Other: Hematology 2 Flashcards

Terms Definitions
CLL
B-cells
MCH
27-31 pg
THROMBOCYTE
PLATELETS-COAGULATION OR CLOTTING
Hodgkin's Lymphoma
Reid-Sternberg cells
New HIV Cases
4.2-5.8 million
What activates Protein C
Thrombin
Leukocytosis
increased number of WBCs
Cyanide-resistant peroxidase
Eos positive
Others neg
Acanthocyte
projections; no central pallor
-poiesis
cell production, formation, and development
What is ITP?
idiopathic thrombocytopenia purpura
Reed Sternberg Cell
Mixed cellularity HL
neutrophils life span is
12 hrs
inherited autosomal recessive disorder that causes an impaired rate of synthesis of one of the 2 chains (alpha or beta) of adult hemoglobin
thalassemia
ALL - FAB L1
pre-B cells
Thalessemias
Quantitative disorder of globin synthesis
Not enough is made
(Hemoglobinopathies are qualitative)
D-dimer
Fibrin breakdown product
Elevated in acute thrombosis

Also
DIC, cancer, infection, surgery, inflammation
Thrombocytopenia Nursing Management
Nutrition
Promote erythropoiesis –
Folic acid, B12 - RBC maturation
Iron, B6 - Hgb synthesis
Vitamin C - converts folic acid to active form, Fe absorption
red cell procursers
defined as erthropoesis
Would splenomegaly be an expected finding in a pt w/ ITP?
No
Excessive heme degradation will result in...
Jaundice
Chromosome anomaly with myelodysplastic syndromes
5 or 7
Mesna
Used in adjunct with cyclophosphamide to trap the active/toxic metabolite acrolein
Neutrophil
A medium-sized mature leukocyte, with a three-to-five-lobed nucleus, and cytoplasm containing small lilac-staining granules; normally constitutes 50 to 70 percent of leukocyte in blood.
aplastic anemia is related to exposure to
myelotoxin
What type of anemia?
Hemolytic anemia
Normocytic anemia
calculating MCHC
MCHC = Hemoglobin X 10/Hematocrit
Primary myelofibrosis
Metaplasia of the bone marrow--replacement with fibroblasts

Smear: teardrops, nucleated red cells, early white cells

Usually with large spleen (extramedullary hematopoesis)
Not possible to get aspirate from bone marrow, need to core
Hereditary spherocytosis

Cause
Labs

Treatment
Genetic disorder, usually autosomal dominant
Spectrin deficiencies result in less membrane stability
Splenic macrophages chomp of bits of cells

Anemia with high MCHC, extravascular hemolytic anemia

Splenectomy
Waldenstrom Macroglobemia
IgM disease
Post germinal center memory cell
Ranging from gammopathy to lymphoplasmacytic lymphoma with IgM

Hyperviscosity syndrome is a problem - plasmaphorese
INR
Standardization of PTs based on analysis of the thromboplastin reagent based on a WHO standard

Normal INR - 1
Normal for anticoagulates 2-3
Neutropenia Collaborative Care
Prevention of infection
Hand washing
Private room if possible
Medications
Antibiotics
Analgesics
Antipyretics
G-CSF (Neupogen)
What is another name for immunoglobulin
antibody
Whick poikilocyte is classically associated with hemoglobinopathies and liver disease?
Target cells
What is crossmatching?
Immediate spin (IS) or AHG
Reed-Sternberg Cells
The malignant cell of Hodgkin's Disease; RS cells are required for the diagnosis in all cases of Hodgkin's; They are binucleated, single cytoplasm cells with large nucleoli (classic RS cells); Variant RS cells can have >2 nuclei or they may be mononuclear "lacunar" cells; When diagnosing Hodgkin's - once you find a RS cell, you need to look at the background cells to make sure that they are benign, reactive cells since NHL can have Reed-Sternberg-Like cells (but in NHL the background would be malignant tumor cells and you don't want to be fooled since the treatment and prognosis for HD and NHL are very different)
MALT Lymphoma
An important NHL Subtype; Chronic infection of the stomach by Helicobacter pylori; Localized to the stomach, indolent course; Can be cured in many cases with antibiotics against H.pylori
Alpha globin gene loci on which chromosome
16
B and T cell markers
Bcell CD20.TCell CD3
Anisocytosis
Variation in the size of erythrocytes when observed on a peripheral blood smear.
folic acid helps with?
red blood cell maturation
MCHC
the percentage of a RBC's volume which contains hemoglobin. Normal values: Usually between 31-37%.
Hemoglobin Synthesis
Heme -synthesized in motochondria from amino acids -one iron for each heme (80% of iron in hemoclobin, recycled) -4 heme complexes + globin = hemoglobin molecule Globin -polypeptide chain synthesized by cytoplasmic ribosomes -combines with heme
What is the growth factor for Eosinophils?
IL-5
multiple myeloma treatment
younger patients --> transplant (pre-treat with thalidomide, dexamethasone)
older patients --> melphalon, prednisone, thalidomide
treat hypercalcemia with loop diuretics
Naphthol AS-D chloroacetate esterase
Neutrophils pos
Basos weak pos/neg
Lymphs/Monos/Eos neg
Evidence of dyserythropoiesis may include any or all of the following:
-oval macrocytes
-hypochromic microcytes
-dimorphic erythrocyte population
-erythrocyte precursors with >1 nucleus
-abnormal nuclear shapes
-nuclear bridging
-uneven cytoplasmic staining
-ringed sideroblasts
Myelodysplastic disorders
Malignant change in hemotopoetic stem cell
Myeloid precursors in bone marrow are dysplasic
Inadequate blood cell production

