CKPeds > Hema > Flashcards

Hema Flashcards

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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
Cx of Fanconi anemia
increased risk of leukemia (AML) and other cancers, organ complications, and bone-marrow failure consequences (infection, bleeding, severe anemia
26
Tx of Fanconi anemia
Corticosteroids and androgens | − Bone marrow transplant definitive
27
• Transient hypoplastic anemia between 6 months–3 years − Transient immune suppression of erythropoiesis − Often after nonspecific viral infection (not parvovirus B19)
Transient Erythroblastopenia of Childhood (TEC)
28
Recovery period of Transient Erythroblastopenia of Childhood (TEC)
Recovery generally within 1–2 months
29
Labs of Anemia of Chronic Disease and Renal Disease
Hb typically 6–9 g/dL, most normochromic and normocytic (but may be mildly microcytic and hypochromic
30
What is the cause? •RBCs at every stage are larger than normal; there is an asynchrony between nuclear and cytoplasmic maturation. • Ineffective erythropoiesis
MEGALOBLASTIC ANEMIAS
31
MCC of MEGALOBLASTIC ANEMIAS
Almost all are folate or vitamin B12 deficiency
32
Labs of MEGALOBLASTIC ANEMIAS
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
33
Presentation of Folic Acid Deficiency
• Peaks at 4–7 months of age—irritability, failure to thrive, chronic diarrhea
34
Causes of Folic Acid Deficiency
Cause—inadequate intake (pregnancy, goat milk feeding, growth in infancy, chronic hemolysis), decreased absorption or congenital defects of folate metabolism
35
Hypersegmented neutrophils have ___ lobes | in a peripheral smear.
>5
36
• Only animal sources; produced by microorganisms (humans cannot synthesize
Vitamin B12 (Cobalamin) Deficiency
37
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 __
4–5 months
38
SSx of Vitamin B12 (Cobalamin) Deficiency
Clinical—weakness, fatigue, failure to thrive, irritability, pallor, glossitis, diarrhea, vomiting, jaundice, many neurologic symptoms
39
Hereditary Spherocytosis and Elliptocytosis Abnormal shape of RBC due to ________ → decreased deformability → early removal of cells by spleen
spectrin deficiency
40
SSx of Hereditary Spherocytosis and Elliptocytosis
− Anemia and hyperbilirubinemia in newborn − Hypersplenism, biliary gallstones − Susceptible to aplastic crisis (parvovirus B19)
41
Labs of Hereditary Spherocytosis and Elliptocytosis
``` Labs − Increased reticulocytes − Increased bilirubin − Hb 6–10 mg/dL − Normal MCV; increased mean cell Hb concentration (MCHC ```
42
Confirmation of Hereditary Spherocytosis and Elliptocytosis
Confirmation—osmotic fragility test
43
What is the dx? − Increased reticulocytes, mild macrocytosis, polychromatophilia • Diagnosis—pyruvate kinase (PK) assay (decreased activity)
Pyruvate kinase (glycolytic enzyme) deficiency
44
Tx of Pyruvate kinase (glycolytic enzyme) deficiency
Treatment—exchange transfusion for significant jaundice in neonate; transfusions (rarely needed), splenectomy
45
2 syndromes of Glucose-6-phosphate dehydrogenase (G6PD) deficiency
− Episodic hemolytic anemia (most common) | − Chronic nonspherocytic hemolytic anemia
46
Inheritance of G6PD?
