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321 Cards in this Set
- Front
- Back
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if you suspect hemolytic anemia, what diagnostic test should you do? |
peripheral smear |
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In autoimmune hemolytic anemia (AIHA) RBCs are destroyed by antibodies made by a person against their own RBCs. there are 2 types: warm (Ig__) and cold (Ig__) |
warm = IgG cold = IgM |
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IgG vs. IgM = which is warm and which is cold? |
IgG is warm; IgM is cold (dictatestemperature for maximal antibody binding to occur) |
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+ direct Coombs test means what? + indirect Coombs test means what? |
direct + = something wrong with the RBC (cells) indirect + = problem with serum |
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agglutination in a Coombs test means a positive or negative result? |
positive |
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= Dueto mutations resulting in abnormal synthesis of hemoglobin |
hemoglobinopathies |
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examples of hemoglobinopathies |
sickle cell anemia, thalassemia, G6PD deficiency |
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name the 3 types of hemoglobin diseases (letters) what is the most common |
C, S-C, and E S-C most common |
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Biggestproblem we find with sickle cell disease is... |
vaso-occlusive problems (builds dams in vessels leading to an array of problems) |
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sickle cell disease is stimulated by changes in... |
cellular hydration (dehydration), oxygen (hypoxia, acidosis, high altitude, etc.), fever |
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acute chest syndrome is an acute manifestation of which disease? what is physiologically going on? can it be life-threatening? how do you treat this? |
sickle cell disease, occlusion of PULMONARY CIRCULATION, can be life threatening since positive feedback of hypoxemia and more sickeling, requires exchange transfusion (exchange transfusion = slowly removing the patient's blood and replacing it with fresh donor blood or plasma) |
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= slowly removing the patient's blood and replacing it with fresh donor blood or plasma using aphaeresis |
exchange transfusion |
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= the removal of blood plasma from the body by the withdrawal of blood, its separation into plasma and cells, and the reintroduction of the cells, used especially to remove antibodies in treating autoimmune diseases. |
aphaeresis |
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= a diseased state or symptom. |
morbidity |
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what neurological/hemorrhagic event are sickle cell diseased individuals at risk for? |
stroke (occluding vessels) |
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acute large vessel occlusion in sickle cell disease is more common in children or adults? |
children |
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what organs are more susceptible to sickle cell disease complications? |
most susceptible according to Dr. L = kidneys and lungs but also spleen |
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sickle cell disease can affect renal medulla and spleen resulting in...? |
urinary concentrating ability impaired if renal medulla is impaired splenic infarction, increased risk of infections, prone to salmonella |
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which part of the kidney impacted by sickle cell disease |
renal medulla (an area of low O2 tension) |
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Sickle Cell disease treatment is generally supportive. what does this mean? |
Youcannot pick out the sickled cells that are already there so you try to stop it from gettingworse. |
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what does HbF stand for? |
fetal hemoglobin |
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analgesics = |
painkillers |
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Withpainful sickle cell crisis: treatment = |
Fluids,O2, analgesics, antibiotics with infection |
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In sickle cell disease = acute chest syndrome, stroke, bone marrow necrosis, or intractablepain without response to supportive treatment may require.... |
exchange transfusion |
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newer treatment for sickle cell disease |
hydroxyurea to increase HbF (fetal hemoglobin) to reduce vaso-occlusive crises |
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Hemoglobin C vs S-C which is more mild? |
hemoglobin C |
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Hemoglobin C sickle cell = more mild. most do NOT have symptoms. but if they do, what are they? |
Most people do not have symptoms. It can cause a mild to moderate splenomegaly and/or hemolytic anemia |
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beta thalassemia minor or beta thalassemia trait = happens when you get a normal gene from one parent and a thalassemia gene from the other. this results in...? |
mild anemia and probably won't need treatment |
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what is another name for beta thalassemia major? |
Cooley's anemia |
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Beta thalassemia = 1 defective gene = 2 (both) defective genes = |
1 = minor/trait 2= intermedia (moderate) or major (severe) aka Cooley's |
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what is more common: alpha or beta thalassemia? |
beta |
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Thalassemia geography alpha = beta = |
a = southeast Asia B = mediterranean |
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alpha thalassemia consists of 4 genes: what happens when 1, 2, 3, or 4 or missing? |
1 defective/missing: Your red blood cells might be smaller than normal. no symptoms, no treatment. you are a silent carrier. 2 defective/missing: very mild anemia that will typically not need treatment. This is called alpha thalassemia minor or alpha thalassemia trait. 3 defective/missing: mild to moderately severe anemia. This is sometimes called hemoglobin H disease. If it is severe, you may need blood transfusions. 4 defective/missing: alpha thalassemia major or hydrops fetalis. The fetus will be stillborn, or the child will die soon after birth. |
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the severe hemolytic anemias (alpha major and beta major) are typically noticed when? |
childhood |
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which Beta thalassemias require transfusions? |
just major (intermedia and minor do not) |
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how do you distinguish B thalassemia minor from Fe deficiency? |
B minor has normal Fe saturation |
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= is a red blood cell parameter that measures variability of red cell volume/size (anisocytosis). |
RDW (red cell distribution width) |
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how do you distinguish iron def. anemia vs. thalassemia? |
RDW = high in IDA, low in thalassemia RBC = low in IDA, normal/slightly elevated in thalassemia |
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to definitively diagnose IDA, you need to do ? to definitively diagnose thalassemia, you need to do ? |
iron studies hemoglobin electrophoresis |
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is a blood test that can detect different types of hemoglobin. [useful for thalassemias, sickle cell, defective hemoglobinopathies.] |
hemoglobin electrophoresis |
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what is more mild: alpha or beta thalassemia? |
alpha |
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what is more mild: alpha or beta thalassemia intermedia? |
alpha (-Dr. L lecture comment) |
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α chain not as soluble as β chain so generally there is no.... |
generally no hemolysis in alpha |
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1 missing or abnormal alpha globin gene = 2 missing or abnormal alpha globin gene = 3 missing or abnormal alpha globin gene = 4 missing or abnormal alpha globin gene = |
1 = silent carrier 2 = alpha thalassemia minor/trait 3 = hemoglobin H disease 4 = alpha thalassemia major or hydrops fetalis |
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another name for alpha thalassemia major = |
hydrops fetalis |
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a shift to the left means... |
increased neutrophil bands |
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what is the predominant leukocyte in peripheral blood |
neutrophils |
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location of majority of neutrophils |
Themajority of the neutrophils are in the bonemarrow or tissue; they are not typically in the blood as much |
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more neutrophils in blood or tissue
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tissue |
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= if there is an areathat needs the neutrophils to fight infection, the neutrophils will pick upthis signal and come here (do not need to tell them twice) |
Chemotaxis |
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what is it called when neutrophils adhere to endothelial surface? if there is a deficiency in adhesion, then... |
margination Deficiencyin adhesion – leukocyte-adhesion deficiency with defective bacterial killing |
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Phagocytosis Neutrophilwith surface receptors for C__b and a portion of Ig__ for recognition and binding |
C3b, IgG |
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neutrophils have primary and secondary granules. primary granules are also present in...? secondary granules give the neutrophils their...? |
monocytes color |
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which cells present in allergic and inflammatory reactions? |
eosinophils and basophils |
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= increase in numbers in parasitic infections andallergies |
eosinophils |
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= have HISTAMINE, which is why thecell has dark-blue basophilic granulations |
basophils |
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Eosinophils and basophils are both present in small numbers normally.
