Other: Hematology Flashcards

Bone marrow
Terms Definitions
splen-
spleen
phago-
eat, ingest
Polycythemia Clinical manifestations
Headaches
Dizziness
Parasthesias
Visual disturbances
Hypertension
Thrombophlebitis
Joint pain
Hepatosplenomegaly
Ruddy complexion
Angina
Pruritis
M5 genetics
11q23 translocation
MCH calculation
Hemoglobin/RBC count
What is secondary hemostasis?
coagulation
Lepirudin
Direct thrombin inhibitorsindicated for patients with heparin-induced thrombocytopenia (HIT). should be usedcautiously in patients with renal failure, since it can accumulate andcause bleeding in these patients. Patients may develop antihirudinantibodies that occasionally cause a paradoxical increase in theaPTT; therefore, daily monitoring of the aPTT is recommended.There is no antidote for lepirudin.
Describe the blood picture.
Eosinophilia
Lymphocytosis

define, causes
>4000 cells/ul
Acute infection--pertussis, EBV, hepatitis
Chronic infection - brucellosis, TB, syphillis
Hypersensitivity rxn to drug
Cancer - lymphomas, leukemias
Anemia
Nursing Diagnoses
Activity intolerance
Imbalanced nutrition: Less than body requirements
Ineffective therapeutic regimen management
Potential complication: Hypoxemia
Widespread hyaline thrombi formation, made up of platelet aggregates, which lodge in the microvasculature is the underlying cause of what two conditions?
TTP-HUS
normal PT and aPTT in
Thrombocytopenia
Antbiotic Anthracyclines
Doxorubicin, •Mechanism: DNA intercalator, DNA breaks by free radical damage andTopo-interference.Topo II •Toxicity: DLT - cardiotoxicity (totaldose, often irreversible),myelosuppression
Hyperplasia
increase in number of cells
GPIb/IX
Binds platelets to vWF multimers
Normal platelet count
150,000 - 400,000/mm^3
Treating autoimmune hemolytic anemias
Underlying condition
Splenectomy
Steroids
Immunosuppresion
Anti-CD20 (rituximab)
Folic acid
vWD treatment
DDAVP (desmopressin, synthetic vasopressin)
Stimulates release of vWF
Good in 1 and mild 2, BAD in 2b

Factor replacement
Anti-fibrinolytics to stabilize clots
Contraceptive for mennorhagia

Avoid aspirin
Complex protein-iron compound that binds with oxygen and carbon dioxide
Hemoglobin
Hematopoiesis
- formation, production, and maintenance of blood cells - 3-12 weeks: yolk sac (megaloblastic period); 3-6 months: liver (hepatic period); > 6 mo: bone marrow (myeloid period)
Which intermediate in heme synthesis is water soluble and can turn up in urine in case of excess?
Uroporphyrinogen
What causes hemophilia B?
Factor IX deficiency
abnormality of 1-3 cell lines
myelodysplastic syndrome
This thrombin induced enzyme activates Protein C
Thrombomodulin
Microspherocytes
Small, sphere-shaped red blood cells seen in certain kinds of anemia.
Pancytopenia
decreased number of all blood cells
What type of anemia?
Lead poisoning
Microcytic anemia
Describe prolymphocytic leukemia (PLL).
-absolute lymphocytosis
-relatively large cells having one prominent nucleolus
-chromatin structure intermediate between that of blast and mature lymphocyte
-relatively uniform within a given patient
-anemia
-thrombocytopenia
Neutropenia caused by bone marrow infiltration by
Tumor
Sarcoidosis
Tuberculosis
M6: Acute Erythrocytic leukemia
Rare

Erythroleukemia
>50% erythroid precursors in marrow
>20% of myeloblasts are non-erythroid

