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136 Cards in this Set
- Front
- Back
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Clinical triad of leukemia
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1) Anemia (from lack of RBC production)
2) Infection (lack of normal WBCs) 3) Bleeding/bruising (lack of platelets) |
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4 types of leukemia
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1) Acute myeloblastic leukemia (AML)
2) Chronic myelogenous leukemia (CML) 3) Chronic lymphocytic leukemia (CLL) 4) Acute lymphoblastic leukemia (ALL) |
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rapidly progressing diseases that result from the accumulation of immature, functionless blast cells in bone marrow and blood
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acute leukemia
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T/F Acute leukemias frequently respond to chemo and other forms of treatment
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T
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T/F Chronic leukemias are usually not cured with chemo
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T
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___ is primarily a pediatric disease while _____ is primarily adults
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ALL; AML
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Hematopoietic transcription factors are disrupted in _____ leukemia
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acute
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Lymphoblasts in peripheral blood with increased serum LDH and uric acid are characteristic of what?
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ALL
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Acute promyelocytic leukemia associated with
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DIC
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Signs and Symptoms of acute myeloid leukemia
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Fever
Gingival hyperplasia Gingival bleeding Epistaxis Infections Weakness, Fatigue Bone pain Menorrhagia Extramedullary masses (monoblastic leuk) Ecchymoses |
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Risk factors for acute myeloid leukemia
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Down syndrome
Prior chemo Prior radiation chemical exposure Aplastic anemia Myelodysplastic syndrome Paroxysmal nocturnal hemoglobinuria |
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Signs and Symptoms of ALL
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Headache
Nosebleed Gingival bleeding Mediastinal mass Fever Weakness, Fatigue Infections bone pain Testicular enlargement Menorrhagia Ecchymosis |
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clumps of azurophilic granular material that form elongated needles seen in the cytoplasm of leukemic blasts
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auer rods
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in what diseases can auer rods be seen?
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acute leukemia
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chromosomal translocations resulting in specific fusion genes are a hallmark of
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leukemia
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best for identifying specific chromosomal abnormalities
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FISH
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Cytochemistry (chem stains for cell enzymes)
What enzymes will be seen in A) AML cells B) ALL cells |
A) Myeloperoxidase
B) Terminal deoxytransferase (TdT) |
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ALL cells are positive for _____ while AML cells are positive for _________
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TdT; Myeloperoxidase
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What antigens are characteristics of T cells?
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CD2 and CD3
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What antigens are characteristics of B cells?
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CD10,19,20
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What antigens are characteristics of myeloid cells?
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CD13 and CD33
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What antigen is characteristics of stem cells, found in almost all cases of acute leukemia?
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CD34
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85% of ALLs have the immunophenotype of immature ___ cells
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B
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Burkitt leukemia/lymphoma is what type of leukemia?
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ALL
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Criteria for leukemia diagnosis
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>30% blast cells in bone marrow or peripheral blood
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Burkitt lymphoma cells contain what translocation?
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t(8;14)
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Hyperdiploidy (>50 chromosomes) is a (good, poor) prognostic sign
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good
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Philadelphia chromosomes (t(9;22))is a (good, poor) prognostic sign in ALL
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poor
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5 year survival rate for kids with ALL
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80%
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Classic triad of profound anemia, leukopenia, and thrombocytopenia is characteristic of ____
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AML
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term used for cells normally found in the hypergranular form of acute promyelocytic leukemia (FAB - M3). This term is applied to these blast cells because of the presence of numerous Auer rods in the cytoplasm. The accumulation of these Auer rods gives the appearance of a bundle of sticks
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Faggot cell
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Characteristics of acute promyelocytic leukemia (APL), a type of AML
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Proliferation of malignant promyelocytes.
Cells contain multiple Auer rods (faggot cells). <b>Associated with DIC</b> |
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Retinoic acid receptor gene dysfunction is a characteristic of ___
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Acute Promyelocytic Leukemia
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What is pathognomonic for AML?
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Auer rod, comprised of primary granules, appears as small red rod in cytoplasm of myeloblast.
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In flow cytometry, cells of AML will be (positive, negative) for B and T lineage markers
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negative
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CML; Polycythemia vera; essential thrombocytopenia; and myelofibrosis are all what?
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Chronic myeloproliferative disorders
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What are the stages of CML?
