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CKPeds > Hema > Flashcards

Hema Flashcards

1
Q 
Progressive drop in Hb over first 2–3 months until tissue oxygen needs are greater
than delivery (typically 8–12 weeks in term infants, to Hb of 9–11 g/dL
A

Physiologic Anemia of Infancy

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2
Q

Physiologic Anemia of Infancy

• Exaggerated in preterm infants and earlier; nadir at _____ to Hb of 7–9 g/dL

A

3–6 weeks

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3
Q

Reason for IDA in non-BF babies

A

− Higher bioavailability of iron in breast milk versus cow milk or formula

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4
Q

Infants with decreased dietary iron typically are anemic at_______

A

9–24 months of age.

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5
Q

MC SSx of Fe deficiency

A

Clinical appearances—pallor most common; also irritability, lethargy, pagophagia,
tachycardia, systolic murmurs; long-term with neurodevelopmental effects

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6
Q

Lab findings of IDA

A

− First decrease in bone marrow hemosiderin (iron tissue stores)
− Then decrease in serum ferritin
− Decrease in serum iron and transferrin saturation → increased total iron-binding
capacity (TIBC)
− Increased free erythrocyte protoporhyrin (FEP)

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7
Q

PBS findings in IDA

A

− Microcytosis, hypochromia, poikilocytosis
− Decreased MCV, mean corpuscular hemoglobin (MCH), increase RDW, nucleated
RBCs, low reticulocytes

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8
Q

Within 72–96 hours—peripheral reticulocytosis and increase in Hb over ____

A

4–30 days

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9
Q

IDA Tx

Continue iron for ____weeks after blood values normalize; repletion of iron in 1–3
months after start of treatment

A

8

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10
Q

Lead Poisoning

• Blood lead level (BLL) up to_____ is acceptable

A

5 μg/dL

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11
Q

_______—gold standard blood lead level

A

Confirmatory venous sample

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12
Q

Indirect assessments of Pb poisoning—
1
2

A
  1. x-rays of long bones (dense lead lines);

2. radiopaque flecks in intestinal tract (recent ingestion)

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13
Q

Labs of Pb poisoning

A

− Microcytic, hypochromic anemia
− Increased FEP
− Basophilic stippling of RBC

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14
Q

Treatment for Lead Poisoning:

5–14 (μg/dL)

A

Evaluate source, provide education, repeat blood lead level in 3 months

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15
Q

Treatment for Lead Poisoning:

≥70 μg/dL)

A

Immediate hospitalization plus 2-drug IV treatment:

– ethylenediaminetetraacetic acid plus dimercaprol

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16
Q

• Increased RBC programmed cell death → profound anemia by 2–6 months

A

Congenital Pure Red-Cell Anemia (Blackfan-Diamond)

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17
Q

Congenital Pure Red-Cell Anemia (Blackfan-Diamond)

Sx

A

− Short stature
− Craniofacial deformities
− Defects of upper extremities; triphalangeal thumbs

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18
Q

Congenital Pure Red-Cell Anemia (Blackfan-Diamond)

Labs

A

− Macrocytosis
− Increased HbF
− Increased RBC adenosine deaminase (ADA)

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19
Q

Congenital Pure Red-Cell Anemia (Blackfan-Diamond)

Other Labs

A

− Very low reticulocyte count
− Increased serum iron
− Marrow with significant decrease in RBC precursors

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20
Q

Congenital Pure Red-Cell Anemia (Blackfan-Diamond)

Tx

A

− Corticosteroids

− Transfusions and deferoxamine

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21
Q

MCC of Congenital Pancytopenia

A

• Most common is Fanconi anemia—spontaneous chromosomal breaks

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22
Q

Physical abn of Fanconi anemia

A

− Hyperpigmentation and café-au-lait spots
− Absent or hypoplastic thumbs
− Short stature

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23
Q

Labs abn of Fanconi anemia

A

− Decreased RBCs, WBCs, and platelets
− Increased HbF
− Bone-marrow hypoplasia

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24
Q

Dx of Fanconi anemia

A

bone-marrow aspiration and cytogenetic studies for chromosome breaks

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25
Q

Cx of Fanconi anemia

A

increased risk of leukemia (AML) and other cancers, organ complications,
and bone-marrow failure consequences (infection, bleeding, severe anemia

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26
Q

Tx of Fanconi anemia

A

Corticosteroids and androgens

− Bone marrow transplant definitive

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27
Q

• Transient hypoplastic anemia between 6 months–3 years
− Transient immune suppression of erythropoiesis
− Often after nonspecific viral infection (not parvovirus B19)

A

Transient Erythroblastopenia of Childhood (TEC)

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28
Q

Recovery period of Transient Erythroblastopenia of Childhood (TEC)

A

Recovery generally within 1–2 months

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29
Q

Labs of Anemia of Chronic Disease and Renal Disease

A

Hb typically 6–9 g/dL, most normochromic and normocytic (but may be mildly
microcytic and hypochromic

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30
Q

What is the cause?

•RBCs at every stage are larger than normal; there is an asynchrony between nuclear
and cytoplasmic maturation.

• Ineffective erythropoiesis

A

MEGALOBLASTIC ANEMIAS

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31
Q

MCC of MEGALOBLASTIC ANEMIAS

A

Almost all are folate or vitamin B12 deficiency

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32
Q

Labs of MEGALOBLASTIC ANEMIAS

A

Macrocytosis;
nucleated RBCs;
large, hypersegmented neutrophils;
low serum folate; iron and vitamin B12 normal to decreased; marked increase in lactate dehydrogenase; hypercellular bone marrow with megaloblastic changes

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33
Q

Presentation of Folic Acid Deficiency

A

• Peaks at 4–7 months of age—irritability, failure to thrive, chronic diarrhea

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34
Q

Causes of Folic Acid Deficiency

A

Cause—inadequate intake (pregnancy, goat milk feeding, growth in infancy, chronic
hemolysis), decreased absorption or congenital defects of folate metabolism

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35
Q

Hypersegmented neutrophils have ___ lobes

in a peripheral smear.

A

> 5

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36
Q

• Only animal sources; produced by microorganisms (humans cannot synthesize

A

Vitamin B12 (Cobalamin) Deficiency

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37
Q

Vitamin B12 (Cobalamin) Deficiency

Sufficient stores in older children and adults for 3–5 years; but in infants born to mothers with deficiency, will see signs in first __

A

4–5 months

38
Q

SSx of Vitamin B12 (Cobalamin) Deficiency

A

Clinical—weakness, fatigue, failure to thrive, irritability, pallor, glossitis, diarrhea,
vomiting, jaundice, many neurologic symptoms

39
Q

Hereditary Spherocytosis and Elliptocytosis

Abnormal shape of RBC due to ________ → decreased deformability →
early removal of cells by spleen

A

spectrin deficiency

40
Q

SSx of Hereditary Spherocytosis and Elliptocytosis

A

− Anemia and hyperbilirubinemia in newborn
− Hypersplenism, biliary gallstones
− Susceptible to aplastic crisis (parvovirus B19)

41
Q

Labs of Hereditary Spherocytosis and Elliptocytosis

A 
Labs
− Increased reticulocytes
− Increased bilirubin
− Hb 6–10 mg/dL
− Normal MCV; increased mean cell Hb concentration (MCHC
42
Q

Confirmation of Hereditary Spherocytosis and Elliptocytosis

A

Confirmation—osmotic fragility test

43
Q

What is the dx?

− Increased reticulocytes, mild macrocytosis, polychromatophilia
• Diagnosis—pyruvate kinase (PK) assay (decreased activity)

A

Pyruvate kinase (glycolytic enzyme) deficiency

44
Q

Tx of Pyruvate kinase (glycolytic enzyme) deficiency

A

Treatment—exchange transfusion for significant jaundice in neonate; transfusions
(rarely needed), splenectomy

45
Q

2 syndromes of Glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

− Episodic hemolytic anemia (most common)

− Chronic nonspherocytic hemolytic anemia

46
Q

Inheritance of G6PD?