Pure erythroid leukemia
80% of precursors are erythroid
Faggot cell
multiple auer rods (azurophilic granules)
Amount (%) of WBC's in blood(Leukocytes, thrombocytes, platelets)
<1%
what is severe neutropenia?
less than 500
DIC- triggers
acidosis, poor perfusion, or endotoxins; noted in infants with sepsis, RDS, MAS, and amniotic fluid aspiration
What condition is called "Christmas factor"?
Hemophilia B
Leukemoid Reaction
Called leukemoid because it resembles leukemia, but it is benign; Severe leukocytosis with immature cells seen in peripheral blood
S/S of Megaloblastic Anemias
Fatigue, exaggerated cardiovascular problems, exaggerated pulmonary problems, sore smooth tongue, CNS affects (in B12 deficiency)
COMPOSTION OF RBC'S
95% HEMOGLOBIN-OXYGEN CARRIER CONTAINS IRON
Improves engraftment in patient with aplastic anemia
Antithymocyte globulin
Sorafenib tosylate
inhibits signaling in both the Raf/MEK/ERK pathway (Ser/Thr kinase) and the VEGFR/PDGFR (Tyr kinase) pathway Uses: Renal cell carcinoma, hepatocellular carcinoma, malignant melanoma, in trials for others Metabolized by CYP3A4, inhibits several CYPs DLT - bone marrow suppression, mostcommon toxicity is skin rash
Toxic Granulation
Medium to large metachromatic granules that are evenly distributed through the cytoplasm; may be seen in severe bacterial infections, severe burns, or other conditions.
folic acid deficiency is promonent in who
alcoholics
calculating MCH
MCH = Hemoglobin X 10/RBC Count
RBCS with normal amounts of hemoglobin are termed ________________.
normochromic
How is hereditary spherocytosis confirmed?
Osmotic fragility test
chronic myelogenous leukemia presentation
leukocytosis and sometimes thrombocytosis lead to
fatigue, night sweats, low-grade fever
abdominal pain, splenomegaly, bone pain
rare --&gt; lymphadenopathy, infection or bleeding
leukostasis has dyspnea, blurry vision, priapism, thrombosis, stroke
Describe rouleaux.
Erythrocytes arranged in rows like stacks of coins; increased proteins in patients with rouleaux may make background of slide appear blue.
Three catagories of causes of thrombocytopenia
Underproduction
Peripheral destruction
Splenic sequestration
AML, therapy related
Generally poor prognosis

1) with ionizing radiation or alkylating agents (cyclophosphamide, chlorambucil)
--presents as MDS years later

2) with topoisomerase 2 inhibitors (often associated with 11q23 changes)
--presents as overt leukemia, sometimes as soon as 12 months out
HD subgroups
Nodular sclerosis - most common neck/mediastinum, fibrous capsule and bands of expanding cells
Lymphocyte predominant - neck limited disease, young adults
Lymphocyte depleted - oft retroperitoneal aggresive
Mixed celluarlity - very aggressive
Burkitt's general features
Highly aggressive
High LDH, rapid doubling time
Presents with bulky disease can involve CNS, bone marrow, extranodal sites
Hogkins's disease pathology
Reed-Sternberg cell
a minor population but diagnostic
altered B cell
CD15 and 30 positive

