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Pedia > Hematology and Oncology > Flashcards

Hematology and Oncology Flashcards

1
Q

Progressive decline in Hgb that starts during 1st week of life that persists 6-8 weeks and usually lasts 8-12 weeks

A

Physiologic Anemia of Infancy

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2
Q

The MC childhood malignancy

anorexia, irritability, lethargy

signs of marrow failure - anemia, bleeding, purpuric/petechial lesions, low grade fever

signs of infiltration - bone pain, lymphadenopathy - splenomegaly > hepatomegaly

A

Acute Lymphocytic Leukemia (ALL)

pancytopenia + hypercellular (blasts)

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3
Q

The single most important prognostic factor in ALL

A

TREATMENT

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4
Q

3 of the most important predictive factors in ALL

A
  1. Speed of response to treatment
  2. Age of the patient at the time of diagnosis
  3. Initial leukocyte count
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5
Q

Poor Prognostic Factors

A 
< 2 yrs or >10 yrs
male
WBC > 100 000 u/L on presentation
(+) CNS leukemia
(+) mediastinal mass
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6
Q

Sites of relapse in ALL

A

bone marrow

CNS – increased ICP and isolated nerve palsies

testes - painless swelling of one or both testes in 1-2 % of males

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7
Q

Sites of metastases in ALL

A

liver
spleen
lymph nodes

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8
Q

The MC hematologic disease of infancy and childhood

MC in 9-24 mos of age

MCC – poor dietary intake

A

Iron Deficiency Anemia (IDA)

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9
Q

Most important sign of IDA

A

PALLOR - 7-8 mg/dL

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10
Q

Reduction of the hemoglobin concentration or RBC volume below the range of values occurring in healthy persons

A

Anemia

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11
Q

Macrocytic Anemia

MCV > 100

A

Folate Deficiency
Vitamin B12 Deficiency
Drug and alcohol induced anemia

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12
Q

Microcytic Anemia

MCV < 80

A 
Thalassemia
Anemia of Chronic Disease
IDA
Lead poisoning
Sideroblastic anemia
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13
Q

Normocytic Anemia

MCV 80-100

A

Anemia of Chronic Disease
Uremia
Hypothyroidism
BM failure (aplastic anemia)

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14
Q

Non hematologic effects of IDA

A

Pica
Pagophagia - ICE
Neurocognitive effects

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15
Q

Diagnostic findings in IDA

A 
low reticulocyte count
microcytic, hypochromic RBCs
high RDW
high TIBC
low serum iron and ferritin
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16
Q

microcytic, hypochromic
low reticulocyte count
HIGH RDW

A

Iron of Deficiency Anemia

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17
Q

microcytic, hypochromic
low reticulocyte count
NORMAL - DECREASED RDW

A

Thalassemia

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18
Q

IDA vs Chronic Disease of Anemia

A

IDA - TIBC - HIGH

Anemia of Chronic Disease - TIBC - LOW

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19
Q

Inherited microcytic hemolytic anemia d.t. abnormal hemoglobin synthesis

Result when 1 or more of globin gene mutates

A

Thalassemia

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20
Q

Due to decrease in alpha-globin chain synthesis

A

Alpha Thalassemia

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21
Q

Diagnosis of Alpha Thalassemia

A 
DECREASED reticulocyte count (d.t. ineffective hematopoiesis)
microcytic hypochromic RBCs
normal RDW
TARFET CELLS
HEINS BODIES
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22
Q

Treatment of Alpha Thalassemia

A

frequent transfusions
chelation therapy - Deferoxamine
Splenectomy

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23
Q

Due to decrease in beta-globin chain synthesis or absence of beta chain of hemoglobin

A

Beta Thalassemia

Beta thalassemia major (Cooley anemia)
Beta thalassemia minor

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24
Q

Treatment of Beta Thalassemia

A

aggressive transfusions
chelation therapy as needed
hydroxyurea
splenectomy

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25
Q

Normochromic, normocytic RBCs

Increased MCHC

A

Spherocytosis

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26
Q

Results from decrease in RBC survival

pallor
jaundice d.t. increased indirect bilirubin
increased LDH

A

HEMOLYTIC ANEMIA

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27
Q

Characteristic findings in Hemolytic Anemia

A

normocytic, normochromic RBCs

increased reticulocyte count

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28
Q

Etiologies of Hemolytic Anemia

A

Intrinsic RBC defects – structural or metabolic abnormalities

Autoimmune destruction

Intravascular destruction

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29
Q

Hemolytic anemia d.t. intrinsic RBC defects

A

Spherocytosis

Sickle Cell Disease

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30
Q

Complications of Sickle Cell Disease

A

painful (vaso-occlusive) crisis

splenic sequestration crisis – auto-splenectomy – increased susceptibility to encapsulated organisms

aplastic crisis

priapism

stroke

leg ulcers

acute chest syndrome

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31
Q

Idiopathic
Usually with VIRAL trigger
MUCOSAL bleeding

normal Hgb and WBC
DECREASED platelet

A

Idiopathic Thrombocytopenic Purpura

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32
Q

Treatment of ITP

A

Supportive
Steroids
IVIG

33
Q

X linked recessive deficiencies of factor VIII and factor IX

A

HEMOPHILIA

Hemophilia A (factor VIII) - MC

Hemophilia B or Christmas Disease (factor IX)

34
Q

The MC hereditary BLEEDING disorder

A

von Willebrand disease

35
Q

The MC and most serious congenital and coagulation factor deficiencies

A

Hemophilia A

36
Q

The MC hereditary HYPERCOAGULABLE disorder

A

Factor V Leiden

37
Q

Hallmark of Hemophilia

A

PROLONGED BLEEDING

38
Q

Earliest joint hemorrhages in children

A

ANKLES

39
Q

Diagnosis of von Willebrand disease

A 
DECREASED vWF and factor VIII levels
PROLONGED bleeding time
ABNORMAL platelet adhesion
INCREASED PTT
Ristocetin cofactor assay - measures vWF antigen levels and activity
40
Q

