HEMA ONCO Flashcards

1
Q

It is the most common extracranial solid tumor in children

Astrocytoma
Craniopharyngioma
Medulloblastoma
Neuroblastoma

A

Neuroblastoma

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1
Q

Of the following, the LEAST likely feature of acute immune thrombocytopenia (ITP) would be:

a. a preceding viral infection ,
b. isolated thrombocytopenia c.mucocutaneous bleeding
d. Isolated splenomegaly
e. increased number of megakaryocytes in the
bone marrow

A

d. Isolated splenomegaly

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2
Q

True Hodgkin Lymphoma except:

is the most common cancer seen in adolescents
Associated with Epstein-Barr virus (EBV)
The Reed-Sternberg (RS) cell, a pathognomonic feature
None of the above

A

None of the above

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3
Q

To diagnose Acute Lymphoblastic Leukemia, this feature alone is adequate.

Morphology
Signs and symptoms
Chromosomal abnormalities
Diagnostic procedures

A

Morphology

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4
Q

This is the form of Von Willebrand disease that presents symptoms similar to those seen in mild hemophilias

Type 1 VWD
Type 2A VWD
Type 2B VWD
Type 3 VWD

A

Type 3 VWD

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5
Q

A 16-year-old female is found to have multiple bruising and hypochromic microcytic anemia; during her first pregnancy she noticed disappearance of the bruises; she has a past history of
uncomplicated emergency appendectomy at the age of 10 year. Of the following, the drug MOST likely beneficial for alleviation of her symptoms would be:

Ferrous sulphate
Aminocaproic acid
Recombinant factor VIII
Recombinant factor IX
Desmopressin acetate (DDAVP)

A

Desmopressin acetate (DDAVP)

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6
Q

Splenectomy is recommended in all the following conditions EXCEPT:

a. an 8-year old child with hereditary stomatocytosis with hemolysis
b. a 6-year-old child with thalassemia major with splenomegaly and
frequent blood transfusion
requirement
c. a 6-year-old child with hereditary spherocytosis and significant hemolysis
d. a 7-year-old child with hereditary elliptocytosis and a hemoglobin level of 7g/dL and corrected reticulocytes count of more than 15%
e. a 12-year-old child with chronic immune thrombocytopenia not
responding to all modalities of
medical treatment

A

a. an 8-year old child with hereditary stomatocytosis with hemolysis

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7
Q

This is the valuable diagnostic feature that differentiate anemia of chronic disorder from iron deficiency anemia :

leukocytosis
Low serum iron
Low or normal serum transferrin
Normal bone marrow cellularity
Normochromic normocytic RBC

A

Low or normal serum transferrin

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8
Q

Vitamin K-dependent clotting factors include all the following EXCEPT

Factor I
Factor II
Factor VII
Factor IX
Protein S

A

Factor I

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9
Q

Transfusion of fresh frozen
plasma (FFP) is efficacious for the
treatment of deficiency of all the
following coagulation factors
EXCEPT:

A. factor V
B. factor X
C. factor XI
D. factor XIII
E. protein C

A

D. factor XIII

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10
Q

The following conditions may be
associated with iron deficiency
EXCEPT one:

A. celiac disease
B. hookworm infestation
C. prolonged intravascular
hemolysis
D. congenital heart disease
with right to left shunt
E. prolonged use of isoniazid
(INH)

A

E. prolonged use of isoniazid
(INH)

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11
Q

This is the mainstay of treatment for congenital hypoplastic anemia:

A. Androgen
B. corticosteroids
C. antithymocyte globulin
(ATG)
D. fully matched-related stem
cell transplantation fully
matched-unrelated stem cell
E. transplantation

A

B. corticosteroids

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12
Q

One of the ff/s is a physiologic
adaptation to anemia

a. Tachycardia
b. Decrease Arteria-Venous
oxygen diferrence
c. Shift to the Left of the
oxygen dissociation curve
d. All of the above

A

a. Tachycardia

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13
Q

Anemia of inflammation is also
referred to this type of anemia..

a. Fanconi syndrome
b. Pearson syndrome
c. Transient anemia of
childhood
d. Anemia of chronic
disease

