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Blood Flashcards

1
Q

What is the physiological definition of anemia?

A

Hemoglobin level too low to meet cellular oxygen demand.

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2
Q

What is the practical definition of anemia?

A

Hemoglobin level at least 2 standard deviations (SD) below the mean for age, gender, and race.

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3
Q

What are three reductions that characterize anemia?

A
  • Red cell/mm3
  • Hematocrit
  • Blood hemoglobin concentration
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4
Q

What is the hemoglobin threshold for anemia in newborns?

A

<13.5 g/dL

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5
Q

What is the most common type of anemia in infants and children?

A

Iron deficiency anemia.

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6
Q

What are the main causes of impaired red cell function leading to anemia?

A
  • Deficiencies
  • Decreased dietary intake
  • Increased demand
  • Decreased absorption
  • Impairment of red cell production
  • Increased loss
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7
Q

What are the dietary factors that enhance iron absorption?

A
  • Meat
  • Fish
  • Poultry
  • Seafood
  • Gastric acid
  • Ascorbic acid
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8
Q

What are the dietary factors that inhibit iron absorption?

A
  • Phosphate
  • Calcium
  • Tea (tannic acid)
  • Coffee
  • Colas
  • High doses of minerals
  • Bran/fiber
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9
Q

What is the primary nutrient deficiency in iron deficiency anemia?

A

Iron.

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10
Q

What is the typical iron absorption requirement during the first 15 years of life?

A

0.8 mg of iron daily.

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11
Q

What is the most common nutritional disorder in the world?

A

Iron deficiency anemia.

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12
Q

What is the major clinical manifestation of iron deficiency anemia?

A

Pallor.

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13
Q

Fill in the blank: The term for the desire to ingest non-nutritive foods is _______.

A

Pica.

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14
Q

What is the typical hemoglobin level associated with severe iron deficiency anemia?

A

<5 g/dL

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15
Q

What is the best test to confirm storage iron in the laboratory diagnosis of iron deficiency anemia?

A

Serum ferritin.

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16
Q

What is a common laboratory finding in peripheral blood for iron deficiency anemia?

A

Hypochromic, microcytic red blood cells.

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17
Q

What is the Mentzer Index value indicating iron deficiency anemia?

A

> 13.

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18
Q

What is the recommended therapeutic dose of iron for mild iron deficiency anemia?

A

3 mg/kg/day.

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19
Q

What is the recommended iron supplementation for full-term infants after 4 months?

A

15 mg/day.

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20
Q

What condition is caused by a loss of function mutation in the TMPRSS6 gene?

A

Iron refractory iron deficiency anemia.

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21
Q

What are the causes of vitamin B12 deficiency?

A
  • Inadequate nutrients
  • Malabsorption
  • Failure to secrete intrinsic factor
  • Inherited defects
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22
Q

What is the significance of elevated methylmalonic acid in laboratory findings?

A

Indicates vitamin B12 deficiency.

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23
Q

What are common clinical features of cobalamin and folate deficiency?

A
  • Pallor
  • Lethargy
  • Fatigability
  • Anorexia
  • Sore red tongue
  • Glossitis
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24
Q

Fill in the blank: The recommended intake of folate for pregnant women is _______.

A

3000–4000 μg/day.

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25
Q

What is the treatment for folic acid deficiency?

A

Folic acid: 2-5 mg/day x 2-3 weeks.

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26
Q

What is the treatment for vitamin B12 deficiency with neurological manifestations?

A

1 mg IM x 2 weeks.

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27
Q

What type of anemia is characterized by congenital red cell aplasia?

A

Congenital hypoplastic anemia.

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28
Q

What is the term for the condition characterized by an increased destruction of red blood cells?

A

Hemolytic anemia.

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29
Q

What condition is associated with hereditary spherocytosis?

A

Membrane defect leading to hemolytic anemia.

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30
Q

What is the daily folate requirement for adults?

A

3.1 μg/kg/day

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31
Q

What is the daily cobalamin requirement for adults?

A

1 μg/kg/day

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32
Q

What is the folate requirement for pregnant or lactating women?

A

3000–4000 μg/day

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33
Q

What is the primary etiology of congenital hypoplastic anemia?

A

Autosomal dominant (50%)

34
Q

What mutation is associated with Diamond-Blackfan anemia?

A

Mutation on gene RPS19

35
Q

What are two common clinical manifestations in infants with congenital hypoplastic anemia?

A
  • Pallor * Rare hydrops
36
Q

What percentage of patients with congenital hypoplastic anemia have congenital anomalies?

37
Q

What is the hallmark laboratory finding in Diamond-Blackfan anemia?

A

Elevated erythrocyte adenosine deaminase enzyme

38
Q

What are the key diagnostic criteria for Diamond-Blackfan anemia?

A
  • Normochromic macrocytic anemia * Reticulocytopenia * Normocellular marrow with selective deficiency of red cell precursors
39
Q

What is the definitive therapy for Diamond-Blackfan anemia?