Can become an actue leukemia (30-40%)
Chromosomal changes important
ALL treatment
Rapid resistance is a problem

Use multidrug induction and then long consolidation
CNS prophylaxis with XRT or intrathecal chemotherapy
-25% CNS relapse without
Many adults need allogenic transplant to survive
Thrombin clotting time
Exogenous thrombin added to plasma
Time to clot

Detect fibrinogen abnormalities
hypo, dys, heparin, thrombin inhibitors
Heinz body
denatured hemoglobin; can ONLY see with Supravital stain (like Brilliant Cresyl blue)
Anemia of Chronic Disease
Underproduction of RBCs
Mild shortening of RBC survival

Causes
End-stage renal disease
Primary factor: ↓ Erythropoietin
Chronic liver disease
Chronic inflammation
Malignant tumors
Chronic endocrine diseases
s/s of anemia? (4)
1. pallor
2. jaundice
3. pruiritus
4. heart problems
The morphological classification of anemia in the myelodysplastic syndorme is:
Macrocytic and Normochromic
.9%
what is the normal value for the salinity of blood
What is the end product of coagulation?
Fibrin clot
Clinical Course of CLL
Generally indolent with gradual increase in lymphocytosis, adenopathy and splenomegaly; May be years before Rx is required; Occasionally see transformation to ALL, Prolymphocytic Leukemia, or Richter Syndrome (~5% transform to aggressive large cell lymphoma/ leukemia)
Examples of Plasma Cell Dyscrasias
Multiple Myeloma, Waldenstrom's Macroglobulinemia, Monoclonal Gammopathy of Undetermined Significance
Morphology of Folate Deficiency
Peripheral Blood Smear - macro-ovalocytes, hypersegmented neutrophils; Bone marrow - megaloblasts (with "salami-like" chromatin), giant bands and metamyelocytes
What tests for G6PD deficiency
Rapid flourescent screening test
Pure Topo-I Inhibitor
Irinotecan. DLT - bone marrowsuppression, but also GI toxicity, severe diarrhea.
Is anemia considered a sx or a dz?
sx
Hemolytic Anemia
Anemia caused by hemolysis of red blood cells resulting in reduction of normal red cell lifespan.
megablastic anemias include
pernicious anemia and folic acid anemia
results of increased intravascular hemolysis
increased indirect bilirubindepleted haptoglobin and hemopexinincreased LDHincreased retic countmeasurable methemalbuminhyperplastic bone marrow
What are some symptoms of thrombotic thrombocytopenia purpura (TTP)?
thrombocytopenia
neurologic abnormalities
fever
renal problems

**This is an emergency because bleeding and clotting occur at the same time!
Reticular Dysgenesis
Failure of stem cell committed to myeloid and lymphoid development
beta thalassemia diagnosis and treatment
anemia + microcytosis
electrophoresis shows increased HbA2 and HbF
beta thalassemia major requires monthly transfusions and deferasirox for iron overload
may do splenectomy or marrow transplant
ALL - L3
Burkitt's lymphoma - leukemia phase (B-cells)
What disease states are associated with hypochromasia?
-iron deficiency anemia
-thalassemias
-sideroblastic anemia
-lead poisoning
-some cases of chronic disease
Chronic lymphocytic leukemia/ small lymphocytic lymphoma
Indolent course, not curable
Leukemia Etiology
Type of white blood cell involved (WBC)
Acute lymphocytic leukemia (ALL)
Acute myelogenous leukemia (AML)
Also called acute nonlymphoblastic leukemia (ANLL)
Chronic myelogenous leukemia (CML)
Chronic lymphocytic leukemia (CLL)
what is thalassemia?
inadequate production of normal hgb that causes decreased erythrocyte production