A

d. Anemia of chronic
disease

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14
Q

A 5-year-old child, with
hemophilia A of severe type,
presents to the emergency
unit with a groin pain after a minor
trauma to his back; his blood
pressure is 60/30 mm Hg; his pulse rate is 180/min; he holds his right hip in a flexion position with internal rotation.Of the.following, the NEXT step in the management of this child would be:

A. factor VIII replacement therapy
B. abdominal ultrasonography
C. abdominal computed
tomography (CT) scan
D. intravenous 1-deamino-8-d-arginine
vasopressin (DDAVP)
factor VIII assay

A

A. factor VIII replacement therapy

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15
Q

Which of the following is the
best treatment for a 5-year-old girl
with acute immune thrombocytopenia (ITP) and platelets count of 30,000/ml
and mild petechial rash all over her body?

a. corticosteroids
b. anti-D gamma globulin
c. platelets transfusion
d. intravenous
immunoglobulin (IVIG)
e. no treatment

A

e. no treatment

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16
Q

All the following may occur as
a renal manifestation in a child with
sickle cell anemia EXCEPT:

A. polyuria
B. hematuria
C. hyposthenuria
D. nephrotic syndrome
E. acute renal injury

A

A. polyuria

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17
Q

All the following are true regarding Transient Erythroblastopenia of Childhood(TEC) EXCEPT

a. corticosteroid therapy is of no value
b. virtually all children recover within 1-2 months
c. Parvovirus B19 infections is a common causative agent
d. most of the affected children are older than 12 mo at onset
e. it is more common than congenital hypoplastic (Diamond-Blackfan)

A

c. Parvovirus B19 infections is a common causative agent

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18
Q

A 5-year old male was seen
with multiple ecchymosis all over
the extremities and trunk. On
examination reveal a palpable
spleen 3 cm below the left costal
margin. Lab findings include:
hemoglobin, 11.3 g/dL; white blood
cell count, 8700/mm3; platelets
count, 21000/mm3.Of the
following, the most proper NEXT
step is:

A. bleeding time
B. coagulation profile
C. bone marrow study
D. platelets aggregation test
E. abdominal ultrasonography

A

C. bone marrow study

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19
Q

A 3-year-old male child
develops hematoma and bruising
of his right hand next day after
falling on the ground; the mother
stated that her child has a
poorwound healing and a history of
delayed umbilical separation
during the neonatal period. Of the
following, the MOST valuable test
for this case is

a. bleeding time
b. prothrombin time
c. clot solubility test
d. partial thromboplastin time
e. thrombin time

A

c. clot solubility test

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20
Q

Spontaneous intracranial
hemorrhage is more likely to occur
in

a. Factor VII deficiency
b. Factor VIII deficiency
c. Factor IX deficiency
d. Factor XI deficiency
e. Factor XIII deficiency

A

a. Factor VII deficiency

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21
Q

A 9-month-old child with a
hemoglobin concentration of 10
gm/dL and marked microcytosis;
serum iron and total iron binding
capacity are within normal mits;serum ferritin and emoglobin electrophoresis are lso normal. Of the following,the
MOST likely diagnosis would be:

a. iron deficiency anemia
b. sideroblastic anemia
c. ß-thalassemia minor
d. α-thalassemia trait
e. anemia due to chronic

A

d. α-thalassemia trait

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22
Q

Fresh frozen plasma (FFP)
transfusion is the best treatment
for which of the following conditions

A. hemophilia a
B. hemophilia b
C. factor VII deficiency
D. immunoglobulin replacement therapy
E. bleeding due to warfarin therapy

A

E. bleeding due to warfarin therapy

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23
Q

True of Von Willebrand disease
is/are:

a. Thrombocytopenia
occasionally present
b. most common inherited bleeding
c. Treated with Desmopressin
d. All of the above

A

d. All of the above

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24
Q

In hemophilia A, factor VIII
level activity should be increased
to 100% in.

a. Epistaxis
b. Hematuria
c. gum bleeding
d. tooth extraction
e. iliopsoas bleeding

A

e. iliopsoas bleeding

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25
Q

A pathognomonic feature of Hodgkin’s lymphoma is:

A. Multinucleated cells
B. Reed-sternberg cell
C. Ann Arbor cells
D. B or T cells

A

B. Reed-sternberg cell

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26
Q

A 1-year-old child has folic acid deficiency since the age of 4 months, the best indicator of this deficiency would be:

a. decrease level of RBC folate
b. significant fall of reticulocytes count
c. high level of lactate dehydrogenase LDH
d. increase number of hypersegmented neutrophils
e. significant increment of mean corpuscular volume MCV

