leukemia_06-07.ppt
TRANSCRIPT
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LEUKEMIALEUKEMIA
Masatoshi Kida, M.D.Dept. of Pathology
University of Vermont
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““leukoproliferative disorders”leukoproliferative disorders”
lymphoid lymphoid neoplasms acute
chronic
myeloidmyeloid neoplasms acute
chronic
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Lymphoid NeoplasmsLymphoid Neoplasms
general aspects of lymphoid neoplasms1. histologic examination is required for diagnosis
2. wide range of behavior
3. majority are of B-cell origin (80-85%)
4. disruption of normal immune regulatory mechanisms
5. deriving from a single transformed cell (monoclonal)
6. homing to and growing in the areas of origin
7. tends to spread through lymphatics and peripheral blood to distant lymphoid tissue
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Acute LeukemiaAcute Leukemia
• monoclonal proliferation of immature “blast” cells that fail to participate in the normal maturation process
• as the cells accumulate, they spill over into the peripheral blood
• morbidity and death are rarely caused by leukemic cells
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Acute LeukemiaAcute Leukemia
general overview• abrupt stormy onset• depression of marrow function
– fatigue anemia– fever, infection WBC– bleeding platelet
• bone pain and tenderness marrow expansion
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Acute LeukemiaAcute Leukemia
- generalized lymphadenopathysplenomegaly ALL > AMLhepatomegaly
- testicular involvement ALL > AML
- CNS involvement ALL > AMLheadachevomitingnerve palsie
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Acute Lymphocytic Acute Lymphocytic Leukemia/Lymphoma (ALL)Leukemia/Lymphoma (ALL)
• 40% of acute leukemia• children, young adults• most frequent malignancy of childhood• 80% of childhood acute leukemia• 50% of cancer deaths in children <15 y/o• white : non-white = 2 : 1• male > female
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Acute Lymphocytic Acute Lymphocytic Leukemia/Lymphoma (ALL)Leukemia/Lymphoma (ALL)
pre B-cell
pre T-cellNull cell
80-85%
15% <5%
child ALL: almost curable
adult ALL: recent improvement in prognosis, but still with significant mortality
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Acute Lymphocytic Acute Lymphocytic Leukemia/Lymphoma (ALL)Leukemia/Lymphoma (ALL)
• BM failure (pallor, lethargy, abnormal bleeding)• bone & joint pain• peripheral lymphadenopathy• splenomegaly• CNS involvement
CSF : increased pressure and cellularity
decreased glucose
normal protein
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Acute Lymphocytic Acute Lymphocytic Leukemia/Lymphoma (ALL)Leukemia/Lymphoma (ALL)
• Peroxidase-granule negative ( AML)• terminal deoxynucleotidyl transferase (TdT)• common acute lymphoblastic leukemia antigen (CALLA)• T-cell antigen (T) --- mostly negative• surface Ig (sIg) --- mostly negative• cytoplasmic Ig (cIg)• PAS-pos cytoplasmic aggregate
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ALL ALL peripheral blood smearperipheral blood smear
• normocytic, normochromic anemia• thrombocytopenia• WBC may be low, normal, or high
acid phosphatase PAS ++
peripheral B-cell
pre B-cellpre T-cell
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ALL ALL clinical courseclinical course
• Remission in the majority of children• 5 yrs disease free period in 50% of cases• complete cures possible• significant recent improvement in survival rate for T-cell type
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ALL ALL prognostic factorsprognostic factors
good bad
age 2-10 y/o <1 y/o; adult
sex female male
WBC >50,000 blast/L
phenotype early precursor B-cell mature B-cell, null cell
cytogenetics high hyperploidy pseudo-diploid
t(9;22) 3% of pediatric ALL 25% of adult ALL
t(8;14)
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““leukoproliferative disorders”leukoproliferative disorders”
lymphoid lymphoid neoplasms acute
chronic
myeloidmyeloid neoplasms acute
chronic
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[Lymphoproliferative Disorders][Lymphoproliferative Disorders]
• clonal expansions of antigen stimulated lymphocytes• maturation arrest due to molecular derangements• primarily B cell origin• may be induced by chromosomal translocations, retrovirus,
or oncogene activation
1. chronic lymphocytic leukemia (CLL)
2. hairy cell leukemia
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Chronic Lymphocytic Leukemia (CLL)Chronic Lymphocytic Leukemia (CLL)
• most common form of leukemia in North America and Northern Europe• essentially identical to small lymphocytic lymphoma (SLL)• M > F (2 : 1)• elderly (>60 y/o)• considered incurable• mostly asymptomatic• hepatosplenomegaly may be present• peripheral lymphocytosis (>200,000)• increased susceptibility to bacterial infection (most frequent cause of
death)• may associated with autoimmune hemolytic anemia
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Chronic Lymphocytic Leukemia (CLL)Chronic Lymphocytic Leukemia (CLL)
• Indolent clinical course• median survival : 4-6 yrs• occasional transformation to large non-Hodgkin’s lymphoma (Richter’s
syndrome) --- 3 to 5 %
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Hairy Cell LeukemiaHairy Cell Leukemia
uncommon variant of peripheral B-cell neoplasmclinically middle age to elderly (younger than CLL)
splenic red pulp involvement
histologically lymphocyte with finger-like projections
phenotypically TRAP (Tartrate Resistant Acid Phosphatase)
CD19, CD20
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Hairy Cell Leukemia Hairy Cell Leukemia clinicalclinical
• M > F (3-5 : 1)• splenic red pulp involvement red pulp “lake”• tends to follow an indolent course
• pancytopenia - most prominent feature
- granulocytopenia recurrent bacterial infection
- anemia fatigue
- thrombocytopenia bleeding• good response to some chemotherapy regimen
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