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1) Chronic/stable phase
2) Accelerated phase 3) Blast phase 4) Spent phase |
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Clinical presentation of CML
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Insidious onset, non-specific findings. Often discovered incidentally.
Fever and night sweats, progressive and massive splenomegaly |
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What is the spent phase of CML?
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Marrow becomes fibrotic, patient becomes pancytopenic because normal marrow elements are pushed out of the bone marrow.
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Lab features of CML?
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Increased basophils, high platelet count. Hypercellular bone marrow due to proliferation of myeloid cells.
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What is the leukemoid reaction?
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Body's response to infection is to increase WBCs from the bone marrow, sometimes a bit too premature.
The peripheral blood picture might look like CML. Lab test Leukocyte Alkaline phosphatase (LAP) will help distinguish. |
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what is the Leukocyte Alkaline phosphatase (LAP) test?
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Helps distinguish between CML and a leukemoid reaction to infection.
Neoplastic granulocytes of CML have NO LAP. Reactive neutrophils, however, do. |
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Molecular hallmark of CML
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Philadelphia chromosome
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What is the oncogene encoded protein product in CML?
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type of tyrosine kinase that initiates transformation of the stem cell to a leukemic cell
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Chemo for CML
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hydroxyurea, alkalating agents, interferon alpha
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Best tx for CML
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HSCT
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Clinical picture of Polycythemia vera
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Insidious onset, very high Hb and hct.
Plethora, erythromelalgia, headache and dizziness Thrombotic tendencies. Splenomegaly, hepatomegaly. |
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About 90% of polycythemia vera patients ahve a mutation in _____
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JAK2
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Disease primarily involving the megakaryocytic cell line
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Essential thrombocythemia
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Characterized by collagen deposition in bone marrow, squeezing out normal hematopoietic precursors
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Primary myelofibrosis
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Disease in which TEARDROP shaped cells are seen in peripheral blood. And why?
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Primary myelofibrosis. This is because cells are squeezing through the reticulin (collagen) fibers
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Hairy cell leukemia, mantle cell lymphoma, and CLL are all what?
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several chronic clonal lymphoproliferative diseases with elevated lymphocyte count
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Malignant clonal disorder of relatively mature, indolent B cells (95%) or T cell (5%)
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CLL
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Pathogenesis of CLL
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It depresses immunoglobulin levels, impairing humoral immunity.
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Clinical features of CLL
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Generally discovered during routine peripheral blood exam; generalizd nontender adenopathy and splenomegaly.
Will see complications of pancytopenia, including hemorrhage and infection. |
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Lab features in CLL
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Absolute lymphocytosis. Small lymphocytse (appear relatively mature). Will see <b>smudge cells</b> due to CLL cells being more fragile and easily damaged during prep.
Also express CD5. Will weakly express kappa or lambda Ig. |
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In what disorder are smudge cells commonly seen?
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CLL
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What is a common cytogenetic abnormality in CLL?
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Trisomy 12
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What is Richter's syndrome?
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Transformation of CLL to Large Cell Lymphoma in a minority, and it is a poor prognostic indicator.
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Plasma cell myeloma is aka
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multiple myeloma
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cancer of plasma cells (b cell differentiated enough to secrete and Ig - either kappa or lambda)
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Myeloma
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Paraproteins: defn
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The monoclonal Igs produced from Myeloma cells
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Clinical presentation of multiple myeloma
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Some are asymptomatic at presentation, first detected on routine chem panel.
Many patients become anemic from decreased RBC production, develop bone fractures from tumorous masses and lytic lesions to the bone, complain of bone pain, or develop renal failure from effect of the protein on the kidney. Production of normal Ig is usually suppressed, leading to susceptibility to infection. Also hypercalcemia from increase bone resorption |
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Monoclonal protein in urine
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Bence-Jones protein
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Lab features of multiple myeloma
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Demonstration of paraprotein on electrophoresis.
M spike in Ig region of the gell. Immunofixation electrophoresis confirms monoclonality of the band and to determine its composition. Increased numberso f plasma cells May see rouleaux formation - phenomenon whereby red cells are sticking to each other like stacks of coins |
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Why does the rouleaux formation occur?
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Because disruption of neg electrical charge that keeps proteins apart is disrupted
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T/F Neoplastic plasma cells can be seen in the blood of patients with multiple myeloma
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F. Only if VERY advanced (plasma cell leukemia)
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Tx for multiple myeloma
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localized radiotherapy and chemotherapy for older patients.