A

X-linked; a number of abnormal alleles

47
Q

SSx of G6PD deficiency

A

Within 24–48 hours after ingestion of an oxidant (acetylsalicylic acid, sulfa drugs,
antimalarials, fava beans) or infection and severe illness → rapid drop in Hb,
hemoglobinuria and jaundice (if severe)

48
Q

Labs of G6PD deficiency after attack

A

free Hb and hemoglobinuria, Heinz

bodies, increased reticulocytes

49
Q

Genetic problem in Sickle Cell Anemia (Homozygous Sickle Cell or S-Beta
Thalassemia)

A

• Single base pair change (thymine for adenine) at the sixth codon of the beta gene
(valine instead of glutamic acid)

50
Q

SSx of Sickle Cell Anemia (Homozygous Sickle Cell or S-Beta

Thalassemia)

A

Newborn usually without symptoms;

development of hemolytic anemia over
first 2–4 months (replacement of HbF);

as early as age 6 months; some children
have functional asplenia;

by age 5, all have functional asplenia

51
Q

First SSx of Sickle Cell Anemia (Homozygous Sickle Cell or S-Beta
Thalassemia)

A

First presentation usually hand-foot syndrome (acute distal dactylitis)—symmetric,
painful swelling of hands and feet (ischemic necrosis of small bones)

52
Q

What are the acute presentation of Sickle Cell Anemia (

A

° Younger—mostly extremities
° With increasing age—head, chest, back, abdomen
° Precipitated by illness, fever, hypoxia, acidosis, or without any factors (older)

53
Q

Sickle Cell Anemia

° Infarction of bone and marrow (increased risk of_______

A

Salmonella osteomyelitis)

54
Q

Sickle Cell Anemia

_____ (peak age 6 mos to 3 yrs); can lead to rapid death

A

− Acute splenic sequestration

55
Q

Altered splenic function → increased susceptibility to infection, especially with
__________

A

encapsulated bacteria (S. pneumococcus, H. influenzae, N. meningitidis

56
Q

Aplastic crisis—after infection with _________; absence of reticulocytes
during acute anemia

A

parvovirus B19

57
Q

Labs of Sickle Cell Anemia

A

If severe anemia: smear for target cells, poikilocytes, hypochromasia, sickle
RBCs, nucleated RBCs, Howell-Jolly bodies (lack of splenic function); bone
marrow markedly hyperplastic

58
Q

Dx for Sickle Cell Anemia

A

° Confirm diagnosis with Hb electrophoresis (best test)

° Newborn screen; use Hb electrophoresis

59
Q

Tx for Sickle Cell Anemia

A

° Immunize (pneumococcal regular plus 23-valent, meningococcal)
° Start penicillin prophylaxis at 2 months until age 5
Folate supplementation
Hydroxyurea

60
Q

Tx for Sickle Cell Anemia

Bone-marrow transplant in selected patients age ______

A

<16 years

61
Q

–deletion of 2 genes
– Common in African Americans and those of Mediterranean descent
– Mild hypochromic, microcytic anemia (normal RDW) without clinical problems

A

Alpha thalassemia trait

62
Q

______ deletion of 3 genes; Hgb Barts >25% in newborn period and easily
diagnosed with electrophoresis

A

HgB H disease:

63
Q

_______: deletion of 4 genes; severe fetal anemia resulting in hydrops fetalis

A

Alpha-thalassemia major

64
Q

Alpha-thalassemia major prognosis

A

immediate exchange transfusions are required for any possibility of survival;
transfusion-dependent with only chance of cure (bone marrow transplant)

65
Q

Problem with Beta Thalassemia Major (Cooley Anemia)

A

Excess alpha globin chains → alpha tetramers form; increase in HbF (no problem
with gamma-chain production)

66
Q

SSx ov with Beta Thalassemia Major (Cooley Anemia)

A

• Presents in second month of life with progressive anemia, hypersplenism, and cardiac decompensation (Hb <4 mg/dL)

67
Q

Labs of Beta Thalassemia Major (Cooley Anemia)