Spread continguously via lymphatics
Neutropenia Nursing Management Discharge teaching
Meticulous personal hygiene
Avoid crowds
Avoid persons known to have a cold or other contagious illness
Avoid swimming in lakes and crowded pools
Keep home free of dust/dirt
What is the cytoplasm of basophil
Gray blue
hemoglobin D
cannot seperate from hgbs through electrophoesis
Hemolytic anemiaauto-immun disease
hypogammaglobulinemia is a significant feature in half of patients with CLL can lead to
alleregy or parasites
an elevated amount of eosinophils means that what is wrong?
What is the first cell to have receptors for erythropoetin?
CFU-E
Diagnosis of Beta Thalassemia
Clinical/ Family History; Peripheral Blood Smear; Elevated Levels of HgA2 (compensation becuase HgA levels are decreased in Beta thal); Alpha:Beta chain ratios
Leukopenia
A reduction in WBC number; Can have neutropenia, lymphocytopenia, eosinopenia, or monocytopenia; Neutropenia and Lymphocytopenia are the most common types
Demographics of Pernicious Anemia
Northern European descent; Occurs equally in both sexes; Average age of onset is 60
What does vWF do?
Facilitates platelet adhesion to vessel
Mycosis Fungoides immuno markers
"CD3+, 4+, 7-, (T cell)"
follicular lymphoma translocation
t(14;18)» bcl-2 gene» BCL-2 protein, anti-apoptosis signal, constantly expressed» Cells don’t apoptose normallly-40% of NHL, median age 55, bone marrow involvement is common.
Dacrocyte
The abnormal red cell is seen with frequency in the myeloproliferative disorders, it is shaped like a tear. Synonym: Teardrop cell.
Basophilic Stippling
Red blood cell inclusion that consists of precipitated ribonucleoprotean and mitochondrial remnants. Stippling may be fine, coarse, or punctuate in form and is seen in toxic states such as metal poisoning, severe bacterial infection, drug exposure, and so forth.
Major fucntion of Rapapart-Leubering Pathway
production of 2,3 diphosphoglycerate
Acute Leukemia
Rapid growth of immature blood cells, crowding makes the bone marrow unable to produce healthy blood cells. Acute onset. Most common type in children (85%). Rapid progression, cells spill over into bloodstream. Fatal if left untreated
What are the side effects of treatment for polycythemia?
anemia
leukopenia
thrombocytopenia
acute leukemia
What factor increases the speed of factor IX activation of factor X?
Factor VIII
acute leukemia etiology
most have no apparent cause; associated with radiation, benzene, melphalan, etoposide, retroviruses, Down and Klinefelter, myelodysplasia and sideroblastic anemia
Evidence of dysmegakaryopoiesis may include any or all of the following:
-giant platelets
-platelets with abnormal granulation
-circulating micromegakaryocytes
-large mononuclear megakaryocytes
-abnormal nuclear shapes
What are the functions of eosinophils?
Chemotaxis
Phagocytosis
Killing of phagocytosed bacteria
Antibody dependent damage to parasites
Regulation of immediate-type hypersensitivity reactions
Beta thalassemia
Minor: one mutation in beta globin gene
microcytic anemia, usually normal longetivity

Major: two mutations, marked hemolysis, extramedullary hematopoesis (facial bones, hepatospenomegaly, cardiac failure)
Beta thalassemia major is also known as:
Cooley's anemia
What is normal amt of lobes of neutrophil
3-4
type AB has what kind of antigens?
both antigens
Features of the myelodysplastic syndromes:
Multineuclearity, asyncrony similar desinigration of nucleus, nuclear bridging between cells
RBC, WBC,Platelets
what makes up formed elements in the blood?
What is the shape of an RBC?
a biconcave disc
Structural ALL Karyotypic Abnormalities
Translocations with a BAD prognosis: t(4;11), t(1;19), t(9;22), t(8;14); Translocaitons with a GOOD prognosis: t(12;21);NOTE: t(4;11) was in his handout, but not the powerpoint, and t(8;14) was in his powerpoint but not his handout
T-Cell Antigen-Dependent Development
Occurs in lymph node paracortex; Blast transformation (become mature T effector cells, either CD4+ or CD8+); Memory Cells are generated
MALT can be caused by
H Pylori infection of stomach
What are the sx of G6PD def. if hemolytic anemia is not present?
asymptomatic
signs of anaphylactic blood reaction
flushing, bronchospasms, chest pain, loss of airway
Extravascular Hemolysis
normal processes of removal of old or damaged cells from circulation by spleen or liver.components are saved and reutilized.increased serum bilirubin, carboxyhemoglobin, methemoglobin
condition in which platelets aggregate and occlude the microcirculation

platelet aggregation occurs without activation of the coagulation cascade and is related to hemolytic syndromes and low platelet syndromes
thrombotic thrombocytopenia purpura (TTP)
What is a heinz body?
Intracellular inclusions of denatured hemoglobin
a/w thalassemias, sickle cell, G6PD, splenectomy
reticulocyte count
The proportion (%) of RBC's in a specimen of blood that are immature reticulocytes

