Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

#DAY4_PEDIATRICS > HEMATOLOGY/ONCOLOGY > Flashcards

HEMATOLOGY/ONCOLOGY Flashcards

1
Q

Most common soft tissue tumor?

A

Rhabdomyosarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

EVALUATION OF MICROCYTIC ANEMIA

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

SIGNS/SYMPTOMS OF ALL

A
  • acute onset < 4 wks duration of Sx
  • non-specific (anorexia, irritability, lethargy)
  • signs of marrow failure (anemia, bleeding, purpuric/petechial lesions, low-grade fever)
  • signs of infiltration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the most common and most serious congenital coagulation factor deficiencies?

A

Hemophilia A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
  • renal tumor of embryonal origin; 2nd most common malignant abdominal tumor in childhood
  • 2-5 years old
A

WILMS TUMOR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
  • Painless, non-tender, firm, rubbery cervical or supraclavicular adenopathy
  • symptoms important in staging (weight loss >10% of TBW over 3 months, unexplained high-grade fever, drenching night sweats)
A

Hodgkin / Non-hodgkin Lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

most important sign of IDA

A

Pallor (7 - 8 mg/dL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Hemophilia factor levels and severity of bleeding

A

o mild (5-25% normal activity): bleeding only after major trauma or surgery

o moderate (1-5%): bleeding with mild trauma

o severe (<1%): spontaneous bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Frontal bossing, prominence of malar and maxillary bones are seen in

A

Beta thalassemia major

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Why are the measurements of the total iron binding capacity important?

A

TIBC is high in iron deficiency anemia and low in anemia of chronic disease. Both illnesses have low serum iron levels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Hemoglobin H disease

A

3/4 alpha globin chains deleted or mutated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

MICROCYTIC ANEMIA MCV <80

A

CLITS

  • Chronic disease
  • Lead poisoning
  • IDA
  • Thalassemia
  • Sideroblastic Anemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Malignancy with highest mortality?

A

Brain (PNET)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Most common solid tumor outside CNS?

A

Neuroblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Most common malignancy in children?

A

Leukemia (ALL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Where does ALL spread?

A

Liver, spleen, lymph nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

PHYSIOLOGIC ANEMIA OF INFANCY

A
  • 1st week of life – progressive decline in Hgb that persists 6-8 weeks
  • Usually lasts 8-12 weeks
  • Hb levels around 11g/dL, rarely falls below 10g/dL
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

sites of relapse in ALL

A

bone marrow, CNS (increased ICP and isolated cranial nerve palsies), testes (painless swelling of one or both testes in 1-2% of males)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Wilms Tumor vs. Neuroblastoma

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

DIFFERENTIAL DIAGNOSIS OF SMALL, ROUND, BLUE-CELL TUMORS:

A
  • Wilms tumor
  • Acute leukemia
  • Rhabdomyosarcoma
  • Mesothelioma / Medulloblastoma
  • Ewing sarcoma
  • Retinoblastoma
  • Primitive neuroectodermal tumor (PNET)
  • Neuroblastoma

*** W-A-R-M-E-R-P-N (warmer in the Philippines)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

hydrops fetalis

A

Hemoglobin Bart (alpha thalassemia major)

4/4 alpha globin chains deleted or mutated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Rhabdomyosarcomas are most commonly found in

A

the head and neck

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

TREATMENT OF VON WILLEBRAND DISEASE

A
  • Mild bleeding in type 1: desmopressin (causes release of vWF from endothelial stores)
  • Severe disease: Factor VIII concentrates which contain high vWF Ag
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

poor prognostic factors of ALL

A

o < 2 yrs or > 10 yrs

o male

o WBC > 100,000 u/L on presentation

o presence of CNS leukemia

o presence of a mediastinal mass

25
Q

Cooley anemia

A

Beta-thalassemia major

  • no beta-globin production
  • anemia at 6 mos (decline in normal Hgb F & rise in Hgb A), splenomegaly, extramedullary hematopoiesis)
  • ↓ or absent beta chain of Hgb → normal alpha Hgb chains build up → form insoluble aggregates that precipitate within the RBCs → hemolysis or damage to RBCs → susceptible to macrophage destruction & splenic sequestration → small & pale RBCs
26
Q

t (9:22)

A

AML/CML

*Philadelphia chromosome

27
Q
A
28
Q

What is the most common hereditary hypercoagulable disorder?

A

Factor V Leiden

29
Q

most common sites of metastasis of neuroblastoma

A

long bones & skull, BM, liver, lymph nodes, skin

30
Q

invaginate and migrate along the neuroaxis where neural crest cells are present – sympathetic ganglia, adrenal glands

A

Neuroblasts (pluripotent stem cells)

31
Q

What is the most common hereditary bleeding disorder?

