Pediatrics - Hematologic and oncologic disorders Flashcards
PED - 10.1
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
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.
PED - 10.2
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
C) intravenous immunoglobulin preparations
EXPLANATION
Contemporary, virus-inactivated/eliminated intravenous immunoglobulin (IVIG) preparations obtained for screen donors do not result in aplastic anaemia. Application of IVIG is recommended in aplastic anaemia (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.
PED - 10.3
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
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.
PED - 10.5
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
D) Beckwith-Wiedemann syndrome
EXPLANATION
Conditions listed in points A, B, C represent different forms of constitutional hypoplastic anaemias 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).
PED - 10.6
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
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.
PED - 10.8
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
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.
PED - 10.10
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
A) craniotabes
EXPLANATION
Craniotabes (A) is a sign of rickets.
PED - 10.11
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
D) hemolysis
EXPLANATION
Hemolysis may be associated with iron overload, in particular if patient is transfusion-dependent (D).
PED - 10.12
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
B) glucose-6-phosphate-dehydrogenase deficiency
EXPLANATION
Glucose-6-phosphate-dehydrogenase deficiency (B) results in normochromic normocytic anemia.
PED - 10.13
Frequent signs and complications of hemolytic anaemia, 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
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.
PED - 10.14
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
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).
PED - 10.15
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
E) methemoglobinemia
EXPLANATION
Methemoglobinemia (E) does not result in hemolysis.
PED - 10.16
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
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.
PED - 10.18
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
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.
PED - 10.20
Signs of hyperleukocytosis syndrome, with the exception of:
A) central nervous system bleeding
B) ARDS
C) polyuria
D) thrombosis
E) lung hemorrhage
ANSWER
C) polyuria
EXPLANATION
Hyperleukocytosis syndrome can be associated with oligo-anuria rather than with polyuria (C).
PED - 10.21
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
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).
PED - 10.22
The tumor lysis syndrome triad, with the exception of:
A) hyperbilirubinemia
B) hyperkalemia
C) hyperphosphatemia
D) hyperuricemia
ANSWER
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.
PED - 10.23
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
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.
PED - 10.24
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
B) hemophilia A
EXPLANATION
Hemophilia is characterized by deep muscolo-skeletal bleeding and not by platelet-type bleeding signs.
PED - 10.25
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
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.
PED - 10.26
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
A) hemolysis
EXPLANATION
Hemolysis (A) is not a characteristic finding in patients with neuroblastoma.
PED - 10.27
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
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).
PED - 10.28
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
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 of the eyes of the patients or if the disease infiltrates the optic nerve or other surrounding or brain tissues (E).
PED - 10.29
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
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).
