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48 Cards in this Set

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