Pediatrics- hematologic and oncologic disorders

Question 1

Diagnostic criteria of severe aplastic anaemia, with the exception of:
A) absolute neutrophil count < 0,5 G/L
B) platelet count < 20 G/L
C) corrected reticulocyte ratio < 1%
D) absolute CD4-positive lymphocyte count < 0,2 G/l

Answer 1

D) absolute CD4-positive lymphocyte count < 0,2 G/l
EXPLANATION
Severe aplastic anaemia (SAA) is characterized by bone marrow failure involving each three lineages, i.e. the erythroid, the myeloid and the megakaryopoietic lineages. Accordingly, there is severe anaemia, neutropenia and thrombocytopenia present in the peripheral blood (A ,B ,C). Changes in lymphocyte subpopulations are not characteristic for SAA, decreased absolute CD4-positive lymphocyte count (D) is a diagnostic criterion of AIDS.

Question 2

Factors below may result in the development of aplastic anemia, with the exception of:
A) ionizing radiation
B) chloramphenicol
C) intravenous immunoglobulin preparations
D) parvovirus B19
E) hepatitis C virus

Answer 2

C) intravenous immunoglobulin preparations
EXPLANATION
Contemporary, virus-inactivated/eliminated intravenous immunoglobulin (IVIG) preparations obtained for screen donors do not result in aplastic anemia. Application of IVIG is recommended in aplastic anemia (SAA) caused by parvovirus B19 infection. All other factors listed maybe in a causative relationship with SAA, although the exact cause of bone marrow failure mains remains mostly unexplained, the SAA is most frequently idiopathic.

Question 3

Most frequent indications of allogeneic hematopoietic stem cell transplantation, with the exception of:
A) severe aplastic anemia
B) acute lymphoblastic leukemia in second remission
C) high-risk acute myeloid leukemia in first remission
D) high-risk acute lymphoblastic leukemia in first remission
E) thrombotic thrombocytopenic purpura

Answer 3

E) thrombotic thrombocytopenic purpura
EXPLANATION
Thrombotic thrombocytophenic purpura (TTP) is treated by plasmapheresis and not by allogeneic hematopoetic stem cell transplantation (HSCT). HSCT is indicated in the other conditions listed.

Question 4

The syndromes listed below are characterized by the association of hematopoietic and skeletal anomalies, with the exception of:
A) Diamond–Blackfan-anemia
B) Fanconi-anemia
C) „thrombocytopenia with absent radii” (TAR) syndrome
D) Beckwith-Wiedemann syndrome

Answer 4

D) Beckwith-Wiedemann syndrome
EXPLANATION
Conditions listed in points A, B, C represent different forms of constitutional hypoplastic anemias characterized by single-or multiple lineage bone marrow failure and frequently associated with skeletal anomalies. Bone marrow function is not affected in patients with Beckwith-Wiedemenn syndrome (D).

Question 5

In addition to the signs of bone marrow failure, Fanconi anemia is characterized by signs and symptoms listed below, with the exception of:
A) skeletal malformations
B) hyperpigmentation
C) oculocutaneous albinism
D) spontaneous and induced chromosome fragility

Answer 5

C) oculocutaneous albinism
EXPLANATION
Anomalies listed under points A and B are frequently associated with signs of bone marrow failure in Fanconi-anemia. Oculocutaneous albinism characterizes Hermansky-Publack syndrome, a platelet storage of disease (C). Chromosome fragility (D) is a pathognomonic characteristic of Fanconi-anemia.

Question 6

Average hemoglobin concentration in trimenon (‘physiological’) anemia:
A) 80 g/L
B) 114 g/L
C) 140 g/L
D) 155 g/L
E) 168 g/L

Answer 6

B) 114 g/L
EXPLANATION
The proper replay is B. A is characteristic for the late anemia of premature babies, C and D are the average hemoglobin concentrations of healthy adult females and males, respectively. A represents the average hemoglobin concentration in the umbilical blood of a healthy mature newborn.

Question 7

Clinical signs of iron deficiency anemia in infants and toddlers, with the exception of:
A) craniotabes
B) restlessness
C) psychomotor retardation
D) pale skin and mucous membranes
E) somatic retardation

Answer 7

A) craniotabes
EXPLANATION
Craniotabes (A) is a sign of rickets.

Question 8

Factors listed below may result in iron deficient anemia, with the exception of:
A) excessive cow milk diet in toddlers
B) celiac disease
C) periods of rapid growth (e.g. infancy and puberty)
D) hemolysis
E) blood loss

Answer 8

D) hemolysis
EXPLANATION
Hemolysis may be associated with iron overload, in particular if patient is transfusion-dependent (D).

Question 9

Hypochromic, microcytic anemias, with the exception of:
A) β-thalassemia
B) glucose-6-phosphate-dehydrogenase deficiency
C) lead poisoning
D) copper deficiency
E) iron deficiency

Answer 9

B) glucose-6-phosphate-dehydrogenase deficiency
EXPLANATION
Glucose-6-phosphate-dehydrogenase deficiency (B) results in normochromic normocytic anemia.

Question 10

Frequent signs and complications of hemolytic anemia, with the exception of:
A) jaundice
B) acholic feces (clay-colored stools)
C) early development of gallstones
D) dark urine with elevated urobilinogen level
E) splenomegaly

Answer 10

B) acholic feces (clay-colored stools)
EXPLANATION
The color of feces is usually darken than normal in patients with hemolytic anemia. Acholic faces (B) is characteristic for post hepatic jaundice.

Question 11

Signs of hemolytic crisis in case of severe intravascular hemolysis, with the exception of:
A) fever, fever chills accompanied by severe abdominal or flank pain
B) sudden onset and enhancement of jaundice and splenomegaly
C) shock with or without DIC
D) onset of hyperkalemia, oliguria, anuria
E) reticulocytopenia

Answer 11

E) reticulocytopenia
EXPLANATION
Hemolysis including hemolytic crisis is associated with elevated reticulocyte count as a sign of increased compensatory erythropoiesis and not with reticulocytopenia (E).

Question 12

Hemolytic diseases in children, with the exception of:
A) Evans-syndrome
B) glucose-6-phosphate-dehidrogenase deficiency
C) hereditary spherocytosis
D) Kasabach–Merritt-syndrome
E) methemoglobinemia

Answer 12

E) methemoglobinemia
EXPLANATION
Methemoglobinemia (E) does not result in hemolysis

Question 13

Characteristic clinical and biological findings in patients with hereditary spherocytosis, with the exception of:
A) increased osmotic fragility of the red cells due to cell membrane defect
B) presence of microspherocytes in the blood smear
C) both hemolytic and aplastic crisis may occur
D) microspherocytes will vanish from peripheral blood after splenectomy
E) hemolysis will stop after splenectomy

Answer 13

D) microspherocytes will vanish from peripheral blood after splenectomy
EXPLANATION
Decay of microspherocytes occurs in the spleen. Therefore, the presents of microspherocytes in the peripheral blood will increase after splenectomy (D), but the hemolytic anemia will improve because of a decreased rate of splenic eliminations of microspherocytes.

Question 14

Eosinophilia is frequently present in the conditions listed below, with the exception of:
A) allergic disorders
B) inflammatory bowel diseases
C) measles
D) scarlet fever
E) toxocariasis

Answer 14

C) measles
EXPLANATION
Among contagious childhood infections only scarlet fever is associated with eosinophilia (D); therefore, measles (C) is not characterized by eosinophilia. Conditions listed under A, B and E are associated with eosinophilia.

Question 15

Signs of hyperleukocytosis syndrome, with the exception of:
A) central nervous system bleeding
B) ARDS
C) polyuria
D) thrombosis
E) lung hemorrhage

Answer 15

C) polyuria
EXPLANATION
Hyperleukocytosis syndrome can be associated with oligo-anuria rather than with polyuria (C)

Question 16

Measures to be taken to prevent tumor-lysis syndrome, with the exception of:
A) start immediately full-dose combined cytostatic treatment
B) start single-agent low-dose chemotherapy
C) apply allopurinol in 300-400 mg/m2/day
D) use diuretics
E) hydration with 3000 ml/m2/day fluid intake

Answer 16

A) start immediately full-dose combined cytostatic treatment
EXPLANATION
The metabolic load (hyperuricemia, hyperkalemia and hyperphosphatemia) resulting from the rapid lysis of a big tumor burden may result in or increased the severity of the tumor lysis syndrome. Therefore, we don’t want to start full dose combined cytostatic treatment (A).