___________= increased in myeloproliferativesyndromes ____________= elevated in allergic response, parasites |
basophils, eosinophils |
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basophils are increased in ____________ syndromes |
myeloproliferative (abnormal growth in bone marrow) syndromes |
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myeloproliferative means... |
abnormal growth in bone marrow |
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Hypereosinophilicsyndromes = common or rare? associate with....? |
rare Associatedwith damage to lung, peripheral nervous system, endocardialtissue, drug induced, malignancies (paraneoplasticsyndromes, hodgkins) |
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monocytes are agranular but in the same myeloid cell line t or f |
false = they are granular AND in same myeloid cell line |
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do monocytes have granules? |
yes. Monocytes do have GRANULES…they are just hard tosee |
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which cell line are monocytes = myeloid or lymphoid? |
myeloid |
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most circulating monocytes are marginated. what does this mean clinically? |
when you get a blood sample, may show a lower number in the blood than it actually is because they are stuck to the walls and not flowing |
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monocytes that move into tissues and develop turn into... |
macrophages |
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monocytes have similar function to what other cell? but monocytes have an increased importance in killing the following: |
neutrophils
Increasedimportance in mycobacteria, fungi, protozoa |
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monocytes = small or large in size? |
large |
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monocytes can do the following: |
antigen presentation, capable of cytotoxicity, secrete cytokines |
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monocytes have a rolein tumor cytotoxicity against tumor cells t or f |
true |
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name some monocytes/macrophages = whose name changes only based on location |
Langerhans cells of skin, Kupffer cells of liver, alveolar macrophages |
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monocytes contain small numbers of proteins t or f |
false |
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Granulocytemass Marrowgranulocyte precursors about ___% of granulocyte mass Storagepool about ___% of granulocyte mass Peripheralneutrophils only about ___% of total granulocyte mass |
20% 75% 5% (smaller number in blood because shorter life in blood and longer life in tissues) |
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Peripheralwhite cell count reflects granulocyte status t or f |
false Peripheralwhite cell count does not adequately reflect granulocyte status |
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-cytosis means |
increase in cell numbers |
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-penia means |
decrease in cell numbers |
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-moid means |
resembles |
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= is a reactive increase in the WBC, which can mimic leukemia. The reaction is actually due to an infection or another disease and is not a sign of cancer. Blood counts usually return to normal when the underlying condition is treated. |
leukemoid reaction |
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leukocytosis is typically due to primary hematologic disorders t or f |
false Generallydue to other process and not due to primary hematologic disorder Causes include = Infection Acutestress Drugs Chronicinflammaiton Malignancy Marrowhyperstimulation Postsplenectomy Smoking |
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which drugs can cause leukocytosis? |
corticosteroids (because the steroids cause leukocytes to stick to the walls) |
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Since the steroids prevent the cells from sticking to the walls ofvessels/margination = fewer cells can migrate to interstitium so there is an increased risk of ..... |
infection |
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CML = test leukocyte alkaline phosphatase levels to help support/confirm CML, they will be high or low? |
low (they are normalto high in other myeloproliferativedisorders/leukemic reaction) |
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acute leukocytosis usually reflects = chronic reflects = |
acute leukocytosis = acute infection, stress, steroid administration chronic leukocytosis = chronic bone marrow stimulation |
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neutrophil count may vary among ethnic groups. it tends to be lower in... |
african americans |
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Generallybenign cyclical changes in all hematopoietic cell lines but most in neutrophils |
cyclic neutropenia |
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Congenitalneutropenia/agranulocytosis associated with.... |
infections perinatally, increased risk of acute leukemia |
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leukopenia can occur after infection t or f |
true |
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if leukopenia occurs during overwhelming infection = good or poor prognosis? |
poor |
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what drugs can cause leukopenia? |
chemotherapy, chloramphenicol (Mostdrug induced neutropenia responds to d/c agent) |
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autoimmune neutropenia is associated with which autoimmune disorders? |
SLE and RA |
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Your patients lab results come back showing neutropenia. What should you do? |
discontinue any offending drugs, perform serologic testing to r/o collagen vascular disease, bone marrow exam for diagnosis |
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Neutropenia counts: between 1000-1500= between 500-1000 = below <500 = |
usually w/o impairment; no intervention slightly increased risk of infection more serious risk of infection |
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Even though _________ are normallybad for infection (because they prevent margination), you still give them sothe body makes even more cause it finally realizes how low it is. [Sometimes you have to do things that seemcontradictory to what you think] |
steroids |
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Sometimes the WBC count may be normal or low (whenit should be higher with infection) during infection so you think the patientdoes not have infection but they do. Soyou have to think outside the box. t or f |
true |
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what does G-CSF or GCSF stand for? |
