Pediatric Hematology and Oncology

Question 1

Acute leukemia is defined as a neoplastic proliferation of blasts > […]% on bone marrow biopsy.

Answer 1

Acute leukemia is defined as a neoplastic proliferation of blasts > 25% on bone marrow biopsy

e.g. ALL is diagnosed by presence of > 25% lymphoblasts in the bone marrow

Question 2

Aplastic anemia may be caused by Fanconi anemia, which occurs due to a […] defect, resulting in bone marrow failure (pancytopenia).

Answer 2

Aplastic anemia may be caused by Fanconi anemia, which occurs due to a DNA repair defect, resulting in bone marrow failure (pancytopenia).

defect in nonhomologous end joining (double stranded break repair)

Question 3

Aplastic anemia may be caused by […] anemia, which occurs due to a DNA repair defect, resulting in bone marrow failure (pancytopenia).

Answer 3

Aplastic anemia may be caused by Fanconi anemia, which occurs due to a DNA repair defect, resulting in bone marrow failure (pancytopenia).

defect in nonhomologous end joining (double stranded break repair)

Question 4

Aplastic crisis is often seen in patients with sickle cell disease and is characterized by […] reticulocyte count.

Answer 4

Aplastic crisis is often seen in patients with sickle cell disease and is characterized by decreased reticulocyte count.

important distinguishing feature from splenic sequestration

Question 5

Aplastic crisis is often seen in patients with sickle cell disease and is characterized by […] platelet count.

Answer 5

Aplastic crisis is often seen in patients with sickle cell disease and is characterized by normal platelet count.

important distinguishing feature from splenic sequestration and aplastic anemia

Question 6

At baseline, patients with sickle cell disease have […] levels of HbF.

Answer 6

At baseline, patients with sickle cell disease have increased levels of HbF.

due to prolonged lifespan of HbF-containing RBCs

Question 7

Avascular necrosis of the femoral head results in pain on hip […] and […]. (movements).

Answer 7

Avascular necrosis of the femoral head results in pain on hip abduction and internal rotation. (movements)

there is no erythema, swelling, or point tenderness, which helps differentiate avascular necrosis from other etiologies

Question 8

Children with sickle cell disease should take daily oral […] prophylaxis until 5 years of age.

Answer 8

Children with sickle cell disease should take daily oral penicillin prophylaxis until 5 years of age.

Question 9

Cytoplasmic aggregates of periodic acid Schiff (PAS) positive material are characteristic of […]-blasts.

Answer 9

Cytoplasmic aggregates of periodic acid Schiff (PAS) positive material are characteristic of lympho-blasts.

versus myeloblasts, which often have peroxidase positive granules and/or Auer rods

Question 10

Cytoplasmic aggregates of peroxidase positive granules and/or Auer rods are characteristic of […]-blasts.

Answer 10

Cytoplasmic aggregates of peroxidase positive granules and/or Auer rods are characteristic of myelo-blasts.

versus lymphoblasts, which often have aggregates of periodic acid Schiff (PAS) positive material

Question 11

Diamond-Blackfan anemia is associated with congenital anomalies, such as short stature, webbed neck, cleft lip, shield chest, and […] thumbs.

Answer 11

Diamond-Blackfan anemia is associated with congenital anomalies, such as short stature, webbed neck, cleft lip, shield chest, and triphalangeal thumbs.

suspect in children with the aforementioned anomalies and a macrocytic pure red cell aplasia

Question 12

Heinz bodies form due to precipitation of denatured […] within RBCs.

Answer 12

Heinz bodies form due to precipitation of denatured hemoglobin within RBCs

Hb sulfhydryl groups donate an electron to hydrogen peroxide, forming hemoglobin crosslinks

Question 13

How do the following laboratory values change in anemia of chronic disease?

Serum iron: […]

TIBC: […]

% Saturation: […]

Ferritin: […]

Answer 13

How do the following laboratory values change in anemia of chronic disease?

Serum iron: decreased

TIBC: decreased

% Saturation: decreased

Ferritin: increased

high ferritin and low TIBC help distinguish ACD from iron deficiency anemia

Question 14

How do the following laboratory values change in iron deficiency anemia?

