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A. PEDIA BOARD REVIEW > Blood Chap 446-490 > Flashcards

Blood Chap 446-490 Flashcards

1
Q

Reduction of the hemoglobin concentration on the RBC volume below the range of values occurring in healthy persons

A

Anemia

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2
Q

Hemoglobin level wherein clinical findings of pallor, sleepiness, irritability and decreased exercise tolerance

A

Hgb <7-8g/dl

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3
Q

Most useful indicator of iron stores

A

Serum ferritin

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4
Q

Reliable but late indicator of iron deficiency

A

Mean corpuscular volume (MCV)

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5
Q

Rare, congenital bone marrow failure syndrome, symptomatic in early infancy
Associated with craniofacial abnormalities (hypertelorism, cleft palate), skeletal anomalies, noteable ABSENT RADIAL PULSE
MACROCYTIC anemia
Elevated Erythrocyte adenine deaminase activity, low reticulocyte

Dx & Tx?

A

Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia)

Tx: Corticosteroid
*if unresponsive- hematopoietic stem cell transplantation

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6
Q

MC ACQUIRED red cell aplasia

A

Transient Erythroblastopenia of Childhood (TEC)

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7
Q

Severe, hypoplastic anemia in a previously healthy 1 year old boy
Noted to have a VIRAL ILLNESS prior to anemia

Normal MCV (Normocytic), with thrombocytosis
RBC adenosine deaminase is normal

Recovered after 2mos

Dx?

A

Transient Erythroblastopenia of childhood (TEC)

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8
Q

Usual VIRAL cause of red cell aplasia in patients with chronic hemolysis, who are immunocompromised, and fetus in utero

A

Parvovirus B19 (causes erythema infectiosum/fifth disease)

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9
Q

Brief cessation of erythropoiesis in patients with hemolysis causing severe anemia

A

Aplastic crisis

*Parvovirus induced aplastic crisis usually occurs only once

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10
Q

Low serum iron
Low to normal serum ferritin

A

Anemia of chronic disease

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11
Q

Site of erythropoietin production in fetus

A

Liver

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12
Q

Site of erythropoietin production in infants

A

Kidney

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13
Q

Progressive decline in Hgb in infants that starts during the first week of life and persists for 6-8wks, with major during 8 & 12 weeks

A

Physiologic anemia of infancy

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14
Q

MC cause of folate deficiency in older children

A

Malnutrition

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15
Q

Anemia in folate deficiency

A

Megaloblastic anemia

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16
Q

T/F chronic diarrhea may cause folic acid deficiency

A

True, it disrupts enterohepatic circulation of folate enhancing loss

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16
Q

T/F chronic diarrhea may cause folic acid deficiency

A

True, it disrupts enterohepatic circulation of folate enhancing loss

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17
Q

Vegetarian or vegan diet

A

Vitamin B12 (Cobalamin) deficiency

*Megaloblastic anemia

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18
Q

Excessive excretion of methylmalonic acid in the urine (0.3-5mg/24h)

A

Vitamin B12 deficiency (Cobalamin)

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19
Q

Most important clinical sign of iron deficiency

A

Pallor

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20
Q

Pallor is visible at Hgb of?

A

7-8g/dL

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21
Q

Desire to invest nonnutritive substances

A

Pica

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22
Q

Serum ferritin is
A. Decreased
B. Normal
C. Increased

in what type of anemia

A

A. IDA
B. THALASSEMIA
C. CHRONIC DISEASE

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23
Q

Estimate of the body’s iron stores in the absence of inflammation

A

Serum Ferritin

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24
Q

⬇️ serum ferritin
⬆️ serum transferrin

Hypochromic, microcytic

A

Iron deficiency anemia

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25
Q

Regular response of iron deficiency anemia to oral iron

A

Increase in Hgb >/=1g/dL after one month

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26
Q

Therapeutic dose of elemental iron for IDA

A

3-6mg/kg in 3 divided doses max 150-200mg daily

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27
Q

IDA response to oral iron after
A. 12-24 hrs
B. 36-48 hrs
C. 48-72 hrs
D. 4-30 days
E. 1-3 mos

