Hematology 4 Flashcards

Terms Definitions
-emia
blood
thromb(o)-
clot, thrombus
Immature RBC's
Reticulocytes
Hypoxia
Deficiency of oxygen.
Hemoglobin C
"""washington monument"""
Circulating plasmin inactivated by
a2-antiplasmin
L-asparaginase
Mechanism: hydrolyzes L-asparagine toaspartic acid and ammonia – depletes Lasparagine,. Cytotoxicity due to protein synthesis inhibition. Resistance: expression of L-asparagine synthetase in tumor cells
What is this cell?
Monocyte
Infectious causes of lymphopenia
HIV
TB
Malaria
Focus Assessment/Complications
Integument: petechiae/ecchymosis
Hematological: prolonged bleeding w/ invasive procedures; occult blood
Gastrointestinal: splenomegaly; abdominal pain & distention
Neuro: CVA - cerebral bleed; retinal bleed
Musculoskeletal: hemarthrosis
HAART
Refers to Highly Active Anti-Retroviral Therapy (for HIV/ AIDS); Successful viral eradication by use of multiple drugs in different classes
M7 increased incidence w
Down Syndrome
Immediate Generalized Reactions(IGR) (transfusion)
A.K.A. allergic>anaphylactoid>anaphylactic Initiating event (theory) Patient = performed IgE Donor = allergen histamine and enzymes released from mast cells
malignant disorder involving abnormal overproduction of specific wbc.
leukemia
GPIIb/IIIa
Binds platelets to collagen, fibrinogen and vWF multimers
Sideroblastic anemia causes
Congenital ALA deficiency
Pyridoxine/copper deficiency
Lead, ETOH, chloremphenicol, isoniozid
RA, myeloma, myelo/lymphoproliferative diseases
Platelets
General description
Anucleate
150k-400k is normal
Life 7-10 days

Alpha, dense, glycogen granules
What does serum =
Plasma- Fibrinogen
granulocytes
WBC; include basophils, eosinophils, and neutrophils
Diagnosis of B12 Deficiency
Macro-ovalocytes (macrocytosis); Hypersegmented neutrophils; Neurologic manifestations, Serum autoantibodies (with Pernicious anemia); Eleveated serum methylmalonic acid; Low serum B12; Schilling Test
Diffuse Large B Cell Lymphoma Tx
Rituximab
Labile/Stable coagulation factor replacement
Fresh Frozen Plasma
Site of iron absorption
Duodenom absorbs this
Hemolytic
Pertaining to, characterized by, or producing hemolysis.
Erythropoiesis
Formation of RBC's in bone marrow
This RBC form indicates?
Schistocyte
DIC, traumatic hemolysis
Describe microangiopathic hemolytic anemia (MAHA) in PB.
schizocytes, spherocytes, polychromasia, nucleated erythrocytes, decreased platelet count.
Causes of acquired, extrinsic neutropenia
Chemotherapy
Infection
Drugs, toxins
Immune mediated
Hypersplenism
Idiopathic
Follicular hyperplasia

Define
Associated with
Type of reactive hyperplasia
Expansion of germinal centers (can go into paracortex, medullary areas

Collagen vascular disease
Systemic toxo
Syphillus
Alder-Reilly bodies
mucopolysaccharides in PMNs; dark blue, obscures nucleus; looks like basophil, but will see too many of them; genetic disorder
What is an ^ in neutrophils
Neutrophilia
where does iron absorption most occur?
duodenum
Physiologic Anemia- definition
postnatal suppression of hematopoiesis; aymptomatic; more dramatic, happens sooner, and is prolonged in preterm infants
What is the stem cell for platelets?
Hemocytoblast
Subtypes of Hodgkin's Disease
Lymphocyte predominant, Nodular sclerosis, Mixed cellularity, Lymphocyte depleted; Unlike with non-Hodgkin's Lymphoma, in Hodgkin's Disease the histologic subtype does NOT determine how the disease is treated
What is a Normal Hematocrit?
Women: 40-44%;Men: 42-52%
How is Hem B treated?
factor IX replacement
Chemotherapy that causes blood in urine
"cyclophosphamide, ifosfamide"
Circulating iron is loosely bound to the transport protein _________.
Transferrin
Defribrinated
Deprived of fibrin. The conversion of fibrinogen into fibrin is the basis for clotting blood.
lymphocytes make up what percent of the diff
20-40
Eosinophils
0 – 4 %0-0.5 x 10 3rd/μl
the study of blood and blood-forming tissues

includes blood cells, bone marrow, spleen, and lymphatic system
hematology
What blood cell?
Clock face chromatin distribution
Plasma cell
MCV
The average size (volume) of an RBC

