Term
|
Definition
| Hgb concentration more than 2 standard deviations below the normal range |
|
|
Term
| Symptoms related to hypoxia |
|
Definition
|
|
Term
|
Definition
| Hemorrhage, hemolysis and decreased production of RBCs |
|
|
Term
| Initial focus of pt with anemia |
|
Definition
|
|
Term
| Diagnostic test to differentiate between intra and extravascular hemolysis |
|
Definition
|
|
Term
|
Definition
RBCs are destroyed within the circulation
extrinsic (acquired)
More pronounced symptoms
pt is sick right away |
|
|
Term
|
Definition
RBCs are removed from circulation by the spleen
jaundice and splenomegaly
intrinsic (congenital) |
|
|
Term
|
Definition
| intrinsic RBC deformity, but is an intravascular hemolysis and congenital (oddball) |
|
|
Term
| Autoimmune hemolytic anemia |
|
Definition
| extrinsic RBC deformity, but is an extravascular hemolysis and acquired (oddball) |
|
|
Term
| Lab values of hemolytic anemia |
|
Definition
decreased Hb and Hct
increased or unchanged MCV
increased reticulcyte count
polychromatophilia - high reticulocytes
increased indirect bilirubin and LDH
decreased plasma haptoblobin - primarily in intravascular hemolysis
increased plasma Hb
Hemosiderinuria - intravascular hemolysis |
|
|
Term
|
Definition
| Direct antiglobin test detects IgG or complement bound to RBC membrane - hallmark of autoimmune hemolytic anemia |
|
|
Term
|
Definition
| Indirect antiglobin test detects RBC antibodies in serum of patient with immune-mediated hemolytic anemia - ABO blood typing |
|
|
Term
| RBC types associated with hemolytic anemia |
|
Definition
| Spherocytes, Acanthocytes, Target cells, Schistocytes - all caused by membrane problems leading to hemolysis |
|
|
Term
|
Definition
| most common cause of decreased production of RBCs leading to anemia |
|
|
Term
| Causes of decreased RBC production |
|
Definition
B12 or iron deficiency
kidney disease (decreased EPO)
chronic illness (diabetes)
bone marrow disorder (aplastic anemia) |
|
|
Term
| Mean Corpuscular Volume (MCV) |
|
Definition
Measure of mean RBC volume (size of RBC)
divide Hct by RBC count
80-100 = normocytic
less than 80 = microcytic
greater than 100 = macrocytic |
|
|
Term
| Mean Corpuscular Hemoglobin Concentration (MCHC) |
|
Definition
amount of Hb in the total RBC (amount of redness in the blood)
divide Hb by Hct
32-36 = normochromic
less than 32 = hypochromic
greater than 36 = hyperchromic |
|
|
Term
| Red cell distribution width (RDW) |
|
Definition
Identifies subpopulations of RBCs of different sizes (MCVs) not identified by MCV alone - if size and shape of RBC is off it will be elevated
normal in thallesemia
elevated in iron deficiency anemia |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| Elevated reticulocyte count |
|
Definition
| in a patient with normal marrow, suggests hemorrhage or hemolysis as cause of anemia |
|
|
Term
| Normal or decreased reticulocyte count |
|
Definition
| if not elevated with anemia, suggests defective production of RBCs (hypoproliferative anemia) due to acute blood loss, vitamin deficiency, bone marrow depression, defective RBC production, destruction of marrow, replacement of marrow by malignancy |
|
|
Term
|
Definition
reflects deficiency in Hgb synthesis, either in globins or heme
inherited causes: α and β thallesemias
acquired causes: iron deficiency, anemia of chronic disease, lead poisoning, drugs that interfere with heme synthesis (isoniazid) |
|
|
Term
|
Definition
greatest diagnostic challenge: due to underlying systemic illness or complex hematologic disorder
low reticulocyte count - usually requires marrow biopsy |
|
|
Term
|
Definition
megaloblastic caused by impaired DNA synthesis (B12 deficiency)
nonmegaloblastic (liver disease) |
|
|
Term
|
Definition
Iron deficiency
