Transfusion Medicine and Granulocyte Disorders Flashcards
Storage of Whole Blood
35 days/4-6C
Storage of PRBCs
35 days/4-6C
A left shift in the WBC differential indicates a ______ in neutrophils and a _______ in lymphocytes.
increase; decrease
A right shift in the WBC differential indicates a ______ in neutrophils and a _______ in lymphocytes.
decrease; increase
Adult neutrophilia ANC
> 7500/mcL
Blood product used for increasing oxygen capacity to treat low HCT patients
PRBCs
Blood product used for low or absent fibrinogen associated with disorders and replacement for factor XIII deficiency
Cryoprecipitate
Blood product used for severe bacterial or fungal infection in patients with ANC <500/μL
Granulocyte concentrates
Blood product used to in assive transfusions to replace oxygen carrying capacity and blood volume
Whole Blood
Blood product used to treat bleeding associated with thrombocytopenia or platelet dysfunction
Platelets
Blood product used to treat coagulopathy related to procoagulant deficiency
FFP
Child/Adolescent/Adult ANC
<1500/mcL
Defect in which complement factor(s) describes the following symptoms: recurrent bacterial infections?
C3
Defect in which complement factor(s) describes the following symptoms: severe infections with Neisseria
C5-C9
Defect in which complement factor(s) describes the following symptoms: SLE, autoimmunity, and inflammatory vascular disease?
C1q, C2, C4
Eosinophil Count for Eosinophilia
> 350/mcL
For severe cases, _______ at a dose of 3-5mcg/kg subcutaneously every day will normalize production of neutrophils.
G-CSF
HCT of PRBCs
70%
Infant neutropenia ANC
<1100/mcL
Monocyte Count for Monocytosis
> 500 (Child/adult), >1000 (newborn)
Newborn neutropenia ANC
<3000/mcL
Newborn neutrophilia ANC
> 13,000/mcL
Storage of Cryoprecipitate
18 hours/4C then 1 year/-18C
Storage of FFP
1 year/18C
Storage of Granulocyte Concentrate
8-12hours/Room temp
Storage of Platelet Concentrate
22-24C/5-7days
The adverse reaction characterized by alloantibody production and slow destruction of sensitizing cells is _______
Delayed hemolytic Transfusion reaction
The adverse reaction characterized by infiltrates, dyspnea, and difficulty maintaining peripheral oxygen saturation is ________.
Transfusion-Related Lung Injury (TRALI)
The adverse reaction involving bronchospasm that is frequently seen in IgA-deficient individuals is _______.
Anaphylactic reaction
The adverse reaction likely to occur with incompatible blood products is _______.
Immediate hemolytic transfusion reaction
The advsere reaction characterized by fluid overload related to excessive amounts of products or cardiac dysfunction is _______.
Transfusion Associated Circulatory Overload (TACO)
The most common adverse reaction to transfusion is _______.
febrile non-hemolytic reactions/mild allergic reactions
What condition is caused by abnormal transferase resulting in altered fucosylation of adhesion molecules and poor interaction with selectins?
Leukocytosis Adhesion Deficiency II
What condition is caused by allergic reactions, dermatitis, parasitic causes, tumor?
Eosinophilia
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?
Leukocytosis Adhesion Deficiency I
What condition is caused by decreased rolling on endothelial surfaces and is characterized by recurrent infections, mental retardation, short stature, craniofacial abnormalities?
Leukocytosis Adhesion Deficiency II
What condition is caused by hematologic disorders, collagen vascular disease, granulomatous disease, infection?
Monocytosis
What condition is caused by hypersensitive reaction, inflammation and infection, myeloproliferative diseases?
Basophilia
What condition is caused by mutation in CSH1 gene causes alterations in membrane fusion with formation of giant, leaky granules?
Chediak-Higashi Syndrome
What condition is caused by neutrophilia with a defect in oxidase system with no toxic oxygen metabolites produced?
Chronic Granulomatous Disease (CGD)
What condition is characterized by apoptosis of myeloid precursors associated with elastase (ELA-2) or HAX-1gene mutations? (Rarely: G-CSF receptor mutation)
Kostmann Syndrome
What condition is characterized by ELA-2 mutations and apoptosis in precursors and cyclic hematopoiesis in cycles of ~21 days?
Cyclic Neutropenia
What condition is characterized by FAS associated apoptosis of marrow precursors, ↓ CD34+ cells, marrow stromal defect?
Schwachman-Diamond Syndrome
What condition is characterized by generally health and increased fungal infections when associated with diabetes?
Myeloperoxidase Deficiency
What condition is characterized by multisystem disease: neutropenia (90-95%), pancreatic insufficiency, metaphyseal chondrodysplasia, other dysmorphic features?
Schwachman-Diamond Syndrome
What condition is characterized by neutropenia, giant granules in all leukocytes, and abnormal degranulation?
Chediak-Higashi Syndrome
What condition is characterized by oculocutaneous albinism, nystagmus photophobia, and recurrent infections of skin, mucous membranes, and respiratory tract by bacteria
Chediak-Higashi Syndrome
What condition is characterized by recurrent fever, pharyngitis, aphthous ulcers, gingivitis, periodontitis?
Neutropenia
What condition is characterized by recurrent severe infection (skin, sepsis) and poor wound healing as a consequence of decreased chemotaxis and ingestion?
Actin Dysfunction
What condition is characterized by recurrent skin and deep tissue infections as caused by decreased chemotaxis and microbicidal activity?
Specific Granule Deficiency
What conidition is caused by an autosomally recessive inheritance of defects in the CD18 genes?
Leukocytosis Adhesion Deficiency I
What conidition is characterized by recurrent purulent infections with catalase positive bacteria and fungi involving skin and mucous membranes?
Chronic Granulomatous Disease (CGD)