Normal values: 80-100 fl. (a femtoliter [fl] = 10-15 liter)
What disease states are associated with basophilic stippling?
-lead intoxication
-thalassemia
-abnormal heme synthesis
What are significant causes of lymphocytopenia?
Immunodeficiencies (HIV/AIDS)
Immunosuppressive drugs
Lymphomas
Granulomatous diseases, including TB and sarcoidosis
Adhesion

define and mechanism
Platelets bridging to surface

GPIb-IX (also IIb/IIIa) binds to vWF whcih connect to subendothelial collagen
Smudge cell
Seen on smear
Fragile cell gets destroyed by process
Characteristic of CLL
Megaloblastic anemias
Anemias where problem is DNA synthesis, end up with mature cytoplasm, immature nuceli

Macrocytic anemia

B12 deficiency, folate deficiency, drug induced, some hereditary
MMM signs
a chronic myeloproliferative; MMM has dacryocytes, and large platlets; MMM shows a leukoerythroblastic response (all cell lines are increased); BM biopsy usually has a dry tap
Neutropenia Nursing Management Nutrition
Low microbial
High nutritional value
What do granules of neutrophil look like?
Pale-Pinkish
hemoglobin f
electrophoresis is the majority of hemoglobin
Anemia of prematuriy- prevention
conservative transfusion guidelines; decrease iatrogenic losses; 3.5 g/kg protein each day
polycythemia
what is the overproducation of RBC and other cells?
TRAP stain 
(tartrate-resistant acid phosphatase)
positive in hairy cell leukemia
Hematologic Consequences of Thalassemia
Low Intracellular Hg (hypochromia, microcytosis); Relative Excess of the Normal Chain (ex: excess alpha chains in beta thalassemia) - insoluble complexes (eg the excess chains that come together precipitate out), ineffective hematopoiesis, hemolysis
Common Features of AML
Morphology - myeloblast (immature cell, delicate chromatin, 3-5 nucleoli, AUER RODS); Defining Characteristics - Blasts comprise at least 20% of nucleated marrow elements, with or without peripheral blood involvement
What is another term for primary thrombocytosis?
essential thrombocytosis
Blasts in spinal fluid only in which AML
M5
Clopidogrel mechanism of action
Antiplatelet. Irreversibly inhibits P2Y12 ADP receptor and inhibits activation of the glycoprotein IIb/IIIa pathway.
Cabot Rings
A red blood cell inclusion resembling a figure eight. It is usually found in heavily stippled cells.
metaplasia
change from one adult cell type to another
Blood Tests
Complete blood count MCV = mean corpuscular vl -sze of the RBC's MCH/MCHC - mean corpuscular hemoglobin (concentration) -hemoglobin countent or color of RBC's Bone marrow aspiration / biopsy -best way to look at cells
Name some causes of folate deficiency anemia.
malabsorption
drugs
alcohol
poor nutrition
What is factor II more commonly known as?
Prothrombin
acute leukemia presentation
fatigue, anemia and bleeding from thrombocytopenia +- leukopenia or leukocytosis
ALL --> in children, hepatosplenomegaly, lymphadenopathy, bone pain
AML --> DIC (promyelocytic M3), CNS meningitis-like (monocytic M4, M5)
What are the significant causes of acquired defects in neutrophil function?
Corticosteroid use
Alcoholism
Leukemias
Myelodysplasia
Myeloproliferative disorders
CLL refractory to fludarabine
BAD
prognosis less than a year
Eosinophilia
causes

Most common?
Types?
>1500 cells/ul for 6 weeks, severe is >5000
Most common - atopic in developed nations, parasites in developing