A

A. Improvement of symptoms (subjective); decreased irritability, increased appetite; repletion of intracellular iron enzymes

B. Erythroid hyperplasia; initial bone marrow response

C. Reticulocytosis, peaking at 5-7 days

D. Increase in hemoglobin level

E. Repletion of stores

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28
Q

Premature destruction of RBC

A

Hemolysis

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29
Q

Laboratory evidence when seen denotes inravascular hemolysis

A

Free hemoglobin in plasma (plasma appears pink after centrifugation)

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30
Q

MC abnormality of the RBC membrane

A

Hereditary spherocytosis

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31
Q

Evidence of hemolysis

A

Reticulocytosis
Indirect hyperbilirubinemia

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32
Q

MC inherited abnormality of RBC membrane

A

Hereditary Spherocytosis

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33
Q

Anemia, hyperbilirubinemia, splenomegaly
Risk for GALLSTONE

Reticulocytosis, spherocutyes on blood smear, inc MCHC

A

Hereditary Spherocytosis

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34
Q

Resistance to malarial infection

A

Hereditary Elliptocytosis

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35
Q

Extreme microcytosis
Extraordinary variation of cell size & shape
INCREASED THERMAL INSTABILITY

A

Hereditary pyropoikilocytosis

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36
Q

Fever is a medical emergency in this hemoglobinopathy

A

Sickle cell anemia

*high risk for infection with encapsulated organisms due to functional asplenia

37
Q

Life-threatening complication of Sickle cell anemia

A

Acute splenic sequestration

38
Q

Left sided abdominal pain, rapid spleen enlargement, decline in hemoglobin at least 2g/dl
Reticulocytosis
Thrombocytopenia
History of fever, bacteremia, or viral infection

A

Acute splenic sequestration

39
Q

First manifestation of pain in infants & young children with Sickle cell anemia

A

Dactylitis (hand-foot syndrome)

*unilateral or symmetric swelling of the hands &/or feet

40
Q

Cardinal feature of Sickle cell anemia

A

Acute vasoocclusive pain

41
Q

Cause of osteomyelitis in children with sickle cell anemia

A

S. Aureus
Salmonella

42
Q

Only drug proven effective in reducing the frequency of painful episodes in Sickle cell anemia

A

Hydroxyurea

43
Q

Renal cell carcinoma is associated with

A

Sickle cell trait

44
Q

T/F: 70% Methemoglobin is lethal

A

True

45
Q

Blood is chocolate brown even when exposed to oxygen

A

Methemoglobin

46
Q

Treatment to reduce Methemoglobin in Hereditary Methemoglobinemia

A

Ascorbic acid (200-500 mkday)

47
Q

Treatment for Toxic Methemoglobinemia

A

Methylene blue (initially 1-2mg/kg IV, then 100-300mg PO as maintenance)

*except for G6PD (ineffective)

48
Q

Quantity of globin chains produced

A

Thalassemia

49
Q

Quality of globin chains produced

A

Sickle cell disease

50
Q

Maxillary hyperplasia, flat nasal bridge frontal bossing
Hepatosplenomegaly

A

Thalassemic facies

51
Q

Best indicator of total body iron stores

A

Quantitative liver iron

52
Q

Major cause of death in thalassemia

A

Cardiac disease

53
Q

Persistently low RDW and low MCV despite iron treatment

A

Beta thalassemia trait

54
Q

Heinz bodies
Polychromasia
Bite cells

A

G6PD

55
Q

Hallmark of autoimmune hemolytic anemia

A

Positive Direct Coomb’s test

56
Q

MC Cause of drug immune hemolytic anemia

A

Cephalosphorins

57
Q

Autoimmune hemolytic anemia with concomitant ITP

A

Evans syndrome

58
Q

Treatment for autoimmune hemolytic anemia

A

Glucocorticoids (Prednisone 2mkday up to 6 mkday)
IVIG if severe despite steroids
Rituximab in chronic refractory cases