Question 17

The tumor lysis syndrome triad, with the exception of:
A) hyperbilirubinemia
B) hyperkalemia
C) hyperphosphatemia
D) hyperuricemia

Answer 17

A) hyperbilirubinemia
EXPLANATION
The triad of tumor lysis syndrome is hyperuricemia, hyperkalemia, hyperphosphatemia (D, B and C, respectively). Hyperbilirubinemia (A) is not characteristic for tumor lysis syndrome.

Question 18

Mark the proper answer. More frequently manifesting clinical signs in children with acute lymphoblastic leukemia than in children with acute myeloid leukemia:
A) chloroma
B) hemorrhagic hyperplasia of the gingiva
C) hepatosplenomegaly
D) bone and/or joint pain

Answer 18

D) bone and/or joint pain
EXPLANATION
Bone and/or joint pain is more frequent in children with acute lymphoblastic leukemia (ALL) than in children with acute myeloid leukemia (AML) (D). Hepatosplenomegaly (C) can be observed with similar frequency in ALL and AML. Chloroma (A) and hemorrhagic hyperplasia of the gingiva (B) are characteristic for AML.

Question 19

Platelet-type bleeding signs can be seen in conditions listed below, with the exception of:
A) acute leukemia
B) hemophilia A
C) immune thrombocytopenia
D) side effect of salicylate treatment
E) von Willebrand disease

Answer 19

B) hemophilia A
EXPLANATION
Hemophilia is characterized by deep muscolo-skeletal bleeding and not by platelet-type bleeding signs.

Question 20

Frequent clinical signs in children with intracranial tumors, with the exception of:
A) endocrine disorders
B) signs of extracranial, extramedullary metastases
C) focal neurologic signs
D) signs of elevated intracranial pressure
E) character change

Answer 20

B) signs of extracranial, extramedullary metastases
EXPLANATION
Extracranial, extramedullary metastasis (B) are very rare in children with intracranial tumors. They may occur however, with the presence of ventriculo peritoneal or ventriculo atrial shunts or in case of intracranial gem cell tumors and primary central nervous system lymphoma.

Question 21

Characteristic presentation signs of neuroblastoma, with the exception of:
A) hemolysis
B) Horner-triad
C) hypertension
D) opsoclonus-myoclonus (dancing eyes-dancing feet) syndrome
E) palpable abdominal mass

Answer 21

A) hemolysis
EXPLANATION
Hemolysis (A) is not a characteristic finding in patients with neuroblastoma.

Question 22

Adverse prognostic factors of neuroblastoma, with the exception of:
A) onset in infancy
B) bone metastases
C) N-myc amplification
D) high levels of serum ferritin and LDH

Answer 22

A) onset in infancy
EXPLANATION
Neuroblastoma among infants (up to 1.5 years of age) is associated with a more favorable clinical outcome than is case of older children (A).

Question 23

The following statements are characteristic for retinoblastoma, with the exception of:

A) The first suspicious sign is the onset of strabismus in an infant who was not amblyopic previously.
B) The pathognomonic clinical sign, leukocoria (cat’s eye or white eye) characterizes an advanced disease, which can be treated with enucleation
C) The unilateral, unifocal retinoblastoma is usually sporadic
D) The bilateral, multifocal retinoblastoma is usually inherited due to germline mutations of the RB1 gene
E) Retinoblastoma usually does not exceed the boundaries of the orbit; therefore, there is no role of chemotherapy in the management

Answer 23

E) Retinoblastoma usually does not exceed the boundaries of the orbit; therefore, there is no role of chemotherapy in the management
EXPLANATION
Chemotherapy can and should be applied in children with retinoblastoma if there is a chance to preserve the vision of at least one of the eyes of the patients or if the disease infiltrates the optic nerve or other surrounding or brain tissues (E).