Primary - clonal disease of myeloid lineage
Secondary- infections, allergies, toxins, reactive to lymphomas
Howell-Jolly bodies
piece of nucleus left from nucleus expulsion; can be seen with BOTH Wrights or Supravital
What is another name for mononuclear phagocyte system
Reticulo-endothelial system
what is stem cell aka?
nondifferentiated immature blood cell
Chronic illnesses
All different immature cells of each line
hemocrit
what is the term for the percent of formed elements?
What is the life span of an RBC?
120 days
Immunologic Characteristics of AML
Myeloblasts have CD13, 15 and 33
Antigen-Independent Lymphoid Cell Development
Occurs largely during fetal/ neonatal and childhood periods for both B and T cells. As a result, malignancies of cells derived from antigen-independent development are seen more commonly in kids (except for mature virgin B and T cells - cancer of these (CLL) is usually seen in adults/ the elderly)
Multiple Myeloma
Plasma cells in the bone marrow. Incidence increases with age peak 80yrs. Monoclonal antibodies seen on electrophoresis. Most have osteolytic lesions that lead to fractures. Shows multistep progression MGUS/smoldering to Intramedullary to malignant. Best to treat at intramedullary. beta-2 microglobulin above 5.5 mg/dL is marker for stage III, poor prognosis. Increased free light chains = poor prognosis. Cytogenetics is greatest prognostic factor.
What is heme degraded into?
a yellow pigment called bilirubin
ferous sulfate can result in what
constipation and dark stools.
Basophils
0 – 2 %0 – 0.2 x 10 3rd/μl
Blood test that measures the depletion of body iron (potential deficiency of heme synthesis)
Serum ferritin determination
What blood cell?
Important in defense against helminths & protozoans
Eosinophil
Uses Major basic protein
Basophils
0 – 2 %

0 – 0.2 x 10 3rd/μl
Differentiating between extravascular and intrasvascular hemolysis
Haptoglobin will be decreased in intravascular
Hemoglobinuria or hemoglobinemia in intravascular only
Diagnostic findings
Severe = Hb <6 g/dl
Involve multiple body systems
Integument
Eyes
Mouth
Cardiovascular
Pulmonary
Neurologic
Gastrointestinal (GI)
Musculoskeletal
What are Lymphokines?
Secreted by mediator T cells and secrete a substance called chemotaxis that activate other leukocytes
Acute leukemic processes are marked by:
Anemia, thrombocytopenia, hypercellular bone marrow greater than 20
physiologic anemia- mechanisms
rapid turnover of RBC's, rapid growth, increased pO2 at birth suppresses erythropoeisis
granular and agranular
what are the two different types of leukocytes (WBC)
What is the type of thrombocytopenia w/ hemolytic anemia?
Thrombocytic Thrombocytopenia Purpura (TTP)
Hemoglobin H Disease
A form of Alpha Thalassemia; (a_/_ _); Moderate to severe lifelong hemolytic anemia, splenomegaly, bone changes and ineffective erythropoiesis
Predisposing Factors for Iron Deficiency Anemia
Heavy menstrual bleeding; 4 positive guiac stools (chronic use of NSAIDs, recurrent peptic ulcer disease); Childbearing; Antacids; Tetracycline
What is the mechanism of warfarin induced skin necrosis
decreased protein C levels
Hyper-segmented PMNs are seen in this condition, along with increased erythropoietin and increased iron absorption
Features of megaloblastic anemia
circulatory overload is due to
fluid being administered faster than the circulation can accommodate.
an acquired clinical syndrome in which the manifestations are the result of abnormal clotting that causes a serious bleeding disorder--thrombosis (clot formation) and hemorrhage
Disseminated intravascular coagulation (DIC)
disseminated intravascular coagulation treatment
fresh frozen plasma and maybe platelet transfusions; correct underlying disorder
A delayed PT indicates a deficiency or defect in what?
Fibrinogen
Prothrombin
Factor V
Factor X
Factor VII
Pathogenesis of kidney failure in hemoytic transfusion reaction
Hypotension - acute tubular necrosis
Immune complex glomerulonephritis
DIC -glomerular capillary thrombosis
Free Hgb-- leads to tissue damage and ischemia
Where are all lymphocytes made after birth
Extramedullary, Lymphnodes, Spleen, Peyer's patches
s/s of folic acid deficiency?
1. GI disturbances
2. smooth beefy tongue

