Transfusion Medicine and Granulocyte Disorders Flashcards

1
Q

Storage of Whole Blood

A

35 days/4-6C

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2
Q

Storage of PRBCs

A

35 days/4-6C

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3
Q

A left shift in the WBC differential indicates a ______ in neutrophils and a _______ in lymphocytes.

A

increase; decrease

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4
Q

A right shift in the WBC differential indicates a ______ in neutrophils and a _______ in lymphocytes.

A

decrease; increase

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5
Q

Adult neutrophilia ANC

A

> 7500/mcL

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6
Q

Blood product used for increasing oxygen capacity to treat low HCT patients

A

PRBCs

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7
Q

Blood product used for low or absent fibrinogen associated with disorders and replacement for factor XIII deficiency

A

Cryoprecipitate

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8
Q

Blood product used for severe bacterial or fungal infection in patients with ANC <500/μL

A

Granulocyte concentrates

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9
Q

Blood product used to in assive transfusions to replace oxygen carrying capacity and blood volume

A

Whole Blood

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10
Q

Blood product used to treat bleeding associated with thrombocytopenia or platelet dysfunction

A

Platelets

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11
Q

Blood product used to treat coagulopathy related to procoagulant deficiency

A

FFP

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12
Q

Child/Adolescent/Adult ANC

A

<1500/mcL

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13
Q

Defect in which complement factor(s) describes the following symptoms: recurrent bacterial infections?

A

C3

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14
Q

Defect in which complement factor(s) describes the following symptoms: severe infections with Neisseria

A

C5-C9

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15
Q

Defect in which complement factor(s) describes the following symptoms: SLE, autoimmunity, and inflammatory vascular disease?

A

C1q, C2, C4

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16
Q

Eosinophil Count for Eosinophilia

A

> 350/mcL

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17
Q

For severe cases, _______ at a dose of 3-5mcg/kg subcutaneously every day will normalize production of neutrophils.

A

G-CSF

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18
Q

HCT of PRBCs

A

70%

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19
Q

Infant neutropenia ANC

A

<1100/mcL

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20
Q

Monocyte Count for Monocytosis

A

> 500 (Child/adult), >1000 (newborn)

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21
Q

Newborn neutropenia ANC

A

<3000/mcL

22
Q

Newborn neutrophilia ANC

A

> 13,000/mcL

23
Q

Storage of Cryoprecipitate

A

18 hours/4C then 1 year/-18C

24
Q

Storage of FFP

A

1 year/18C

25
Q

Storage of Granulocyte Concentrate

A

8-12hours/Room temp

26
Q

Storage of Platelet Concentrate

A

22-24C/5-7days

27
Q

The adverse reaction characterized by alloantibody production and slow destruction of sensitizing cells is _______

A

Delayed hemolytic Transfusion reaction

28
Q

The adverse reaction characterized by infiltrates, dyspnea, and difficulty maintaining peripheral oxygen saturation is ________.

A

Transfusion-Related Lung Injury (TRALI)

29
Q

The adverse reaction involving bronchospasm that is frequently seen in IgA-deficient individuals is _______.

A

Anaphylactic reaction

30
Q

The adverse reaction likely to occur with incompatible blood products is _______.

A

Immediate hemolytic transfusion reaction

31
Q

The advsere reaction characterized by fluid overload related to excessive amounts of products or cardiac dysfunction is _______.

A

Transfusion Associated Circulatory Overload (TACO)

32
Q

The most common adverse reaction to transfusion is _______.

A

febrile non-hemolytic reactions/mild allergic reactions

33
Q

What condition is caused by abnormal transferase resulting in altered fucosylation of adhesion molecules and poor interaction with selectins?

A

Leukocytosis Adhesion Deficiency II

34
Q

What condition is caused by allergic reactions, dermatitis, parasitic causes, tumor?

A

Eosinophilia

35
Q

What condition is caused by decreased adherence to endothelial surface and is characterized by soft tissue infections (skin, mucous membranes), gingivitis, mucositis, periodontitis, cellulitis, abscesses, omphalitis, poor wound healing?

A

Leukocytosis Adhesion Deficiency I

36
Q

What condition is caused by decreased rolling on endothelial surfaces and is characterized by recurrent infections, mental retardation, short stature, craniofacial abnormalities?

A

Leukocytosis Adhesion Deficiency II

37
Q

What condition is caused by hematologic disorders, collagen vascular disease, granulomatous disease, infection?

A

Monocytosis

38
Q

What condition is caused by hypersensitive reaction, inflammation and infection, myeloproliferative diseases?

A

Basophilia

39
Q

What condition is caused by mutation in CSH1 gene causes alterations in membrane fusion with formation of giant, leaky granules?

A

Chediak-Higashi Syndrome

40
Q

What condition is caused by neutrophilia with a defect in oxidase system with no toxic oxygen metabolites produced?

A

Chronic Granulomatous Disease (CGD)

41
Q

What condition is characterized by apoptosis of myeloid precursors associated with elastase (ELA-2) or HAX-1gene mutations? (Rarely: G-CSF receptor mutation)

A

Kostmann Syndrome

42
Q

What condition is characterized by ELA-2 mutations and apoptosis in precursors and cyclic hematopoiesis in cycles of ~21 days?

A

Cyclic Neutropenia

43
Q

What condition is characterized by FAS associated apoptosis of marrow precursors, ↓ CD34+ cells, marrow stromal defect?

A

Schwachman-Diamond Syndrome

44
Q

What condition is characterized by generally health and increased fungal infections when associated with diabetes?

A

Myeloperoxidase Deficiency

45
Q

What condition is characterized by multisystem disease: neutropenia (90-95%), pancreatic insufficiency, metaphyseal chondrodysplasia, other dysmorphic features?

A

Schwachman-Diamond Syndrome

46
Q

What condition is characterized by neutropenia, giant granules in all leukocytes, and abnormal degranulation?

A

Chediak-Higashi Syndrome

47
Q

What condition is characterized by oculocutaneous albinism, nystagmus photophobia, and recurrent infections of skin, mucous membranes, and respiratory tract by bacteria

A

Chediak-Higashi Syndrome

48
Q

What condition is characterized by recurrent fever, pharyngitis, aphthous ulcers, gingivitis, periodontitis?

A

Neutropenia

49
Q

What condition is characterized by recurrent severe infection (skin, sepsis) and poor wound healing as a consequence of decreased chemotaxis and ingestion?

A

Actin Dysfunction

50
Q

What condition is characterized by recurrent skin and deep tissue infections as caused by decreased chemotaxis and microbicidal activity?

A

Specific Granule Deficiency

51
Q

What conidition is caused by an autosomally recessive inheritance of defects in the CD18 genes?

A

Leukocytosis Adhesion Deficiency I

52
Q

What conidition is characterized by recurrent purulent infections with catalase positive bacteria and fungi involving skin and mucous membranes?

A

Chronic Granulomatous Disease (CGD)