Serum iron: […]

TIBC: […]

% Saturation: […]

Ferritin: […]

Answer 14

Serum iron: decreased

TIBC: increased

% Saturation: decreased

Ferritin: decreased

low ferritin and high TIBC help distinguish iron deficiency from anemia of chronic disease

Question 15

How do the following laboratory values change in sideroblastic anemia?

Serum iron: […]

TIBC: […]

% Saturation: […]

Ferritin: […]

Answer 15

Serum iron: increased

TIBC: decreased

% Saturation: increased

Ferritin: increased

“iron overload”

Question 16

In patients with sickle cell disease, chronic damage and autoinfarction typically causes functional asplenia by age […].

Answer 16

In patients with sickle cell disease, chronic damage and autoinfarction typically causes functional asplenia by age 5.

Question 17

Iron deficiency anemia can be differentiated from thalassemia by an elevated […].

Answer 17

Iron deficiency anemia can be differentiated from thalassemia by an elevated red cell distribution width (RDW).

typically > 20% in iron deficiency

Question 18

Iron deficiency anemia in children is often caused by excess consumption of […].

Answer 18

Iron deficiency anemia in children is often caused by excess consumption of cow’s milk (> 24 ounces/day).

Question 19

Osteosarcoma arises in the […] of long bones, often around the knee.

Answer 19

Osteosarcoma arises in the metaphysis of long bones, often around the knee

i.e. distal femur or proximal tibia

Question 20

Polycythemia in term neonates is defined as a hematocrit > […]%.

Answer 20

Polycythemia in term neonates is defined as a hematocrit > 65%.

risk factors include delayed cord clamping, maternal hypertension, and maternal diabetes mellitus

Question 21

Pure red cell aplasia is associated with […], lymphocytic leukemias, and parvovirus B19 infection.

Answer 21

Pure red cell aplasia is associated with thymoma, lymphocytic leukemias, and parvovirus B19 infection.

severe anemia with normal granulopoiesis and thrombopoiesis

Question 22

Risk factors for neonatal polycythemia include delayed cord clamping, maternal […], and maternal […].

Answer 22

Risk factors for neonatal polycythemia include delayed cord clamping, maternal hypertension, and maternal diabetes mellitus.

Question 23

Splenectomy is associated with a long-term risk for sepsis with […] bacteria.

Answer 23

Splenectomy is associated with a long-term risk for sepsis with encapsulated bacteria.

risk is present for up to 30 years, and perhaps longer

Question 24

Splenic sequestration is characterized by normocytic anemia with […] reticulocyte count.

Answer 24

Splenic sequestration is characterized by normocytic anemia with increased reticulocyte count.

important distinguishing feature from aplastic crisis

Question 25

Splenic sequestration is characterized by [...] platelet count.

Answer 25

Splenic sequestration is characterized by **decreased** platelet count. platelets are trapped in the spleen along with RBCs

Question 26

Thalassemia minor is characterized by [...] total RBC count and [...] RDW.

Answer 26

Thalassemia minor is characterized by **normal** total RBC count and **normal** RDW. important distinguishing feature from iron deficiency anemia

Question 27

The combination of a vascular tumor (e.g. hemangioma) and consumptive thrombocytopenia is indicative of [...] syndrome.

Answer 27

The combination of a vascular tumor (e.g. hemangioma) and consumptive thrombocytopenia is indicative of **Kasabach-Merritt** syndrome. large or rapidly-growing tumors may trap platelets causing severe thrombocytopenia Kasabach-Merritt syndrome is characterised by the combination of rapidly growing vascular tumour, thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy. The blood clotting disorder results from platelets and other clotting factors of the blood being used up within the tumor

Question 28

The diagnosis of sickle cell disease can be confirmed with [...].

Answer 28

The diagnosis of sickle cell disease can be confirmed with **hemoglobin electrophoresis**.

Question 29

The symptoms of Wiskott-Aldrich syndrome may be remembered with the mnemonic "WATER": W: Wiskott A: Aldrich T: [...] E: Eczema R: Recurrent (pyogenic) infections

Answer 29

The symptoms of Wiskott-Aldrich syndrome may be remembered with the mnemonic "WATER": W: Wiskott A: Aldrich T: **Thrombocytopenia (fewer and smaller platelets)** E: Eczema R: Recurrent (pyogenic) infections

Question 30

The symptoms of Wiskott-Aldrich syndrome may be remembered with the mnemonic "WATER": W: Wiskott A: Aldrich T: Thrombocytopenia (fewer and smaller platelets) E: [...] R: Recurrent (pyogenic) infections

Answer 30

W: Wiskott A: Aldrich T: Thrombocytopenia (fewer and smaller platelets) E: **Eczema** R: Recurrent (pyogenic) infections

Question 31

The symptoms of Wiskott-Aldrich syndrome may be remembered with the mnemonic "WATER": W: Wiskott A: Aldrich T: Thrombocytopenia (fewer and smaller platelets) E: Eczema R: [...]

