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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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