Sideroblastic anemia
Anemia of chronic disorder
Thalassemia |
|
|
Term
|
Definition
Infiltrative bone marrow disorder
Nutritional anemia
Anemia of chronic disorder
Hemolytic anemia
Anemia of renal insufficiency
Myelodysplastic anemia |
|
|
Term
|
Definition
Drugs (metformin)
Alcohol
Malabsorption syndromes
Nutritional anemias |
|
|
Term
|
Definition
| diagnostic for iron deficiency anemia if low |
|
|
Term
|
Definition
in the adult is almost always due to chronic blood loss (peptic ulcer, colorectal neoplasia, gastric surgery, hiatal hernia, upper GI malignancy)
microcytic, hypochromic anemia
increased RDW
serum iron decreases, TIBC increases |
|
|
Term
| Glossitis, angular chelitis and koionychia |
|
Definition
| common symptoms of iron deficiency anemia |
|
|
Term
|
Definition
RBCs are larger than normal due to a defect in DNA synthesis (elevated MCV)
Vitamin B12 and folic acid deficiency are most common
Beefy, red smooth tongue
neuropsychiatric disorders
macrocytic, hyperchromic anemia
hypersegmented PMNs |
|
|
Term
|
Definition
| serum homocysteine and methylmalonic acid levels are both elevated |
|
|
Term
|
Definition
| serum homocysteine elevated, serum methylmalonic acid is normal |
|
|
Term
| Glucose 6-Phosphate deficiency |
|
Definition
glutathione stores are epleted resulting in oxidative damage to Hgb, RBC proteins, and membrane lipids
Heinz bodies attach to membrane and contribute to membrane damage (bite cells)
damaged RBCs removed by spleen |
|
|
Term
| Pyruvate kinase deficiency |
|
Definition
only disorder of the glycolytic pathway
very rare
results in increased 2,3-DPG which actually facilitates O2 delivery in tissues, so symptoms are mild |
|
|
Term
| Hb electophoresis - Hg A2 increased |
|
Definition
| marker for beta-thalassemia |
|
|
Term
|
Definition
determined by typing RBC antigens with antisera
e.g. Patient is type A |
|
|
Term
|
Definition
determined by family studies
e.g. patient is AO |
|
|
Term
|
Definition
membrane bound
some absorbed onto membrane from plasma
Lewis
HLA |
|
|
Term
|
Definition
|
|
Term
| Secretion antigens (saliva, urine, tissues) |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
given the names Kell, Duffy or P
Kell are K or k
Duffy are Fya or Fyb
P are P, p1, p2p1k and p2k |
|
|
Term
| Main considerations in transfusion Tx |
|
Definition
|
|
Term
| Causes immediate hemolytic transfusion rxn |
|
Definition
|
|
Term
| Results in immunization and possible delayed transfusion rxn |
|
Definition
|
|
Term
| Glycoprotein precursor type 1 chains |
|
Definition
|
|
Term
| Glycoprotein precursor type 2 chains |
|
Definition
|
|
Term
|
Definition
precursor for ABO
large amounts present on type O RBCs |
|
|
Term
|
Definition
Lack substance H
hh genotype |
|
|
Term
|
Definition
|
|
Term
|
Definition
Develops around 6th week of fetal life
less than 50% present at birth
adult levels reached by 3 years |
|
|
Term
|
Definition
Naturally occuring
develop shortly after birth
generally IgM
may weaken with age |
|
|
Term
| Severe hemolytic transfusion rxns |
|
Definition
due to IgM binding compliment
as little as 150 cc can be fatal |
|
|
Term
|
Definition
Leukemia pts have progressively decreased antigen levels
Carcinoma of the stomach and intestinal disorders cause a decrease in antigen levels and make it difficult to get an accurate blood type
Antibody levels are altered in any disease resulting in hypogammaglobulinemia - CLL or non-Hodgkins lymphoma |
|
|
Term
| ABO forward (front) typing |
|
Definition
| testing of RBCs to determine the presence of A or B antigens |
|
|
Term
| ABO reverse grouping (back typing) |
|
Definition
testing of serum for the naturally occurring antibodies
uses cells of known ABO type |
|
|
Term
|
Definition
| Presence (+) or absence (-) of the D antigen |