Normal Range = 0.5% - 1.5%
What are the characteristics of basophils?
-10-14 micrometers
-nucleus usually has 2 lobes connected by thin filaments without visible chromatin
-coarsely clumped chromatin
-lavender to colorless cytoplasm
-rare primary granules
-secondary granules are variable in number with uneven distribution; may obscure nucleus or washout during staining, giving appearance of empty areas
-0-1% in PB
Factors effecting extent of hemolytic transfusion reaction
Antibody titers
Antibody class
Antigen concentration on cells
Antigen position on cells
Number of cells transfused
Neutropenia Nursing Management
Fluids & Electrolytes
Adequate intake to prevent dehydration & possible urinary tract infection; up to 3-4L daily
Monitor I&O
What is contained in plasma
Plasma Proteins, electrolytes, vitamins minerals carbs fats hormones
congential hemlic anemia
one of the leasts severe of g6pd
Cryoprecipitate- contains & dose
factor VIII, factor XIII, and fibrinogen; dose: 5 ml/kg
iron, vitamin b, folic acid, amino acids and lipids
what does erythropoiesis require?
What is a controversial tx for DIC?
Heparin (stop thrombin formation)
Laboratory Diagnosis of Iron Deficiency Anemia
Decreased serum iron, Decreased serum ferritin (an iron storage protein); Increased total iron binding capacity; Decreased transferrin saturation (a transport protein); Decreased MCV; Absent storage iron in the bone marrow
Megaloblastic anemia of infancy caused by
Deficiency of Transcobalamin II (peripheral tissues)
This mutation creates resistance to activated protein C, causing a hypercoaguable state
Factor V leiden mutation (Arg506Gln)
treatment for sicke cell is
hydration, avoid stress, pain med. give folic acid, genetic counceling
Acute myelogenous leukemia
more common in adults, can occur in children as wellOften linked wtih toxins geographical links - environmental Precursor cells, pluipotential stem cells
causes of prolonged PTT only
inherited --&gt; vWF, factors VIII, IX, XI or XII deficiencies
acquired --&gt; heparin, antiphospholipid antibody
Describe Gaucher disease.
The Gaucher cell is a macrophage 20-80 microns in diameter, with one or more small, round to oval eccentric nuclei; cytoplasm has crompled tissue paper appearance; found in bone marrow, spleen, liver and other affected tissue.
Iron studies in anemia of chronic disease
Low iron
Low TIBC
High ferritin
WHO lymphoma classification
Cell of origin: B vs T/NK vs Hog

Peripheral vs precursor
What are the common nonprotein nitrogens in plasma
urea, amino acids, and creatinine
in what kind of anemia is there a decrease in all cells (RBC, WBC, and platelets) <pantocytopenia?
why?
aplastic anemia
P50
the pO2 at which 50% of the hgb is saturated with oxygen; decreased at birth but gradually increases during first week of life; preterm infants have decreased concentration of 2,3 DPG, increased amounts of hgb F, and shift the dissociation curve to left
Other than EBV, what viruses are belived to be linked to the dev. of HL?
Chicken pox, Rubella, and Pertussis
Methylmalonate in the Urine
Suggests a B12 deficiency; If there is no B12, L-methylmalonyl CoA can't be converted into Succinyl CoA, so it builds up and then (after a few intermediate steps) is excreted in the urine as methylmalonate, which can be measured
How do RBCs get NADPH?
It comes from the Pentose Phosphate Pathway, G-6-P dehydrogenase produces it
ALL bad prognostic indicator and Tx
"Philly Chr, allogeneic stem cell transplant"
Can supplementation reverse s/sx in pts w/ neurologic damage d/t Bqw/folate def.?
slow reversal, up to 6 mths.
Granulocytes - cytoplasmic granules - segmented
Neutrophils - 55-65%- also called polymorphonuclear leukocytes (PNM) - precursos - myeloblast (not normally circulating) - destroy foreign substances, phagocytic - surround, ejgulf, destroy - bands &lt; 5% = immature neutrophils Eosinophils - 1-3% -allergic reactions - release chemical mediators Basophils - 0.3-0.5% -contain heparin and histamine - allergic reaction - i.e. asthma
Hodgkin and non-Hodgkin staging
stage I --&gt; one lymphatic group or extra-lymphatic tissue
stage II --&gt; two lymphatic groups or extra-lymphatic tissue on same side of diaphragm
stage III --&gt; lymph nodes on both side of diaphragm or extralymphatic involvement contiguous to primary site
stage IV --&gt; widespread lymph involvement or diffuse extra lymphatic sites
Distribution in Igs of multiple myeloma
60% IgGs
20% IgAs - worse prognosis
Few IgDs
Rest are light chains
What cells cannot differentiate?
The dumb cells which can no longer differentiate: granulocytes, Plasma cells, Effector cells(Helper,Mediator,Killer,Suppressor)
The marginating pool of neutrophils is located:
Along the vessel walls endothelium
4 risk factors of GVHD TA?
1) Immunocompromised 2) Newborns 3) CA (leukemia and lymphoma) 4) Blood from close relative
T or F: The highest sensitivity transferrin receptors are found in the liver and bone marrow
True, remember transferrin is the protein that transports iron in the blood
 