there will be no problems with neuro
Fresh frozen plasma- uses
DIC, vit K deficiency bleeding, and inherited deficiencies of coagulation factors
Is HL more common in men or women? What race is it less common?
Men, African American
What is another name for Factor 2?
Prothrombin (Factor 2a- activated factor 2 - is known as thrombin)
Triad of B12 Deficiency Symptoms
Weakness and/ or dyspnea; Sore tongue; Symmetric numbness or tingling in extremities (stocking/glove neuropathy)
Replace blood volume and O2 carrying capacity
Whole blood (has no platelets/granulocytes)
Differentitate b/t absolute and relative anemia.
1) Relative - normal RBC mass/excess plasma volume (pregnancy) 2) Absolute - actual dec. in RBC #
What can occur with platelet counts of 50,000 or less?
hemorrhage from minor trauma
P50 value for hemoglobin
pressure at which 50% of hemoglobin is oxygenated.

P50 for hgb=26 torrs
Where are T cells in LN?
Paracortex
A few in the germinal center
What is the Process of Cellular Immunity?
Lymphoid stem cells&gt; preprocessed in thymus&gt;T cells= cellular immunity
Asynchrony in the bone marrow is defined as:
Unbalanced nuclear hemoglobin development
Describe mixed cellularity type of HL.
RS cells easily identified and band forming sclerosis is absent
In Which Conditons are Folate Requirements Increased?
In conditions of high metabolic rate or increased rate of cell division: Pregnancy (16ug/kg/day or 1mg/day), Lactation (400 to 800 ug/day), Infection, Malignancies, Hemolytic Anemias
Fetal RBC's are made here from weeks 3-7 and are nucleated.
Extraembryonic yolk sack makes these
what is the causes for an acute hemolytic reaction
ABO compatibility problem, or improper storage of blood.
Should platelet transfusions be used to treat TTP?
ABSOLUTELY NOT
doing so only fuels the fire
What is an immunocyte?
A B or T lymphocyte that has been ACTIVATED after being exposed to an antigen
90% water, 7% protein, 3% other
what makes up the plasma part of blood?
When does the intrinsic pathway start?
Exposure of collagen as a result of endothelial damage (slow)
What are the 3 big indicators of Iron Deficiency?
Decreased Iron, Increased TIBC, Decreased Ferritin
Small Lymphocytic Lymphoma (SLL)
Type of NHL. Like CLL only in the tissue
signs of delayed hemolytic reaction include
extreme fatigue 6 weeks to 6 months, gradual decline in cbc.persistent low grade fever.
Prolonged PTT and normal PT
Hemophilia -- A (def in VIII), B (IX), C (XI)

vWF disease

Inhibitor of intrinsic pathway

Heparin

HMWK, pre-kal, XII deficiencies
What effect does the pH chromosome have on the prognosis of patients wchronic myelocytic leukemia?
Prognosis is better if pH chromosome is present
What happens to RBC production in renal failure?
it will decrease b/c the growth factor erythropoetin is made in the kidney, this is known as the anemia of kidney failure
Cause of Hemoglobinuria in Hemolytic Anemia
The excess free Hg in the serum spills over into the urine
what to do if you suspect allergic urticaria
stop blood, keep vein open with ns,notify blood bank or dr. give antihistamine and/or corticosteroid turn blood back on and monitor.
what is the tx for aplastic anemia? (2)
1. hemaopoietic stem cell transplant + immunosuppressive therapy + antithymocyte globulin + cyclosporine

2. high dose cyclophosphamide

3.
Why will you see changes in the PT/ INR before changes in the aPTT in a patient on Warfarin?
Because PT and INR measure the extrinsic pathway which involves factor 7, and factor 7 has the shortest half-life of any of the clotting factors.
What is Rouleaux look like and what is it common in?
Stacked coin appearance of cells, and common in horses cats and pigs
Anisocytosis is often seen on RDW in this micro-nutrient deficiency anemia
This feature is often seen on RDW in iron deficiency anemia
Hematology
-chromic
hemoglobin content
increased WBC
marrow fibrosis
increased LAP
nRBC
teardrop cells, schistocytes
myelocytes, bands
myelofibrosis
Hct
42-52% Male

