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Hematology and Oncology > Hematology > Flashcards

Hematology Flashcards

1
Q

physiologic nadir in term infants

A

6-10 weeks low hemoglobin (9-11mg/dl)

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2
Q

physiologic nadir in preterm infants

A

4-8 weeks (7-9mg/dl)

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3
Q

When do we give Rhogam to mothers

A

administered to all Rh negative mothers at risk for Rh alloimmunization

IM injection of 300ug (1ml) human anti-D globulin within 72hours delivery of Rh positive

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4
Q

What is neonatal polycythemia?

A

Full term with hgb >/= 22mg/dl or Hct >65%
measure central hemoglobin
Hct peaks during 1st 2-3 weeks of life

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5
Q

Manifestations of early onset Vitamin K deficiency

A

bleeding in 1st 24 hours
mother chronically given anticoagulants, anticonvulsant, cholesterol lowering
can have severe bleeding

*should be given 1-2mg Vitamin K IV
if still prolonged –> 10-15ml/kg Fresh frozen plasma

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6
Q

Responses to iron therapy in IDA:

replacement of intracellular iron, decrease irritability, increase appetite, increase serum iron

A

12-24hr

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7
Q

Responses to iron therapy in IDA:

initial BM response; erythroid hyperplasia

A

36-48hr

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8
Q

Responses to iron therapy in IDA:

reticulocytosis peaking at 5-7 days

A

48-72hr

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9
Q

Responses to iron therapy in IDA:

increased hemoglobin level, inc MCV, inc ferritin

A

4-30 days

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10
Q

Responses to iron therapy in IDA:

repletion of stores

A

1-3 months

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11
Q

normal development switch from fetal to adult hemoglobin synthesis at birth that results to

A

replacement of high-oxygen affinity fetal hemoglobin with lower affinity adult hemoglobin capable of delivering more oxygen to tissues

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12
Q

physiologic anemia of infancy

A

inc blood O2 content and delivery –> downregulation of EPO production –> suppression of erythropoiesis –> aged RBCs that are removed from circulation are not replaced –> Hgb level decreases

point is reached between 8-12 weeks when Hgb is 11g/dL

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13
Q

what is physiologic anemia of prematurity?

A
  • Hgb decline is more extreme and rapid
  • min Hgb level 7-9g/dL commonly reached by 3-6 wk of age
  • blood loss from repeated phlebotomies
  • premature infant’s rbc life span 40-60 days
  • plasma EPO levels are lower than would be expected
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14
Q

RBC life span in premature

A

40-60 days

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15
Q

half life of transfused RBC in early preterm infants (<1250g)

A

30 days

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16
Q

most widespread and common nutritional disorder in the world

A

Iron deficiency anemia

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17
Q

dietary intake of Iron should be

A

8-10mg iron daily

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18
Q

infections that contribute to IDA

A

hookworm, trichiuris trichiura, plasmodium and H. pylori

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19
Q

desire to ingest ice

A

pagophagia

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20
Q

desire to ingest non-nutritive substances

A

Pica

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21
Q

what happens when iron stores are depleted

A
  • tissue iron stores depleted
  • reduced serum ferritin
  • serum iron binding capacity (serum transferrin) increases
  • transferrin saturation falls below normal
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22
Q

findings in IDA

A
  • reduced ferritin
  • reduced serum iron
  • increased TIBC
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23
Q

routine screening using hemoglobin or hematocrit is done

A

at 12 months of age or earlier if at 4 months the child is assessed to be at risk for iron deficiency

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24
Q

in mild anemia, when do we repeat cbc after treatment?