it is due to the absence of or reduction of globulin protein

abnormal hgb synthesis
The hemolytic anemias are generally classified morphologically as:
Normocytic normochromic
Prenatal Anemia: Twin-twin transfusion
15-33% of all monochorionic, monozygotic twins; anastomosis between artery of one placenta and vein of the other; >20% size difference; smaller twin is "donor"
What type of CA can be a complication of polycythemia vera?
AML
Immunologic Classification/ Differentiation of Acute Lymphoid Leukemia Subtypes
Based on antigen expression of tumor cells as detected by flow cytometry; Precursor B-Cell ALL - CD19, CD20, CD22 positive; Precursor T-Cell ALL - CD2, CD3, CD4 or CD7, CD8 positive; CHEESY PNEUMONIC: B-CELL has BIG number CDs, T-CELL has TINY number CDs
Reasons to Treat Advanced Indolent Lymphomas
Constitutional Sxs; Anatomic obstruction; Organ dysfunction; Cosmetic considerations; Painful lymph nodes; Cytopenias; NOTE - in most cases you wouldn't treat NHL if they didn't have one of these reasons, even stages I or II are usually incurable
What are the abnormal blood tests with Hem B?
prolonged PTT
Acquired intrinsic defect in cell membrane that makes cells vulnerable to lysis by compliment and predisposes to DVT.
Paroxysmal Nocturnal Hemoglobinurea (PNH)
Sickle Cell
An abnormal red cell seen in patients who possess high quntities of hemoglobin S, an abnormal hemoglobin; red cell is crescent or sickle shaped.
major functions of Hexose Monophosphate Shunt
prevent injury from oxidative factors, provides reducing power to protect Hgb & RBC membrane from oxidant injury
Blood test that detects the presence of hemoglobin S in erthyrocytes
Sickle cell test
This RBC form indicates?
Basket cell
Oxidative damage (G6PD deficiency); unstable hemoglobin
chronic lymphocytic leukemia diagnosis
suspected in older patient with leukocytosis (80-98% lymphocytes)
bone marrow biopsy confirms leukemic lymphocytes
smudge cells on peripheral smear
Relapsed large B cell lymphoma treatment
Chemo + autologous transplant

Transplant helps up cure rate by 20%
Donors at risk for causing TRALI
multiparous women
recipients of multiple transfusions
M:E Ratio range
the ratio of myeloid to erythroid precursors in bone marrow; normally it varies from 2:1 to 4:1; we learned 1.5:1 to 3:1;
What are the effector cells?
Helper, Suppressor, Mediator, Plasma, Killer(Cytotoxic) these are dumb cells
what level of hgb is low enough to allow pt to maintain own erythropoiesis w/o enlarging the spleen?
10g/dl
What chromosome disorder in MDS has a poor predictive influence?
Chromosome 7
thrombocytopenia- definition & symptoms
platelet count < 150,000; petechiae, hematuria, pulmonary hemorhage
What causes secondary polycythemia?
Chronic hypoxemia w/ relative inc. in erythropoietin production.
Morphology of Bone Marrow in B12 Deficiency
Hypercellular, Megaloblasts, Giant Bands
Normal PT and abnormal PTT
"Defect in factor 8,9,10,11, heparin therapy"
Differential Diagnosis for increased PT and PTT and a decreased platelet count in a patient with a bleeding disorder
DIC, liver disease, heparin therapy with associated thrombocytopenia
When might a BMT be performed for thalassemia?
for severe beta-thalassemia
how long does it take to see en graftment with bone marrow transplant
14 days
What is HbBarts?
Hemoglobin that is a tetramer of gamma globin chains; seen in alpha-thalassemia w/ 4/4 genes deleted
Describe Howell-Jolly bodies.
Dark blue to purple color. Round to oval. 1 micron. Usually 1, but may be multiple, per cell. Composed of DNA.
When to treat follicular lymphoma
treat with gentle chemo including rituximab when
bulky, painful adenopathy
organ system invovlement
cytopenias
severe B symptoms
high FLIPI score
Essential thrombocythemia

Treatment and prognosis
Low risk --
&lt;60, no h/o thrombosis, plts &lt;1000K
No treatment