37-47% Female
MCH calculation
HGB (g/L)
RBC (106/μL)
AML with gum bleeding
M5
interleukins
mediate complex communications between classes of WBCs and WBC effect on hematopoeisis
Tay-Sachs Disease
hexosaminidase A def.
(monos)
Gaucher's disease
Lysosomal storage disorder
glucocerebrosidase builds up

Bone marrow fills with fat not hematopoetic cells
hgb to crit ratio
1:3
What is DVT?
Deep Vein thrombosis
Popcorn Cell
Nodular Lymphocyte Predominant HL
increased number in reticulocytes means what?
anemia
Macrocytic
Abnormally lg erythrocyte &gt;10 microns (megaloblastic 11-20 microns)
ALL - FAB L2
Adult T-cell
GP6D deficiency
Deficiency of glucose-6-phosphate dehydrogenase
X-linked (affected males or female mosaics w/ mutation in RBCs)
Extravascular hemolysis either after stress or all the time

Hemoglobin oxidize to methHbg--sulfHbg--Heinz bodies
Poor prognosis mutations in AML
FLT3
KIT
P53
Pelger-Huet PMNs
genetic; no associated problems
norma cromicnorma cytic
morphology classified anemias
DAT finding in DHTR?
DAT (+), IgG
R-CHOP
R= Rituxan (Rituximab), C = Cyclophosphamide (Cytoxan), H = Hydroxydaunorubicin (Adriamycin), O = Oncovin (Vincristine), P = Prednisone
AML with abundance of auer rods
M3
Myelofibrosis (MF)
Marrow fibrosis with extramedullary hematopoiesis, stem cells are colonizing other organs (metastasize),Splenomegaly and fibrotic marrow,JAK2 V617F. Tear drop eryththrocytes.
Monoclonal
Antibody derived from a single ancestral antibody-production parent cell.
Euchromatin
uncoiled DNA strands within the nucleus
What type of cell is this?
Basophil
Microcytic
Abnormally small RBC's (corresponds to an MCV &lt; 80)
Folded nucleus with uneven countour; slate grey cytoplasm; there may be vacuoles = ________
Monocyte
Haldane effect
deoxygenated hemoglobin has higher affinity for CO2 than oxygenated
Leukemia
A group of malignant disorders affecting the blood and blood-forming tissues of
Bone marrow
Lymph system
Spleen
Occurs in all age groups
Fatal if untreated
Progressive
Which Leukocytes are granular?
Neutrophils, Eosinophils, Basophils
When the bone marrow temporarily ceases to produce cells in a sickle cell patient, an crisis has occurred.
Aplastic
four
each hemoglobin molecule can bind with up to how many oxygen molecules?
Which enzyme facilitates the rate limiting step in heme production?
ALA synthase
High Grade Lymphomas
So-called because of aggressive behavior and poor prognosis. These, however, are treatable with many long-term remissions and cures; Many arise in the background of a pre-existing immunologic disorder such as Sjogrens, Hashimotos thyroiditis, transplant patients (PTLD) and AIDS; Show "fish flesh" grossly and "starry sky" histologic pattern
Hairy Cell Leukemia
Characterized by pancytopenia, massive splenomegaly and accumulation of lymphoid-appearing cells with "hairy" cytoplasmic projections; Bone marrow is often difficult to aspirate ("dry tap") secondary to myelofibrosis and infiltration of hairy cells; Cells typically B cell in origin, stain strongly for tartaric acid-resistant acid phosphatase
Increase risk of thrombosis w/ Factor 5 Leiden
Estrogens
Causes of follicular hyperplasia
Nonspecific Autoimmune: rheumatoid arthritis HIV (early stages) Other infectious causes: toxoplasmosis, syphilis
Oxyhemoglobin
The combined form of hemoglobin and oxygen.
neutrophils make up what percent of the differential.
40-70
Intravascular Hemolysis
occurs when severely damaged cells lyse in circulation.components are found in circulation.hemoglobin bound to haptoglobin and transported to liverhemoglobinemia, hemoglobinuria, hemosidinuria
Hemophilia A
Defect in clotting factor VIII Etiology -X linked recessive inherited disorder -Affects mostly males -Female carriers Lab findings - deficiency in factor VIII, prolonged bleeding time Manifestations - spontaneous or traumatic hemmorrhages, intramuscular, subcuntaneous
Name the stage of neutrophilic maturation.
Neutrophilic promyelocyte
von Willedrand disease presentation
petechiae, echymoses, epistaxis, bruising
hematuria, dysfunctional uterine bleeding, GI bleeding
increased with aspirin
shift to the right
decreased hgb-oxygen affinity
DPG ↑
pH ↓
temp ↑
Describe Waldenstrom macroglobulinemia.
-leukocyte count usually normal
-rare plasmacytoid cell or plasma cell
-normcytic/normochromic anemia w/ rouleaux
-normal to decreased platelet numbers
-excess protein may cause blue precipitate
Normal lymphocyte circulation
Blood into
Lymph node
Surveilling for presentation of specific antigen
Leave through efferent lymphatics
Surface markers for t cell lineage
CD3, CD4
Causes of intravascular hemolysis
Transfusion reactions
G6P deficiency w/ oxidative stress
Paroxysmal nocturnal hemolysis
Infection (clostridial sepsis, severe malaria)
Microangiopathy (aortic stenosis, artifical valve leak)
Amyloid AL
Light chain deposition in tissues
With or without myeloma