granulocyte colony-stimulating factor |
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to manage neutropenia, what can you prescribe? |
GCSF (neulasta, neupogen) |
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function of G-CSF |
stimulations production of granulocytes and then pushes them from marrow into blood alsostimulates the survival, proliferation, differentiation, and function ofneutrophil precursors and mature neutrophils. |
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= central cell of the immune system |
lymphocytes |
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location of mature B cells? |
mature B cells leave bone marrow and migrate to lymphoid tissue |
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how are B cells identified |
cell surface Ig and antibodies CD19, CD 20, CD 21 |
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Whenantigen binds to cell surface Ig... |
activation and proliferation of B cells, which differentiate into plasma cells |
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B cells = sincefrom different stages in development, have a variable mechanism and expression t or f |
true |
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precursors of T cells migrate from where to where |
bone marrow to thymus |
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surface proteins of T cells = |
CD3, CD4, CD8 |
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helper cells = |
CD4 |
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MatureT cells comprise approximately: ___%of peripheral blood lymphocytes ___%of lymph node cells ___%of spleen lymphoid cells |
80%, 40%, 25% |
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CD 4 = MHC class I or II CD 8 = MHC class I or II |
II, I |
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ClassI or II ?????– from intracellular endogenous antigens processed in cytosol and travelthrough endoplasmic reticulum |
Class I |
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Class I or II ????? from antigens from extracellular sources and engulfed via endocytosis andprocessed in intracellular vesicles |
Class II |
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the outer cortex of lymph nodes primarily consits of....
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B cells (activated B cells proliferate after encountering antigen) |
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Lymph nodes organization: outer cortex = cortex = |
outer B cells T cells around cortex |
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Spleen's lymphoid system function: traps antigens from lymphatic system or blood?? the lymphocytes are located in what part of the spleen? spleen also important in the disposal of.... |
blood white pulp of spleen disposal of RBC |
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what does MALT stand for?
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mucosa-associated lymphoid tissue
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= the diffusion system of small concentrations of lymphoid tissue found in various sites of the body,
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MALT
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what organs are part of the MALT system
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tonsils, adenoids, appendix, Peyer's patches (of the small intestine) [along with others] |
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what percentage of circulating/peripheral leukocytes are lymphocytes?
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20-40% in adults (higher in children/infants)
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the majority of circulating lymphocytes are B cells or T cells
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T cells (80-90%)
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the majority of circulating lymphocytes are...
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mature resting lymphocytes
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how are NK cells different from T and B cells
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no specific surface cell marker; indiscriminate killers
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name the neoplasias/malignancies of the lymphoid system |
lymphomas (Hodgkin's, non-Hodgkin's), lymphoid leukemias (ALL, CLL), plasma cell dycrasias (e.g. multiple myeloma) |
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= produced as a result of abnormal proliferation of a monoclonal population of plasma cells that may or may not secrete detectable levels of a monoclonal immunoglobulin or immunoglobulin fragment (paraprotein or M protein). |
plasma cell dycrasias |
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Most common presentation of lymphoid malignancy in adult = |
painless lymphadenopathy |
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a disease resulting in lymph nodes which are abnormal in size, number or consistency and is often used as a synonym for swollen or enlarged lymph nodes. |
lymphadenopathy |
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lymphadenopathies are always malignant t or f |
false lOthercauses of lymphadenopathy non-malignantlThereforehistory is helpful |
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____________ or lymphadenitis refers to lymph nodes which are abnormal in size, number orconsistency and is often used as a synonym for swollen or enlarged lymphnodes. |
lymphadenopathy |
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most likely causes of cervical lymphadenopathy |
Mostdue to infection of the upper respiratory tract, mononucleosis, other viralsyndromes, bacterial pharyngitis |
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most likely cause of unilateral axillary or femoral lymphadenopathy |
unilateral means one side so suggests LOCAL. mostly due to skin infections |
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generalized lymphadenopathy suggests... |
suggests systemic = systemic infections, HIV, cytomegalovirus,autoimmune disease, drug reactions, lymphoma |
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enlarged supraclavicular node suggests.. |
highly suggestive of malignancy |
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The __________ lymph node is the hypothetical first lymph node or group of nodes draining a cancer. |
sentinel |
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which nodes are the ones we worry about? |
supraclavicular nodes |
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swollen occipital nodes can be indicative of... |
rubella |
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where do GI tracts drain = which lymph nodes |
supraclavicular |
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Excisionalbiopsy = |
takeout the entire thing |
