Brainscape's Knowledge GenomeTM

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


See full index

pediatrics II > peds hematology > Flashcards

peds hematology Flashcards

1
Q

Define anemia. What is the cutoff [Hgb]

A

reduction in RBC mass or blood hemoglobin concentration

  • [Hgb] < 11 g/dL for male and female children < 5 yrs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

microcytic, hypochromic anemia comprises of what conditions

A
  • iron deficiency
  • thalassemia
  • lead intoxication
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

macrocytic anemia comprises of what conditions

A
  • vit B12 and folate deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is fanconi anemia

A
  • inherited bone marrow failure syndrome
  • autosomal recessive
  • defective DNA repair
  • 75-90% develop bone marrow failure in the first 10 yrs of life
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

clinical presentation

  • progressive pancytopenia
  • severe congenital malformations
    • abnormal pigmentation of skin
    • short stature
    • skeletal malformation
  • increased incidence of malignancies
A

fanconi anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what lab findings are typical of fanconi anemia

A
  • thrombocytopenia or leukopenia typically occurs first
    • followed by anemia -> severe aplastic anemia
  • bone marrow hypoplasia or aplasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

treatment of fanconi anemia

A
  • supportive: treat based on how they present
    • anemia: transfusion
    • thrombocytopenia: platelet transfusion
  • hematopoeitic stem cell transplant: definitive treatment
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

clinical presentation

  • peripheral pancytopenia with a hypocellular bone marrow
  • fatigue, pallor
  • frequent or severe infections
  • purpura, petechiae, and bleeding
A

acquired aplastic anemia

  • 50% idiopathic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what labratory findings are consistent with acquired aplastic anemia

A
  • all cell lines are affected early
    • anemia, usually normocytic
    • leukopenia with marked neutropenia
    • thrombocytopenia
    • low reticulocyte count
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are the leading causes of death in acquired aplastic anemia

A
  • overwhelming infection
  • severe hemorrhage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

treatment for acquired aplastic anemia

A
  • referral
  • Abx for infection
  • immunosuppressant agents
  • hematopoietic stem cell transplant (HSCT)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what is the most common nutritional deficiency in children

A

iron deficiency

  • prevalence is greatest among toddlers aged 1-2 yo; higher in african americans and hispanics, and those living in poverty
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

clinical presentation

  • pallor
  • fatigue, irritability
  • delayed motor development
  • hx of pica
A

iron deficiency

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

when is anemia first screened for

A

screening for anemia performed at 12 months

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

what are some risks for iron deficiency anemia

A
  • prematurity/low birth weight (iron absorbed in 3rd trimester)
  • lead exposure
  • exlcusive breast feeding beyond 4 mo w/o iron supplementation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what labratory findings are consistent with iron deficiency anemia

A
  • microcytic, hypochromic anemia
  • hemoglobin < 11 g/dL
  • ferritin < 12 mcg/L (iron stores will fall first)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

treatment for iron deficiency anemia

A
  • iron 6 mg/kg/d, 3 divided daily doses
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what are the common causes of vitamin B12 (cobalamin) deficiency

A
  1. intestinal malabsorption
  2. dietary insufficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what are the common causes of folic acid deficiency

A
  • increased folate requirements
    • rapid growth, chronic hemolytic anemia
  • malabsorptive syndromes (celiac disease)
  • inadequate dietary intake (rare)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

clinical presentation

  • pallor
  • glossitis
  • older children
    • paresthesias, weakness, or unsteady gait
    • decreased vibratory sensation and proprioception on neurologic exam
A

megaloblastic anemia

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

what condition is consistent with large neutrophils that have hypersegmented nuclei

A

megaloblastic anemia

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

treatment of megaloblastic anemia

A
  • **must treat vit B12 deficiency to avoid permanent neurological deficits
  • B12 and/or folic acid supplementation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

peripheral smear that shows spherocytes with increased osmotic fragility is consistent with what condition

A

Hereditary spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what is Hereditary spherocytosis

A
  • red cell membrane defect that makes it deformed. When it goes through spleen, RBC get stuck and phagocytized
  • type of hemolytic anemia
    • see jaundice, splenomegaly, and gallstones
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

treatment of Hereditary spherocytosis

A
  • supportive care +/- RBC transfusion
  • may need a splenectomy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

how is thalassemia diagosed

A

hemoglobin electrophoresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

thalassemia is what type of anemia

A
  • microcytic, hypochromic anemia
  • hemolytic anemias
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

thalassemia treatment

A
  • RBC transfusion?
  • iron monitoring + chelation
  • splenectomy may help (wait till age 3-6 yo)
  • hematopoietic stem cell transplant for severe beta thalassemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what is sickle cell disease

A
  • homozygous, Hb SS-hemolytic anemia
  • “sickle-shaped” RBCs when deoxygenated
  • splenic infarcts -> functional asplenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

chronic hemolysis with vaso-occlusion -> pain is consistent with what condition

A

sickle cell disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

how is sickle cell disease diagnosed

A

hemoglobin electrophoresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

treatment for sickle cell disease

A
  • avoid precipitating factors
  • oxygen
  • hydroxyurea for painful vaso-occlusive episodes
  • stem cell transplant
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what is G6PD deficiency

A
  • defect in an enzyme in RBC that protects cell from oxidative stress
  • X-linked recessive inheritance
  • episodic hemolysis at times of exposure due to
    • oxidant stress of infection
    • certain drugs and food substances
34
Q

G6PD deficiency is highest among what populations

A
  • african, mediterranean, and asian ancestry
35
Q

clinical presentation

  • neonatal jaundice, hyperbilirubinemia
  • episodic hemolysis
    • pallor, jaundice, hemoglobinuria
A

G6PD deficiency

  • RBC lysis -> dump of hemoglobin -> hyperbilirubinemia -> too much biliruben for liver to handle -> jaundice
36
Q

peripheral smear showing Heinz bodies is consistent with what condition

A

G6PD deficiency

37
Q

peripheral smear showing basophilic stippling is consistent with

A

lead poisoning

38
Q

lead poisoning treatment

A

chelation

39
Q

what is congential erythrocytosis (familial polycythemia)

A
  • only RBC affected (lots of RBC)
  • hemoglobin may be as high as 27 g/dL
    • concern: blood viscosity and thrombosis
40
Q

clinical presentation

  • plethora
  • splenomegaly
  • HA, lethargy
A

congential erythrocytosis (familial polycythemia)

41
Q

treatment of congential erythrocytosis (familial polycythemia)

A

phlebotomy (take blood away)

42
Q

what is secondary polycythemia

A
  • acquired, more common
  • occurs in response to hypoxemia
    • cyanotic congenital heart disease
    • chronic pulmonary disease
43
Q

treatment for secondary polycythemia

A
  • correct underlying disorder
  • phlebotomy when indicated
44
Q

risk of spontaneous bleeding when platelet count is below

A

< 20,000

  • nml 150,000 - 400,000 / mm3
45
Q

prothrombin time is a measure of what pathway? what are the factors of that pathway

A
  • PET - > extrinsic and common
    • I, II, V, VII, X, and tissue factor
46
Q

activated partial thromboplastin time (PTT) is a measure of what pathway? what are the factors of that pathway

A
  • PITT: intrinsic and common pathways
    • I, II, V, VIII, IX, X, XI, XII
47
Q

what are the factors of the common pathway

A
  • V, X, II (prothrombin), IIa (thrombin); I (fibrinogen)
48
Q

INR (international normalized ratio) is used to monitor

A

warfarin treatment

49
Q

“bleeding time” function

A
  • measures time for hemostasis
  • screens test for platelet dysfunction
  • prolonged in platelet disorders (vW disease) and severe thrombocytopenia
50
Q

what is the most common bleeding disorder of childhood

A

acute idiopathic thrombocytopenic purpura

  • most frequent in children 2-5 yo
51
Q

etiology of idiopathic thrombocytopenic purpura

A
  • often follows viral infection
  • immune mediated
  • antibodies bind to platelets
52
Q

prognosis of idiopathic thrombocytopenic purpura

A
  • 90% of children will have spontaneous remission
  • chronic ITP occurs n 10% of patients
53
Q

clinical presentation

  • multiple petechiae
  • ecchymosis
  • epistaxis
  • thrombocytopenia
  • normal WBC, normal hemoglobin
  • PT and PTT are normal
A