High risk
Hydroxyurea, sometimes aspirin, anagrelide

Usually normal life span, rarely get clotting complication, AML, ALL, myelofibrosis
Sodium citrate mode of action
removes Calcium (used in coag pathway)
What type of cell is most common in ruminants
Large Lymphocytes
Pure erythroid leukemia is defined as?
Erythroid precursors greater than 80
When does hypocalcemia occur in blood transfusion? Why?
Rapid/large blood transfusion. Anticoagulant will bind out ionized Ca.
What is a heme?
it is a complex of iron and protoporphyrin
Why is Fibrinolysis important?
In tissues without a lot of collateral circulation, you don't want the clot formed during hemostasis to overgrow and completely occlude the vessel (similarly, you don't want clots to grow beyond the area of the vascular endothelial damage), so fibrinolysis breaks down the clot once fibrin has been laid down and collagen begins to re-grow
What causes a delayed hemolytic reaction to a blood transfusion?
Delayed hemolytic reactions to blood transfusions occur when the recipient has plasma cells that recognize donor RBC Ag's
The functional role of mitochondria
main site of energy production for the cell
What cell is being stained positive in this image?
Activated neutrophils. LAP stain
Diagnosis of multiple myeloma
Big M spike
Lots of plasma cells in marrow
Hypogammaglobinemia
Plasmacytoma
Lytic lesions/end organ damage
2 Primary functions of RBCs
transport O2 to tissue and remove CO2
anemia of chronic disease is primarily ___driven
why?
immune driven

cytokines are released and cause an increased and retention of iron which is then stored in storage sites rather than used in erythropoiesis
Name 3 uses for cryoprecipitated AHF.
1) Fibrin glue for surgery 2) Hemophilia A 3) von Willebrands dz
What is the initial tx consideration for ITP?
Prednisone until plts are normal (>50,000)
What is the probably most common cause of anemia in the hospital setting?
Anemia of Chronic Disease (AOCD)
Factors that cause HbS precipitation in sickle cell anemia
Sickling caused by low O2 content, intracellular dehydration (increased MCHC) or a decrease in pH.
What cardiac signd may be present in a pt w/ anemia?
orthostatic hypotension, tachycardia, systolic ejection murmur, bounding pulses
Why do you give Hydroxyurea to sickle cell patients?
Increases concentraion of fetal Hgb
Bone Marrow and Stem Cell Transplantation Nursing Management
Assess for signs and symptoms of disease progression,infection,bleeding,Provide nutrition,Prevent fatigue,Promote safety
Observe for side effects of tx
what can cause iron deficiency anemia?(3)
1. GI surgery of duodenum
2. blood loss
3. pregnancy
what are the most common causes of aquired immunodeficiency?
Malnutrition, some CA, chicken pox, CMV, Rubella, Measles, TB, Mono, chronic hepatitis, lupus, bact. and fungal infxns.
Where is the hemoglobin alpha gene locus and what two proteins does it produce?
chromosome 16 and globins alpha and sigma(only during first 8 weeks of fetal development and will mature into alpha)
What is the Role of Antithrombin?
It "mops up" thrombin so that you don't continue to clot when you no longer need/ want to; Antithrombin needs heparin sulfate (located on intact vascular endothelium) in order to work; Without antithrombin you form clots (causes 2-4% of hereditary thrombophilia)
Name 5 clinical signs of hereditary spherocytosis.
1) Anemia 2) Jaundice 3) Splenomegaly 4) Gallstones 5) Chronic leg ulcers
Describe acute lymphoid leukemia in PB.
-blasts (2-3 times the size of a resting lymphocyte)
-moderate cytoplasm
-irregular nuclear membrane
-prominent nucleoli
-thrombocytopenia
-morphologicall difficult to distinguish from acute myeloid leukemia
what is anemia of chronic disease associated with?
underproduction of RBC and a shorter lifespan of a RBC
Name 4 medical conditions that may result from polycythemia vera.
1) TIA/CVA 2) MI 3) DVT 4) Splenomegaly
What should you do if B12 levels look normal, but you're still suspicious that the patient has a B12 deficiency?
Get a serum methylmalonic acid level
What is the tx for iron-def. anemia?
Oral ferrous sulfate w/ Vit. C, tx underlying cause
What keeps teh vasculature a closed system to guard against bugs
Platelets plug up any leaks in vessel walls
Des. the genetic transmission of vWD. What is it a def. of?
Autosomal dominant. Red. or abn fxn of von Willebrands factor (vWF)
what to do do if you suspect acute hemolytic reaction
stop blood, keep vein open, change tubes, hang D5W, give o2, give epinephrine and nitro, insert foley, monitor I and O. may need clotting factors infused. notify lab immediatley..
T or F: Hemoglobin B is the most common form of human hemoglobin
False, hemoglobin A which has 2 alpha and 2 beta chains
T or F: Iron is absorbed in the distal ileum 
F, absorbed in the duodenum by the DMT transporter on the brush border, where it is taken up into enterocytes
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