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Pediatrics > Hema-Onco > Flashcards

Hema-Onco Flashcards

1
Q

diagnostic test to distinguish ACD from IDA

A

Soluble transferring receptor (sTfR)
- high in IDA, normal in ACD

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2
Q

progressive decline in Hb levels in normal full term infants persists until what age?

A

persists for 6-8 weeks

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3
Q

Hemoglobin concentration continues to decline until tissue oxygen needs become greater than oxygen delivery. This point is reached between what age?

A

8 and 12 weeks of age

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4
Q

blood smear in IDA

A

Hypochromic, microcytic RBCs

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5
Q

most accurate method of diagnosing IDA

A

Bone marrow iron staining

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6
Q

most practical means to establish the diagnosis of IDA

A

Increase in hemoglobin >1 g/dL after 1 month of iron therapy

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7
Q

therapeutic dose of Iron for IDA

A

3-6 mg/kg elemental Iron in 1 or 2 doses

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8
Q

reticulocytosis after initiation of iron therapy in IDA happens when?

A

48-72 hours

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9
Q

Subjective improvement, decreased irritability, increased appetite and serum iron after initiation of Iron therapy in IDA happens when?

A

12-24 hours

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10
Q

describe the primary pathologies in thalassemia and sickle cell disease

A

Thalassemia: quantity of globin produced
Sickle cell disease: quality of globin produced

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11
Q

major clinical complication of transfusion-dependent thalassemia

A

Hemosiderosis

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12
Q

major cause of death in thalassemia

A

Cardiac disease

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13
Q

Common presentation/manifestation of G6PD

A

acute episodic hemolytic anemia
neonatal jaundice

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14
Q

this constitutes the most important therapeutic measure in G6PD

A

prevention of hemolysis

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15
Q

Deficient factor in Hemophilia A

A

Factor VIII

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16
Q

Deficient factor in Hemophilia B

A

Factor IX

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17
Q

most common and serious congenital coagulation factor deficiencies

A

Hemophilia A and B

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18
Q

hallmark of hemophilic bleeding

A

hemarthrosis

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19
Q

Major long-term disability associated with hemophilia

A

chronic arthropathy

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20
Q

most common inherited bleeding disorder

A

Von Willebrand disease (VWD)

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21
Q

Patients with VWD typically present with this type of bleeding

A

Mucosal bleeding

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22
Q

most common type of VWD

A

Type 1
- mild to moderate quantitative defect

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23
Q

most severe type/form of VWD

A

Type 3
- VWF is completely absent

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24
Q

most common cause of acute onset of thrombocytopenia in an otherwise well child

A

Idiopathic thrombocytopenic purpura (ITP)

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25
Q

Most common viruses assicated with ITP

A

EBV
HIB

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26
Q

In some patients, ITP appears to arise in children infected with this organism

A

Helicobacter pylori

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27
Q

classic presentation of ITP

A

sudden onset of generalized petechiae and purpura

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28
Q

What is Evans Syndrome?

A

Autoimmune hemolytic anemia + thrombocytopenia

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29
Q

first line of treatment for ITP presenting with mucocutaneous bleeding

A

IVIG (0.8-1 g/kg) single dose for 1-2 days or
short course of corticosteroids

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30
Q

most common malignant neoplasms in childhood

A

leukemias

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31
Q

accounts for 775 of cases of childhood leukemia

A

Acute Lymphoblastic Leukemia (ALL)

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32
Q

this infection is associated with B-cell ALL in certain developing countries

A

Epstein-Barr virus (EBV) infection

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33
Q

ITP is common in this age group

A

1-4 years old

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34
Q

most common immunophenotype of ALL

A

B-lymphoblastic leukemia

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35
Q

Diagnosis of ALL based on bone marrow evaluation

A

> 25% lymphoblasts

36
Q

single most important prognostic factor in ALL

A

treatment
-without it, the disease is fatal

37
Q

definition of standard risk of ALL

A

Age 1-10 yr
Leukocyte count <50,000/uL

38
Q

definition of high risk for ALL

A

Children <1 yr or >10 yr
Leukocyte count >50,000/uL

39
Q

definition of remission in ALL

A

<5% blasts in the marrow

40
Q

what are the phases of therapy in ALL and what are the drugs being given for each phase

A

Remission induction phase - weekly vincristine + steroids + aspariginase
Consolidation phase -with intrathecal chemo
Maintenance phase - daily mercaptopurine + weekly methotrexate

41
Q

which among the types of ALL has worse outcome, B-ALL or T-ALL?

A

T-ALL

42
Q

Characteristic feature of AML based on bone marrow evaluation

A

> 20% myeloid-monocyte-megakaryocyte blast cells

43
Q

which is more common in Down syndrome during the 1st 3 yr of life, ALL or AML?

