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Nurs 648 > Heme > Flashcards

Heme Flashcards

1
Q

Signs and symptoms include: Vary based on severity of factor deficiency 1. Easy bruising at injection sites 2. Prolonged bleeding following circumcision 3. Excessive bruising after child begins walking 4. Mucosal bleeding 5. Prolonged bleeding in any part of the body 6. May have pain at the site of the bleed 7. Hemarthrosis (bleeding into the joint spaces)

A

Hemophilia

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2
Q

Particularly effective in areas where fibrinolysis appears to contribute to bleeding., i.e. in mucous membranes, trauma, and dental procedures

A

Antifibrinolytic Agents

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3
Q

Diagnostic platelet count <10,000

A

Chronic ITP

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4
Q

Moderate; gross bleeding following moderate trauma; some hemarthrosis; rare spontaneous hemorrhage

A

1-5% Factor VIII/IX

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5
Q

Management/treatment includes: 1. Children should be screen between the ages of 9-12 months and 1-5 years for at risk 2. Maintain breastfeeding for at least 6 months with iron-fortified cereal by 4-5 months; No cows milk before 1 year, limit to 18-24 oz 3. Administer iron until HMG normal and then additional 3-4 months to replace iron stores; give on empty stomach

A

Iron-Deficiency Anemia

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6
Q

Physical findings include: 1. Petechiae, purpura, and ecchymoses 2. Hemorrhages in mucous membranes 3. Pallor usually not present (usually there has been significant bleeding) 4. Splenomegaly

A

ITP

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7
Q

Diagnostic tests/findings include: 1. Variation in findings by specific variant as well as within same patient over time 2. Bleeding time - usually prolonged 3. VWF- usually decreased or absent 4. VWF antigen - usually decreased or absent 5. Blood group - VWF decreased in type O

A

VWD

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8
Q

Children should with this illness should also receive PPV every 5-10 years as recommended by current guidelines

A

SCD

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9
Q

Most severe ocular change in SCD; treated with laser therapy.

A

Proliferative Sickle Retinopathy

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10
Q

Prevention includes: Education regarding precipitating factors like dehydration, hypoxia, fever, exposure to extreme temperatures

A

Acute Painful Events

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11
Q

Regular infusions of factor VIII or IX given to prevent joint hemorrhage and bleeding episodes; initiated before 3 years old

A

Primary prophylaxis

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12
Q

Thrombocytopenia Platelet count associated with spontaneous bleeding; Risk for ICH

A

<10

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13
Q

Management includes: 1. PRBCs transfusion 2. Exchange transfusion for severe vaso-occlusive associated problems

A

Acute Exacerbations of Anemia

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14
Q

Affinity to platelet binding

A

Type 2M VWD

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15
Q

Asymptomatic with sickle hemoglobin 35-45% and hemoglobin A 55-65%; AS

A

Sickle Cell Trait

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16
Q

Diagnostic tests/findings include: 1. G6PD fluorescence-based screen (may give false negative) 2. RBC indices during or just after hemolytic episode (Heinz bodies present, fragmented cells and blister cells) 3. Reticulocytosis 4. Normal HMG between episodes 5. Acute self-limiting hemolytic anemia with hemoglobinuria

A

G6PD

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17
Q

Referral to hematologist 1. When thrombocytopenia worsens during viral illness or prior to elective surgery, prednisone or IVIG can be administered 2. Low-dose steroids may be necessary for for a prolonged period of time; antacids, H2 blockers, or PPI need to be taken with steroids 3. Rituximab may be effective in refractory instances 4. Splenectomy - treatment of choice when disease is severe or symptomatic (prophylactic PCN/AMX daily)

A

Chronic ITP

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18
Q

Severe anemia in-utero caused by alpha thalassemia; fatal without intervention

A

Hydrops Fetalis

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19
Q

Curative treatment for SCD that eliminates sickle cell erythrocytes and cellular progenitors and replaces with donor hematopoietic stems cells that give rise to erythrocytes

A

Stem Cell Transplant

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20
Q

Initiated for musculoskeletal bleeds

A

Physical therapy

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21
Q

Alternative treatment for VWD if desmopressin is not indicated or effective

A

Factor Concentrate with VWF

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22
Q

Diagnostic tests/findings include: 1. Anemia, hypochromia, microcytosis, low MCV, target cells, increased reticulocyte

A

Beta Thalassemia Major (Cooley’s Anemia) and Intermedia

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23
Q

Symptoms include chest pain, shortness of breath, chills, productive cough, hemoptysis, hypoxia, tachypnea, and wheezing; 1/3 do not exhibit symptoms.

