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Block 6 - Week 7
Hematology 2
73
Bartending
4th Grade
09/17/2009

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Term
Name this leukemia - symptoms include dyspnea, fatigue weight loss, arthritis, ab pain due to spleen, neurologic findings, confusion, signs include pallor, spleno, sternal tenderness; neutrophilia in all cell stages, thrombo/baso/eosinophlia, no fat in marrow, mild anemia; marrow shows hypercells, myelos, megakaryos, reticulin fibrosis, increased progenitors; Philadelphia chromosome in all myeloid cells from translocation of 9 to 22 which is short.
Definition
CML, this disease is a myeloproliferative disease but can become a true leukemia cancer
Term
What is this phase of CML - more malignant, more chrome abnormalities, disordered growth and diminished maturation, with fever weight loss adenopathy, extramedullary blast crisis; labs show anemia, leuko/thrombopenia, basophilia, blasts; treatment is supportive, chemo, or if early on then bone marrow transplant.
Definition
Accelerated phase
Term
T or F: the bcr-abl gene causes tyrosine kinase to activate cell pathways causing CML.
Definition
True
Term
What drug is a competitive inhibitor of BCR-ABL tyrosine complex that treats CML?
Definition
Imatinib, although there is resistance it is not common
Term
Name this myeloproliferative disease - hematopoeitic stem cell problem with sustained erythrocytosis and other cellular proliferation, peak onset at 50, symptoms include headache, visual, dizzy, abdominal pain due to splenomegaly, pruritis, thrombosis, hemorrhages; signs show retinal hemorrhages and hepatospleno; treated with phlebotomy, phosphorus, allopurinol.
Definition
Polycythemia vera, detected with normal O2 sat, high b12, hyperuricemia, and very low Epo; course progresses to spent phase of polycythemia, thrombosis is main cause of death
Term
Name this myeloproliferative disease - clonal disorder of platelet production, persistent elevation of platelet, neuro problems, hemorrhagic problems, thrombosis; labs show high hematocrit and RBC masss, neutrophilia, thrombocytosis; bone marrow shows hypercellularity in all cell lines but mostly megakaryocytes.
Definition
Primary thrombocythemia
Term
Name this leukemia - primarily due to exposure of offending agent, present similarly to aplastic anemia, rising incidence over time and age, more males, due to chrome breakage (bloom, fanconi, downs syndrome, immuno) or rads/chems; fatigue, infection, adeno, LUQP, leukostasis, rectal lesions; physical reveals spleno, lymphadeno, neuro, granulocytic sarcoma, dermatoses, CHF; labs show anemia, low WBC, thrombopenia, hypercellular undifferentiated blasts marrow, hyperuricemia, HOUR RODS typical.
Definition
AML, very heterogenous leukemia that is treated with induction via chemo and consolidation aka remission.
Term
Name this leukemia - leukemia subtype, distinct DIC, azurophilic granules, Auer RODS, translocation of 15:17 or 11:17, bad Retinoic Acid receptor alpha, treated with all trans retinoic acid, arsenic, or chemo.
Definition
Actue promyelocytic leukemia, a form of AML
Term
T or F: the most powerful prognostic indicators for leukemia remission are age, prior chemo, and chrome 5 or 7 abnormalities.
Definition
True, poor survive ability for AML
Term
Name this leukemia - malignant transformation of pre-B cell blasts with CD10 markers, most common childhood malign is CD10 positive, 30% adults is Ph pos, burkitts lymphoma c-myc subtype, malignant cells go to sanctuary sites like CNS/testis, treat with steroids, prolonged anti-metabolite therapy, anthracycline, CNS prophylaxis, and maintenance therapy, allogeneic bone transplant in worse off patients due to low success.
Definition
Acute lymphoblastic leukemia, survival is 3-6 months untreated
Term
What does petichiae indicate?
Definition
Low platelets
Term
In patients with high white cell count can plug vessels, so what type of transufusion do you want to withhold until the white count is brought down?