Question 24

The following statements are characteristic for Wilms tumor, with the exception of:
A) the most frequent presentation sign is a painless abdominal mass
B) clinical signs, such as hypertension, hematuria, abdominal pain, weakness and fever may accompany the development of Wilms tumor in 20% of cases
C) the patients are almost always anemic due to decreased erythropoietin production
D) Wilms tumor may be associated with aniridia, hemihypertophy and urogenital malformations
E) treatment intensity is influenced by the stage and histological type of the disease

Answer 24

C) the patients are almost always anemic due to decreased erythropoietin production
EXPLANATION
Wilms tumor may produce erythropoietin as a paraneoplastic sign. Such patients have an increased hemoglobin concentrations and hematocrit level. Patients, rarely may develop anemia due to excessive hematuria or tumor rupture (C).

Question 25

The following statements are characteristic for pediatric soft tissue sarcomas, with the exception of:
A) 50% of pediatric soft tissue sarcomas are rhabdomyosarcoma
B) rhabdomyosarcoma may arise exclusively from striated muscles
C) manifestation signs are very variable according to the localization of the tumor
D) the clinical outcome of rhabdomyosarcoma is influenced by the stage, the histological type, the localization and the response to treatment
E) the treatment of rhabdomyosarcoma involves chemotherapy, surgery and irradiation

Answer 25

B) rhabdomyosarcoma may arise exclusively from striated muscles
EXPLANATION
Rhabdomyosarcoma arises from neoplastic transformed mesenchymal precursor cells and contains tumor cells that resemble striated muscle cells. It arises frequently from striated muscle of the limbs and the trunk but it may take an origin from organs, such as urogenital tract and nasopharynges which normally do not contain striated muscle (B).

Question 26

The following statements are characteristic for bone tumors, with the exception of:
A) the two most frequent bone tumors in children are osteosarcoma and Ewing-sarcoma
B) most frequent manifestation signs are: pain of the involved bone accompanied by swelling and hyperemia
C) osteosarcoma arises mainly from flat bones and the diaphysis of long bones, whereas Ewing-sarcoma arises from the metaphyseal part of the long bones
D) ‘Codman triangle’ and ‘sunburst’ appearance may often characterize radiographs of patients with osteosarcoma
E) Radiographic picture of Ewing-sarcoma is usually characterized by ‘moth-eaten’ destructive lucent lesions with large soft tissue component, and typical ‘onion skin’ periostitis

Answer 26

C) osteosarcoma arises mainly from flat bones and the diaphysis of long bones, whereas Ewing-sarcoma arises from the metaphyseal part of the long bones
EXPLANATION
In point (C) the opposite is true: osteosarcoma arises mainly from the metaphyseal part of the lung bones whereas Ewing sarcoma arises frequently from flat bones and the diaphysis of long bones (C).

Question 27

Clinical presentation, laboratory findings and management of patients with Hemophilia:
1) frequent joint and intramuscular bleeds
2) frequent manifestation of purpuras and petechiae without treatment
3) X-linked recessive inheritance, 30% of cases represent new mutations
4) normal platelet count, bleeding time and prothrombin time, prolonged activated partial thromboplastin time
5) the mainstream of treatment is factor VIII supplementation
A) 1st, 2nd and 3rd answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 27

B) 1st, 3rd, 4th and 5th answers are correct
EXPLANATION
The improper answer is 2., since hemophilia does not present with platelet type bleeding signs. Therefore, the proper answer is (B).

Question 28

Choose statements characteristic for von Willebrand-disease (vWD)!
1) decreased activity of vitamin K-dependent coagulation and anticoagulant factors due to von Willebrand factor (vWF) deficiency
2) both quantitative and qualitative anomalies of vWF may result in vWD
3) due to dual function of vWF, hemophilia-like bleeding signs may also be seen in severe forms of vWD, in addition to superficial and mucosal bleeds
4) minor bleeds in type-I vWD can be treated with desmopressin infusion, whereas only vWF-containing factor concentrates can be applied in type-II and –III vWD
5) intravenous vitamin K application results in rapid elevation in the levels of large vWF multimers therefore, it may restore bleeding control
A) 1st, 2nd and 3rd answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 28

D) 2nd, 3rd and 4th answers are correct
EXPLANATION
Von Willebrand Factor (vW) is not a vitamin K-dependent coagulation factor

Question 29

Factors increasing the risk of deep venous thrombosis in children:
1) activated protein C resistance (factor V Leiden-mutation)
2) deficiencies of anticoagulant factors (ATIII, PC, PS)
3) nephrosis syndrome
4) prothrombin gene mutation
5) newborn period
A) 1st, 2nd and 4th answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 29

E) all of the answers are correct
EXPLANATION
Each choice represents a true statement.