Answer 31

W: Wiskott A: Aldrich T: Thrombocytopenia (fewer and smaller platelets) E: Eczema R: **Recurrent (pyogenic) infections**

Question 32

What bone tumor is characterized by a "sunburst" pattern and Codman triangle on X-ray?

Answer 32

Osteosarcoma typically presents with pain and soft-tissue swelling without systemic symptoms

Question 33

What demographic (age group) is most commonly associated with acute lymphoblastic leukemia (ALL)?

Answer 33

Children versus AML, which typically arises in older adults

Question 34

What demographic (age group) is most commonly associated with acute myeloid leukemia (AML)?

Answer 34

Older adults (median onset is 65 years old) versus ALL, which typically arises in children

Question 35

What finding on blood smear is strongly suggestive of physical or functional hyposplenism (e.g. splenectomy)?

Answer 35

Howell-Jolly bodies fragments of nuclear materials in RBCs that may be seen with Wright stain; normally removed by splenic macrophages

Question 36

What hematologic pathology is characterized by normocytic anemia with a low reticulocyte count in preterm infants?

Answer 36

Anemia of prematurity due to diminished EPO levels, short RBC life span, and frequent phlebotomy in the NICU; diagnosis of exclusion

Question 37

What hematologic pathology is characterized by profound hypocellularity with fatty infiltration on bone marrow biopsy?

Answer 37

Aplastic anemia

Question 38

What hematologic pathology is characterized by thrombocytopenia, MAHA, and acute renal failure following a diarrheal illness?

Answer 38

Hemolytic uremic syndrome typically due to endothelial damage by drugs or infection; similar clinical presentation to TTP

Question 39

What inclusion body is often found inside the RBCs of patients with G6PD deficiency?

Answer 39

Heinz bodies visible on peripheral smear after using a crystal violet dye

Question 40

What inherited hematologic pathology is associated with an increased MCHC?

Answer 40

Hereditary spherocytosis i.e. mean corpuscular hemoglobin concentration; elevated due to loss of RBC surface area

Question 41

What inherited hematologic pathology is associated with avascular necrosis (osteonecrosis)?

Answer 41

Sickle cell disease patients typically present with chronic pain without signs of inflammation (e.g. warmth, erythema, leukocytosis, elevated ESR)

Question 42

What is the dose-limiting side effect of hydroxyurea?

Answer 42

Myelosuppression e.g. neutropenia, anemia, thrombocytopenia

Question 43

What is the likely diagnosis in a 5-day-old baby that did not receive perinatal care and presents with easy bruisability with an elevated prothrombin time?

Answer 43

Vitamin K-deficient bleeding previously known as hemorrhagic disease of the newborn; deficiency occurs due to combination of poor placental transfer, absent gut flora, immature liver function, and inadequate levels of vitamin K in breast milk

Question 44

What is the likely diagnosis in a child that presents with bitemporal hemianopsia and diabetes insipidus with calcified pituitary mass on imaging?

Answer 44

Craniopharyngioma

Question 45

What is the likely diagnosis in a child that presents with scattered petechiae with isolated thrombocytopenia and enlarged platelets following a viral infection?

Answer 45

Immune thrombocytopenia (ITP) most common occurs in children age 2 - 5 following a viral infection

Question 46

What is the likely diagnosis in a child that presents with short stature, hypoplastic thumbs, and aplastic, macrocytic anemia?

Answer 46

Fanconi anemia

Question 47

What is the likely diagnosis in a child with a family history of anemia that presents with Coombs-negative hemolytic anemia, jaundice, and splenomegaly?

Answer 47

Hereditary spherocytosis

Question 48

What is the likely diagnosis in a child with a history of sickle cell disease that presents with acute, severe anemia in the setting of a low reticulocyte count and no hepatosplenomegaly?