A

Bone marrow transplant (HSCT)

40
Q

What is the median survival for patients with Diamond-Blackfan anemia?

41
Q

What is the most common age group affected by transient erythroblastopenia?

A

6-30 months

42
Q

What is a common clinical feature of transient erythroblastopenia?

A

Moderate to severe normocytic anemia

43
Q

What is the prognosis for transient erythroblastopenia?

A

Spontaneous recovery within weeks to months

44
Q

What laboratory evidence indicates extravascular hemolysis?

A
  • Increased B1 (indirect bilirubin) * Increased LDH * Increased fecal and urinary urobilinogen
45
Q

What are the two main classifications of hemolytic anemia?

A
  • Cellular defects (intrinsic) * Extracellular defects (extrinsic)
46
Q

What is the most common defect in hereditary spherocytosis?

A

Defect in RBC skeletal proteins

47
Q

What is the prevalence of hereditary spherocytosis?

48
Q

What laboratory test is used to diagnose hereditary spherocytosis?

A

Peripheral blood smearo
Hallmark: plenty spherocytes
Reticulocytosis
Bonemarrow: erythroid hyperplasia
Osmotic fragility test

49
Q

What is the hallmark laboratory finding in G6PD deficiency?

A

Increased reticulocyte count

50
Q

What are the clinical symptoms of class I G6PD deficiency?

A

Chronic hemolytic anemia

51
Q

What common food triggers hemolysis in G6PD deficiency?

A

Fava beans

52
Q

What is the treatment for paroxysmal nocturnal hemoglobinuria?

A

Splenectomy

53
Q

What is the main clinical manifestation of paroxysmal nocturnal hemoglobinuria?

A

Intravascular hemolysis

54
Q

What is the primary laboratory test for diagnosing paroxysmal nocturnal hemoglobinuria?

A

Flow cytometry

55
Q

What is a major group of disorders related to hemoglobin abnormalities?

A

Hemoglobinopathies

56
Q

What is the predominant hemoglobin in adults?

A

HbA (α2β2)

57
Q

What is the basic pathology of sickle cell disease?

A

Substitution of valine instead of glutamic acid in #6 position of β chain

58
Q

What are the three categories of sickle cell anemia?

A
  • Vaso-occlusive sickle cell crisis * Acute sequestration * Aplastic crisis
59
Q

What is the hallmark of vaso-occlusive sickle cell crisis?

A

Painful crisis

60
Q

What is the hallmark of Sickle Cell Disease (SCD)?

A

Painful crisis

61
Q

What is acute sequestration?

A

Acute splenic sequestration, leading to splenomegaly

62
Q

What is an aplastic crisis?

A

Temporary cessation of bone marrow activity due to suppression by infection

63
Q

What are the most frequent sites of occlusion in vaso-occlusive sickle cell crisis?

A
  • Lumbosacral
  • Spine
  • Ribs
  • Knees
  • Clavicle
  • Shoulder
  • Iliac crest
  • Femur
64
Q

What is priapism?

A

Trapped blood in the cavernous blood vessels of the penis, causing rigidity and erection

65
Q

What is acute chest syndrome?

A

New pulmonary infiltrate on chest X-ray involving at least 1 lung segment with symptoms

66
Q

What are the symptoms required to diagnose acute chest syndrome?

A
  • Temp. >38.5°C
  • Chest pain
  • Respiratory symptoms
  • Hypoxemia
67
Q

What causes acute splenic sequestration?

A

Destruction of RBCs leading to splenomegaly

68
Q

What is the main cause of aplastic crisis?

A

Human parvovirus B19

69
Q

What is the most common cause of death in children with SCD?

70
Q

What are common chronic organ damages due to SCD?

A
  • Cardiovascular
  • Renal
  • Hepatobiliary
  • Skin
  • Ear
  • Skeletal
  • Lungs
  • CNS
71
Q

What are the laboratory diagnosis findings for SCD?

A
  • Target cells
  • Sickle cells
  • Poikilocytes
  • Nucleated RBC
  • Howell Jolly bodies
72
Q

What is the treatment for painful crises in SCD?

73
Q

What is the only curative hope for SCD?

A

Bone marrow transplant (BMT)

74
Q

What is the survival rate probability at 20 years for SCD patients?

75
Q

Fill in the blank: The most common hematologic disease of infancy and childhood is _______.

A

Iron Deficiency Anemia

76
Q

True or False: Folate supplementation reduces the incidence of neural tube defects by 50-70%.

77
Q

What condition is caused by the substitution of valine instead of glutamic acid in the β chain?

A

Sickle Cell Disease

78
Q

What is the treatment of choice for hereditary spherocytosis?

A

Splenectomy

79
Q

What leads to development of megaloblastic anemia in a strict vegetable diet?

A

Folic acid deficiency

80
Q

Which food has the highest iron content?

81
Q

True or False: The world’s leading cause of iron deficiency anemia is blood loss.

Pediatric blood disorders (1 decks)

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