A

4 weeks after initiating therapy

  • 1-2g/dL rise in hgb
  • iron medication should be continued for 8 weeks
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25
Q

disorders of globin chain production

A

Thalassemia

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26
Q

absence of B globin production

A

Beta thalassemia

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27
Q

absence or partial reduction in alpha globin

A

Alpha thalasemia

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28
Q

symtoms of thalassemia major

A

profound weakness and cardiac decompensation during the 2nd 6 months of life

thalassemic facies (maxilla hyperplasia, flat nasal bridge, frontal bossing, pathologic bone fractures, marked hepatosplenomegaly, cachexia

spleen may be enlarged

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29
Q

deletion of 1 alpha globin

A

alpha thalassemia silent trait

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30
Q

deletion of 2 alpha globin

A

alpha thalassemia trait

*microcytic anemia

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31
Q

deletion of 3 alpha globin

A

Hemoglobin H disease

*marked microcytosis, anemia, mild splenomegaly and occasionaly scleral icterus or cholelithiasis

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32
Q

deletion of 4 alpha globin

A

Hydrops fetalis

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33
Q

treatment for alpha thalassemia

A

folate acid supplementation
possible splenectomy
intermittent transfusion
chronic transfusion therapy

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34
Q

most common manifestation of G6PD deficiency

A

neonatal jaundice
episodic acute hemolytic anemia induced by infections, certain drugs, fava beans

*hemolysis ensues about 24-48hr after ingestion

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35
Q

most common and serious congenital coagulation factor deficiences

A 
Hemophilia A (Factor VIII def)
Hemophilia B (Factor IX def)
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36
Q

hallmark of hemophilic bleeding

A

hemarthrosis

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37
Q

what to request for possible Factor 8 or 9 deficiency

A

PTT

*specific assays to confirm diagnosis of hemophilia

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38
Q

most common cause of acute onset thrombocytopenia

A

ITP

39
Q

most common viruses associated with ITP

A

EBV and HIV

40
Q

indications for bone marrow aspiration in ITP

A

abnormal WBC
unexplained anemia
history and PE suggestive of bone marrow failure or malignancy

41
Q

management of ITP

A

IVIG induces rapid rise in platelet count

Prednisone 4mg/kg/24hr

42
Q

role of splenectomy in patients with ITP should be reserved for 1 of 2 circumstances

A
  1. older child (>4yrs) with severe ITP that lasted more than 1 year (chronic ITP) and symptoms not easily controlled with therapy
  2. life threatening hemorrhage complicating acute ITP, if PC cannot be corrected rapidly with transfusion
43
Q

decreased EPO production by diseased kidneys

A

Anemia of renal disease

tx: oral iron therapy in all pedia pxs with CKD w/ anemia
IV iron therapy- receiving dialysis

44
Q

mainstay treatment in ESRD

A

erythrocyte stimulating agents (ESA)

Darbepoietin- synthetic form of EPO

45
Q

mucosal bleeding, epistaxis, easy bruising, menorrhagia

A

Von Willebrand disease

46
Q

what are the types of Von Willebrand disease

A

Type I and Type II - mild-moderate

Type III - severe

47
Q

type of VWD: most common type with mucosal bleeding, epistaxis, menorrhagia

A

Type I VWD

Type I: desmopressin

48
Q

type of VWD: most severe form; symptoms of mucosal bleeding, epistaxis, menorrhagia but with joint bleed, CNS hemorrhage

A

Type II VWD

49
Q

findings in Von Willebrand

A

PTT prolonged

50
Q

Low hemoglobin, low hematocrit with high RDA

A

IDA

51
Q

Low hgb, low hct, normal RDW

Normal or high serum ferritin

A

Thalassemia

52
Q

Low MCV

A

Iron deficiency anemia
Thalassemia
Sideroblastic anemia
Anemia of chronic disease

53
Q

High MCV

A

Vitamin B12/Folate
Immune hemolytic
Diamond-Blackfan

54
Q

Reticulocyte count <3%

A 
Infection
Drugs
Lead poisoning
Acute blood loss
Anemia of chronic disease
Renal disease
55
Q

Reticulocyte count >3%

A

Are there clinical signs of hemolysis?

If yes: 
Hereditary spherocytosis
Hereditary elliptocytosis
G6PD
Sickle cell disease 
HUS/TTP
Mechanical heart valve

If no:
Hemorrhage

56
Q

CBC findings in IDA

A

Low RBC, Low MCV, low Reticulocyte count

Increased RDW

57
Q

When can we repeat CBC after starting iron therapy?