A

a. decrease level of RBC
folate

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27
Q

This is the MOST specific test that is helpful to differentiate Diamond-Blackfan Anemia from transient erythroblastopenia of childhood:

a. reticulocytes count
b. hemoglobin electrophoresis
c. bone marrow examination
d. mean corpuscular volume (MCV)
e. erythrocyte adenosine deaminase (ADA) enzyme level assay

A

e. erythrocyte adenosine
deaminase (ADA) enzyme level assay

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28
Q

True of Pediatric malignancies EXCEPT:

A. Epithelial Tumors most common
B. Usually associated with Ionizing radiation exposure and several chemotherapeutic agents
C. usually originate from the deeper, visceral structures
D. Npne of the above

A

A. Epithelial Tumors most
common

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29
Q

True of chemotherapy in children EXCEPT:

A. Sequential single-drug therapy is recommended
B. The most common acute adverse effects are myelosuppression
C. All of the above

A

A. Sequential single-drug therapy is recommended

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30
Q

Anemia due to decrease RBC production

a. Heriditary Spherocytosis
b. Thallasemia
c. Megaloblastic Anemia
d. None of the above

A

c. Megaloblastic Anemia

31
Q

The standard care for MOST children with severe hemophilia would be:

A. avoid trauma
B. avoid aspirin and other NSAID
C. avoid violent contact sports
D. aggressive treatment by F VIII replacement therapy when significant bleeding occurs
E. prevention by F VIII replacement therapy to prevent spontaneous
bleeding and early joint
deformities

A

E. prevention by F VIII
replacement therapy to
prevent spontaneous
bleeding and early joint
deformities

32
Q

This is the first laboratory marker in progressive iron deficiency anemia:

A. falling of serum ferritin
B. hypochromic microcytic anemia
C. decrease hemoglobin synthesis
D. depletion of bone marrow hemosiderin
E. decrease of serum iron and increase of the iron-binding capacity

A

D. depletion of bone marrow hemosiderin

33
Q

True of Childhood leukemias

a. ALL most common (77% of cases)
b. Genetic abnormalities in a hematopoietic cell give rise to an unregulated clonal proliferation of cells
c. 31% of all malignancies that occur in children younger than 15 yr
d. All of the above

A

d. All of the above

34
Q

This is the most common clinical presentation of children with Nephroblastoma

a. Fever
b. Abdominal pain
c. Abdominal mass
d. Easy fatigability

A

c. Abdominal mass

35
Q

The following are hematologic features of congenital hypoplastic anemia (Diamond-Blackfan Anemia) include all the following EXCEPT

a. Reticulocytopenia
b. normocytic anemia
c. elevated serum iron levels
d. deficiency or absence of red blood cell bone marrow precursors elevated fetal hemoglobin (Hb F)

A

b. normocytic anemia

36
Q

A healthy 5-mo-old boy appears pale. Examination is unremarkable. Lab findings include: Hb 8.1 g/dl; WBC
4,800/mm3; platelets 144,000/mm3; MCV, 111 fl; blood film showed hypersegmented neutrophils; serum B12, 65 pg/mL (low). The infant is vigorously breast fed. Of the following, your NEXT step of the management
would be:

a. performing Schilling test
b. transfuse packed RBCs
c. check serum B12 of the mother
d. parenteral administration of vitamin B12
e. administration of both folic acid and vitamin B12

A

c. check serum B12 of the mother

37
Q

The classic manifestation of
osteosarcoma is:

Sunburst pattern
Sun setting pattern
Cauliflower pattern
Onion-skinning pattern

A

Sunburst pattern

38
Q

You are evaluating a 4-year-old
child with ß-thalassemia major; he
is on chronic transfusion therapy
since the age of 1 year; you
suspect transfusion-induced
hemosiderosis. Of the following,
the organ that is LEAST likely to be
affected by iron deposition at this
time would be:

A. liver
B. heart
C. pancreas
D. pituitary gland
E. thyroid gland

A

B. heart

39
Q

An initial laboratory tests to be requested on a patient who comes in due to pallor EXCEPT:

A. WBC
B. Bone marrow smear exam
C. Reticulocyte count
D. Red cell indices

A

B. Bone marrow smear exam

40
Q

This is the most common anomaly in Fanconi anemia.

a. Short stature
b. Renal malformations
c. Skin pigment changes
d. Cardiopulmonary
abnormalities

A

C. Skin pigment changes

41
Q

The most common manifestation/s of Glucose-6-Phosphate Dehydrogenase
deficiency(G6PD) is/are:

a. RBCS are microcyic, normochromic
b. It has an autosomal recessive inheritance
c. Majority of patients are symptomatic
d. Patients present with jaundice and hemolytic anemia

A

d. Patients present with jaundice and hemolytic anemia

42
Q

Pediatric Cancer Management
involves:

a. Multimodal, Multidisciplinary Approach
b. Diagnostic imaging is a critical phase of evaluation
c. systemic multiagent chemotherapy usually is necessary
d. All of the above

A

d. All of the above

43
Q

One of the following is NOT a
subtype of NonHodgkin Lymphoma

a. Lymphocytic lymphoma
b. Burkitt’s lymphoma
c. Anaplastic large cell
d. Diffuse large cell lymphoma

A

a. Lymphocytic lymphoma

44
Q

Hodgkin lymphoma in children
usually has favorable outcome; however, the prognosis may be grim in some cases.Poor prognostic factors in HodgkinLymphoma include all the following EXCEPT

A. scan positivity
B. stage IV disease
C. presence of bulky
mediastinal mass
D. presence of “B” symptoms
E. age of more than 15 year at the time of the diagnosis
F. poor response to therapy
manifested by positron
emission tomography
(PET)

A

E. age of more than 15 year at the time of the diagnosis

45
Q

The most common primary malignant renal tumor in children is:
a. Wilm’s tumor
b. Neuroblastoma
c. Germ cell tumor
d. Teratoma

A

a. Wilm’s tumor

46
Q

Transient erythroblastopenia of
childhood has the following
feature.

A. It has familial predisposition
B. It requires regular blood transfusion
C. Anemia is evident by age 2-5months old
D. It is not associated with congenital anomalies

A

D. It is not associated with congenital anomalies

47
Q

This is the best assessment of
iron overload for patients with
thalassemia major:

A. Liver MRI
B. Serum iron
C. Serum ferritin
D. Bone marrow biopsy
E. Total iron binding
capacity

A

A. Liver MRI

48
Q

One of the ff is/are anemia
secondary to increase RBC
destruction :

A. low reticulocyte count
B. G6PD Deficiency
C. Physiologic anemia of the
newborn
D. All of the above

A

B. G6PD Deficiency

49
Q

True of Neuroblastoma is/are:

A. embryonal cancers of the peripheral sympathetic nervous
B. Crosses the midline of the abdomen
C. can result in Horner syndrome
D. All of the above

A

D. All of the above

50
Q

Physical Findings of anemia are the ff. EXCEPT:

a. Pallor in Hb = 10 g/dl
b. Flow murmur
c. Hepatosplenomegaly
d. None of the above

A

a. Pallor in Hb = 10 g/dl

51
Q

Microcytes are frequently seen in:

a. Thallasemia
b. Diamond Blackfan Anemia
c. Liver Diseases
d. Folate dificiency

A

a. Thallasemia

52
Q

True of Hemophilia B EXCEPT:

a. factor VIII deficiency
b. Hemarthrosis is the hallmark finding
c. X-linked traits
d. None of the above

A

a. factor VIII deficiency

53
Q

True of Idiopathic (Autoimmune)Thrombocytopenic Purpura is/are:

a. 1-4 wk after exposure to a common viral infection
b. Common initial manifestation - generalized petechiae and purpura
c. rarely with hepatosplenomegaly
d. All of the above

A

d. All of the above

54
Q

COAGULATION FACTORS

A

Factor I =Fibrinogen
Factor II =Prothrombin
Factor V =Labile Factor
Factor VII =Stable Factor
Factor VIII =Antihemophilic Factor
Factor IX =Christmas Factor
Factor X =Stuart Factor
Factor XI =Plasma Thromboplastin Antecedent
Factor XII =Hageman Factor
Factor XIII =Fibrin Stabilizing Factor