Prognosis is months if cardiac involvement

Treat like myeloma
Green vacutainer
heparin; used for osmotic fragility (and a lot of chemistry)
Aplastic Anemia Collaborative Care
Remove the cause
Treat symptoms
Transfusions
RBCs
Platelets
Bone marrow transplant/peripheral stem cell transplant
Which cells have aggregate or punctate organelles
Reticulocytes
A peripheral blood picture dominated by cells that appear convoluted with an ovoid nucleus and clefting would most likely be seen in:
Sezary syndrome
Platelet transfusion
never filter through micropore blood filter; dose: 5 ml/kg should raise counts by 20-60,000
During coagulation phase I, once factor __ has been activated, it complexes with Ca2+, PF3, and factor V to form _______ _______.
X, prothrombin activator
Ingestion of B12
During gastric digestion, cobalamin in food is released and forms a complex with gastric R binder. On entering the duodenum, the cobalamin-R binder complex is digested, releasing the cobalamin, which then binds to intrinsic factor (IF). IF binds to the B12, protecting it from digestion, and eventually binds to receptors on the brush borders of mucosal cells lining the ileum, facilitating the absorption of B12 and its transport into the bloodstream.
Alpha Thalassemia Minor
(a_/a_) or (aa/_ _); Clinically normal, but may have mild anemia and decreased MCV
Which proteins' bindings are affected in PNH
CD55 and CD59
Platelet and vascular type bleeding results in
petechiae, mucosal bleeding
What organ to a lesser extent, also produces erythropoetin?
liver
Lymphocyte
A white blood cell formed in lymphoid tissue throughout the body, generally described as non granular and including small and large varieties; comprises approximately 20 to 45 percent of the total leukocyte count.
hi lymph and hi mono mean
shift to right
Anisocytosis
Variation in RBC size. The higher the RDW, the greater the amount of anisocytosis
Blood test that measures the duration of bleeding following a standardized superficial puncture wound of the skin, integrity of the platelet plug, measured in minutes following puncture
Bleeding time
What is the growth factor for B cells?
IL-7
idiopathic thrombocytopenic purpura
antibodies against platelets leads to destruction and bleeding

epistaxis, bruising, petechiae, echymoses
hematuria, dysfunctional uterine bleeding, GI bleeding

thrombocytopenia is found and splenomegaly is absent

exclude HUS, TTP, DIC --&gt; peripheral smear without schistocytes and normal creatinine
exclude marrow thrombocytopenia --&gt; marrow biopsy with normal megakaryocytes

treat with prednisone or splenectomy if unresponsive
MCHC
the percentage of a RBC's volume which contains hemoglobin.