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= can be hit or miss because youare sticking a needle in a random part of the lymph node. Because if it is not near the needle, it maybe inaccurate |
needle biopsy |
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Ifcause of lymphadenopathy is not apparent, then.... |
biopsy warranted |
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the analysis of heterogeneous populations of cells for the purpose of identifying the presence and proportions of the various populations of interest. Antibodies are used to identify cells by detecting specific antigens expressed by these cells, which are known as markers. |
immunophenotyping |
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Characterizescell surface antigens on malignant lymphocyte Alsodetermines B cell, T cell, NK cell or non-malignant |
immunophenotyping |
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what is the most common lymphoma in young adults? |
Hodgkin's Lymphoma |
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Hodgkin's lymphoma = bimodal incidence peaks. when are they? |
1st peak: 15-35 yoa 2nd peak: > 50 yoa Over80% success rate in the younger age group (15-35yoa) Older individuals are less likelyto survive/overcome Hodgkin’s lymphoma |
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which virus is frequently found in Hodgkin's lymphoma malignant cells? |
Epstein Barr virus (EBV) Nodirect causative link established |
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Hodgkin's lymphoma = there is an increase in frequency in patients with congenital immunodeficiency syndromes t or f |
false |
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Hodgkin's lymphoma = lincreasein frequency in patients immunosupressedafter organ transplant t or f |
false
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Hodgkin's lymphoma = increased presence in HIV patients t or f |
false |
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do we know the cause of Hodgkin's lymphoma? if so, what is it? |
no (etiology unknown) |
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how do you identify the irregular lymphocytes from EB virus? |
more cytoplasm, irregular shape |
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what is the pathogonomic for hodgkins lymphoma? |
Reed-Sternberg cells (w/ owl eye appearance, binucleate cells) |
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you notice that the peripheral smear of your patient shows "owl eye" appearance. what cells are these? what would your diagnosis be? |
Reed-Sternberg cells, Hodgkin's lymphoma |
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EBvirus in Reed-Sternberg cells in about ___% of patients with Hodgkin’sLymphoma |
50% |
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the bulk of infiltrated cells in lymph nodes of Hodgkins lymphoma are.... |
benign reactive inflammatory cells (SomeReed-Sternberg cells but may be on occasion difficult to find) |
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what are the 4 pathological variants of hodgkin's lymphoma |
nodular sclerosis (most common type) mixed cellularity lymphocyte depleted type lymphocyte predominant type |
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Histologicallyfibrous bands separating node into noduleslMostcommon type in young adultslTypicallyinvolve mediastinal and supradiaphragmaticnerves |
nodular sclerosing Hodgkin's lymphoma (80% of patients) |
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which nerves are typically involved in nodular sclerosis of HL |
mediastinal and suprdiaphragmatic nerves |
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MostHodgkin’s lymphoma start above the diaphragm. Some may travel below the diaphragm in Stage ___? |
3 |
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lNoband forming noduleslDiffuseheterogeneous infiltration of lymph nodeslOccursat any agelAdvancedstage with more subdiaphragmaticinvolvement |
mixed cellularity type of HL |
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In stage 4 of Hodgkin's lymphoma... |
spread to non-lymphatic parts ofthe body throughout the body |
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<1% rare formlWithsheets of Reed-Sternberg cells and few inflammatory cellslMorein older individualslOrin patients with HIV |
lymphocyte depleted type of Hodgkin's lymphoma |
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Closerindolent form of Non-Hodgkin's LymphomalNodulargrowth with Reed-Sternberg variant “Popcorncells”lStrongmale predominancelInvolveslargely peripheral nodes and spares mediastinumlGenerallywith excellent prognosislHoweveroften with late relapses |
Lymphocyte predominant type (5% of patients with HL) |
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lymphocyte predominant type of HL = strong male or female presence? |
male |
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lymphocyte predominant type of HL = good or poor prognosis |
generally excellent prognosis (but with late relapses) |
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lymphocyte predominant type of HL = which nodes? |
largely peripheral nodes |
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lymphocyte predominant type of HL = what is the pathogonomic? |
nodular growth w/ Reed-Sternberg variant "popcorn cells" |
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Hodgkin's lymphoma usually arises in lymph nodes of which parts of the body? and then spreads where? spreads hematogenously to which areas? |
mediastinum and neck Spreadsto adjacent/contiguous or non-contiguous nodal sites includingretroperitoneal nodes and spleen bone marrow, liver, lung |
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which lymphoma tends to be local clumps of involved nodes? which tends to be more disseminated? |
Hodgkins = local clumps Non = disseminated |
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clinical features of Hodgkins lymphoma = painless lymphadenopathy, which is mostly noted in which part of the body? mediastinal nodes are generally identified by...? |
the neck, routine CXR OR if large then respiratory symptoms |
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about 1/3 of patients with Hodgkins lymphoma have ___________ symptoms; this is designated by what letter? |
constitutional, b (e.g. Stage 3b) |
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what fraction of patients with Hodgkins lymphoma have constitutional symptoms? what are these symptoms? |
1/3 fever, night sweats, weight loss, pruritus (most common w/ nodular sclerosing form) |
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pruritus means.... |
itch |
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clinical features of untreated or advanced Hodgkins lymphoma: |
slowly progressive to multiple nodal sites followed by hematogenous spread to bone marrow, liver, other viscera [w/ progression = more symptoms arise which includes malaise, cachexia, infection complications] |