X-linked; a number of abnormal alleles
47
SSx of G6PD deficiency
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
Labs of G6PD deficiency after attack
free Hb and hemoglobinuria, Heinz | bodies, increased reticulocytes
49
Genetic problem in Sickle Cell Anemia (Homozygous Sickle Cell or S-Beta Thalassemia)
• Single base pair change (thymine for adenine) at the sixth codon of the beta gene (valine instead of glutamic acid)
50
SSx of Sickle Cell Anemia (Homozygous Sickle Cell or S-Beta | Thalassemia)
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
First SSx of Sickle Cell Anemia (Homozygous Sickle Cell or S-Beta Thalassemia)
First presentation usually hand-foot syndrome (acute distal dactylitis)—symmetric, painful swelling of hands and feet (ischemic necrosis of small bones)
52
What are the acute presentation of Sickle Cell Anemia (
° Younger—mostly extremities ° With increasing age—head, chest, back, abdomen ° Precipitated by illness, fever, hypoxia, acidosis, or without any factors (older)
53
Sickle Cell Anemia ° Infarction of bone and marrow (increased risk of_______
Salmonella osteomyelitis)
54
Sickle Cell Anemia _____ (peak age 6 mos to 3 yrs); can lead to rapid death
− Acute splenic sequestration
55
Altered splenic function → increased susceptibility to infection, especially with __________
encapsulated bacteria (S. pneumococcus, H. influenzae, N. meningitidis
56
Aplastic crisis—after infection with _________; absence of reticulocytes during acute anemia
parvovirus B19
57
Labs of Sickle Cell Anemia
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
Dx for Sickle Cell Anemia
° Confirm diagnosis with Hb electrophoresis (best test) | ° Newborn screen; use Hb electrophoresis
59
Tx for Sickle Cell Anemia
° Immunize (pneumococcal regular plus 23-valent, meningococcal) ° Start penicillin prophylaxis at 2 months until age 5 Folate supplementation Hydroxyurea
60
Tx for Sickle Cell Anemia Bone-marrow transplant in selected patients age ______
<16 years
61
–deletion of 2 genes – Common in African Americans and those of Mediterranean descent – Mild hypochromic, microcytic anemia (normal RDW) without clinical problems
Alpha thalassemia trait
62
______ deletion of 3 genes; Hgb Barts >25% in newborn period and easily diagnosed with electrophoresis
HgB H disease:
63
_______: deletion of 4 genes; severe fetal anemia resulting in hydrops fetalis
Alpha-thalassemia major
64
Alpha-thalassemia major prognosis
immediate exchange transfusions are required for any possibility of survival; transfusion-dependent with only chance of cure (bone marrow transplant)
65
Problem with Beta Thalassemia Major (Cooley Anemia)
Excess alpha globin chains → alpha tetramers form; increase in HbF (no problem with gamma-chain production)
66
SSx ov with Beta Thalassemia Major (Cooley Anemia)
• Presents in second month of life with progressive anemia, hypersplenism, and cardiac decompensation (Hb <4 mg/dL)
67
Labs of Beta Thalassemia Major (Cooley Anemia)
− 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
BMA of Beta Thalassemia Major (Cooley Anemia)
Bone-marrow hyperplasia; iron accumulates → increased serum ferritin and transferrin saturation
69
Tx of Beta Thalassemia Major (Cooley Anemia)
− Transfusions − Deferoxamine (assess iron overload with liver biopsy) − May need splenectomy − Bone-marrow transplant curative
70
SSx of von Willebrand disease (vWD) or platelet dysfunction → _________
mucous membrane bleeding, petechiae, small ecchymoses
71
SSx of Clotting factors deficiency
deep bleeding with more extensive ecchymoses and hematoma
72
______—platelet function and interaction with vessel walls; qualitative platelet defects or vWD (platelet function analyzer)
Bleeding time
73
_______ is the most common acquired cause of bleeding disorders in children
thrombocytopenia
74
What lab test ________ from initiation of clotting at level of factor XII through the final clot (prolonged with factor VIII, IX, XI, XII deficiency)
PTT—intrinsic pathway:
75
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)
PT—
76
________—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)
Thrombin time
77
* X-linked | * Clot formation is delayed and not robust → slowing of rate of clot formation
Hemophilia
78
Hemophilia − 2× to 3× increase in ______ − Correction with mixing studies
PTT (all others normal)
79
Hemophilia ________ sometimes used to diagnose carrier state
° Ratio of VIII:vWF
80
Hemophilia _____ increases factor VIII levels in mild disease
DDAVP
81
• Most common hereditary bleeding disorder; autosomal dominant, but more females affected
von Willebrand Disease (vWD)
82
SSx of von Willebrand Disease (vWD)
Clinical presentation—mucocutaneous bleeding (excessive bruising, epistaxis, menorrhagia, postoperative bleeding)
83
Labs of von Willebrand Disease (vWD)
Labs—increased bleeding time and PTT
84
VWD Tx − Most with type 1 ____ induces release of vWF − For types 2 or 3 need replacement → _______
DDAVP plasma-derived vWF-containing concentrates with factor VIII
85
Vitamin K is fat soluble so deficiency associated with a decrease in factors _______
II, VII, | IX, and X, and proteins C and S
86
All clotting factors produced exclusively in the liver, except for ______
factor VIII
87
Tx of bleeding from liver disease
Treatment—fresh frozen plasma (supplies all clotting factors) and/or cryoprecipitate (supplies fibrinogen
88
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
Immune Thrombocytopenic Purpura (ITP)
89
BMA of ITP
Bone marrow—normal to increased megakaryocytes
90
What shouldnt be given to pts with ITP
Transfusion contraindicated unless life-threatening bleeding (platelet antibodies will bind to transfused platelets as well)
91
Treatment of ITP If very low platelets, ongoing bleeding that is difficult to stop or life-threatening:
– Intravenous immunoglobulin for 1–2 days | ° If inadequate response, then prednisone
92
Treatment of ITP _____ reserved for older child with severe disease
– Splenectomy

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