A

4 weeks after iron therapy; usually risen by atleast 1-2g/dL and often within normal levels

58
Q

CBC findings in G6PD

A

low hemoglobin
Heinz bodies
anisopoikilocytosis
bite cells

59
Q

definitive diagnosis of Thalassemia

A

hemoglobin electrophoresis

60
Q

microcytic hypochromic RBCs
Target cells
Heinz bodies

A

Thalassemia

61
Q

most common cause of hemolytic anemia due to a red cell membrane defect

A

Hereditary spherocytosis

62
Q

test for Hereditary spherocytosis

A

Osmotic fragility test:

confirms presence of fragile sphere-shaped RBCs

63
Q

management for Hereditary spherocytosis

A

splenectomy is curative and recommended for:

transfusion dependent patients
severe disease
moderate disease with frequent hypoplastic or aplastic crises, poor growth or cardiomegaly

Folate supplementation

64
Q

clinical hallmarks of sickle cell disease

A

vasooclusive phenomena and hemolysis

65
Q

Howell Jolly bodies is seen in

A

sickle cell disease

66
Q

management for sickle cell disease

A

analgesia and hydration
Hydroxyurea to promote production of fetal hemoglobin
vaccination against encapsulated organisms
Folate supplementation

67
Q

normal PT
normal PTT
prolonged bleeding time
decreased platelet count

A

ITP

68
Q

normal PT
prolonged PTT
normal bleeding time
normal platelet count

A

Hemophilia

69
Q

normal PT
prolonged PTT
prolonged bleeding time
Normal or decreased platelet count

A

vWD

70
Q

prolonged PT
prolonged PTT
prolonged bleeding time
decreased platelet count

A

DIC

71
Q

prolonged PT
Normal or prolonged PTT
normal bleeding time
normal platelet count

A

Vitamin K deficiency

72
Q

bruising and petechiae

A

mild ITP

73
Q

more severe skin and mucosal lesions, troublesome epistaxis, meorrhagia

A

moderate ITP

74
Q

bleeding episodes requiring transfusion or hospitalization

A

severe ITP

75
Q

splenectomy indicated

A

life-threatening hemorrhage (intracranial bleed)
children >/=4 years old with chronic ITP lasting >1yr
children whose symptoms are difficult to control

76
Q

Five cardinal symptoms of TTP

A

FAT RN

Fever
Anemia
Thrombocytopenia
Renal dysfunction
Nervous system changes
77
Q

sex-linked hematologic disorder occurring most exclusively in males; marked by delayed clotting of blood

A

Hemophilia

78
Q

first sign of early joint hemorrhage

A

warm, tingling sensation in the joint;

easy bruising

79
Q

hallmark of hemophilia

A

hemarthrosis

most common earliest joint involved: ankle
older children and adolescents: knees and elbows

80
Q

common manifestations of ALL

A

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

signs of infiltration (bone pain, lymphadenopathy, hepatosplenomegaly)

81
Q

tumor lysis syndrome

A

hyperphosphatemia
hypocalcemia
hyperuricemia
hyperkalemia

82
Q

painless lymphadenopathy
mediastinal mass
presence of B symptoms

A

Hodgkin lymphoma

83
Q

most common solid tumor in children

A

Brain tumors

84
Q

most common malignant brain tumor in childhood

A

Medulloblastoma

85
Q

treatment for osteosarcoma

A

chemotherapy and surgery

86
Q

treatment for Ewing sarcoma

A

chemotherapy
radiation
surgery

87
Q

abdominal mass that crosses midline

A

Neuroblastoma

88
Q

abdominal pain, proptosis, periorbital ecchymoses (raccoon eyes), Horner syndrome

A

Neuroblastoma

89
Q

painless abdominal enlargement with flank mass that does not cross the midline

A

Wilms Tumor

*hematuria and hypertension

90
Q

most sensitive test for IDA

A

serum ferritin

91
Q

most common population group assoicated with priapism

A

males with sickle cell anemia

92
Q

most common disease involving hexose monophosphate pathway

A

G6PD deficiency

93
Q

most common cause of drug-immune hemolytic anemia

A

Cephalosporins

94
Q

most common inherited abnormality of the RBC membrane

A

hereditary spherocytosis

Hematology and Oncology (2 decks)

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