Treatment of von Willebrand disease

A

MILD - DESMOPRESSIN - causes release of vWF from endothelial stores

SEVERE - Factor VIII (contain high vWF Ag)

41
Q

Platelet dysfunction

A

bruising

mucosal bleeding – epistaxis, menorrhagia

42
Q

Coagulation disorder

A

joint bleeding

muscle bleeding

43
Q

Normal PT, bleeding time and platelet count

PROLONGED PTT

A

Hemophilia A and B

44
Q

normal PT
normal or decreased platelet count

PROLONGED PTT and BLEEDING TIME

A

von Willebrand disease

45
Q

normal PT and PTT

PROLONGED BLEEDING TIME

DECREASED platelet count

A

ITP

46
Q

normal bleeding time and platelet count

normal or prolonged PTT

PROLONGED PT

A

Vitamin K deficiency

47
Q

PROLONGED PT, PTT and BLEEDING TIME

DECREASED PLATELET COUNT

A

DIC

48
Q

Renal tumor of embryonal origin

2nd MC malignant abdominal tumor in childhood

A

WILMS TUMOR

Associated with:

Neurofibromatosis

Beckwith Wiedemann syndrome (hemihypertrophy, visceromegaly, macroglossia)

WAGR syndrome (Wilms tumor, Aniridia, Genitourinary abnormalities, Mental retardation)

49
Q

PAINLESS abdominal enlargement with flank mass that does NOT cross the midline

Hematuria, Hypertension

A

WILMS TUMOR

kidneys - origin/location

50
Q

Embryonal tumor of neural crest cell origin

3rd MC pediatric cancer

A

NEUROBLASTOMA

Associated with:
N-myc oncogene - tuberous sclerosis
Neurofibromatosis - pheochromocytoma
Hirschprung disease

51
Q

Abdominal mass that CROSSES the midline

Horner syndrome
HPN
cord compression (from a paraspinal tumor)

A

NEUROBLASTOMA

anywhere along the neuroaxis (adrenals- MC) - origin/location

52
Q

Site of metastasis in Wilms tumor

A

LUNGS

53
Q

Site of metastasis in Neuroblastoma

A

BONES

54
Q

Diagnosis of Wilms tumor

A

Imaging

Biopsy

55
Q

Diagnosis of Neuroblastoma

A

Imaging
Biopsy (BMA)
Urine VMA and HVA

56
Q

Differential Diagnosis of Small Round Blue Cell Tumors

A

WARMERPN

Wilms tumor
Acute leukemia
Rhabdomyosarcoma
Mesothelioma/Medulloblastoma
Ewing Sarcoma
Retinoblastoma
Primitive Neuroectodermal tumor (PNET)
Neuroblastoma
57
Q

Typical PBS findings in APLASTIC ANEMIA

A

pancytopenia

HYPOCELLULAR

58
Q

Pancytopenia

A

Fanconi anemia

Aplastic Anemia

59
Q

Treatment of Aplastic Anemia

A

RBC and platelet transfusion
Allogenic BM transplant (sibling)
Granulocyte colony-stimulating factor or Granulocyte macrophage colony-stimulating factor

60
Q

MC solid tumor

A

Brain tumors

61
Q

MC solid tumor OUTSIDE CNS

A

Neuroblastoma

62
Q

MC soft tissue tumor

A

Rhabdomyosarcoma

63
Q

Malignancy with HIGHEST mortality

A

Brain (PNET)

64
Q

2nd decade
MALES
spindle cell producing OSTEOID
METAPHYSIS of long bones

local pain and swelling often with history of injury

SUNBURST PATTERN

A

OSTEOSARCOMA

65
Q

2nd decade
MALES
undifferentiated small round cell of neural origin
DIAPHYSIS of long bones

local pain and swelling with fever

ONION SKINNING/MOTH EATEN APPEARANCE

A

EWING SARCOMA

66
Q

Minimal Hgb levels in physiologic anemia of prematurity

A

7-9 g/dL

67
Q

RESPONSE TO IRON THERAPY

A

12-24 hrs - subjective improvements

26-48 hrs - initial bone marrow response

28-72 hrs - reticulocytosis

4-30 days - increasing Hgb levels

1-3 mos - repletion of stores

68
Q

1st lab value to decline in IDA

A

serum FERRITIN

69
Q

Definitive diagnosis of thalassemia and SCD

A

Hb Electrophoresis

70
Q

Hereditary Spherocytosis Confirmatory Test

A

Osmotic Fragility Test

71
Q

Curative for hereditary spherocytosis

A

Splenectomy

72
Q

Mutation in Sickle Cell Disease

A

MISSENSE mutation

73
Q

At the end of induction therapy, peripheral blast count or minimal residual disease must be

A

<0.01 %

74
Q

WAGR syndrome

A

Wilms tumor
Aniridia
GU malformation
Mental retardation

75
Q

Beckwith-Wiedemann syndrome

A 
Visceromegaly
Macroglossia
Omphalocele
Hyperinsulinemic hypogycemia
Wilms tumor, Hepatoblastoma
76
Q

Denys-Drash syndrome

A

Nephropathy
Renal Failure
Male pseudohermaphrodism
Wilms tumor

77
Q

Malignancy that may present with raccoon eyes, subcutaneous tumor nodules, “dancing eyes, dancing feet”

A

Neuroblastoma

78
Q

Treatment for neuroblastoma

Stage 4S

A

Observation

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