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48 Cards in this Set
- Front
- Back
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define: leukemia
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- malignancies of either lymphoid or hamatopoietic origin
- usually see increased circulating leukocytes |
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where does leukemia spread?
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- infiltration of leukemic cells in the liver, spleen, lymph nodes, and other organs is common
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what are the two acute leukemias?
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1. Acute lymphoblastic leukemia
2. Acute myeloid leukemia |
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what are general characteristics of acute leukemias
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- predominance of blasts in BM and peripheral blood
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define: blasts
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- immature blood cells
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what is the epidemiology of acute leukemias?
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- occur most often in children
- second peak incidence after 60 |
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what are the cytogenetic abnormalities seen in acute leukemias?
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- 9:22 translocation -> philadelphia chromosome (Ph1)
- Ph1 is usually associated with chronic myelogenous leukemia. if you see it in acute leukemias, it is indicative of a poorer prognosis |
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what is the progression of acute leukemias without intervention?
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- anemia, infection, and hemorrhage
- death within 6-12 months |
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what is the incidence of ALL?
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- most common malignancy of children
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what do you see in the circulating blood and BM in ALL?
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- predominance of lymphoblasts
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which acute leukemia is most responsive to therapy?
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- ALL
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how do you classify ALL?
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- classification is based on differences in morphology, cytogenetic changes, antigenic cell surface markers, or rearrangemnet of the Ig heavy cains or T cell receptor genes
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which form of ALL is most receptive to therapy?
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- blast cells that are positive for the CD10 marker
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what is the incidence of AML?
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- occurs most often in adults
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what do you see in circulating blood and bone marrow in AML?
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- predominance of myeloblasts and early promyelocytes
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define: myeloblast
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a precursor of leukocytes that normally occurs only in bone marrow
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how do you classify AML?
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- classification based on morphology, cytochemical characteristics, surface markers, genetic alterations
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what characterizes chronic leukemias?
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- proliferations of lymphoid or hematopoietic cells that are more mature than those of the acute leukemias
- have longer, less devastating clinical course, but are less responsive to intervention |
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list the chronic leukemias
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1. Chronic lymphocytic leukemia
2. Hairy cell leukemia 3. Chronic meylogenous leukemia |
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what are the general considerations for chronic lymphocytic leukemia?
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- CLL is characterized by proliferation of neoplastic lymphoid cells (almost always B cells)
- infiltration of BM, peripheral blood, lymph nodes, spleen, and liver |
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what is different about the proliferative potential of CLL cells?
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- less capable of differentiating into antibody producing plasma cells
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what is the epidemiology of CLL?
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- most often occurs in people > 60 years of age
- more frequent in men |
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what are the characteristics of leukemic cells in CLL?
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- look like normal mature peripheral blood lymphocytes
- express surface Igs and pan-B-cell markers like CD19, and CD20 - CD5 positive - CD10 negative |
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what is different about the leukemic cells in CLL?
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- more susceptible to mechanical damage
- often appear on the peripheral blood smear as 'smudge cells' |
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where do you see smudge cells?
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- in chronic lymphocytic leukemia (CLL)
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what is the peripheral WBC count in CLL?
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- 50,000/ul - 200,000/ul
- preponderance of leukemic cells |
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where do leukemic cells spread in CLL?
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- diffusely infiltrate the BM
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what are complications associated with CLL?
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- warm antibody autoimmune hemolytic anemia
- hypogammaglobulinemia (occurs early in CLL) |
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what are the clinical features of CLL?
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- indolent, often with few symptoms and minor disability for a long time
- will get generalized lymphadenopathy and moderate hepatosplenomegaly - mean survival is 3-7 years - treatment relieves symptoms, but has little effect on overall survival |
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what is Hairy cell leukemia?
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- B cell disease where leukemic cells have hair-like filamentous projections
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how do you identify the afflicted leukemic cells in hairly cell leukemia?
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- look for positive staining for tartrate resistant acid phosphatase (TRAP)
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what is the epidemiology of hairy cell leukemia?
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- affects middle aged men that present with splenomegaly and pancytopenia
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how do you treat hairy cell leukemia
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- dramatic response to several agents:
- a-interferon - 2-chlorodeoxyadenosine - deoxycoformycin |
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what is chronic myelogenous leukemia?
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- neoplastic clonal proliferation of myeloid stem cells
- it is one of the myeloproliferative syndromes |
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what are myeloid stem cells?
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- the precursor cell of erythrocytes, granulocytes, monocytes, and platelets
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what are molecular changes seen in CML?
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- reciprocal translocation between chr 9 and 22 (Philadelphia chr)
- found in all blood cell lineages, but not in the majority of CIRCULATING B or T lymphocytes |
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what is the philadelphia chromosome?
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- represents a remnant of chr 22 and a small segment of 9
- found in all blood cell lineages, but not in the majority of CIRCULATING B or T lymphocytes |
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what derives from a blood cell lineage?
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- erythroblasts
- granulocytes - monocytes -megakaryocytes - B and T cell progenitors |
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describe in more detail, the translocation seen in CML
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- c-abl proto-oncogene on chr 9 is transposed to an area on chr 22 adjacent to an oncogene (bcr)-> forms hybrid bcr-abl
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what does bcr stand for?
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- breakpoint cluster region
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what does bcr-abl code for?
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- protein p210 with tyrosine kinase activity
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what do you see in the peripheral blood smear of CML patients?
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- marked leukocytosis with WBC counts from 50,000/ul to 200,000/ul
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what types of cells do you see in CML, and where are they located?
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- leukemic cells are seen in the peripheral blood and BM, mainly middle-to-late myeloid (granulocytic) precursor cells (myelocytes, metamyelocytes, bands, segmented forms)
- few blasts and promyelocytes |
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where do you find the philadelphia chromosome in CML?
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- in granulocytic and erythroid precursor cells and in megakaryocytes
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what do you see a reduction of in leukemic leukocytes of CML patients?
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- reduction in leukocyte alkaline phosphatase activity
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what are the clinical features of CML?
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- SPLENOMEGALY
- modestly enlarged liver and lymph nodes |
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what is the progression of CML?
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- terminates in an accelerated phase leading to blast crisis (increased primitive blast cells adn promyelocytes)
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what is the epidemiology of CML?
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- peak incidence at middle age (35-50)
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