A

Acute Chest Syndrome

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24
Q

Physical findings include: Signs of bleeding, including ecchymosis, swelling and pain in joints, and prolonged bleeding from lacerations or injections

A

Hemophilia

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25
Q

Hemoglobinopathy caused by decreased synthesis of beta-like globins, ineffective erythropoiesis, and shortened red cell life span

A

Beta Thalassemia

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26
Q

Initiated by 2 months of age and continued until at least 5 years of age for children with SCD

A

Prophylactic PCN

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27
Q

Mild to moderate clinical severity with sickle hemoglobin 45-55% and hemoglobin C 45-55%; near normal lifespan; SC

A

Sickle Hemoglobin C

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28
Q

Signs and symptoms include: 1. Bruising 2. Nosebleeds 3. Bleeding gums and lips 4. Petechiae 5. Menorrhagia 6. Child appears well except for signs of bleeding

A

ITP

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29
Q

A microcytic, hypochromic anemia caused by inadequate supply of iron, associated with low reticulocyte count and elevated red cell distribution width

A

Iron-Deficiency Anemia

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30
Q

Acute onset of fever with accompanying pulmonary symptoms including tachypnea, wheeze or cough, and new alveolar infiltrate suggestive of consolidation on chest x-ray; may result from bacterial or viral infection, fat embolism from bone infarction and necrosis, and vaso-occlusion of he pulmonary vasculature.

A

Acute Chest Syndrome

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31
Q

Management/treatment includes: 1. Generally mild symptoms require minimal intervention 2. Identification and avoidance of foods and drugs that cause hemolysis 3. Transfusion for severe hemolysis 4. Genetic counseling; routine screening not generally recommended

A

G6PD

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32
Q

Usually marked clinical severity with sickle hemoglobin equal to 80-90%; life span usually about 40 years; SS

A

Sickle Cell Anemia

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33
Q

Detects elevated blood velocity in middle cerebral arteries secondary to elevated CBF and/or stenosis; completed yearly (toddler-teens) as primary stroke prevention

A

Trans-Cranial Doppler Ultrasound

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34
Q

Bleeding disorder caused by congenital deficiency or absence of clotting factor VIII or IX

A

Hemophilia

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35
Q

Hemoglobinopathy that is usually asymptomatic with normal physical examination

A

Beta Thalassemia Minor (trait), Alpha Thalassemia (trait), HMG C/E

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36
Q

A collection of autosomal recessive, recessive genetic disorders characterized by the hemoglobin S variant of the beta-globin gene

A

Sickle Cell Disease

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37
Q

Rare form of VWD

A

Type 2B VWD

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38
Q

Physical findings include: 1. Hyperbilirubinemia in infants - usually associated with Med. and Canton individuals 2. Older children are asymptomatic between episodes of hemolysis

A

G6PD

39
Q

Immune-mediated disorder characterized by production of antiplatelet antibodies caused by an autoimmune disorder sometimes related to sensitization by viral infection, most prevalent during early-mid childhood

A

Immune Thrombocytopenia

40
Q

Diagnostic tests/findings include: 1. mild or no anemia, increased HbA2, microcytosis, hypochromia No management needed

A

Beta Thalassemia Trait

41
Q

ITP that continues between 3-12 months from diagnosis

A

Persistent ITP

42
Q

Hemoglobinopathy associated with occasional splenomegaly and microcytic anemia

A

HMG C/E and A-Thalassemia Hb H

43
Q

Isolated thrombocytopenia (platelet count of 100,000 in the absence of other causes)

A

Primary ITP

44
Q

Severe; spontaneous hemarthrosis and deep-tissue hemorrhages

A

< 1% Factor VIII/IX

45
Q

Mucocutaneous bleeding: 1. Epistaxis 2. Easy bruising 3. Petechiae 4. Menorrhagia 5. Gingival bleeding

A

Thrombocytopenia

46
Q

Symptoms develop 24-48 hours after ingestion of substance having oxidant properties: 1. Weakness 2. Pale appearance 3. Severe case - blood in urine, yellow discoloration of skin, whites of eyes, and gyms or inside of mouth

A

G6PD

47
Q

Sudden intrasplenic pooling of blood leading to acute drop in circulatory volume with rigid distended abdomen. Management includes prompt correction of hypovolemia, RBC transfusion, chronic transfusion therapy for recurrent episodes, and/or splenectomy (>2 moderate episodes or after 1 severe episode). Must avoid contact sports.

