Leukemia 06 07
Transcript of Leukemia 06 07
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LEUKEMIALEUKEMIAMasatoshi Kida, M.D.
Dept. of Pathology
University of Vermont
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leukoproliferative disordersleukoproliferative disorderslymphoid lymphoid neoplasms acute
chronic
myeloid myeloid neoplasms acute
chronic
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Lym phoid Neoplas m sLym phoid Neoplas m sgeneral aspects of lymphoid neoplasms
1. 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 mechanisms5. 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 distantlymphoid tissue
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Ac ute Leuke m iaAc ute Leuke m iamonoclonal proliferation of immature blast cellsthat fail to participate in the normal maturationprocessas the cells accumulate, they spill over into theperipheral bloodmorbidity and death are rarely caused by leukemiccells
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Ac ute Leuke m iaAc ute Leuke m iageneral overview
abrupt stormy onset
depression of marrow function fatigue n anemia fever, infection n q WBC bleeding n q platelet
bone pain and tenderness n marrow expansion
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Ac ute Leuke m iaAc ute Leuke m ia- generalized lymphadenopathy
splenomegaly ALL > AML
hepatomegaly
- testicular involvement ALL > AML
- CNS involvement ALL > AMLheadachevomitingnerve palsie
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Ac ute Lym pho cy tic Ac ute Lym pho cy tic Leuke m ia/ Lym pho m a ( ALL)Leuke m ia/ Lym pho m a ( ALL)40% of acute leukemiachildren, young adults
most frequent malignancy of childhood80% of childhood acute leukemia50% of cancer deaths in children female
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Ac ute Lym pho cy tic Ac ute Lym pho cy tic Leuke m ia/ Lym pho m a ( ALL)Leuke m ia/ Lym pho m a ( ALL)
BM failure (pallor, lethargy, abnormal bleeding)bone & joint pain
peripheral lymphadenopathysplenomegalyCNS involvement
CSF : increased pressure and cellularitydecreased glucosenormal protein
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Ac ute Lym pho cy tic Ac ute Lym pho cy tic Leuke m ia/ Lym pho m a ( ALL)Leuke m ia/ Lym pho m a ( ALL)
Peroxidase-granule negative ( m AML)terminal deoxynucleotidyl transferase (TdT)
common acute lymphoblastic leukemia antigen (CALLA)T-cell antigen (T) --- mostly negativesurface Ig (sIg) --- mostly negativecytoplasmic Ig (cIg)
PAS-pos cytoplasmic aggregate
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ALL ALL peripheral blood s m ear peripheral blood s m ear normocytic, normochromic anemiathrombocytopenia
WBC may be low, normal, or high
a c idphosphatase PAS ++
peripheral B-cell
pre B-cellpre T-cell
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ALL ALL c lini c al c oursec lini c al c ourse R emission in the majority of children
5 yrs disease free period in 50% of cases
complete cures possiblesignificant recent improvement in survival rate for T-cell type
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leukoproliferative disordersleukoproliferative disorderslymphoid lymphoid neoplasms acute
chronic
myeloid myeloid neoplasms acute
chronic
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[Lym phoproliferative Disorders][Lym phoproliferative Disorders]
clonal expansions of antigen stimulated lymphocytesmaturation arrest due to molecular derangements
primarily B cell originmay be induced by chromosomal translocations, retrovirus,or oncogene activation
1. chronic lymphocytic leukemia (CLL)2. hairy cell leukemia
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Chroni c Lym pho cy tic Leuke m ia ( CLL)Chroni c Lym pho cy tic Leuke m ia ( CLL)
most common form of leukemia in North America and Northern Europeessentially identical to small lymphocytic lymphoma (SLL)M > F (2 : 1)elderly (>60 y/o)considered incurablemostly asymptomatichepatosplenomegaly may be presentperipheral lymphocytosis (>200,000)increased susceptibility to bacterial infection (most frequent cause of death)may associated with autoimmune hemolytic anemia
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Chroni c Lym pho cy tic Leuke m ia ( CLL)Chroni c Lym pho cy tic Leuke m ia ( CLL)
Indolent clinical coursemedian survival : 4-6 yrsoccasional transformation to large non-Hodgkins lymphoma ( R ichterssyndrome) --- 3 to 5 %
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Hair y Cell Leuke m iaHair y Cell Leuke m ia
uncommon variant of peripheral B-cell neoplasmclinically middle age to elderly (younger than CLL)
splenic red pulp involvement
histologically lymphocyte with finger-like projectionsphenotypically TR AP (Tartrate R esistant Acid Phosphatase)
CD19, CD20
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