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EOR Pediatrics > Chapter 11: Hematology-RBC disorders > Flashcards

Chapter 11: Hematology-RBC disorders Flashcards

1
Q

When does physiologic anemia occur?

A

Nadir at 6-8wks of life in premature infants

Nadir at 2-3 mos in term infants

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2
Q

What should you think of that would cause anemia in young child?

A

excessive consumption of cow’s milk

prolonged exclusive breast feeding (>6mo)

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3
Q

What labs to order to evaluate anemia?

A
  • CBC (including RBC indices)
  • Differential WBC count
  • Reticulocyte countmost important
  • peripheral blood smear
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4
Q

Labs in anemia due to hemolysis

A
  • lactate dehydrogenase increased
  • indirect bilirubin increased
  • haptoglobin decreased
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5
Q

Who should you consider G6PD assay in?

A

AA and Mediterranean individuals w/ hemolytic anemia

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6
Q

Macrocytic anemia is most worrisome for what in children?

A

-Bone marrow failure or infiltration
(so bone marrow examination is often needed)
-B12 and flat are less common in children living in developed nations

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7
Q

Most common microcytic anemias=

A
  • iron deficiency
  • recurrent or chronic inflammation
  • Thalassemia trait
  • sideroblastic states
  • copper deficiency
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8
Q

Lead intoxication hematologic feature?

A

-basophilic stippling not microcytosis

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9
Q

Most common cause of anemia during childhood?

A

Iron deficiency

  • usually seen between 6-24mo of life
  • adolescent females because of menstruation
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10
Q

Clinical manifestations of iron deficiency anemia

A
  • decreased appetite
  • irritability
  • fatigue
  • decreased exercise tolerance
  • skin and mucous membrane pallor
  • tachycardia
  • systolic ejection murmur along LSB

SEVERE:

  • CHF
  • tachycardia
  • S3 gallop
  • cardiomegaly
  • hepatomegaly
  • distended neck veins
  • rales

(koilonycia, angular stomatitis, glossitis uncommon in kids)

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11
Q

Tx of mild to moderate iron deficiency anemia w/o evidence of CHF

A

3-6mg/kg/day of elemental iron by mouth

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12
Q

Improvement of labs w/ iron supplementation

A
  • Reticulocyte count increases in 2-3 days
  • Hemoglobin concentration normalizes w/in 1mo
  • *must continue iron for 2-3 months after the hemoglobin normalizes to replenish tissue stores and prevent recurrent iron deficiency
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13
Q

Alpha versus Beta Thalassemia

A

(named after the one that is deficient)

  • Alpha thalassemia = more beta than alpha
  • Beta thalassemia = more alpha than beta
  • excess pairs w/ itself, becomes unstable, precipitates and damages the membrane inside the developing erythroblast = ineffective erythropoiseis and hemolysis
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14
Q

look up thalassemias more & look up tx

A

pg 184-85; see pharm lectures

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15
Q

Who is thalassemia more common in?

A

-African, Southeast asian, mediterranean, and middle eastern populations

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16
Q

Anemia of inflammation is caused by

A
  • Chronic inflam disorders (IBD, JIA, chronic infection, malig)
  • acute or recurrent viral infections
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17
Q

how to distinguish iron deficiency anemia from anemia of inflammation

A

Iron deficiency = TIBC high; Ferritin low

inflam = TIBC is low; Ferritin high or normal

18
Q

Tx of anemia of inflammation

A

direct at cause of inflammation

-will resolve spontaneously when the underlying inflammatory condition resolves

19
Q

Normocytic anemias w/ decreased RBC production

A

Common theme is impaired or inadequate bone marrow response to anemia
(replacement of the marrow by fibrosis or infiltration of the marrow by malignant cells or deficiency of erythropoietin)
-Transient Erythroblastopenia of childhood
-Toxic insults (drug tox=myelosuppresion or chemo agents)
-Human parvovirus induced aplastic crisis

20
Q

What is transient erythroblastopenia of childhood?

A
  • pure red cell aplasia due to temporary suppression of bone marrow erythropoiesis
  • exact cause is unknown
  • self limited and associated w/ normal WBC and platelets
21
Q

Normocytic anemias w/ increased red cell production

A

Most commonly caused by hemolysis and can be divided into intrinsic and extrinsic subtypes

1) Intrinsic (defect of RBC component)
- herditary spherocytosis
- herditary elliptocytosis
- hereditary stomatocytosis
- paroxysmal nocural hemoglobinuria (only one not inherited)
- hemoglobinopathies (sickle cell, thalassemias)
- enzyme disorders (G6PD deficiency, pyruvate kinase deficiency)
2) Extrinsic hemolysis
- nonimmune: DIC, HUS, Toxin related, parasites, burns
- immune: deposition of antibody, complement or both on RBC

22
Q

What is a spherocyte?