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death from Hodgkin's lymphoma is typically due to.... |
bone marrow failure, infection [because it had spread to other areas like bone marrow] |
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Hodgkins lymphoma = which one has symptoms present? a or b |
b (a = asymptomatic) |
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IIb has a worse prognosis than IIa in Hodgkins lymphoma t or f |
true (presence of symptoms = worse prognosis) |
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how many stages in Hodgkins lymphoma |
4 (w/ a and b so 8 total) |
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the stages of Hodgkins lymphoma: stage 1 = stage 2 = stage 3 = stage 4 = |
stage 1 = single site stage 2 = 2 or more sites; only one side of diaphragm (above typically) stage 3 = multiple sites on both sides of diaphragm w/ splenic and/or contiguous extra nodal (spread outside lymph nodes) site involvement stage 4 = extensive involvement of extra nodal sites w or w/o lymph node involvement |
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the work up of hodgkins and non-hodgkins are similar t or f |
true |
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what areas are you going to pay specific attention to when looking at Hodgkins lymphomas? |
lymphoid areas, Waldeyer's ring, liver, spleen |
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= are groups of blood tests that giveinformation about the state of a patient's liver. These tests include prothrombintime (PT/INR), aPTT, albumin, bilirubin (direct andindirect), and others. |
LFTs (liver function tests) |
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is an enzyme found in nearly allliving cells (animals, plants, and prokaryotes); catalyzes the conversionof pyruvate to lactate and back, as it converts NADH to NAD+ and back |
LDH (lactate dehydrogenase) |
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if you suspect Hodgkin's lymphoma, what are you going to do? |
standard blood work (CBC, LDH, LFTs, renal function, Ca+, uric acid) then a biopsy |
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Along with a biopsy of lesion w/ histological examination of Hodgkins lymphoma, you are also going to do a radiographic evaluation, which includes? |
CXR (lateral and PA)
chest, abdominal, pelvic CT gallium scan or PET scan bilateral bone marrow aspiration and biopsy |
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if there are bone symptoms of Hodgkin's lymphoma...what should you do |
bone scan |
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=does NOT need to be done as much due to newerevaluation techniques; currently NOT routine for Hodgkin's lymhoma |
staging laparotomy |
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= A procedure in which a particular body region is surgically examined to assess the extent of disease with the purpose of determining the stage or extension of a cancer. |
staging laparotomy |
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staging laparotomy (not really used anymore) for HL involves... |
splenectomy, liver biopsy, sampling of retroperitoneal nodes [puts patient at risk for infection] |
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factors of HL that give WORSE PROGNOSIS |
mixed cellularity type lymphocyte depleted type male large number of nodes involved over 40 yoa presences of constitutional symptoms (type B) elevated sed rate large mediastinal nodes |
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which types of HL have worse prognosis? |
mixed cellularity and lymphocyte depleted |
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which types of HL have better prognosis? |
nodular sclerosing and lymphocyte predominant |
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Sed ratewill increase with increasing levels of.... |
inflammation |
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rheumatoid arthritis vs. osteoarthritis:
which is local, which is systemic? |
osteoarthritis is local RA is systemic |
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Sincemost with Hodgkin's are young adults with goal of long term survival = Usetherapy that minimizes treatment-associated morbidity/mortality withoutsacrificing cure potential t or f |
true |
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HL is generally highly curable t or f |
true (cure rate > 80%) |
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Treatment for Stages IA, IB, and IIA in HL |
Radiation (involved sites and contiguous nodal regions BUT radiation increases risk of breast cancer and lung cancer) Due to risk of carcinomas/other sequelae of radiation = combine w/ chemotherapy |
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a condition that is the consequence of a previous disease or injury. |
sequela |
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sequelae of HL |
thyroid dysfunction (hypothyroid), accelerated CAD (coronary artery disease), risk of carcinomas |
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treatment for advanced stages (III and IV) of HL OR lower stage w/ other risk factors (B symptoms, Large mediastinalnodes, histological type MC or lymphocyte depleted) |
ABVD chemotherapy (side effects = pulmonary fibrosis in <5% and cardiac risk) [MOPP chemo used to be used but ABVD is just as effective w/ less complications] |
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is radiation used for advanced HL? |
no (rarely used; only used in bulky mediastinal disease but still combined with chemo) |
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Hallmark of most lymphomas = |
painless lymphadenopathy |
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To evaluate the response to HL treatment, you will do the following.... |
H&P, blood pannel,CT/Gallium/PET scan Bonemarrow bx |
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enlarged nodes in HL after treatment means no cured t or f |
false (Curemay still demonstrate enlarged nodes so evaluate w/ biopsy, PET stand, or gallium) |
|
|
Positivegallium or PET scan with residual radiographic evidence associated with highrelapse rate in which disease? |
Hodgkins lymphoma |
|
|
the majority of relapses occur when? |
with 2 years (rare after 5 years) [20% in early stage HL will relapse if radiation only used] |
|
|
For those who do not respond to initial therapy in HL, then you do __________ therapy. this includes what? |
salvage therapy, high dose chemotherapy [>50% with recurrent Hodgkin’s if chemosensitivecan be cured] |
|
|
lCauseunknown in mostlNogenetic predisposition in mostlNoregular epidemiologic or environmental factorslSomewith similar chromosomal aberrations but ? Involvement or cause for non-HL....t or f? |
true |
|
|
there is no definite cause of NHL but what are some associations/correlations? |
immunodeficiency syndromes (e.g. HIV/AIDS), autoimmune disorders, carcinogenic viruses (HIV, EBV), Karposi's sarcoma, lymphoproliferative disorders |