A

Splenic Sequestration

48
Q

Diagnostic tests/findings include: 1. Prenatal diagnosis - can be done by fetal blood sampling through fetoscopy or by analysis of DNA obtained through chorionic villus sampling or amniocentesis 2. Diagnostic test - direct assay of plasma factor activity for A or B 3. Screening tests - Activated partial thromboplastin time - prolonged; PT - normal; bleeding time - normal

A

Hemophila

49
Q

Factor IX deficiency, Christmas disease; X-linked disorder, with 20-30% spontaneous mutation

A

Hemophilia B

50
Q

ITP lasting for more than 12 months

A

Chronic ITP

51
Q

Management/Treatment includes: 1. Chronic transfusion protocol - to maintain hemoglobin, support growth, and prevent extramedullary hematopoiesis 2. Chelation therapy to remove excessive intracellular iron 3. Prophylactic PCN 4. Splenectomy may help (all immunizations given and daily PCN/AMX)

A

Beta Thalassemia Major

52
Q

Physical findings include: 1. Splenomegaly, occasional hepatosplenomegaly 2. Jaundice, usually mild 3. Abnormal facies secondary to extramedullary hematopoiesis - prominence of malar eminences, frontal bossing, depression of bridge of nose, exposure of upper incisors 4. Growth retardation 5. Bronze skin

A

Beta Thalassemia Major/Intermedia

53
Q

Associated with SCD: 1. Pain - most often in bones, but can occur in any part of body; in children <2 years usually in hands/feet 2. Swelling - sometimes seen at site of pain 3. Low-grade fever-sometimes occurs

A

Vaso-Occlusive Crisis

54
Q

An abnormal VWF molecule that does not bind with factor VIII

A

Type 2N VWD

55
Q

Signs and symptoms vary with severity: 1. Mild - usually asymptomatic 2. Fatigue 3. Irritability 4. Delayed motor development 5. Decreased attentiveness, shorter attention span, poor school performance 6. Eating nonnutrient substances such as ice, plaster, clay, paint, fabric

A

Iron-Deficiency Anemia

56
Q

Signs and symptoms include: Manifestations begin around 4-6 months of life 1. Pale skin or mucous membranes 2. Weakness 3. Characteristic facies (frontal bossing, exposure of frontal teeth, prominence of malar eminences)

A

Beta Thalassemia Major/Intermedia

57
Q

An inherited hemorrhagic disorder characterized by defective primary hemostasis and due to a quantitative or qualitative abnormality in blood factor; most common congenital bleeding disorder

A

Von Willebrand Disease

58
Q

Mild; severe hemorrhage only following moderate to severe trauma or surgery

A

6-25% Factor VIII/IX

59
Q

Administered intravenously or intranasally for mild FVIII deficiency

A

Desmopressin

60
Q

Treatment usually not indicated if platelet count >50,000; considered if platelet count <20,000, especially with extensive cutaneous bleeding or if protective environment cannot be ensured 1. High-dose corticosteroids for 1-3 weeks with slow wean 2. IVIG 1-3 days; treatment of choice for acute bleeds; platelets may be given but are short-lived 3. Anti-D IG

A

Acute ITP

61
Q

Decreased platelet-dependent VWF

A

Type 2A VWD

62
Q

Administered to children with frequent SCD complications to decrease hemolysis and painful events; increases production of Hb F, which retards gelation and sickling. May cause neutropenia, thrombocytopenia, anemia, GI disturbance, rash/nail changes, renal failure, splenic sequestration, and headache

A

Hydroxyurea

63
Q

All forms of immune-mediated thrombocytopenia except primary ITP

A

Secondary ITP

64
Q

Management/treatment includes: 1. Factor IX concentrate intravenously

A

Hemophilia B

65
Q

Hemoglobin S results from a single base pair substitution of valine for glutamic acid of the beta globin gene resulting in distorting RBCs into classic crescent or sickle shapes when deoxygenated; this leads to hemolysis and intermittent episodes of vascular occlusion that can cause tissue ischemia and acute/chronic organ dysfunction

A

Sickle Cell Disease

66
Q

Associated with SCD: 1. Weakness 2. Irritability 3. Unusual sleepiness 4. Paleness 5. Large spleen 6. Fast heart rate 7. Pain in the left side of abdomen (does not always occur)

A

Splenic Sequestration

67
Q

Administered for oral mucosal bleeds

A

Antifibrinolytic therapy

68
Q

Normal platelet count

A

150-400

69
Q

Antibiotic administered to SCD patient with fever

A

Ceftriaxone

70
Q

Diagnostic platelet count <20,000; often 10,000

A

Acute ITP

71
Q

Severe bleeding disorder with deficits in primary and secondary hemostasis

A

Type 3 VWD

72
Q

Management includes aggressive identification and treatment of infection, transfusion to improve hypoxia, oxygen supplementation, and mechanical ventilation when necessary. Airway hyperreactivity should be managed with bronchodilators.