Answer 48

Aplastic crisis characterized by a sudden halt in RBC production (not hemolysis); lack of pancytopenia differentiates aplastic crisis from aplastic anemia

Question 49

What is the likely diagnosis in a child with a history of sickle cell disease that presents with hypovolemic shock in the setting of LUQ tenderness and splenomegaly?

Answer 49

Splenic sequestration hypovolemic shock occurs due to pooling of RBCs within the spleen

Question 50

What is the likely diagnosis in a well-appearing child that presents with a microcytic, hypochromic anemia with normal RBC count and RDW?

Answer 50

Thalassemia minor normal RBC count and RDW help distinguish thalassemia from iron deficiency anemia (low RBC count with high RDW)

Question 51

What is the likely diagnosis in an adolescent with a history of hemophilia A that presents with gradually worsening pain and limited motion of his knee?

Answer 51

Hemophilic arthropathy due to hemosiderin deposition leading to synovitis and fibrosis within the joint; risk is significantly reduced with prophylactic factor concentrates

Question 52

What is the likely diagnosis in an African American infant that presents with symmetric swelling/tenderness of the bilateral hands and feet?

Answer 52

Dactylitis (secondary to sickle cell disease) also known as hand-foot syndrome; usually presents between 6 months to 4 years of age

Question 53

What is the most common cause of pediatric stroke?

Answer 53

Sickle cell disease

Question 54

What is the most common complication of sickle cell trait?

Answer 54

Painless hematuria (microscopic or gross) due to sickling in the renal medulla; isosthenuria (inability to concentrate urine) is common as well, and manifests as nocturia and polyuria

Question 55

What is the most common trigger of aplastic crisis in patients with sickle cell disease?

Answer 55

Parvovirus B19 infection

Question 56

What is the next step in management for a child with suspected immune thrombocytopenia that presents with mucosal bleeding?

Answer 56

IVIg or corticosteroids

Question 57

What is the next step in management for a child with suspected immune thrombocytopenia that presents with scattered petechiae throughout the body?

Answer 57

Observation in children with ITP, skin manifestations require observation regardless of platelet count, as ITP is generally self-limiting

Question 58

What is the recommended treatment for symptomatic neonates with polycythemia (e.g. respiratory distress, hypoglycemia)?

Answer 58

Partial exchange transfusion blood is removed in exchange for normal saline to normalize the hematocrit; asymptomatic infants may be managed with rehydration (e.g. feeding, parenteral fluids)

Question 59

What is the treatment for severe or refractory hereditary spherocytosis?

Answer 59

Splenectomy most cases can be managed with supportive care (e.g. folic acid supplementation, blood transfusions as needed)

Question 60

What non-megaloblastic macrocytic anemia presents with rapid-onset anemia within the 1st year of life?

Answer 60

Diamond-Blackfan anemia (also known as congenital pure red cell aplasia or congenital hypoplastic anemia) due to intrinsic defect in erythroid progenitor cells with normal WBC and platelet counts; patients may have other abnormalities including triphalangeal thumbs

Question 61

What three vaccines should patients receive prior to undergoing splenectomy?

Answer 61

anti-pneumococcal, Haemophilus, and meningococcal vaccines due to risk of overwhelming sepsis; patients should also have daily oral penicillin prophylaxis until 5 years of age or for 3 - 5 years after splenectomy

Question 62

Which coagulation disorder presents with hemarthroses, easy bruising, and bleeding after surgery?

Answer 62

Hemophilia clinical severity depends on degree of deficiency

Question 63

Wiskott-Aldrich syndrome is characterized by inability for leukocytes and platelets to reorganize their [...] cytoskeleton.

Answer 63

Wiskott-Aldrich syndrome is characterized by inability for leukocytes and platelets to reorganize their **actin** cytoskeleton. results in defective antigen presentation

Question 64

[...] is an anemia due to decreased synthesis of globin chains.

Answer 64

**Thalassemia** is an anemia due to decreased synthesis of globin chains.

Question 65

Thalassemia is an anemia due to decreased synthesis of [...].

Answer 65

Thalassemia is an anemia due to decreased synthesis of **globin chains**.

Question 66

Hemolytic uremic syndrome is classically seen in children with [...] dysentery, which results from exposure to undercooked beef.