Normal values: Usually between 31-37%.
Describe thalassemia major in PB.
Numerous nucleated erythrocytes, microcytes, hypochromasia, codocytes, basophilic stippling, many dacrocytes, many schizocytes, polychromasia.
Anemia sings and symptoms
Pale, jaundice, cold skin
Fatigue, SOB, syncope
Low BP, rapid HR, breathing, heart murmur
Process by which WBCs engulf and kill organisms
Phagocytosis
What is the most important tool in developing a hemogram
PCV
One of the primary glands in the infant responsible for lymphocyte origination is the:
Thymus
banctoceniaabnormal reduction
signiicant feature of hairy cells not seen in other acute leukemias
Tx for anaphylactic transfusion reaction?
1) Trendelenberg position 2) Epinephrine 3) If known anti-IgA or IgA def. give product from IgA def. donor or washed RBCs
Oxidant Injury Associated with G6PD Deficiency
Drug Induced: anti-malarials (primaquine, quinacrine), antibiotics (sulfonamides); Infections
What is the major cause of thrombosis?
vessel wall injury
This drug accelerates the activity of antithrombin
Mechanism of Heparin
Asynchrony
The failure of events to occur in time with each other as they usually do. In hematology, nuclear and cytoplasmic development are mismatched.
components of hemoglobin
protein globin (2 dimers of 2 different polypeptide chains)4 molecules of protoporpyrin IX4 iron atoms in ferrous state that combine with protoporphyrin IX to form 4 heme molecules1 2,3 DPG molecule as occasional resident in hemoglobin molecule
Which type of anemia is caused by sudden blood loss with normal iron stores?
posthemorrhagic anemia
What is factor III more commonly known as?
Tissue factor
acute leukemia treatment
AML --&gt; AraC with danourubicin or idarubicin
ALL --&gt; daunorubicin, vincristine and prednisone
add all-trans-retinoic acid in promyelocytic leukemia
remission is removal of 99.9% of leukemic cells and elimination of blasts in peripheral blood
What disease states are macrocytes associated with?
-liver disease
-vitamin B12 deficiency
-folate deficiency
-neonates
M7: Acute Megakaryocytic leukemia
Can get bone marrow fibrosis

Two ages
Infants, kids &lt;3
w/ downs -- good prognosis
t (1,22)
Older adult
usually de novo, occasionally MDS
Treating anaphylactic or analphlactoid transfusion reaction
Stop transfusion, eval patient
Give epinepherine, antihistamines, steroids
Support respiration and BP
Acute anemia of rapid onset has what characteristics?
tachycardia
orthostatic hypotension
faintness
pale cold extremities
What is the N:L Ratio of Bovine
N:L &lt; 1
hgb of less than 6 may indicate?
multiple body involvement
The best test to assess the bone marrow's response to anemic stress is:
Reticulocyte count
hypochromic- definition, diagnosis
RBC containing less than normal amount of hemoglobin; chronic anemia, iron deficiency, thalassemia, lead poisoning
What are the 3 classifications of NHL?
Low-grade, intermediate-grade, and high-grade
Role of vitamin B12
It is a cofactor for methionine synthase, which helps to trap folic acid within the cell (if you can't trap folic acid withing the cell, then you can't complete DNA synthesis); B12 also is required to complete the conversion of homocysteine to methionine, which is used to myelinate nerves (which is the cause of the neuro s/s seen in B12 deficiency).
Clinical Course of Sickle Cell Anemia
Homozygotes - long-term, chronic anemia; Heterozygotes - basically asymptomatic unless they get dehydrated; Patients can develop vaso-occlusive, aplastic, and sequestration crises
What are the lab test results for BV wall fragility?
All normal
Collagen fibers will cause platelets to aggregate by stimulating them to secrete intracellular granular contents which include
adenosine diphosphate (ADP) and thromboxane A2.
What occurs during a sickle-cell crisis?
vasooclussion from sickle-shaped RBCS resulting in capillary stasis, venous thrombosis, and arteriol emboli (occlusion occurs in sm. capillaries and venules)
what do you look for to be sure bone marrow transplant is successful
reticulocytes
Genetics of Von Willebrand disease
Mostly autosomal dominant, but rarely autosomal recessive
Coded on chrom 12
What are the characteristics of eosinophils?
-12-17 micrometers
-nucleus has 2-3 lobes connected by filaments without visible chromatin
-coarsely clumped chromatin
-pink cytoplasm, may have irregular borders
-rare primary granules
-abundant red to orange, round, secondary granules
-0-5% in PB
vWF deficiency type 3
Absent vWF factor and factor VIII activity