A
  • abnormal RBC w/ high surface to volume ratio (globular or spherical rather than dicoid)
  • many causes = hereditary spherocytosis, immune hemolytic anemia, sepsis, burns, toxins
23
Q

How to detect a spherocyte

A

-Osmotic fragility test

does not diagnose hereditary spherocytosis just says there are spherocytes present

24
Q

What is hereditary spherocytosis

A

intrinsic defect due to abnormalities of cyoskeleton of RBC which causes shortened lifespan
-usually autosomal dominant but 25% of cases are caused by new mutations or are autosomal recessive

25
Q

Clinical manifestations of hereditary spherocytosis

A
  • exaggerated or prolonged neonatal jaundice (UNCONJUGATED)
  • hyperhemolytic episodes of increased anemia, pallor, and jaundice
  • mild to moderate normocytic anemia, reticulocytosis, elevated MCHC, and indirect hyperbilirubinemia
26
Q

Complications of Hereditary spherocytosis

A

Chronic hemolysis and increased flux of bilirubin through the hepatobiliary system can cause bilirubinate (pigment) gallstones
even in the first decade of life which may be asymptomatic or cause signs and symptoms of cholecystitis or choledocholithiasis

27
Q

Tx of Hereditary spherocytosis

A
  • expectant management
  • cholecystectomy for cholelithiasis
  • transfusion if aplastic crisis or hyper hemolytic episodes
  • Splenectomy can “cure” hemolytic anemia but underlying defect remains
28
Q

When to do splenectomy for hemolytic anemia

A
  • Symptomatic hemolysis affecting quality of life (fatigue, growth failure, need for frequent transfusions)
  • wait til 2y/o when possible
  • do immunizations before hand!
  • do several years of postsplenectomy prophylactic penicillins
29
Q

Risks of splenectomy

A
  • lifelong risk of overwhelming sepsis by encapsulated bacteria especially s.pneumo
  • thrombosis
  • pulmonary hypertension
30
Q

Sickle cell disease types

A

see pg188

31
Q

What is autoimmune hemolytic anemia

A

-patient produces autoantibodeis against self or antigens on his or her own blood cells
(this can be post infectious, idiopathic, drug induced, or feature of underling autoimmune disease or malignancy)
vs
alloimmune in which individual produces antibodies against red blood cells of someone of the same species

32
Q

Two types of autoimmune hemolytic anemia are cold and warm…what does this mean?

A

Warm = IgG (max efficacy at warm temps)
-autoimmune dz, lymphomas, viral infections

Cold = IgM (max efficacy at cold temps)
-EBV, mycoplasma pneumo, transfusion rxns

33
Q

Presentation of warm vs cold autoimmune hemolytic anemia

A

IgG = mainly jaundice but mild or no hemoglobinuria (extravascular)

IgM = marked hemoglobinuria and mild or no jaundice

34
Q

Tx of warm vs cold autoimmune hemolytic anemia

A

Tx is generally supportive

  • IgG=packed red blood cell transfusions and corticosteroids
  • IgM =nonsteroid responsive; keep pt warm to avoid some hemolysis
35
Q

Drugs that interfere with folate metabolism

A

methotrexate, trimethoprim, phenytoin, phenobarbital

36
Q

Test for B12

A

Schilling Test

  • no longer commercially available in US
  • confirm diagnosis of B12 deficiency anemia w/ low serum level
37
Q

List non megaloblastic macrocytic anemias

A
  • Diamond Blackfan Anemia
  • Fanconi Anemia
  • Severe Aplastic Anemia
38
Q

Diamond Blackfan Anemia characteristics/tx

A

Dominant or recessive pattern
-25% associated w/ congenital anomies (short stature, web neck. cleft lip, shield chest, triphalangeal thumbs)
-90% diagnosed in 1st year of life
Tx: 2/3 respond to oral cortiosteroids

39
Q

Fanconi Anemia characteristics/tx

A

autosomal recessive
resutls in pacytopenia
-mean age of onset is 8y/o
-signs: hyper pigmentation, cafe au lair spots, microcephaly, microphthalmia, short stature, horseshoe or absent kidney, absent thumbs
Tx: frequently require RBC transfusions, some improve w/ androgen therapy
-if progressive dz tx of choice is bone marrow transplant from HLA matched sibling donor

40
Q

Severe Aplastic Anemia characteristics

A

Acquried failure of the hematopoieticc stem cells that results in pancytopenia

  • results from exposure to chemicals, drugs, infectious agents, ionizing radiation
  • without tx 90% die wi/in 3 months of diagnosis from bleeding or infection
  • Tx: Treatment of choice is bone marrow transplant from HLA matching sibling donor

EOR Pediatrics (16 decks)

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