idiopathic thrombocytopenic purpura

54
Q

treatment of idiopathic thrombocytopenic purpura

A
  • avoid medications that compromise platelet function (NSAIDs, ASA)
  • prednisone: first line treatment
  • IVIG
  • splenectomy: emergency
55
Q

what is the most common inherited bleeding disorder

A

von Willebrand disease

56
Q

how is von Willebrand disease inherited

A

autosomal dominant

57
Q

what is von Willebrand factor

A
  • a protein that binds to factor VIII and is a cofactor for platelet adhesion to the endothelium
  • forms platelet plug
58
Q

clinical presentation

  • epistaxis
  • menorrhagia
  • GI bleeding
  • easy bruising
  • normal PT, prolonged or normal aPTT
  • Prolonged bleeding time
A

von Willebrand disease

59
Q

treatment for von Willebrand disease

A
  • Desmospressin (DDAVP): causes release of vWF and factor VIII from endothelial stores
  • vWF-replacement therapy
60
Q

what is Hemophilia A

A
  • factor VIII deficiency (most common)
  • X-linked M > F
61
Q

what is Hemophilia B

A
  • factor IX deficiency
  • X-linked: M > F
62
Q

clinical presentation

  • bleeding into joints and muscles
  • spontaneous hemarthrosis
    • can lead to joint destruction
  • normal platelet count, PT, and bleeding time
  • prolonged aPTT
A

Hemophilia

63
Q

treatment for Hemophilia

A
  • Hemophilia A : Desmopressin
  • factor replacement (VIII and IX)
64
Q

disseminated intravascular coagulation is triggered by

A
  • sepsis
  • trauma and tissue injury
  • malignancies
65
Q

pathophysiology of disseminated intravascular coagulation

A

triggering event -> widespread activation of coagulation cascade -> microthrombi -> massive consumption of platelets, fibrin, and coagulation factors -> severe bleeding

66
Q

clinical presentation

  • shock
  • hematuria, melena, purpura, petechiae
  • persistent oozing form needle punctures or other invasive procedures
  • evidence of thrombotic lesions
    • purpura fulminans
  • decreased platelet count
  • prolonged aPTT and PT
  • elevated D-dimer and fibrin degradation products
A

disseminated intravascular coagulation

67
Q

the liver produces what factors in coagulation cascade

A
  • prothrombin, fibrinogen
  • factors V, VII, IX, X, XII, and XIII
68
Q

what coagulation factors are dependent on vitamin K

A
  • II, VII, IX, and X
69
Q

function of activated protein C

A

inactivates activated factors V and VIII

70
Q

patients with protein C deficiency can develop what if they are given warfarin

A

warfarin-induced skin necrosis

71
Q

what is protein S

A

cofactor for protein C and facilitates the action of activated protein C

72
Q

what is factor V leiden mutation

A
  • point mutation
  • factor V becomes resistant to inactivation by activated protein C
73
Q

risk of venous thromboembolism is increased in those with factor V leiden mutation who

A
  • take oral contraceptives
74
Q

what is antithrombin? what happens if there is a deficiency

A
  • inhibitor of thrombin
  • deficiency causes venous thromboembolism
75
Q

in patients with antithrombin deficiency, what medication’s MOA is affected

A

Heparin efficiency may be diminished: bc it binds to antithrombin

76
Q

common characteristics of thrombotic disorders

A
  • hypercoagulable
  • may see DVTs or PEs
77
Q

treatment for thrombotic disorders

A
  • anticoagulant prophylaxis
  • anticoagulant agents
    • UFH
    • LMWH
    • warfarin
78
Q

what is the most common type of small vessel vasculitis

A

Henoch-Schonlein Purpura

79
Q

what condition often precedes Henoch-Schonlein Purpura

A

URI

80
Q

what is Henoch-Schonlein Purpura

A
  • deposition of IgA immune complexes
    • small vessels of the skin, GI tract, and kidneys
81
Q

clinical presentation

  • palpable purpura
  • arthritis/arthralgias
  • abd pain
  • renal disease
  • normal or elevated platelet count
  • antistreptolysin O titer often elevated
  • serum IgA elevated
  • UA: hematuria, proteinuria
A

Henoch-Schonlein Purpura

pediatrics II (4 decks)

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