55
Q

One of the following presents with normocytic, normochromic anemia:

a. Iron deficiency
b. G6PD deficiency
c. Vitamin B12 deficiency
d. Congenital Aplastic anemia

A

b. G6PD deficiency

56
Q

One of the following is an acquired aplastic anemia.

a. Fanconi anemia
b. Pearson syndrome
c. Diamond-Blackman anemia
d. Transient erythroblastopenia of childhood

A

d. Transient erythroblastopenia of childhood

57
Q

One of the following characterizes Diamond-Blackfan anemia EXCEPT:

a. RBCs are macrocytic
b. Common in 2-6 months old
c. RBCS are microcytic, normochromic
d. Corticosteroids are the main therapy

A

c. RBCS are microcytic, normochromic

58
Q

Megaloblastic anemias include the following EXCEPT:

a. Iron deficiency anemia
b. Folate deficiency anemeia
c. Cobalamin deficiency anemia
d. Vitamin B12 deficiency anemia

A

a. Iron deficiency anemia

59
Q

It is the most common cause of inherited abnormality of the red blood cell membrane.

a. Hereditary spherocytosis
b. Hereditary elliptocytosis
c. Hereditary stomatocytosis
d. Paroxysmal nocturnal hemoglobinuria

A

a. Hereditary spherocytosis

60
Q

Thalassemia in a fetus with hydrops has most likely this form of hemoglobin.

a. Alpha 2
b. Aplha 4
c. Beta 2
d. Beta 4

A

b. Aplha 4

61
Q

Deficiency of this factor is one the common severe inherited bleeding disorders.

a. IX
b. X
c. XI
d. XII

A

a. IX

62
Q

Factor VIII deficiency is also known as: *

a. Hemophilia A
b. Hemophilia B
c. Hemophilia C
d. Pearson syndrome

A

a. Hemophilia A

63
Q

The most common cause of acute onset of thrombocytopenia in children is:

a. Idipathic thmbocytopenic purpura
b. Thrombotic thrombocytopenic purpura
c. drug-induced thrombocytopenia
d. Hemolytic uremic syndrome

A

a. Idipathic thmbocytopenic purpura

64
Q

The most common intraocular tumor in children is:

a. Retinoblastoma
b. Germ cell tumor
c. non-germ cell tumor
d. Germinoma

A

a. Retinoblastoma

65
Q

Hepatoblastoma/Hepatocellular carcinoma is commonly associated with hepatitis:

a. A
b. B
c. D
d. E

A

b. B

66
Q

Bone tumors include:

a. Wilm’s tumor
b. Ependymoma
c. Germonoma
d. Ewing’s sarcoma

A

d. Ewing’s sarcoma

67
Q

CNS tumors include:

a. Ependymoma
b. Ewing’s tumor
c. Wilm’s tumor
d. Germinoma

A

a. Ependymoma

68
Q

Replacement of intracellular iron enzymes occur at this time after iron administration

A. 12-24 hr
B. 36-48 hr
C. 48-72 hr
D. 4 days

A

A. 12-24 hr

B. 36-48 hr- Initial bone marrow response; erythroid hyperplasia
C. 48-72 hr- Reticulocytosis
D. 4 days- Increase in hemoglobin level

69
Q

The second most common malignancy in childhood and adolescence is

A. CNS tumors
B. Bone tumors
C. Renal tumors
D. Hepatomas

A

A. CNS tumors

???

70
Q

It is the most commonly diagnosed malignancy in infants

A. Astrocytoma
B. Neuroblastoma
C. Medulloblastoma
D. Craniopharyngioma

A

B. Neuroblastoma

71
Q

This the most common soft tissue sarcoma in children

A. Liposarcoma
B. Fibrosarcoma
C. Angiosarcoma
D. Rhabdomyosarcoma

A

D. Rhabdomyosarcoma

72
Q

The most common benign tumor in infancy is

A. Hemangioma
B. Lymphangioma
C. Cystic Hygromas
D. Neuromas

A

A. Hemangioma

73
Q

Adrenal tumors include the ff

A. Neuroma
B. Pheochromocytoma
C. Langerhans cytosis
D. Medulloblastoma

A

B. Pheochromocytoma

74
Q

EXAMPLE OF MICROCYTIC ANEMIA

A
75
Q

EXAMPLE OF NORMOCYTIC

A
76
Q

EXAMPLE OF MACROCYTIC

A