Definition
RBC transfusion because of increased viscosity aka leukostasis
Term
Antibodies to what what CD markers are for T cells and all leukocytes in general?
Definition
CD5,3 and CD45
Term
In a bone marrow test what disease would you have if TdT and CALLA-CD10 were positive versus a positive myeloperoxidse and sudan black?
Definition
First two show lymphoblasts, last two show leuko-origin
Term
T or F: The presence of Ph chrome in acute leukemia is poor prognosis versus chronic.
Definition
True
Term
Mutation of c-myc in chrome 8 is typical of which type of lymphoblastic leukemia?
Definition
Burkitts
Term
Which subtype of AML can present with granulocytic sarcoma aka chloroma?
Definition
M5 known as monocytic leukemia, infiltrate of leukemic cells into the skin or gingival but can affect almost every organ
Term
Name this lymphoma - disease of young and old, Reed-Sternberg cell is transformed B cell with faulty Ig gene arrangements, CD20 neg, CD15/30 positive, most common subtype is nodular sclerosis with rare R-S cells, presents as painless rubbery adenopathy in neck or axilla, systemic symptoms, chest symptoms, pruritis and pain with alcohol, associated with mono/EBV.
Definition
Hogdkin Lymphoma, treated with 6-8 cycles of ABVD
Term
T or F: the staging for both Hogdkin and non-Hogdkin is stage 1 for single lymph node region, 2 for two, 3 for crossing diaphragm, 4 multiple foci in extralymp tissues.
Definition
True; type a is asymptomatic, type b is fever, sweat, weight loss
Term
Name this lymphoma - youngest median age for cancers, can be cured with chemo and anti-CD20 Ab called Rituximab, lymph node enlargement often painless, with fever, sweats, fatigue, B and T cells can be affected.
Definition
NHL
Term
Which of these are proto-oncogenes - c-myc, bcl-6, cyclin D1, bcl-2?
Definition
All of them; c-myc=Burkitts, bcl-6 diffuse large B lymphoma, cyclin D1 Mantle cell lymphoma, bcl-2 follicular lymphoma
Term
What are the two most common non-Hodgkins B cell lymphoma and what gene IDs them?
Definition
Follicular - bcl-2, Diffuse Large B cell - bcl-6
Term
What is the difference between aggressive NHL (diffuse large cell) and indolent NHL (follicular)?
Definition
Aggressive is rapid growth, pain, extranodal sites, acute illness; Indolent is slow, limited to nodes, cytopenias from bone marrow.
Term
Name this subtype of NHL - 60% present with nodes only; 40% have extranodal involvement, CD20+ in virtually all cases, Adverse prognostic factors: Age, elevated LDH, poor PS, stage III or IV, > extranodal site. Half cured by R-CHOP; recurrent cases treated with stem cell transplantation. Gene expression profiling defines distinct biologic and prognostic categories.
Definition
Diffuse large B cell NHL, sharp initial dip in survival then plateau
Term
Name this subtype of NHL - Prototype of “indolent” lymphoma; often asymptomatic at diagnosis, low Hb. CD20+ in virtually all cases; highly responsive to rituximab anti-CD20 antibody therapy. Despite good response to initial therapy, usually “incurable”, median survival 12-13 years. Approx. 25% experience “transformation” to higher grade lymphoma; poor prognosis.
Definition
Follicular NHL
Term
Name this NHL subtype - Slow-growing, “indolent” subtype; often asymptomatic at diagnosis; good prognosis. Small to medium-sized cells; infiltrate around “marginal zone” of reactive B-cell follicles. CD20+, but CD5-, CD10-, CD23-. Highly responsive to rituximab anti-CD20 antibody therapy. Gastric MALT lymphoma = driven by Helicobacter pylori infection; responds to antibiotics, Other MALT sites: salivary glands, lung, head & neck, conjunctiva > skin, thyroid, breast.