A

− Infants born with HbF only (seen on Hgb electrophoresis)
− Severe anemia, low reticulocytes, increased nucleated RBCs, hyperbilirubinemia
microcytosis
− No normal cells seen on smear

68
Q

BMA of Beta Thalassemia Major (Cooley Anemia)

A

Bone-marrow hyperplasia; iron accumulates → increased serum ferritin and
transferrin saturation

69
Q

Tx of Beta Thalassemia Major (Cooley Anemia)

A

− Transfusions
− Deferoxamine (assess iron overload with liver biopsy)
− May need splenectomy
− Bone-marrow transplant curative

70
Q

SSx of von Willebrand disease (vWD) or platelet dysfunction → _________

A

mucous membrane bleeding, petechiae, small ecchymoses

71
Q

SSx of Clotting factors deficiency

A

deep bleeding with more extensive ecchymoses and hematoma

72
Q

______—platelet function and interaction with vessel walls; qualitative
platelet defects or vWD (platelet function analyzer)

A

Bleeding time

73
Q

_______ is the most common acquired cause of bleeding disorders in children

A

thrombocytopenia

74
Q

What lab test

________ from initiation of clotting at level of factor XII through the final clot (prolonged with factor VIII, IX, XI, XII deficiency)

A

PTT—intrinsic pathway:

75
Q

What lab test

______measures extrinsic pathway after activation of clotting by thromboplastin
in the presence of Ca2+;

prolonged by deficiency of factors VII, XIII or anticoagulants; standardized values using the International Normalized Ratio (INR)

A

PT—

76
Q

________—measures the final step: fibrinogen → fibrin; if prolonged:
decreased fibrin or abnormal fibrin or substances that interfere with fibrin
polymerization (heparin or fibrin split products)

A

Thrombin time

77
Q
  • X-linked

* Clot formation is delayed and not robust → slowing of rate of clot formation

A

Hemophilia

78
Q

Hemophilia

− 2× to 3× increase in ______
− Correction with mixing studies

A

PTT (all others normal)

79
Q

Hemophilia

________ sometimes used to diagnose carrier state

A

° Ratio of VIII:vWF

80
Q

Hemophilia

_____ increases factor VIII levels in mild disease

A

DDAVP

81
Q

• Most common hereditary bleeding disorder; autosomal dominant, but more
females affected

A

von Willebrand Disease (vWD)

82
Q

SSx of von Willebrand Disease (vWD)

A

Clinical presentation—mucocutaneous bleeding (excessive bruising, epistaxis, menorrhagia,
postoperative bleeding)

83
Q

Labs of von Willebrand Disease (vWD)

A

Labs—increased bleeding time and PTT

84
Q

VWD Tx

− Most with type 1 ____ induces release of vWF
− For types 2 or 3 need replacement → _______

A

DDAVP

plasma-derived vWF-containing concentrates
with factor VIII

85
Q

Vitamin K is fat soluble so deficiency associated with a decrease in factors _______

A

II, VII,

IX, and X, and proteins C and S

86
Q

All clotting factors produced exclusively in the liver, except for ______

A

factor VIII

87
Q

Tx of bleeding from liver disease

A

Treatment—fresh frozen plasma (supplies all clotting factors) and/or cryoprecipitate
(supplies fibrinogen

88
Q

What condition?

− Typically 1–4 weeks after a nonspecific viral infection

− Most 1–4 years of age → sudden onset of petechiae and purpura with or without
mucous membrane bleeding

− Most resolve within 6 months

A

Immune Thrombocytopenic Purpura (ITP)

89
Q

BMA of ITP

A

Bone marrow—normal to increased megakaryocytes

90
Q

What shouldnt be given to pts with ITP

A

Transfusion contraindicated unless life-threatening bleeding (platelet antibodies
will bind to transfused platelets as well)

91
Q

Treatment of ITP

If very low platelets, ongoing bleeding that is difficult to stop or life-threatening:

A

– Intravenous immunoglobulin for 1–2 days

° If inadequate response, then prednisone

92
Q

Treatment of ITP

_____ reserved for older child with severe disease

A

– Splenectomy

CKPeds (20 decks)

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