Answer 66

Hemolytic uremic syndrome is classically seen in children with **E. coli O157:H7** dysentery, which results from exposure to undercooked beef. ## Footnote Shiga toxin induces endothelial damage in renal glomeruli, causing platelet activation with microthrombi formation and microangiopathic hemolytic anemia (MAHA)

Question 67

[...] syndrome is classically seen in children with E. coli O157:H7 dysentery, which results from exposure to undercooked beef.

Answer 67

**Hemolytic uremic** syndrome is classically seen in children with E. coli O157:H7 dysentery, which results from exposure to undercooked beef. Shiga toxin induces endothelial damage in renal glomeruli, causing platelet activation with microthrombi formation and microangiopathic hemolytic anemia (MAHA)

Question 68

Hereditary spherocytosis is diagnosed by the [...] test in combination with eosin-5-maleimide binding test (flow cytometry).

Answer 68

Hereditary spherocytosis is diagnosed by the **acidified glycerol lysis fragility** test in combination with eosin-5-maleimide binding test (flow cytometry). reveals increased spherocyte fragility in hypotonic solution

Question 69

[...] is diagnosed by the acidified glycerol lysis fragility test in combination with eosin-5-maleimide binding test (flow cytometry).

Answer 69

**Hereditary spherocytosis** is diagnosed by the acidified glycerol lysis fragility test in combination with eosin-5-maleimide binding test (flow cytometry). ## Footnote reveals increased spherocyte fragility in hypotonic solution

Question 70

Hydroxyurea is useful in the treatment of sickle cell anemia because it increases levels of [...] (typically greater than 15%).

Answer 70

Hydroxyurea is useful in the treatment of sickle cell anemia because it increases levels of **HbF** (typically greater than 15%). increased HbF dilutes the amount of HbS, thus decreasing pain crises, the need for transfusions, and episodes of acute chest syndrome

Question 71

[...] is useful in the treatment of sickle cell anemia because it increases levels of HbF (typically greater than 15%).

Answer 71

**Hydroxyurea** is useful in the treatment of sickle cell anemia because it increases levels of HbF (typically greater than 15%). increased HbF dilutes the amount of HbS, thus decreasing pain crises, the need for transfusions, and episodes of acute chest syndrome

Question 72

Lympho-blasts are characterized by positive nuclear staining for [...], which is a DNA polymerase.

Answer 72

Lympho-blasts are characterized by positive nuclear staining for **TdT**, which is a DNA polymerase. marker of pre-T and pre-B lymphoblasts; absent in myeloid blasts and mature blasts

Question 73

[...]-blasts are characterized by positive nuclear staining for TdT, which is a DNA polymerase.

Answer 73

**Lympho**-blasts are characterized by positive nuclear staining for TdT, which is a DNA polymerase. marker of pre-T and pre-B lymphoblasts; absent in myeloid blasts and mature blasts

Question 74

Do spherocytes persist following splenectomy in a patient with hereditary spherocytosis?

Answer 74

Yes life-threatening anemia resolves (mild anemia usually persists)

Question 75

Early stages of iron deficiency cause a [...] (MCV) anemia.

Answer 75

Early stages of iron deficiency cause a **normocytic** (MCV) anemia. bone marrow makes fewer, but normal-sized, RBCs

Question 76

Ewing sarcoma arises in the [...] of long bones; other locations include pelvis, scapula, and ribs.

Answer 76

Ewing sarcoma arises in the **diaphysis** of long bones; other locations include pelvis, scapula, and ribs.

Question 77

Fanconi anemia is associated with an increased risk of developing [...].

Answer 77

Fanconi anemia is associated with an increased risk of developing **cancer** (e.g. tumors, leukemia).

Question 78

Folate and vitamin B12 deficiency are characterized by [...] (MCV) RBCs.

Answer 78

Folate and vitamin B12 deficiency are characterized by **macrocytic** (MCV) RBCs.

Question 79

Folate deficiency develops within [...], as body stores are minimal.

Answer 79

Folate deficiency develops within **months**, as body stores are minimal. versus B12 deficiency, which takes years to develop due to adequate stores

Question 80

Folate deficiency is characterized by [...] serum methylmalonic acid.

Answer 80

Folate deficiency is characterized by **normal** serum methylmalonic acid. important distinguishing feature from vitamin B12 deficiency

Question 81

Giant cell tumor arises in the [...] of long bones, often in the knee region.