What is Pagophagia and in Which disorder might you see it?
Eating of ice; Seen in Iron-deficiency
Contact with collagen or other negatively charged substances (like ground glass) activate this arm of the clotting cascade
These activate the intrinsic arm of the clotting cascade
when giving blood start out at what rate.
1ml/ min for 15 minutes.
What are the characteristics of band neutrophils?
-10-15 micrometers
-C or S shaped nucleus, constricted but no threadlike filament, chromatin must be visible in filament
-coarsely clumped chromatin
-pale blue to pink cytoplasm
-few primary granules
-abundant secondary granules
-0-5% in PB
What are examples of non specific innate immunity?
Physical or chemical barriers like skin, MM, enzymes, tears, saliva and hydrochloric acid of stomach
Behaviors that are violations of standard precautions are?
Bringing notebook into lab not wearing PPE
What are the expected lab findings in regards to plt counts, bleeding time, PT, factor VIII, and vWF?
Plt: normal, bleeding time: prolonged, PT: normal, Factor VIII and vWF: decreased.
With which disorder is there commonly a lack of response to Erythropoietin?
Iron deficiency; This is becuase the ability of EPO to stimulate the production of RBCs is highly dependent on the availability of functional iron
What is the low grade and high grade B cell lymphoma
L: follicular; H: Burkitt
What is seen in the bone marrow of a pt w/ thalassemia?
hypercellular w/ profound erythroblastic hyperplasia
Liver disease and coagulation labs
INR is part of the liver disease end stage assessment

Low factors levels including V but not VIII

PT/INR may not accurately predict bleeding
skeletal xray for hematologic system (purpose)
to evaluate lytic lesions of multiple myeloma
What two types of hemoglobin should never be seen in panel?
2 abnormals= HgS (sickle cell) and HgC (abnormal)
What is the role of Factor 13?
Once the fibrin net has initially been formed (ionic bonds between D and E domains of neighboring molecules) it is not especially strong. Factor 13 comes through and forms covalent bonds between adjacent D domains to stabilize things. The fibrin net is then laid down over the platelet plug formed during primary hemostasis and tacks it down.
Name 3 conditions which trigger erythropoesis.
1) Hypoxia d/t low RBC 2) Decreased O2 availability 3) Increased tissue demand for O2
what anemia is due to decreased number of RBC precursors? (2)
1. aplastic
2. anemia of chronc diseases
What are the 3 Ways that Anemias are Classified/ the 3 Things Anemia May be Secondary to?
1) Blood Loss (Acute v. Chronic); 2)Increased Destruction of RBCs (hemolysis) - intrinsic v. extrinsic RBC abnormalities; 3) Impaired Red Cell Production - defective DNA synthesis or defective hemoglobin synthesis
Ig increases will be seen on serum protein elctrophoresis as?
Increases in the beta and or gamma regions
Spikes for monoclonal, more generalized increases for polyclonal
What is a Test for Sickle Cell Anemia used in the Emergency Setting?
Sickledex - it's a 5 minute solubility test used to detect the presence of HbS; It is of little diagnostic value though because it doesn't differentiate between sickle syndromes and the benign carrier state
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