Definition
Marginal Zone, MALT lymphoma
Term
Name this NHL subtype - CD20 low, CD5+, CD23+, Typically widespread involvement of nodes, spleen, liver, bone marrow, peripheral blood shows smudge cells, Richter’s transformation in approx. 5% - survival < 1 year, solid counterpart of CLL.
Definition
Small Lymphocytic Lymphoma
Term
T or F: T cell lymphomas are mostly cutaneous and aggressive with poorer survival than B cell lymphomas.
Definition
True
Term
NHL is treated with CHOP drugs and Rituximab which attacks CD20 cells, but what is the most important in vivo mechanism against lymphomas regarding Rituximab?
Definition
Antibody dependent cellular toxicity
Term
Primary polycythemia such as polycythemia vera has _____ EPO levels, and secondary due to renal neoplasms or hypoxemia has ______ EPO levels.
Definition
Low, high
Term
Many patients with polycythemia vera, essential thrombocythemia, and myelofibrosis are positive for the gain of function JAK2 mutation, but what gene is it located on?
Definition
9p, this mutation helps differentiate between each of the subtypes not between each other.
Term
T or F: Gompertzian kinetics states that growth of cells is greatest at the beginning and slows down.
Definition
True
Term
Which of these infectious agents can cause cancer - HPV, EBV, HTLV, HIV, HBV, HCV, HHV8?
Definition
All, EBV can make Burkitts, HHV8 can cause Kaposi’s
Term
What is adjuvant chemo versus neoadjuvant?
Definition
Chemo given when you no longer see disease versus neo is given before cancer is removed to shrink it
Term
What is salvage chemo?
Definition
Chemo when other therapies didn’t work
Term
What types of cancer have little benefit from treatment?
Definition
Adenocarcinoma and GI adenocarcinomas
Term
T or F: In FDA approval there are 4 phases, 1 is safety, 2 is activity, 3 is efficacy, 4 is post licensing.
Definition
True
Term
T or F: Hairy cell leukemia and mycosis fungiodes are given chemo for prolonged remission.
Definition
True
Term
T or F: Indolent lymphoma, CLL, and multiple myeloma are given chemo despite a lack of cure.
Definition
True
Term
T or F: diseases which are given chemo for cure are CML, lymphoma, leukemia, and pediatric tumors.
Definition
True
Term
Which of the following are given immunotherapy - CML or Hairy cell leukemia?
Definition
Both
Term
All chemo drugs that start with the letter __ cause pulmonary problems.
Definition
B
Term
Cyclophosphamide, ifosfomide, busulfan, and chlorambucil are all alkylating agents what is their mechanism and clinical use?
Definition
They cross link DNA, causing cell death and a toxicity of hemorrhagic cystitis, for NHL
Term
Cisplatin and carboplatin are similar to alkylating agents but what is their unique toxicity?
Definition
Oto/nephrotoxic
Term
What is the mechanism of action and clinical use of antibiotic neoplastics like doxorubicin and dactinomycin?
Definition
They intercalate DNA and inhib topoisomerase, and they are used for Hogdkins and childhood tumors respectively
Term
The plant alkyloid chemos like vincristine and vinblastine have mechanism and use?
Definition
They block microtubules (vines=vin) and treat hogdkins but have neurotoxicity
Term
Etoposide and teniposide have what mechanism since these epipodophyllotoxins can cause leukemogenesis via chrome 11?
Definition
They inhibit topoisomerase2 and decrease DNA in G2
Term
How is the chemo taxane Paclitaxel similar to vincristine in mechanism?
Definition
It prevents microtubule disassembly versus preventing formation
Term
Methotrexate is a famous antimetabolite inhibiting DNA synthesis by inhibiting a folate reductase enzyme, but what are its unique toxicities and what drug is used for rescue from overdose?
Definition
Tox- myelo, mucositis, pulmonary; salvageable via leucovorin
Term
How is 5-flurouracil similar to methotrexate in mechanism so that they work synergistically?