A

von Willebrand Disease

32
Q

o Disturbs both primary & secondary hemostasis

o Role in platelet adhesion to exposed subendothelium leads to increased bleeding time → mucous membrane bleeding, petechiae, purpura

o Often have a (+) family history

A

VON WILLEBRAND DISEASE

33
Q

“crew cut” or “hair on end” appearance on skull radiographs

A

SICKLE CELL DISEASE

* Erythropoiesis must increase to compensate for hemolysis → marrow expansion → resorption of bone → new bone formation on the external aspect of the skull

* Dx: sickle cells and Howell-Jolly bodies on PBS; definitive diagnosis with Hb electrophoresis (HbS)

34
Q

o Develops at around 6 mos old when HbS (result of a single missense mutation in the B-globin gene of Hgb) replaces HbF

o Episodes of painful crisis due to hypoxic tissue injury from microvascular occlusions

o Hgb becomes susceptible to polymerization in conditions of low oxygen or dehydrationàreduces flexibility of the RBC membrane

o Any organ can be affected by vascular congestion, thrombosis, and infarction caused by sickling cells

A

SICKLE CELL DISEASE

35
Q
  • S/Sx: painless abdominal pain with flank mass that does not cross the midline, hematuria (12-25%), hypertension
  • Dx: abdominal UTZ, CT scan or MRI, abdominal X ray, liver & kidney function tests, chest x ray (pulmonary metastasis)
  • Tx: transabdominal nephrectomy & post-surgical chemoTx
  • Worse prognosis: large tumor (>500 gms), st III & IV, unfavorable histologic type (anaplastic)
  • More than 60% of patients generally survive
  • St I-III have a cure rate of >90%
A

WILMS TUMOR

36
Q

a large protein made by endothelial cells and megakaryocytes; a carrier for factor VIII and is a cofactor for platelet adhesion

A

vWF

37
Q

What is the earliest joint hemorrhages in children?

A

Ankles

38
Q

S/Sx: reflect the tumor site & extent of disease; non-tender abdominal mass which may cross the midline, Horner syndrome, hypertension, cord compression (from a paraspinal tumor)

A

NEUROBLASTOMA

39
Q

“birbeck granules”

A

Langerhans cell histiocytosis

40
Q

Most children with leukemia present with:

A

Unexplained fever, pallor, and hemorrhage

41
Q

What is the hallmark of hemophilia?

A

Prolonged bleeding

42
Q

target cells and Heinz bodies on PBS

A

ALPHA THALASSEMIA

Dx: decreased reticulocyte count (due to ineffective hematopoiesis), microcytic, hypochromic RBCs, normal RDW

*definitive diagnosis with Hb electrophoresis

43
Q

IDA vs. THALASSEMIA

A
44
Q
  • autosomal recessive
  • aplastic anemia with microcephaly, microphthalmia, hearing loss, limb anomalies (absent radii & thumbs)
  • Dx: clinical, cytogenetic analysis
  • Tx: steroids, BMT, supportive care
A

FANCONI ANEMIA

45
Q

measures vWF antigen levels and activity

A

Ristocetin cofactor assay

46
Q

Auer rods deriving from the crystallisation of myeloperoxidase (MPO) granules are the hallmark of

A

Acute Myeloid Leukemia (AML)

47
Q

Metastases: periorbital bruising (“raccoon eyes”), subcutaneous tumor nodules, opsoclonus /myoclonus (“dancing eyes, dancing feet”)

A

NEUROBLASTOMA

48
Q

Most common solid tumor in children?

A

Brain Tumors

49
Q

What are the typical peripheral blood smear findings in aplastic anemia?

A

Hypocellularity and pancytopenia

50
Q
  • embryonal tumor of neural crest cell origin
  • 3 rd most common pediatric cancer
  • 8% of childhood malignancies
A

NEUROBLASTOMA

51
Q

International Neuroblastoma Staging System

A
  • St 1: tumors confined to the organ or structure of origin
  • St 2: tumors extend beyond the structure of origin but not across the midline with (2B) or without (2A) ipsilateral lymph node involvement
  • St 3: extend beyond the midline w/ or w/o bilateral lymph node involvement
  • St 4: disseminated to distant sites (bone, BM, liver, distant lymph nodes)
  • St 4S: <1 yr old w/ dissemination to liver, skin, or BM w/o bone involvement & with a primary tumor
52
Q

Diagnostic tests for neuroblastoma

A

Dx: abdominal CT scan (calcification & hemorrhage), 24-hr urine VMA & HVA (elevated in 95% of cases), BUN/Crea, CXR, bone scan, LFTs, CBC

53
Q

associated with:

o Neurofibromatosis

o Beckwith-Wiedemann syndrome (hemihypertrophy, visceromegaly, macroglossia)

o WAGR syndrome (Wilms tumor, Aniridia, Genitourinary abnormalities, mental retardation)

A

WILMS TUMOR

54
Q

Which type of leukemia in childhood will have the best response to chemotherapy?

A

ALL

55
Q

LABORATORY FINDINGS IN BLEEDING DISORDERS

A
56
Q

3 of the most important predictive factors for ALL:

A
  1. Age of the patient at the time of diagnosis
  2. Initial leukocyte count
  3. Speed of response to treatment
57
Q

t (11:22)

A

Ewing Sarcoma

58
Q

• associated with:

o N-myc oncogene – tuberous sclerosis

o Neurofibromatosis – Pheochromocytoma

o Hirschsprung disease

A

NEUROBLASTOMA

#DAY4_PEDIATRICS (15 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions