CNS toxicity

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53
Q

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

A

Growth hormone

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54
Q

What potential side effect is associated with use of methotrexate?

A

Oral and GI ulcers

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55
Q

What is the cause of physiologic anemia of infancy?

A

Low erythropoietin production

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56
Q

What is the normal MCV for babies and toddlers?

A

70-90

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57
Q

What are 4 major causes of microcytic anemia in children ?

A

Iron deficiency
Thalassemia
Anemia of chronic illness
Lead poisoning

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58
Q

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

A

Thalassemia major

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59
Q

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

A

Thickened bone on X-ray

Skull with “hair on end” appearance - secondary to extramedullary hematopoiesis

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60
Q

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

A

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

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61
Q

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

A

Cholelithiasis

Hemosiderosis

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62
Q

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

A

High hemoglobin F

High hemoglobin S

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63
Q

How long should iron deficiency anemia by treated?

A

Until 2 months after hgb levels are normal

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64
Q

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

A

Nutritional - low amount of iron is absorbed from cows milk

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65
Q

When is IV iron the correct treatment for anemia?

A

Only if child is Unable to take PO

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66
Q

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

A

RDW is high in iron deficiency

RDW is low in thalassemia

67
Q

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

A

Ferritin is high, TIBC is low

68
Q

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

A

Iron deficiency

69
Q

What is the appropriate treatment for anemia of chronic disease ?

A

Treatment of the underlying disease

Remember that patient will already have adequate iron stores

70
Q

What is the gold standard for diagnosis of lead poisoning?

A

Whole blood lead level

71
Q

What is this treatment for lead poisoning?

A

Chelation - penicillamine, dimercaprol and EDTA

72
Q

When is the FEP (free erythrocyte protoporphyrin) elevated?

A

In lead poisoning and iron deficiency

73
Q

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

A

Ringed sideroblasts

74
Q

What type of anemia is caused by drinking goats milk?

A

Macrocytic anemia secondary to folate deficiency

75
Q

What is the test that determines B12 absorption ?

A

Schilling test

76
Q

What are 4 causes of B12 deficiency ?

A

Intrinsic factor
Bacterial overgrowth
Bowel resection
Infant of vegetarian mother

77
Q

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

A

Pernicious anemia

78
Q

What is the cause of pernicious anemia?

A

Lack of intrinsic factor leads to impaired b12 absorption

79
Q

What is the treatment of pernicious anemia?

A

IM cobalamin (aka B12)

80
Q

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

A

Erythrocyte folic acid concentration is preferred over serum folate levels

*b12 deficiency must be ruled out before treatment!

81
Q

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

A

G6PD deficiency

82
Q

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

A

G6PD deficiency

83
Q

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

A

Hemosiderin and bilirubin in urine

Low serum haptoglobin

84
Q

When should G6PD testing be performed and why?

A

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

85
Q

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

A

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

86
Q

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

A

Spectrin deficiency

87
Q

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

A

Hereditary spherocytosis

88
Q

Why does hereditary spherocytosis cause hemolysis?

A

Defective red cell surface with fragile cells that break easily

89
Q

What is the diagnostic method for hereditary spherocytosis?

A

Osmotic fragility test

90
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

91
Q

What is the treatment (3) for hereditary spherocytosis?

A

Folic acid
Transfusion PRN
Splenectomy

92
Q

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

A

Parvovirus

93
Q

What amino acid substitution occurs in sickle cell anemia ?

A

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

94
Q

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

A

Pneumococcal vaccine at age 2

95
Q

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

A

Sequestration crisis due to pooling of blood in liver and spleen

96
Q

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

A

Sickle cell anemia

97
Q

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

A

Hemoglobin SC or

Sickle cell plus thalassemia

98
Q

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?

A

ABG

99
Q

What is the treatment for acute chest syndrome ?

A

Transfusion of pRBC or exchange transfusion

100
Q

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

A

Exchange transfusion followed by MRI

101
Q

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

A

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

102
Q

How can u differentiate aplastic anemia from acute leukemia?

A

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

103
Q

What side effect can occur w use of chloramphenicol ?

A

Aplastic anemia

104
Q

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

A

Fanconi anemia

105
Q

What orthopedic problems are common in children with fanconi anemia?

A

Growth retardation

Absent/hypoplastic thumb

106
Q

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

A

AML or myelodysplastic syndrome

107
Q

What is the cure for aplastic anemia?