Definition
They both inhibit thymidine synthesis; it has a unique tox of GI and mucosal
Term
6-MP and Cytarabine both inhibit DNA synthesis making them effective _________ chemo drugs.
Definition
Antimetabolite
Term
What is the purpose of hormonal agents like tamoxifen, prednisone, corticos?
Definition
Trigger intracellular events like apop, and are immunosuppressive.
Term
What is the leading caude of cancer in both new cases and death for men and women?
Definition
Cause in men is prostate, women is breast, both deaths are lung cancer
Term
At what age should would get breat exams annually?
Definition
40
Term
At what age should people get colon cancer screenings involving fecal occult blood and barium enema every five years with colonoscopy every 10?
Definition
50
Term
What are the strategies for cervical cancer screening?
Definition
After age 21, annual exam, after 30 with 3 normal paps you can screen every 2-3 years, DES exposure every year
Term
T or F: Tumor staging involves T for size, N for regional lymph node involvement, and M for level of metastasis, while path staging is given a lower case P to indicate.
Definition
True, however, leukemia, lymphoma, CNS, and peds tumors aren’t staged this way
Term
What is the definition of a complete chemo response versus a parital one, and what is the overall response rate?
Definition
Complete is disappearance of all lesions, parital >30-50% decrease from baseline; overall is complete plus partial; stable disease meets neither complete or partial criteria
Term
What is the role of the targeted therapy Trastuzumab in breast cancer?
Definition
Used in Her2 overexpressed patients, it is an anti-HER2 antibody
Term
In assessing prognostic factors for aggressive NHL what does APLES stand for?
Definition
Age greater than 60, Performance status, Lactact Dehydrogenase elevation, Extranodal sites greater than 1, Staging
Term
What disease has Owl’s eye cells and popcorn nuclei on biopsy?
Definition
Hogdkin’s lymphoma
Term
On biopsy, if there are large, non-cleaved cells called centroblasts what is the diagnosis?
Definition
Follicular lymphoma
Term
T or F: The prognostic risk factors for Follicular Lymphoma are age higher than 60, stage 3/4, LDH higher than normal, Hb<12, number of nodal areas >4.
Definition
true
Term
What is the treatment for asymptomatic patients with NHL?
Definition
Watch and wait until symptoms or blood work changes
Term
What is the difference between autologous, allogeneic, or syngeneic stem cell transplant?
Definition
Auto is cells derived from you, matched or mismatched relative or unrelated matched donor, and syn is from an identical twin
Term
Which class of HLA antigens, derived from the short arm of chrome 6, are important in transplantation tolerance?
Definition
I and II.
Term
There is a 20-30% chance that a patient will have a HLA matched sibling for stem cell transplant, so what are the advantages of allogeneic transplant?
Definition
Donors available immediately, and the beneficial graft vs. tumor effect
Term
The HLA class 1 genes are expressed in all tissues but where are HLA 2 expressed?
Definition
B-cells and monocytes
Term
Stem cells harvested from bone marrow (less GVHD) are used in __________ and those gained from peripheral blood (higher GVHD) are used it ____________.
Definition
Children, adults
Term
T or F: unbilical chord blood is used for patients who need a rapid transfusion and have a lower incidence of GVHD.
Definition
true
Term
T or F: Acute GVHD: occurs in first 3 months following SCT primarily affecting Skin, gut, liver occurring most often with neutrophils.
Definition
True, treatment is immunosuppression to inhibit cytokine storm from damaged tissues during transplant.
Term
What causes chronic GVHD?
Definition
Damage to the thymus during chemo prevents negative selection of new T-cells from donor causing autoimmune like symptoms.
Term
Chemo drugs called 5-azacytine and decitabine are hypomethylating agents, what is each drugs mechanism of action?
Definition
Both are incorporated into DNA during replication causing demethylation causing cell differentiation or apoptosis.
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