Question 30

Neoplastic conditions frequently accompanied by lymph node enlargement in children:
1) acute leukemia
2) brain tumors
3) Langerhans cell histiocytosis
4) Hodgkin lymphoma
5) non-Hodgkin lymphoma
A) 1st, 2nd and 3rd answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 30

B) 1st, 3rd, 4th and 5th answers are correct
EXPLANATION
Brain tumors are not frequently associate lymph node enlargement (2.). Therefore, the proper answer is B.

Question 31

Frequent non-neoplastic causes of lymphadenopathy in children:1) BCG-lymphadenopathy
2) hemolytic uremic syndrome
3) regional lymphadenopathy of inflamed organs and tissues
4) acute, purulent lymphadenitis
5) certain virus and protozoon infections, such as adenovirus, CMV, EBV, HIV, rubella and toxoplasma
A) 1st, 2nd and 4th answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 31

B) 1st, 3rd, 4th and 5th answers are correct
EXPLANATION
Hemolytic uremic syndrome is not frequently associate lymph node enlargement (2.). Therefore, the proper answer is B.

Question 32

Prevention and management of iron deficiency anemia:1) to meet with the daily iron requirements of very low-birth weight premature babies: 0.5 mg/kg bw/day
2) physiological iron requirement in healthy infants is 1 mg/kg bw/day
3) we prescribe 3-6 mg/kg bw/day elemental iron in form of ferrous sulfate to be taken b.i.d., orally, before meals
4) parenteral iron substitution results in a faster correction of iron deficiency than oral substitution
5) iron supplementation is to be suspended temporarily during intercurrent infections
A) 1st, 2nd and 4th answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 32

C) 2nd, 3rd and 5th answers are correct
EXPLANATION
Very low birth rate premature infants should be given 4 mg/kg bw/day iron supplementation after the first months of life (1.). Parenteral iron substitution does not result in a faster correction of iron deficiency than oral substitution. However, we can overcome severe mal absorption or none-compliance (4.). Chooses 2.,3. and 5. are proper once. Therefore, the proper answer is C.

Question 33

Choose proper statements characterizing acute lymphoblastic leukemia!
1) the most frequent presentation signs (anemia, bleeding and fever) result from the infiltration of the bone marrow by leukemic blast cells
2) additional frequent signs are hepatosplenomegaly and lymphadenopathy
3) there are at least 20% atypical leukemic lymphoblasts present in the bone marrow
4) we apply risk-tailored treatment
5) first-line treatment consists of combined chemotherapy, supportive treatment and in certain cases, irradiation, tyrosine kinase inhibitors and allogeneic hematopoietic stem cell transplantation
A) 1st, 2nd and 3rd answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 33

E) all of the answers are correct

Question 34

Differential diagnosis of acute lymphoblastic leukemia:
1) aplastic anemia
2) unstable hemoglobinopathies
3) other neoplastic conditions, such as acute myeloid leukemia, lymphoma, neuroblastoma
4) immune thrombocytopenia
5) juvenile idiopathic arthritisA) 1st, 2nd and 4th answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 34

B) 1st, 3rd, 4th and 5th answers are correct
EXPLANATION
Unstable hemoglobinopathies (2.) share few overlapping clinical signs with acute lymphoblastic leukemia. Therefore, the proper answer is B.

Question 35

Diseases and conditions in children frequently accompanied by severe, life-threatening infections:
1) iron deficiency anemia
2) neutropenia
3) sickle cell disease
4) splenectomy
5) polycythemia
A) 1st, 2nd and 4th answers are correct
B) 1st, 3rd, 4th and 5th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 2nd, 3rd and 4th answers are correct
E) all of the answers are correct

Answer 35

D) 2nd, 3rd and 4th answers are correct EXPLANATION Patients with iron-deficiency anemia (1.) and with polycythemia (5.) are more prone develop infections than healthy persons, but severe lif-threatening infections are not characteristic complications of these to conditions in contrast to patients with neutropenia (2.), sickle cell disease (3.) and splenectomy (4.). Therefore, the proper answer is D.