A

Bone marrow transplant

108
Q

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

A

Diamond Blackman anemia - presents in young infants and is chronic

Transient erythroblastopenia of childhood - presents in toddlers and self resolves

109
Q

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

A

Diamond blackfan

110
Q

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

A

Transient erythroblastopenia of childhood

111
Q

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

A

Diamond blackfan

112
Q

Which type of severe childhood anemia is treated with steroids ?

A

Diamond blackfan

113
Q

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

A

Hemolytic reaction

114
Q

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

A

Allergic reaction

115
Q

How is risk for febrile nonhemolytic transfusion reaction reduced?

A

Use of leukocyte filtered blood

116
Q

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

A

Chediak Higashi

117
Q

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

A

Chediak higashi

118
Q

What are 3 common pathogens in patients with chediak Higashi?

A

Staph aureus
Strep pyogenes
Pneumococcus

119
Q

How is chronic granulomatous disease diagnosed?

A

Nitroblue tetrazolium test measures oxidative burst

120
Q

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

A

Recurrent bacterial (staph and gram negative) and fungal infections

121
Q

What are 3 possible treatment options for chronic granulomatous disease?

A

Infection control
Interferon
Bone marrow transplant

122
Q

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

A

Leukocyte adhesion deficiency

123
Q

What types of infections are common in patients with neutropenia?

A

Gran negative ulcers of the mucosa

124
Q

What are 5 congenital neutropenias?

A 
Cyclic neutropenia
Kostmann syndrome (severe congenital neutropenia)
Benign neutropenia
Shwachman diamond
Chediak Higashi
125
Q

What are the two most common causes of acquired neutropenia?

A

Infection and drugs (macrolides)

126
Q

What is the inheritance pattern of cyclic neutropenia ?

A

Autosomal dominant

127
Q

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?

A

Cyclic neutropenia

128
Q

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

A

Clostridium perfringens

129
Q

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

A

Chronic benign neutropenia

130
Q

What is the inheritance pattern of kostmann agranulocytosis ?

A

Autosomal recessive

131
Q

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

A

Kostmann syndrome

132
Q

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

A

Shwachman Diamond

133
Q

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

A

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

134
Q

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

A

Causes abnormal platelet function. Platelet count will be normal.

135
Q

What is the first line treatment for ITP?

A

IVIG

136
Q

When should platelet transfusion by indicated in patients with ITP?

A

Platelets lower then 20,000 or active bleeding

137
Q

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

A

50% resolve within 3 months in younger children

Can become chronic problem in children older than 10y/o

138
Q

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

A

Intracranial hemorrhage

139
Q

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

A

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

140
Q

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

A

Henoch schonlein purpura (or other vasculites)

141
Q

What is anaphylaxtoid purpura?

A

Henoch Schonlein purpura

142
Q

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

A

Wiskott Aldrich Syndrome

143
Q

How do you distinguish wiskott Aldrich syndrome from ITP?

A

ITP has large platelets

WAS has small platelets

144
Q

What is the treatment of wiskott Aldrich ?

A

Bone marrow transplant

145
Q

What is the inheritance pattern of wiskott Aldrich?

A

X linked (only in boys)

146
Q

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

A

Kasabach Merritt Syndrome - risk for DIC

147
Q

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

A

Congenital factor deficiency

148
Q

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

A

PT is elevated

149
Q

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

A

2, 7, 9 and 10

150
Q

What lab value is abnormal in patients with hemophilia ?

A

Prolonged PTT

151
Q

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

A

Replacement of factors is indicated as soon as possible

152
Q

What is the inheritance pattern of hemophilia ?

A

X linked recessive

153
Q

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

A

TAR

154
Q

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

A

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

155
Q

What type of hemophilia typically presents in females?

A

None - they are X linked and only in males

156
Q

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

A

Factor 8

157
Q

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

A

Hemophilia

158
Q

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

A

Nerve compression - this is a medical emergency

159
Q

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?

A

Von willebrand - order Von willebrand factor activity

160
Q

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

A

Order factor 8 and 9 levels to rule out hemophilia

161
Q

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

A 
Intranasal DDAVP (stimate)
Factor 8 concentrate if major bleeding
162
Q

What is the inheritance pattern of Von willebrand disease?

A

Autosomal dominant

163
Q

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

A

Inhibits fibrinolytic to stop mucosal bleeding

164
Q

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

A

DIC

pediatric Boards (23 decks)

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