A

Acute Chest Syndrome

73
Q

Factor VIII deficiency, classical hemophilia that is 3rd most common X-linked disorder and 4x more common than factor IX deficiency

A

Hemophilia A

74
Q

Treatment for SCD that binds to iron to facilitate elimination (iron overload); monitor serum ferritin levels and renal/hepatic function. Adverse effects - nausea, diarrhea, liver toxicity

A

Chelation Therapy

75
Q

Associated with SCD/Parvovirus B19: 1. Pale 2. Malaise 3. Headache 4. Fever 5. Mild upper respiratory infection symptoms

A

Aplastic Crisis

76
Q

Thrombocytopenia Platelet count associated with increased risk of bleeding, GI bleeding, and hematuria

A

<20

77
Q

Management includes: 1. Home-based - analgesia, hydration, heat, localized massage 2. ED - parenteral fluids, analgesia 3. Inpatient - Hydration, parenteral analgesics and NSAID

A

Acute Painful Events

78
Q

Started after a joint has developed hemarthrosis; used to prevent further bleeding into the joint

A

Secondary prophylaxis

79
Q

Used to treat bleeding complications or as preoperative preparation for Type 1 VWD; contraindicated in the treatment of Type 2B

A

Desmopressin

80
Q

Laboratory findings include: decreased hemoglobin (6.5-9.5), increased reticulocyte count (5-20), and increased MCV (>80)

A

Sickle Cell Anemia/Sickle Beta Zero Thalassemia

81
Q

Signs and symptoms vary with associated problems: 1. Hemolysis 2. Chronic anemia 3. Jaundice 4. Aplastic crisis 5. Cholelithiasis 6. Delayed growth and sexual maturity 7. Vaso-occlusion 8. Recurrent acute pain 9. Functional asplenia 10. Splenic sequestration 11. Acute chest syndrome 12. Stroke 13. Hyposthenuria and enuresis 14. Papillary necrosis of kidneys 15. Chronic nephropathy 16. Priapism 17. Avascular necrosis of humeral heads, femoral heads 18. Proliferative retinopathy 19. Leg ulcers

A

Sickle Cell Disease

82
Q

Diagnostic tests/findings include: 1. Hemoglobin - falls 2 standard deviations below mean for age and gender 2. Peripheral blood smear - hypochromic, microcytic red cells; MCV decreased by 2 3. Wide red cell distribution width >17 4. Decreased serum ferritin (first to fall) 5. Decreased total serum iron 6. Increase iron-binding capacity 7. Decreased iron saturation 8. Elevated free erythrocyte protoporhyrin

A

Iron-Deficiency Anemia

83
Q

Diagnostic tests/findings include: 1. CBC - generally the only required test 2. Platelet count

A

ITP

84
Q

Signs and symptoms include: Great variations in frequency, severity, and bleeding manifestations 1. Nosebleeds 2. Bleeding gums 3. Heavy menstrual bleeding 4. Prolonged oozing from cuts 5. Increased bleeding after trauma or surgery

A

VWD

85
Q

X-linked or autosomal recessive genetic disorder in which activity of red blood cell enzyme is decreased or absent, causing hemolytic anemia; most common among individuals of African, Mediterranean, or Asian descent

A

G6PD

86
Q

Mild to moderate clinical severity with sickle hemoglobin 65-90%, hemoglobin A 5-30%, and fetal hemoglobin 2-20%; near normal lifespan; SB

A

Sickle Beta Thalassemia

87
Q

Physical findings include: jaundice, cardiac murmur, splenomegaly, and pallor

A

SCD

88
Q

Causes functional asplenia, leading to extreme susceptibility to infection. Pneumococcal immunization and PCN prophylaxis initiated for infection prevention.

A

Splenic Infarction

89
Q

Physical findings include: 1. Mild - normal physical examination 2. Pallor 3. Tachycardia 4. Systolic murmur 5. Hepatomegaly 6. CHF

A

Iron-Deficiency Anemia

90
Q

Management/treatment includes: 1. Factor VIII concentrate intravenously

A

Hemophilia A

91
Q

Made in the bone marrow via fragmentation of megakaryocytes; circulate for 7-10 days.

A

Platelets

92
Q

Physical findings include: 1. Easy bruising 2. Multiple sites of bruising 3. Oozing or bleeding at trauma or surgical site

A

VWD

93
Q

Hemoglobinopathy associated with deletion of one or more alpha globin genes, increased but ineffective erythropoiesis, and shortened red cell life span

A

Alpha Thalassemia

94
Q

Mild to moderate decrease in VWF

A

Type 1 VWD

Nurs 648 (6 decks)

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