|
|
Term
|
Definition
lack all Rh antigens
not the same as Rh negative
have RBC membrane abnormalities |
|
|
Term
|
Definition
Immune stimulated
mainly IgG
can cause severe Hemolytic Disease of the Newborn
implicated in hemolytic transfusion rxns |
|
|
Term
|
Definition
centrifugation of primary bag in blood processing
Platelet rich plasma expressed into secondary bag |
|
|
Term
|
Definition
centrifugation of secondary bag in blood processing - brings down platelets
Platelet poor plasma expressed into tertiary bag |
|
|
Term
|
Definition
Hct - 55-65%
shelf life - 42 days
stored at 4 celsius
one unit will increase pt's Hb by 1 g/dL
indication - to increase O2 carrying capacity |
|
|
Term
|
Definition
24 hour outdate to limit bacterial growth if accidentally contaminated
wash with saline
plasma proteins can cause allergic rxns
stored at 4 celsius |
|
|
Term
| Leukodepleted Red Blood Cells |
|
Definition
Leukocytes can induce antibodies to donor HLA antigens
alloimmunized pts have antibodies to most HLA types
White cell filter removes leukocytes to help avoid febrile transfusion rxn |
|
|
Term
|
Definition
Rare blood types can be saved 10 years
glycerol water mixture - glycerol washed off at blood center
two hours extra processing time |
|
|
Term
|
Definition
| individual unit from one unit of whole blood |
|
|
Term
| Indications for platelets |
|
Definition
| bleeding due to thrombocytopenia or thrombocytopathy |
|
|
Term
|
Definition
| sterile platelets docked to units |
|
|
Term
|
Definition
| platelets removed from anticoagulated whole blood |
|
|
Term
|
Definition
collected by an apheresis machine
give to neutropenic pts not responding to two days antibiotic Tx
most effective for neonates |
|
|
Term
|
Definition
seperated from anticoagulated whole blood and frozen within 24 hours
contains all clotting factors indcluding labile factors V and VIII |
|
|
Term
|
Definition
white precipitate that forms when frozen plasma is thawed at 4 celsius
precipitate is centrifuged and separated with 15 mL of plasma
enriched for factor VIII, vWF, factor XIII and fibrinogen
used for von Willebrand disease, fibrinogen deficiency or factor XIII deficiency (rare) |
|
|
Term
| irradiation of blood components |
|
Definition
crosslinks DNA and prevents lymphocytes from replicating
is harmless and a good way to prevent Graft Versus Host Disease |
|
|
Term
Acute hemolytic transfusion rxn
mechanism |
|
Definition
IgM or IgG binds to RBC surface
complement fixed - intravascualr hemolysis with hemoglobinemia
C3a and C5a formed - causing profound hypotension
DIC - clotting within renal glomeruli and arterioles |
|
|
Term
Acute hemolytic transfusion rxn
signs and symptoms |
|
Definition
Fever
chills
pain at IV site
low back and chest pain
renal failure
DIC
hemoglobinuria |
|
|
Term
Acute hemolytic transfusion rxn
Lab evaluation |
|
Definition
Hemoglobinemia
positive direct antiglobulin test (DAT)
increased LDH and potassium ion from intravascular hemolysis
increased indirect bilirubin
decreased haptoglobin
positive urine Hb
increased BUN, Cr in cases of renal failure |
|
|
Term
Acute hemolytic transfusion rxn
Treatment |
|
Definition
STOP the transfusion
put IV at TKO (to keep open)
increase IV fluids
Lasix - increase blood flow to renal arterioles
Mannitol (osmotic diuresis) for hyperkalemia
possibly renal dialysis
monitor closely for DIC |
|
|
Term
| Delayed hemolytic transfusion rxn |
|
Definition
1/20,000 transfusions
onset 5-21 days after transfusion
no clinical signs or symptoms
may have decreased Hb, fever, chills, fatigue, jaundice |
|
|
Term
Delayed hemolytic transfusion rxn
Mechanism |
|
Definition
Pt has prior exposure to RBCs through transfusion or pregnancy - develops IgM, IgG