A

AML

44
Q

Transient leukemia or myeloproliferative disorder in Down syndrome usually resolve within _____

A

1st 3 months of life

45
Q

This type of leukemia is characterized by a specific translocation known as Philadelphia chromosome resulting in a BCR-ABL fusion protein

A

Chronic Myelogenous Leukemia (CML)

46
Q

signs and symptoms only found in AML and uncommon in ALL

A
  • subcutaneous nodules/“blueberry muffin” lesions
  • infiltration of the gingiva
  • discrete masses: chloromas/granulocytic sarcomas
47
Q

current standard treatment for pediatric CML

A

Tyrosine kinase inhibitor such as Imatinib

48
Q

Patients with NF Type 1 and Noonan syndrome have a predilection for this type of leukemia

A

JMML/Juvenile Chronic Myelogenous Leukemia (JCML)

49
Q

most common cancer in adolescents

A

Lymphoma

50
Q

Infectious agents associated with Hodgkin Lymphoma

A
  • Human herpesvirus 6
  • Cytomegalovirus
  • Epstein-Barr virus (EBV)
51
Q

Pathognomic feature of Hodgkin Lymphoma

A

Reed-Sternberg (RS) cell

52
Q

what are B symptoms?

A
  • Unexplained fever >38 C
  • Weight loss >10% of total body weight over 6 months
  • Drenching night sweats
53
Q

3 subtypes of Non-Hodgkin Lymphoma

A

Lymphoblastic Lymphoma (LBL)
Mature B-cell Lymphoma (2 pathologies: Burkitt Lymphoma (BL), diffuse large B-cell lymphoma (DLBCL)
Anaplastic Large Cell Lymphoma (ALCL)

54
Q

electrolyte abnormalities in Tumor Lysis Syndrome

A

Hyperkalemia
Hyperuricemia
Hyperphosphatemia
Hypocalcemia

55
Q

most common primary brain tumor in children with excellent prognosis

A

Pilocystic astrocytoma

56
Q

classic sites of Pilocytic astrocytoma

A

Cerebellum
Optic pathway region

57
Q

most common CNS tumors in children <1 yr old

A

Choroid Plexus Tumor

58
Q

most common group of malignant CNS tumors of childhood, accounting 20% of pediatric CNS tumors

A

Embryonal tumors or Primitive Neuroectodermal tumors (PNET)

59
Q

childhood brain tumor that most often metastasizes extraneurally

A

Medulloblastoma

60
Q

most common extracranial solid tumor in children and the most commonly diagnosed malignancy in infants

A

Neuroblastoma

61
Q

This associated syndrome in neuroblastoma is characterized by limping and irritability in young child with bone and bone marrow metastases

A

Hutchinson syndrome

62
Q

Syndrome seen in neurbolastoma which presents as massive involvement of the liver with or without respiratory distress

A

Pepper Syndrome

63
Q

Signs and symptoms of horner syndrome

A

Unilateral ptosis
Myosis
Anhidrosis

64
Q

half of neuroblastoma tumors arise in what organ?

A

Adrenal glands

65
Q

these tumor markers help to confirm the diagnosis of neuroblastoma

A

HVA and VMA
- elevated in the urine in 95% of patients

66
Q

most common primary malignant renal tumor of childhood

A

Wilms tumor/Nephroblastoma

67
Q

most common initial clinical presentation of Wilms Tumor

A

incidental discovery of an asymptomatic abdominal mass

68
Q

The most common pediatric soft tissue sarcoma

A

Rhabdomyosarcomas

69
Q

tumors that present as small round blue cell tumors in microscope

A

Rhabdomyosarcoma
Neuroblastoma
Non Hodgkin Lymphoma
Ewing sarcoma

70
Q

favorable sites of rhabdomyosarcoma

A

Female genital
Paratesticular
Head and neck (nonparameningeal) regions

71
Q

most common primary malignant bone tumor in children and adolescents

A

Osteosarcoma

72
Q

More common malignant bone tumor among children <10 yr old

A

Ewing sarcoma

73
Q

Patients with _______ have a significantly increased risk for development of Osteosarcoma

A

Hereditary retinoblastoma

74
Q

classic radiographic appearance of this tumor is “sunburst appearance”

A

Osteosarcoma

75
Q

common site of Osteosarcoma

A

Metaphysis

76
Q

common site of Ewing sarcoma

A

Diaphysis

77
Q

Standard chemotherapy regimen for osteosarcoma

A

MAP - Methotrexate, Doxorubicin, Cisplatin

78
Q

Primary tumors arising in the chest wall in Ewing sarcoma are called?

A

Askin tumors

79
Q

this type of bone tumor is often associated with systemic manifestations such as fever and weight loss

A

Ewing sarcoma

80
Q

it has a radiographic appearance of a primary lytic bone lesion with periosteal reaction — “onion-skinning”

A

Ewing sarcoma

81
Q

classic presentation of retinoblastoma

A

leukocoria/white pupillary reflex

82
Q

what is trilateral retinoblastoma

A

primitive neuroectoderal tumor + bilateral retinoblastoma

83
Q

most common benign tumors of infancy

A

Hemangiomas

84
Q

more than 50% of hemangiomas are located in ___ and ___ region

A

Head, neck

85
Q

primary site of visceral involvement in Hemangioma

A

Liver

86
Q

hemangiomas involutes by this age

A

10-15 yr

Pediatrics (17 decks)

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