Answer 81

Giant cell tumor arises in the **epiphysis** of long bones, often in the knee region.

Question 82

Giant cell tumor is associated with a "[...]" appearance on X-ray.

Answer 82

Giant cell tumor is associated with a "soap bubble" appearance on X-ray.

Question 83

Hemophilia (A, B, C) presents with [...] PT and [...] PTT.

Answer 83

Hemophilia (A, B, C) presents with **normal** PT and **increased** PTT. instrinsic pathway coagulation defect

Question 84

Hemophilia A is a coagulation disorder due to a genetic factor [...] deficiency.

Answer 84

Hemophilia A is a coagulation disorder due to a genetic factor **VIII** deficiency.

Question 85

Hemophilia B (Christmas disease) is a coagulation disorder due to a genetic factor [...] deficiency.

Answer 85

Hemophilia B (Christmas disease) is a coagulation disorder due to a genetic factor IX deficiency.

Question 86

Imaging of osteosarcoma may reveal a "[...]" pattern on X-ray.

Answer 86

Imaging of osteosarcoma may reveal a **"sunburst"** pattern on X-ray.

Question 87

Imaging of osteosarcoma may reveal elevation of periosteum, which is known as [...].

Answer 87

Imaging of osteosarcoma may reveal elevation of periosteum, which is known as **Codman triangle**.

Question 88

Impaired division of granulocytic precursors in megaloblastic anemia leads to [...] neutrophils.

Answer 88

Impaired division of granulocytic precursors in megaloblastic anemia leads to **hypersegmented** neutrophils. e.g. folate and vitamin B12 deficiency

Question 89

In addition to symptoms of anemia, iron deficiency also presents with [...] and koilonychia (spoon nails).

Answer 89

In addition to symptoms of anemia, iron deficiency also presents with **pica** and koilonychia (spoon nails). pica is the consumption of non-food substances; image shows koilonychia

Question 90

In addition to symptoms of anemia, iron deficiency also presents with pica and [...].

Answer 90

In addition to symptoms of anemia, iron deficiency also presents with pica and **koilonychia (spoon nails)**. pica is the consumption of non-food substances; image shows koilonychia

Question 91

In hemophilia A, mixing normal plasma with the patient's plasma (mixing study) [...] correct the PTT. (does or doesn't)

Answer 91

In hemophilia A, mixing normal plasma with the patient's plasma (mixing study) **does** correct the PTT. (does or doesn't) normal plasma replaces the deficient factor VIII

Question 92

Iron deficiency anemia is associated with a(n) [...] red cell distribution width (RDW).

Answer 92

Iron deficiency anemia is associated with a(n) **increased** red cell distribution width (RDW). think of the initial normocytic anemia followed by microcytic anemia

Question 93

Later stages of iron deficiency cause a [...] (MCV) anemia.

Answer 93

Later stages of iron deficiency cause a **microcytic** (MCV) anemia. bone marrow makes smaller and fewer RBCs

Question 94

One cause of folate deficiency is increased demand, which is often seen with pregnancy, [...] anemia, and cancer.

Answer 94

One cause of folate deficiency is increased demand, which is often seen with pregnancy, **hemolytic** anemia, and cancer. other causes include drugs (e.g. folate antagonists) and malnutrition (e.g. alcoholics)

Question 95

Osteochondroma arises as a lateral projection of the [...].

Answer 95

Osteochondroma arises as a lateral projection of the **growth plate (metaphysis).**

Question 96

Spherocytes are less able to maneuver through splenic sinusoids and are thus consumed by [...].

Answer 96

Spherocytes are less able to maneuver through splenic sinusoids and are thus consumed by splenic **macrophages**. e.g. hereditary spherocytosis; results in anemia

Question 97

T-ALL usually presents in teenagers (age group) as a [...] mass, thus it is often referred to as acute lymphoblastic lymphoma.

Answer 97

T-ALL usually presents in teenagers (age group) as a **thymic** mass, thus it is often referred to as acute lymphoblastic lymphoma. presents as SVC-like syndrome

Question 98

The peak incidence for primary osteosarcoma is in [...].

Answer 98

The peak incidence for primary osteosarcoma is in **teenagers (10-20 years old)**. most common primary malignancy of bone

Question 99

The peak incidence for secondary osteosarcoma is in [...].