Repeat exposure through transfusion - develops anamnestic response - high IgG titer
IgG coated RBCs destroyed extravascularly in the spleen |
|
|
Term
Delayed hemolytic transfusion rxn
Lab evaluation |
|
Definition
Anemia from destruction of donor RBCs
Spherocytes
positive direct antiglobulin test (DAT) - anti IgG antibodies
May see increased bilirubin, reticulocttes |
|
|
Term
Febrile Transfusion rxn (FTR)
Signs and symptoms |
|
Definition
Frequency - 0.5% of transfusions
1ºC or more rise in temperature
Chills, may see true rigor (shaking chill)
Headache
Nausea
Vomiting |
|
|
Term
Febrile Transfusion rxn (FTR)
Mechanism |
|
Definition
Patient has prior exposure to HLA antigens on white blood cells (WBCs) through transfusion or pregnancy develops IgM, IgG
Repeat exposure through transfusion of WBCs in RBCs or platelets
Patient antibodies bind to donor WBCs, releasing pyrogens (IL-1, IL-6, IL-8, TNF) causing fever / chills |
|
|
Term
Febrile Transfusion rxn (FTR)
Treatment |
|
Definition
STOP transusion
Put IV at TKO (to keep open)
send blood specimens to the bank to rule out acute hemolytic transfusion rxn
Tylenol to resolve fever
Demerol for rigor |
|
|
Term
Allergic Transfusion rxn (ATR)
Mechanism |
|
Definition
1% of all transfusions
IgE antibodies to donor plasma proteins |
|
|
Term
Allergic Transfusion rxn (ATR)
signs and symptoms |
|
Definition
hives/itching, erythema, can see fever
Bronchospasm in 1/50,000 transfusions
anaphylactic shock in 1/150,000 transfusions |
|
|
Term
Allergic Transfusion rxn (ATR)
Treatment |
|
Definition
STOP the transfusion
Put IV at TKO (to keep open)
mild rxn - Benadryl
Bronchospasm - Benadryl, Solu-cortef, epinephrine
Anaphylactic shock - Benadryl, Solu-cortef, epinephrine, dilution IV |
|
|
Term
|
Definition
Rapid infusion of large quantities of cold blood products
can result in cardiac arrhythmia/arrest |
|
|
Term
| Transfusion Citrate toxicity |
|
Definition
large quantities of citrated plasma will reduce ionized calcium
tingling of lips and fingers, may see muscle tremors and tetany
treat with calcium gluconate by IV |
|
|
Term
Transfusion fluid overload
mechanism |
|
Definition
1/10,000 transfusions
increased pulmonary artery and capillary pressures
leads to fluid leaks and pulmonary edema |
|
|
Term
Transfusion fluid overload
signs and symptoms |
|
Definition
Respiratory distress, severe hypoxia
Rales (crackles)
Neck vein distention
CXR – fluffy infiltrates, bilateral bases |
|
|
Term
Transfusion fluid overload
Treatment |
|
Definition
Oxygen 2-4 liters/minute
Lasix to induce diuresis
Phlebotomy
possible intubation |
|
|
Term
Transfusion Related Acute Lung Injury (TRALI)
mechanism |
|
Definition
Donor antibodies to recipient WBCs
Antibody coated WBCs attach to pulmonary endothelium
WBCs release cytokines and proteases
Increased pulmonary capillary permeability – pulmonary edema results |
|
|
Term
Transfusion Related Acute Lung Injury (TRALI)
signs and symptoms |
|
Definition
Respiratory distress, severe hypoxia
Rales
CXR white out
Occurs up to 6 hours after transfusion |
|
|
Term
Transfusion Related Acute Lung Injury (TRALI)
Treatment |
|
Definition
Oxygen 2-4 liters/minute
Close observation
Early intubation frequently required
Reaction resolves spontaneously in 48 hours |
|
|
Term
|
Definition
Unrecognized bacteremia in donor
Hair follicle or sweat gland at donation venipuncture site can have bacteria that chlorhexidine antiseptic doesn’t reach
RBCs or platelets contaminated with bacteria
production of toxins - causes shock and DIC |
|
|
Term
Septic Unit
signs and symptoms |
|
Definition
High fever
Shock
Nausea
Vomiting
DIC |
|
|
Term
|
Definition
STOP the transfusion
Put IV at TKO (to keep open)
Send blood specimen to blood bank to rule out acute hemolytic transfusion reaction