|
|
clinical signs of NHL |
Painlesslymphadenopathy in 1 or more peripheral node sites Possibleinvolvement of extra-nodal sites (more aggressive forms more likely) CNS involvement rare but more common in more aggressive lymphomas |
|
|
similar to HL, NHL has constitutional symptoms (in 20% of patients but more common in aggressive forms), which includes what symptoms? |
fever, weight loss, night sweats |
|
|
the workup of NHL is similar to HL, which includes.... |
thorough H&P (attention to lymphoid areas, Waldeyer's ring, liver, spleen) standard blood work (CBC, LDH, LFTs, renal function, Ca, uric acid) |
|
|
staging for NHL: involves history, PE for size/location/distribution, blood work, CT chest/abdomen/pelvis, bone marrow bx and aspirate, gallium or PET, LP if risk of leptomeningeal involvement t or f |
true |
|
|
ancillary tests for NHL include: |
HTLV-1 (human T-cell lymphotropic virus) or HIV testing, GI evaluation, S. Protein electrophoresis [depending on symptoms the patient is presenting] |
|
|
what does HTLV stand for |
human T-cell lymphotropic virus |
|
|
family of viruses are a group of human retroviruses that are known to cause a type of cancer called adult T-cell leukemia/lymphoma and a demyelinating disease called HTLV-I associated myelopathy/tropical spastic paraparesis |
HTLV |
|
|
poor prognosis/survival in NHL if.... |
stage III or IV multiple extra-nodal sites elevated LDH presence of B symptoms |
|
|
stem cells can renew themselves t or f |
true |
|
|
what are the 3 forms of hematopoiesis disorders ? |
underproduction of mature cells (aplastic anemia) overproduction of mature cells (myeloproliferative disorders) failed differentiation w/ many immature forms (myelodysplasia and acute leukemia) |
|
|
both forms of acute leukemias (ALL, AML) show large numbers of what cells... |
immature forms (blasts) of WBCs |
|
|
l90%of adult leukemia is ____ (10% ALL)l90%of childhood leukemia is _____ (10% AML) |
Think AML = Mature Think ALL = Little |
|
|
how is AML distinguished from ALL? |
presence of Auer rods AND/OR immunophenotyping (uses cell surface antigens to confirm myeloid or lymphoid) |
|
|
name the 2 origins of stem cells |
myeloid (majority) and lymphoid (lymphocytes = T, B, NK, plasma) |
|
|
In leukemias, thebone marrow is being replaced by __________ cells that take up space and do NOTcontribute to normal function. |
immature |
|
|
many acute leukemias have detectable characteristic ___________ _____________ abnormalities. do we know the how these result in malignant transformation? if so, what is it? |
clonal chromosomal abnormalities, no (unknown) |
|
|
what are the risk factors of acute leukemias? |
high radiation exposure occupational exposure to benzene secondary AML due to chemotherapy with alkylating agents chromosomal disorders (Down syndrome, Bloom's syndrome, Fanconi's anemia, Ataxia telangiectasia) |
|
|
clinical features of acute leukemia include = |
anemia, infection, and bleeding (due to peripheral cytopenia) bone pain (from infiltration of bone marrow by malignant cells) |
|
|
name the stages of treatment for acute leukemia [in order] |
induction therapy consolidation therapy intensification therapy maintenance therapy |
|
|
what is the first phase of treatment for acute leukemia? what is the goal of this? |
induction therapy using chemotherapy directed at reducing # of leukemic blasts to undetectable level (but there will still be some "bad" cells left so you will move on to the other phases of treatment) |
|
|
= the process or action of bringing about or giving rise to something |
induction |
|
|
what is included in consolidation therapy of acute leukemia? |
the 2nd step (after induction therapy) continues using chemotherapy w/ same agents used in induction to attempt to kill any existing cancer cell left in the body |
|
|
= combining things/parts to make something stronger as a whole |
consolidation |
|
|
what is involved in intensification therapy of acute leukemia? which step is this? |
3rd step = use wider range of effective agents; high dose therapy; goal is to eliminate cells w/ potential resistance to drugs used in the first 2 phases |
|
|
what is the last phase of treatment for acute leukemias? and then describe it |
maintenance therapy = using low dose intermittent chemotherapy over prolonged period of time to PREVENT RELAPSE, INDUCE REMISSION |
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|
longer time to achieve response to initial/induction therapy means worse prognosis for acute leukemias t or f |
true |
|
|
what are the 3 subtypes of ALL? and describe them [not WHO classification] |
L1 = small, uniform lymphoblasts w/ indistinct nucleoli (25-30% of cases) L2 = larger, more pleomorphic (different size of cells/nuclei) cells, commonly in adults (65% of cases) L3 = large basophilic cells and vacuoles, most infrequent type (2-3%) [in WHO classification = L3 is is not ALL but its own class known as Burkitt's lymphoma/leukemia] |
|
|
= is a term used in histology and cytopathology to describe variability in the size, shape and staining of cells and/or their nuclei. It is a feature characteristic of malignant neoplasms, and dysplasia. |
pleomorphism |
|
|
what does WHO stand for? |
world health organization |
|
|
according to WHO classification, there are only 2 subtypes of ALL (instead of 3)...what are they? |
based on lineage so 2 types = precursor B cell and precursor T cell |
|
|
why is ALL more curable in children? |
different biological mechanisms of disease older patients unable to tolerate aggressive chemotherapy 50%adults with Philadelphia chromosome abnormality with increased chemotxresistance and increased risk of CNS disease |
|
|
treatment for ALL = short or lengthy |
lengthy |
|
|
ALL cells have propensity to reside in which parts of the body? why? |
CNS and testes; chemotx agents generally do not penetrate these sites |
|
|
In ALL, chemotherapy has a hard time penetrating the CNS andtestes so may want to inject ___________ for greater CNS penetration. |
intrathecally |
|
|
Allshould have _?_ with administration of intrathecalmethotrexate and/or whole brain irradiation as adjunct to induction andconsolidation therapy in ALL (acute lymphocytic leukemia) |
LP (lumbar punture) w/ that intrathecal drug |
|
|
ALL relapse tends to occur how long after initial remission? |
months to years after initial remission |
|
|
who benefits from allogenic or autologous stem cell transplantation in ALL? |
those w/ worse prognosis pts. w/ Philadelphia chromosome [HLA-matcheddonor highly beneficial; Non-HLAmatched donor with poor outcome and no benefit over maintenance therapy] |
|
|
which works better for ALL transplants: autologous or allogenic? |
allogenic = Allogeneic works better from a HLA-compatible donor
Autologous stem cell transplantation = has limitations because using the individual’s own cells (autologous) can just be giving the individual back their own cancerous cells |
|
|
how are stem cells are preserved?