59
Q

Warm antibodies

A

IgG

60
Q

Cold antibodies

A

IgM

61
Q

Below normal values of 3 blood cell lineages

A

Pancytopenia

62
Q

MC inherited Pancytopenia

A

Fanconi Anemia

63
Q

Short stature
Skin pigment changes (with café au lait spots(
Upper limb abnormalities (absent of radii and thumbs, radial pulse is weak or bsent)
Hypogonadal and genital changes
Facies: microcephaly, small eyes, epicanthal folds
Kidney abnormalities

PROPENSITY FOR CANCER

A

Fanconi Anemia

64
Q

MC cancer associated with Fanconi Anemia

A

Squamous cell carcinoma

65
Q

Inherited pancytopenia with associated exocrine pancreas insufficiency (fat malabsorption) and skeletal abnormalities (metaphyseal dysplasia)

A

Shwachman-Diamond syndrome

66
Q

Mucocutaneous (ectodermal) triad of Dyskeratosis Congenita

A

Reticulate skin pigmentation of the upper body
Nail dystrophy
Mucosal leukoplakia

67
Q

Factor VIII deficiency

A

Hemophilia A

68
Q

Factor IX deficiency

A

Hemophilia A

69
Q

Measures the activation of factor X by factor VII

A

Prothrombin time

*thus NORMAL in Hemophilia (factor VIII & IX deficiency)

70
Q

Hallmark of hemophilic bleeding

A

Hemarthroses - bleeding in the joint

71
Q

Vague area of referred pain in the groin
Hip is held flex, internally rotated position with inability to extend hip

A

Ilipsoas muscle bleeding

*may lead to hypovolemic shock

72
Q

NORMAL Prothrombin
Prolonged PTT

A

Hemophilia

73
Q

Standard of care for severe hemophilia

A

Prophylaxis

*Factor VIII (A) - 20-40 IU/kg every other day
*Factor IX (B) - 30-50 IU/kg every 2-3 days

74
Q

Failure of a bleeding episode to respond to appropriate replacement is the first sign of an

A

Inhibitor - antibodies directed against factor VIII or IX that blocks the clotting activity

75
Q

Off-label medication used as alternative therapy for patients with high inhibitor titers in whom immune tolerance program have failed

A

Rituximab

76
Q

MC inherited bleeding disorder

A

Von Willebrand disease

77
Q

Presents with mucosal bleeding
(+) family history of bleeding

A

Von Willebrand disease

78
Q

MC type of VWD
*presents with mucosal bleeding

A

Type I

79
Q

Most severe type of VWD

A

Type 3
*may present with joint bleed or CNS hemorrhage

80
Q

MC spontaneous thromboembolic event in neonates

A

Renal vein thrombosis

81
Q

Reverse effect of heparin

A

Protamine sulfate

82
Q

Rat poison

A

Vitamin K deficiency

83
Q

MCC of acute onset thrombocytopenia in a well child

A

Idiopathic Thrombocytopenic Purpura (ITP)

84
Q

MCC of acute onset thrombocytopenia

A

Idiopathic Thrombocytopenic Purpura (ITP)

85
Q

Previously healthy 1 - 4yr old
Sudden onset of generalized petechiae & purpura
History of vital illness
Profound thrombocytopenia in otherwise normal CBC

A

Idiopathic Thrombocytopenic Purpura (ITP)

86
Q

Acute ITP with persistent thrombocytopenia for >12mos

A

Chronic ITP

87
Q

Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function and CNS changes

A

Thrombotic Thrombocytopenic Purpura

88
Q

Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function and CNS changes

A

Thrombotic Thrombocytopenic Purpura

89
Q

Treatment for Thrombotic Thrombocytopenic Purpura (TTP)

A

Plasmapheresis

A. PEDIA BOARD REVIEW (13 decks)

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