Support blood pressure with IV fluids
Antibiotics |
|
|
Term
|
Definition
Vitamin K dependent glycoprotein synthesized in the liver
involved in anticoagulation
Functions as a cofactor to Protein C in the inactivation of activated Factors Va and VIIa
exists in a free and a bound form |
|
|
Term
| Congenital Protein S deficiency |
|
Definition
inheritance is autosomal dominant
3 types - inadequate amounts of protein S in both free and bound form, defective protein molecules, decreased free protein S but normal amount of overall protein S |
|
|
Term
| Acquired Protein S deficiency |
|
Definition
Seen in consumptive processes like DIC or extensive DVT/PE
Patients taking warfarin (other Vitamin K-dependent factors)
Pregnancy
Liver disease |
|
|
Term
| Consequences of Protein S deficiency |
|
Definition
Deep venous thrombosis
Possible association with arterial thrombosis (stroke, heart attack) |
|
|
Term
| Acute Myelogenous Leukemia (AML) |
|
Definition
80% of acute leukemias in adults
increased incidence with ionizing radiation and with Down syndrome |
|
|
Term
| AML clinical presentation |
|
Definition
| Malaise, pallor, weight loss, fever and BLEEDING |
|
|
Term
|
Definition
Infection (70%)
Hemorrhage from thrombocytopenia (10%) |
|
|
Term
|
Definition
typical AML presentation on peripheral smear
Looks like little splinters |
|
|
Term
| Acute Lymphoblastic Leukemia (ALL) |
|
Definition
Primarily a disease of kids
increased incidence with ionizing radiation and with Down syndrome |
|
|
Term
| ALL clinical presentation |
|
Definition
Malaise, pallor, weight loss, fever and BLEEDING
CNS meningeal involvement
Lymphadenopathy
Hepatosplenomegaly |
|
|
Term
|
Definition
better prognosis in children
worse in infants and adults |
|
|
Term
| Clinical finding in all myeloproliferative disorders |
|
Definition
|
|
Term
| Chronic Myelogenous Leukemia (CML) |
|
Definition
Leukocytosis
Basophilia
Low leukocyte alkaline phosphatase
Philadelphia chromosome |
|
|
Term
|
Definition
unusually short autosome in the WBCs of CML patients
t(9,22)
brc-abl oncogene |
|
|
Term
|
Definition
Lethargy
weight loss
increasing abdomen girth
sweating
easy bruising/bleeding
Hepatosplenomegaly |
|
|
Term
|
Definition
Hb and Hct above normal
low EPO |
|
|
Term
|
Definition
Major:
Elevated RBC mass
Normal arterial oxygen saturation
Splenomegaly
Minor:
Thrombocytosis
Leukocytosis
Elevated leuk alk phosphatase
Elevated Vit B12 or Vit B12 binding capacity
*Must have all 3 major or first two major with any 2 minor |
|
|
Term
| PCV clinical presentation |
|
Definition
Patient is typically older
intense pruritis after bathing
Ruddy complexion
Splenomegaly
Macroglossia |
|
|
Term
|
Definition
Periodic phlebotomy
Hydroxyurea as a myelosuppresive agent
Anagrelide - reduces platelet count |
|
|
Term
|
Definition
Thrombosis
less often hemmorhage |
|
|
Term
| Chronic Idiopathic Myelofibrosis |
|
Definition
median age - 65 years
neoplastic stem cells migrate and make marrow in areas outside the bone marrow - primarily liver and spleen |
|
|
Term
Chronic Idiopathic Myelofibrosis
characteristics |
|
Definition
Tear drop RBCs
bone marrow "dry tap" due to increased collagen and reticulin fibers |
|
|
Term
| Essential Thrombocythemia |
|
Definition
Elevated platelets (>600K)
A myeloproliferative disorder with persistent thrombocytosis that is not reactive to another disorder and is not caused by another myeloproliferative disorder
Bone marrow shows megakaryocyte hyperplasia and clustering
Least common and least aggressive
near normal life expectancy |
|
|
Term
Essential Thrombocythemia
complications |
|
Definition
Thrombosis
Hemorrhage
Splenomegaly |
|
|
Term
Essential Thrombocythemia
Treatment |
|
Definition
Interferon-a
Hydroxyurea
Anegrilide
Plateletpheresis |
|
|