Not treated for early disease, but carefully monitored. Bone marrow transplant for younger patients. Median survival is around 3 years |
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What is amyloidosis?
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group of diseases characterized by abnormal production of proteins deposited in tissues as beta-pleated sheets. Amyloid infiltration causes tissue dysfunction and eventual organ failure.
Presence of amyloid can be demonstrated in tissue by staining with Congo red. |
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3 major types of amyloid
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1) AL amyloid: Ig light chains, or a portion of the light chain molecule.
2) AA amyloid - derived from serum amyloid-A related protein, acute phase reactant synthesized in increased quantities in patients with chronic infectious and inflam disorders. 3) Familial amyloidosis |
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How are lymph nodes organizd?
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Into B and T cell subcompartments.
B cells: cortex; T cell: paracortex |
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B cells are in the (paracortex, cortex) while T cells are in the (paracortex, cortex)
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cortex; paracortex
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The Reed-Sternberg cell is associated with
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Hogkin lymphoma
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T/F Lymphomas are clonal
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T. Clonal expansions of a single functional subpopulation
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Tumor of small skin based T cells (mostly CD4+) that occurs in adults
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Mycosis Fungoides
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Tumor in which lymphocytes are small and have a characteristic folded, cerebriform nucleus
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Mycosis Fungoides
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usually a nodal disease of adults, emcompasses a variety of morphological variants
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Peripheral T- cell Lymphoma
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Tumor demonstrating phenotype of loss or aberrant expression of pan T-cell antigens
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Peripheral T Cell Lymphoma
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______ typically effaces nodal architecture, forming a mass of characteristic reactive inflammatory cells with admixed smaller population of large dysplastic RS cells
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Hodgkins Lymphoma
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Characteristics immunophenotype of RS cells
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CD15+, CD30+, CD45-
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4 types of HL
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1) Nodular sclerosis
2) Lymphocyte predominance 3) Mixed cellularity 4) Lymphocyte depleted |
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Most common type of HL
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nodular sclerosis
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Clinical/Epidemiological Characteristics of the nodular sclerosis type of HL
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Most common type
Affects females more than males Occurs most often in young adults Patients usually present with cervical adenopathy and/or mediastinal mass |
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Histological Characteristics of the nodular sclerosis type of HL
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Cpasular fibrosis, dense bands of CT giving nodular appearance.
Have a lacunar appearance where cytoplasm retracts against the nucleus (looks like a hole) |
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Clinical/Epidemiological Characteristics of the mixed cellularity type of HL
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Symptomatic middle-aged to elderly male
Fever, weight loss, night sweats |
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Histological Characteristics of the mixed cellularity type of HL
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Nodal architecture effaced with numerous inflammatory cells and readily identifiable RS-cells including diagnostic binucleate forms.
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Characteristics of the lymphocyte depletion type of HL
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Uncommon (<5%)
Most patients have fever and blood cytopenias. More abdominal adenopathy. Lymphocytes fewer in number. RS cells vary from diagnostic to bizarre, multinucleated cells with sarcomatous appearance. |
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What are the three types of hodgkins disease that are grouped together as "classical"?
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Nodular sclerosis, Lymphocyte depletion, Mixed cellularity
Because they have the characteristics immunophenotype CD45-, CD15+, CD30+ |
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Clinical Characteristics of the lymphocyte predominance type of HL
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10-15% of all HL.
Asymptomatic young males with cervical or axillary adenopathy. Excellent prognosis |
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Histological Characteristics of the lymphocyte predominance type of HL
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"popcorn cells" because RS cells are few in number and have a folded, vesicular nucleus with less prominent nucleoli
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Differential Dx of LAD
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1) Chronic inflammation
2) Lymphoma 3) Metastatic carcinoma |
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Hyperplasia of the lymph node follicles is seen in
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Rheumatoid arthritis and HIV
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Hyperplasia of the lymph node paracortical region is seen in
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viral illnesses
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Cortex is to ___ cells as paracortex is to ___ cells
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B; T
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Where are the histiocytes found in the lymph nodes?
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medullary sinuses
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T/F Small lymphocytes are better differentiated
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T
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Non hodkins lymphomas are broadly grouped into what?
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B or T cells types
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T/F In general, Non hodkins lymphomas (NHLs) in which the predominant cell is small and dormant-appearing tend to be more indolent than those in which the predominant cell is a transformed lymphocyte
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T
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B Cell NHLs comprise ____% of lymphomas
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85-90
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ALl B-NHLs demonstrate clonal rearrangement of __________ gene
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immunoglobulin gene
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Most common type of NHL
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Follicular lymphoma
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Characteristic immunophenotype of follicular lymphoma
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CD20+
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What is the translocation of follicular lymphomas (seen in 90%)?
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t(14;18)
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Follicular lymphoma can progress to __________
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diffuse large B cell lymphoma
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Overexpression of _____ in follicles prevents apoptosis. Seen in ALL follicular lymphomas
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bcl2
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Expands the mantle zone
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mantle cell lymphoma
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cyclin D
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Cell cycle regulator that allows cell to go from G1 --> S phase
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Cyclin D overexpression associated with what lymphoma?
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Mantle cell
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Two forms of Burkitt lymphoma
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African endemic (face)
Sporadic in US (generally abdomen) |
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Burkitt lymphoma is driven by translocation of ______
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C-Myc
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Morphology of burkitt lymphoma
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diffuse proliferation of small noncleaved cells with high mitotic rate, background of "Starry Sky" macrophages
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MALT Lymphoma: characteristics
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usually low-grade lymphomas of adults that arise in extranodal lymphoid tissue, especially in GI tract and lung.
May be associated with autoimmune disorder (hashimotor thyroiditis) or infection (H pylori assoicated) |
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Type of lymphoma associated with H pylori infection
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MALT lymphoma
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Diffuse large B cell lymphoma: characteristics
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Very aggressive, not well differentiated.
CD20+ |
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T/F in HL, Reed-Sternberg cells secrete cytokines that attract reactive cells, causing B symptoms (weight loss, night sweats)
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T
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HL with best prognosis
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Lymphocyte Rich
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HL with worst prognosis
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Lymphocyte poor, seen in elderly and HIV+
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(T, B) cell lymphomas more associated with paraneoplastic syndromes like hypercalcemia, hypergammaglobulinemia, etc
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T
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Osteoclast activating factor is associated with ___________
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multiple myeloma
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Neoplastic proliferation of plasma cells which produce various substances that cause problems
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multiple myeloma
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What is the M spike in serum protein electrophoresis in multiple myeloma due to?
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Increase Ig
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How are T Cell Lymphomas classified?
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According to anatomy compartment from which they arise.
1) Precursor T cell neoplasms: arise in marrow or thymus 2) Peripheral - arise in lymph nodes or extranodal site |
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often present as rapidly growing anterior mediastinal mass in young patients with respiratory problems. Disseminate rapidly.
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Precursor T-lymphoblastic Lymphoma
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T/F For B Cells, Each clone expresses only one antibody with either a kappa or lambda light chain
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T
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B cells
develop in BM, go through affinity maturation and somatic hypermutation in the ______ |
germinal center
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A) B cell component of spleen
B) Part of spleen that filters and contains macrophages |
A) White pulp
B) Red pulp |
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Re lymphomas,
Follicular tends to be (low, high) grade while vs. diffuse tends to be (low, high) grade. |
Low; High
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On What chromosome is the heavy chain locus?
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14
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Match the translocations with the lymphoma:
A) t(14;18) B) t(8;14) C) t(11;14) |
A) Follicular (Fourteen Follicular)
B) Burkitts C) Mantle |
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EBV associated with what lymphoma?
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Burkitt lymphoma
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type of cutaneous lymphoma where affected cells are T-cells that have pathological quantities of mucopolysaccharides.
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Sezary syndrome (associated with Mycosis fungoides )
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Increased small lymphocytes
Smudge Cells Neutropenia associated with what? |
CLL/SLL (Small cell lymphocytic lymphoma)
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CLL is the same disease as
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Small lymphocytic lymphoma (SLL)
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B cell antigens (CD19,CD20) and presence of a κ or λ chain are characteristic of what?
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NORMAL B cells
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B cell antigens (CD19,CD20), CD5, and presence of a κ or λ chain are characteristic of what?
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CLL
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Treatment options for CLL
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1. Watchful waiting
2. Chemotherapy (fludarabine, chlorambucil, combination chemo) 3. Splenectomy, radiotherapy 4. Bone marrow transplantation 5. Anti-CD52 (Alemtuzumab) |