|
cyropreservation = freezing them |
|
|
if relapse occurs after ALL treatment, what are the chances of survival w/ chemotherapy alone |
3 yr survival rate of less than 10% so combined with local irradiation OR should consider allogenic stem cell transplantation |
|
|
what is the median age of diagnosis of AML |
65 yoa |
|
|
original classification of AML according to FAB had how many subtypes? what were they? |
8 = M0-M7 |
|
|
what does FAB stand for? |
Abbreviation for French-American-British (classification of acute leukemias) |
|
|
which leukemia is Myeloblastic,monocytic, erythroleukemic,megakaryoblastic |
AML |
|
|
what is the most common type of AML? |
M2 |
|
|
what is AML M3 subtype associated with? |
spontaneous bleeding from DIC (disseminated intravascular coagulopathy) |
|
|
what are AML M4 and M5 subtypes associated with? |
high level of circulating WBC, swollen gums due to infiltration with blasts |
|
|
= Condition affecting the blood's ability to clot and stop bleeding. |
DIC = disseminated intravascular coagulopathy |
|
|
AML M7 is also known as? what are its features |
megakaryoblastic leukmia, w/ significant marrow fibrosis, usually w/ organomegaly and pancytopenia similar to patients with myelofibrosis |
|
|
WHO classification (compared to FAB) for diagnosing AML |
fewer blast cells needed but more conditions/features (since fewer blast cells needed for diagnosis) |
|
|
although there are numerous varieties of AML, lab results show the same thing t or f |
false (Sincethere are so many varieties of AML, thelaboratory findings will vary) |
|
|
Lab findings of AML include: microcytic, normocytic, or macrocytic? thrombocytosis or thrombocytopenia? abundance of what? |
normocytic, thrombocytopenia, abundance of myeloblasts w/ decreased number of mature, normal cells |
|
|
what are some acute emergencies that patients w/ AML are at risk for? |
leukostasis (Hyperleukocytosis Syndrome) = respiratory distress life threatening CNS bleeding (from blasts surrounding injured vessels) high WBC numbers = increased release of breakdown by products leading to hyperkalemia, acidosis, hyperuricemia ---> renal failure |
|
|
= is a laboratory procedure in which whiteblood cells are separated from a sample of blood. |
leukpheresis |
|
|
Emergency__________ of CNS if intracranial bleeding, cranial nerve involvement,leukemic meningitis in AML |
irradiation |
|
|
should RBC transfusions be given in a AML emergency ? |
no |
|
|
treatment of AML in emergency situations? |
leukapheresis, hydroxyurea, induction therapy to reduce circulating cell numbers hydration |
|
|
maintenance therapy is effective in prolonging remissions in AML t or f |
false |
|
|
maintenance therapy is effective in prolonging remissions in ALL t or f |
true |
|
|
how is AML treatment different from ALL? |
induction therapy w/ consolidation for over 4-6 months no maintenance therapy no CNS prophylaxis needed (this is used in ALL to prevent spread to CNS) |
|
|
treatment for individuals with AML |
induction therapy (complete remission of 60-80% for younger adults) Dependingon prognostic classification, age, etc., may receive intensive consolidationchemotherapy or allogeneic or autologous stem cell transplantation |
|
|
Thereis a wide range of 5 year survivalrates (5-60%) because of the different types of AML. t or f |
true |
|
|
what is really the only long-term cure for patients w/ AML? |
allogenic transplantation |
|
|
is chemotherapy more effective before or after allogenic transplantation? |
before |
|
|
lIfineligible for allogeneic transplant due to advanced age or lack of compatibleHLA donors may receive ..... |
autologous bone marrow or stem cell transplantation [?If autologous transplant with any better survival rates vs. chemotherapy sinceboth about 20-40%] |
|
|
Olderpatients with AML and co-morbidities and/or secondary leukemia maynot tolerate aggressive chemotherapy regimens with or without stem cellproceduresWithlower remission rates (30-50%) and high mortality with aggressive chemotherapymay only receive..... |
supportive/hospice care |
|
|
what ismajor cause of morbidity/mortality during chemotherapy? |
infection |
|
|
APL stands for what....? is it more common in younger or older patients? |
acute promyelocytic leukemia (it is a subtype of AML) younger patients |
|
|
are Auer rods seen in APL? |
yes (because it is a subtype of AML) |
|
|
what subtype of AML is due to chromosomal translocation (15, 17) and composition changes, increased proliferation of immature cells ??? comprises 10-15% of AML cases |
APL (acute promyelocytic leukemia) |
|
|
which chromosomal translocation is associated with APL? |
15, 17 |
|
|
15, 17 translocation associated with... |
APL |
|
|
patients with APL often experience.....? the is due to....? |
life-threateningbleeding caused by disseminated intravascular coagulation (DIC) and procoagulantfactors released by granules in promyelocytes |
|
|
what was the prior tx for APL? what is the current treatment? |
low dose heparin and platelet transfusion [had high mortality and morbidity rates] current treatment ATRA w/ chemo (90% success but high relapse rates if used w/o chemo) |
|
|
what syndrome can result due to ATRA therapy for APL? |
retinoid acid syndrome |
|
|
a potentially life-threatening complication observed in patients with acute promyelocytic leukemia (APML) and first thought to be specifically associated with all-trans retinoic acid (ATRA) (also known as tretinoin) treatment. |
retinoic acid syndrome (RAS) 5 to 10 % mortality rate |
|
|
Retinoic acid syndrome complications = |
acute cardiopulmonary distress, dyspnea (difficult breathing), fever, weight gain, hypotension, and pulmonary infiltrates 5-10% mortality rate |
|
|
dyspnea = |
difficulty breathing |
|
|
what fraction of patients w/ ATRA induction, consolidation and maintenance chemotx have long-term remission |
2/3 |
|
|
treatment for APL |
ATRA ++ induction, consolidation, and maintenance chemotx |
|
|
Thing to remember from AML = clinical picture is quite different in prognosis, treatment, etc. because there are a bunch of different _________ |