Rare
What are transferrin, serum iron and TIBC levels in IDA?
Transferrin: decreased (&lt; 15%)
Serum iron: decreased (&lt; 30)
TIBC: elevated
What do monocytes become
They circulate thru the blood and become tissue macrophages
Acute leukemia is characterized by what of blasts?
Hypercellular bone marrow w20 blasts
What does whole blood used for blood transfusion contain?
RBCs, Plasma, and platelets
What is the standard tx for hemophilia A?
Factor VIII infusion (cryoprecipitate)
In which disorders might you see Jaundice?
Any of the Hemolytic anemias
In this condition IgG Ab's coat RBC's, and splenic macrophages remove chunks of RBC membrane. May be due to drugs acting as Haptens.
Warm Ab immunohemolytic anemia
vit c deficit can lead to what problem with the lips
cheilitis, dry cracking inflamation
Name the cell and the cellular abnormality.
Gaucher cell. Deficiency in beta-glucocerebrosidase leads to the accumulation of the substrate, beta-glucocerebroside.
ABO forward and reverse typing
Forward -- what will clump patients red cells
Reverse - what will patients serum clump
Neutrophilia can be caused by . . .
infection, malignancy (like CML)
wat kind of anemia is due to decreased hbg synthesis? (2)
1. iron
2. thalassemia
A person w/ type AB blood has what type of antigens? Ab?
Antigen: AB, Ab: none
Name 3 sx of organ bleeding d/t thrombocytopenia?
Hematuria, melena (black, tarry stools), hemoptysis
Lipid Abnormalities in Patients Receiving HIV Treatment
PI recipients are 4.2x more likely to develop a 50 point increase in total cholesterol; Ritonavir>Indinavir>Nelfinavir; Treatment - statins, fibrates; Switching to NNRTIs does lower cholesterol
Which alkylating agents show more renal toxicity?
The platinum compounds, but they also have less bone marrow suppression.
Why is Vitamin C given to pts w/ thalasemia?
to increase iron excretion
Quantify the platelets in relation to the disorder.
With May-Hegglin you will observe thrombocytopenia due to the presence of giant platelets.
what are the general characteristics of normochromic normocytic anemias?
these anemias are caused by organ failure or impaired marrow function resulting from systemic disease
what anemias is due to defective DNA synthesis (2)
1. folic deficiency
2. colbain deficiency
5 signs of bacterial sepsis transfusion reaction?
1) Fever 2) Chills 3) Hypotension 4) Tachycardia 5) Shock
What happens to RBC production during hypoxia?
It will increase b/c low oxygen tension is the trigger for the synthesis of erythropoetin by the peritubular cells of the kidney
Molecular Pathogenesis of Beta Thalassemia
The beta chain is encoded for by 2 genes (one on each chr 11)- beta thal results from a single base point mutation; The normal beta gene is "B"; Abnormal genes can either be "B0" (no synthesis of beta chain) or "B+" (low-level synthesis of beta chain)
Do pts w/ G6PD deficiency have hemolytic anemia?
not unless challenged by drugs, infxn, DKA, or fava beans
What mutation has occured in sickle cell anemia?
Single amino acid replacement in beta chain
What type of leukocyte is an immunocyte
A small lymphocyte with deeply blue staining cytoplasm
Name 3 types of lymphocytes.
1) T cells (80-90%) 2) B cells 3) NK cells (don't req. prior antigenic exposure)
Alpha Thalassemia Trait Form of Alpha Thalassemia
Deletion of 2 Alpha globin genes - (___, Alpha/Alpha, Alpha) or (___, Alpha/ ___, Alpha); Mild Anemia; Peripheral blood smear shows hypochromia/ microcytosis and target cells
What physical signs may be present in a pt w/ anemia?
pallor in skin, palmar creases, or mucus membranes (conjunctiva, and pitting of fingernails
How long should iron therapy continue in IDA
Levels will be normal in 2 months, should be continued for 6 months though to replenish stores
When does hemophilia C req. tx? What is the tx?
Dental extractions or surgery. FFP, fector XI concentrate.
Name 2 things caused by enhanced erythropoesis.
1) Inc. RBC in blood 2) Inc. O2 carrying ability of the blood
Describe the A and B component of staging of HL.
A - sx absent. B - sx present.
Name 4 common causes of death from complications of sickle cell anemia.
1) HF 2) Bone marrow and fat emboli 3) Organ failure 4) Shock
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