Answer 99

The peak incidence for secondary osteosarcoma is in the **elderly (\>65 years old).** e.g. secondary to Paget disease of bone and/or radiation

Question 100

The treatment for refractory cases of ITP is [...].

Answer 100

The treatment for refractory cases of ITP is **splenectomy**. eliminates both the source of the antibody and the site of platelet destruction

Question 101

Treatment of hemophilia A involves recombinant factor VIII or [...]. (for mild cases)

Answer 101

Treatment of hemophilia A involves recombinant factor VIII or **desmopressin**. (for mild cases) hemophilia B and C are treated similarly with recombinants to their respective factor deficiencies

Question 102

What inherited cancers (2) are associated with osteosarcoma?

Answer 102

Li-Fraumeni syndrome and familial retinoblastoma

Question 103

What is the mode of inheritance of hemophilia A and B?

Answer 103

X-linked recessive

Question 104

What is the most common tumor of the adrenal medulla in children?

Answer 104

Neuroblastoma

Question 105

What pediatric tumor characterized by increased levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA) in the urine?

Answer 105

Neuroblastoma

Question 106

What tumor is associated with opsoclonus-myoclonus syndrome?

Answer 106

Neuroblastoma sometimes referred to as "dancing eyes, dancing feet"

Question 107

Which bone tumor arises in the epiphysis of long bones?

Answer 107

Giant cell tumor

Question 108

Which bone tumor is associated with a "soap-bubble" appearance on X-ray?

Answer 108

Giant cell tumor

Question 109

[...] leukemia is associated with the t(12;21) translocation.

Answer 109

**Acute lymphoblastic** leukemia is associated with the t(12;21) translocation. specifically the B-ALL subtype

Question 110

[...] is due to an inherited defect of RBC cytoskeleton-membrane tethering proteins.

Answer 110

**Hereditary spherocytosis** is due to an inherited defect of RBC cytoskeleton-membrane tethering proteins. e.g. spectrin, ankyrin

Question 111

[...] is due to autoimmune production of IgG against GPIIb/IIIa.

Answer 111

**Immune thrombocytopenic purpura (ITP)** is due to autoimmune production of IgG against GPIIb/IIIa.

Question 112

Immune thrombocytopenic purpura (ITP) is due to autoimmune production of IgG against [...].

Answer 112

Immune thrombocytopenic purpura (ITP) is due to autoimmune production of IgG against **GPIIb/IIIa**.

Question 113

[...] is a benign bone tumor with an overlying cartilage cap.

Answer 113

**Osteochondroma** is a benign bone tumor with an overlying cartilage cap.

Question 114

Ewing sarcoma is a bone tumor that is associated with t([...];[...]) translocation.

Answer 114

Ewing sarcoma is a bone tumor that is associated with t(**11;22**) translocation. causes a fusion protein (EWS-FLI-1)

Question 115

[...] is a bone tumor that is associated with t(11;22) translocation.

Answer 115

**Ewing sarcoma** is a bone tumor that is associated with t(11;22) translocation. causes a fusion protein (EWS-FLI-1)

Question 116

Fanconi anemia is associated with short stature, café au lait spots, and malformed forearms and/or [...].

Answer 116

Fanconi anemia is associated with short stature, café au lait spots, and malformed forearms and/or **thumbs.**

Question 117

[...] anemia is associated with short stature, café au lait spots, and malformed forearms and/or thumbs.

Answer 117

**Fanconi** anemia is associated with short stature, café au lait spots, and malformed forearms and/or thumbs.

Question 118

What is inhibitor development in Factor VII deficiency?

Answer 118

People with hemophilia, and many with VWD type 3, use treatment products called clotting factor concentrates (“factor”). These treatment products improve blood clotting, and they are used to stop or prevent a bleeding episode. When a person develops an inhibitor, the body stops accepting the factor treatment product as a normal part of blood. The body thinks the factor is a foreign substance and tries to destroy it with an inhibitor. The inhibitor keeps the treatment from working which makes it more difficult to stop a bleeding episode. A person who develops an inhibitor will require special treatment until their body stops making inhibitors. Inhibitors most often appear during the first 50 times a person is treated with clotting factor concentrates, but they can appear at any time. ## Footnote * prolonged PTT * normal PT