subtypes |
|
|
what does M protein stand for? |
monoclonal protein |
|
|
= is a condition in which an abnormal protein (monoclonal protein, or M protein) is in the blood. M protein is produced by plasma cells; MGUS usually causes no problems. |
Monoclonal gammopathy of undetermined significance (MGUS) |
|
|
hallmark of plasma cell disorder = |
presence of homologous M protein or Ig molecule detectable in serum or urine by protein electrophoresis |
|
|
what are some signs/symptoms of multiple myeloma? |
lytic bone lesions serum/urine Ig hypercalcemia renal insufficiency anemia |
|
|
what is the most common plasma cell disorder? |
multiple myeloma |
|
|
how are plasma cell disorders classified? |
classified by Ig (G, A, D, E, or M) |
|
|
presence of M protein is found in the follow benign and malignant conditions: |
plasma cell disorders CLL (IgG or IgM) autoreactive or infectious disorders MGUS (normal, unknown significance) |
|
|
M protein indicated by what on EP (electrophoresis) analysis? |
M spike larger than normal |
|
|
= There are no clinical manifestations (signs,symptoms, etc.) saying they have multiple myeloma but they are 7x more likelyto get multiple myeloma |
MGUS (Monoclonalgammopathy of unknown significance) but only 1% of people w/ MGUS transform into multiple myeloma |
|
|
MGUS = Monoclonalgammopathy of unknown significance what are the signs of this?? |
lowserum M protein, no urinary Bence-Jonesprotein, < 10% bone marrow plasma cells Absenceof:Anemia,hyercalcemia,renal failure, lytic bone lesions |
|
|
Bence-Jones protein present in MGUS t or f |
false |
|
|
presence of protein in the urine is normal physiologically t or f |
false Thereshould be very little or no protein in the urine |
|
|
where is Bence-Jones protein found in patients w/ multiple myeloma |
blood or urine |
|
|
Bence- Jones Proteins are free light chains produced by what cells? |
neoplastic plasma cells |
|
|
bence jones proteins are: a. adhesive light chains b. adhesive heavy chains c. free light chains d. free heavy chains |
c. free light chains |
|
|
TheBence Jones protein is a broken off piece ofthe _____________ (one of the smaller pieces of the Y shape of Ig’s) |
immunoglobulin |
|
|
lMalignantplasma cell disorderlNeoplasticinfiltration of bone marrow and bonelPresenceof monoclonal Ig or light chains in urine or serum |
multiple myeloma |
|
|
Diagnostic criteria for multiple myeloma: Increasein number of plasma cells in bone marrow >___% SerumM protein (other than Ig_) > 3 g/dL forIg__ or > 2 g/dL forIg___ Mprotein > 1 g/24 hrs |
>30% other than IgM IgG >3 IgA> 2 |
|
|
Mayconsider dx with lower M protein or < 30% plasma cells in bone marrow if: Lyticbone lesions, plasmacytoma,hypogammaglobulinemia for which disorder? |
multiple myeloma |
|
|
some of the clinical manifestation of multiple myeloma are due to a deficiency in ___________ immunity |
humoral (B cell, since it is affecting plasma cells which come from B cells this makes sense) |
|
|
what are some of the signs/symptoms of multiple myeloma that are BONE related |
lMostcommon – Bone painlX-rays– “punchedout”lesions (osteolytic)lGeneralizedosteopenialPathologicfractureslOtherbone lesions appear as expansilemasses with possible spinal cord compressionlHypercalcemiadue to bone involvement |
|
|
what causes the anemia seen in multiple myeloma |
marrow infiltration (of cancerous plasma cells) and hematopoietic suppression |
|
|
why are multiple myeloma patients more likely to experience bacterial infection? what are common infections experienced by these people? |
impaired humoral immunity (B cells/plasma cells) increased destruction of Ig respiratory tract infections and UTI (gram neg UTI) |
|
|
renal insufficiency is seen in ___% of multiple myeloma cases. what causes this renal insufficiency? |
hypercalcemia, hyperuricemia, infection, amyloid deposition tubular damage from light chain excretion (M protein) M protein effects = lCryoglobulinemialHyperviscositylAmyloidosislClottingabnormalities |
|
|
negative effects of M protein in multiple myeloma |
Cryoglobulinemia Hyperviscosity Amyloidosis Clottingabnormalities |
|
|
staging of multiple myeloma is based on.... |
lab and bone x-rays |
|
|
multiple myeloma is curable t or f |
false (Multiplemyeloma is not curable but manageable. May not fix the problem but you can monitorand help manage the issue)
|
|
|
treatment for stage I multiple myeloma |
no need for immediate therapy but monitor serial M protein levels |
|
|
treatment for stage II and III multiple myeloma |
systemic therapy and supportive care, which includes VAD chemotherapy |
|
|
Myeloma= NOT uncommon, NOT curable t or f |
true |
|
|
what are the non-chemotherapeutic drugs used for multiple myeloma |
thalidomide and dexamethasone |
|
|
what are the side effects of thalidomide used for multiple myeloma |
birth defects, Peripheralneuropathy, constipation, somnolence, rash DVTin 25% when combined with dexamethasone |
|
|
how do the majority of multiple myeloma patients respond to treatment? |
Majorityrespond with reduced bone pain, reduced hypercalcemia,improved anemia, decreased M protein level |
|
|
what can you combine the chemotx w/ in multiple myeloma to increase survival and quality of life (compared to just standard chemotx) |
combine chemotherapy w/ alkylating agents followed by autologousperipheral stem cell infusion |
|
|
allogenic bone marrow transplants are commonly used in multiple myeloma patients t or f |
false Allogenicbone marrow transplant may be potentially curative but with excessive morbidityand mortality therefore with limited use |
|
|
if a patient w/ multiple myeloma relapses....what do you do? |
alternate chemotherapy regimen Or protease inhibitor (bortezomib) thalidomide analgoues w/ fewer side effects high dose corticosteroids |
|
|
supportive care is provided for older individuals w/ multiple myeloma. what does this care involve? |
biphosphonates to reduce bone resorption pain control, fracture prevention, local radiation renal support vaccines to common pathogens eythropoeitin for anemia |
