acute leukemia.ppt

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    Essentials of Diagnosis

    Short duration of symptoms, including

    fatigue, fever, and bleeding.

    Cytopenias or pancytopenia.

    More than 20% blasts in the bone marrow.

    Blasts in peripheral blood in 0% of

    patients.

    Classify as acute myeloid leu!emia "#M$or acute lymphoblastic leu!emia "#$$.

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    General Considerations

    #cute leu!emia is a hematopoieticprogenitor cell malignancy.

    &ncontrolled proliferation cells and replace

    normal bone marrow elements. #rise with no clear cause. 'owever,

    radiation and some to(ins "ben)ene areleu!emogenic. # number of

    chemotherapeutic agents "especiallycyclophosphamide, melphalan, otheral!ylating agents, and etoposide maycause leu!emia.

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    General Considerations

    Most of the clinical findings in acute

    leu!emia are due to replacement of

    normal bone marrow elements by the

    malignant cell. $ess common manifestations result from

    organ infiltration "s!in, gastrointestinal

    tract, meninges. #cute leu!emia is potentially curable

    with combination chemotherapy.

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    General Considerations

    #$$ comprises *0% of the acute leu!emias

    of childhood. +he pea! incidence is

    between and - years of age. t is also

    seen in adults, causing appro(imately 20%of adult acute leu!emias.

    #cute myeloid leu!emia "#M$ is primarily

    an adult disease with a median age

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    Symptoms and Signs

    Bleeding "usually due to thrombocytopenia /

    S!in

    Mucosal surfaces

    inggival bleeding

    1pista(is

    Menorrhagia

    $ess commonly, widespread bleeding isseen in patients with disseminated

    intravascular coagulation "C.

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    Symptoms and Signs

    nfection is due to neutropenia "falls below

    3004mc$. +he most common pathogens

    are gram5negative bacteria (Escherichia

    coli, Klebsiella, Pseudomonas or fungi"Candida, Aspergillus. Common

    presentations include cellulitis, pneumonia,

    and perirectal infections6 death within a few

    hours may occur if treatment withappropriate antibiotics is delayed.

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    Symptoms and Signs

    7atients may also see! medical attention / um hypertrophy Bone and 8oint pain

    +he most dramatic presentation ishyperleu!ocytosis, in which a mar!edlyelevated circulating blast count "usually 9200,0004mc$ leads to impaired circulation /

    'eadache Confusion yspnea

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    Laboratory Findings

    +he hallmar! of acute leu!emia is the

    combination of pancytopenia with

    circulating blasts

    'owever, blasts may be absent from theperipheral smear in as many as :0% of

    cases ";aleu!emic leu!emia;.

    +he bone marrow is usually hypercellular

    and dominated by blasts. More than 20%

    blasts are re

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    Laboratory Findings

    'yperuricemia may be seen. f C is present / +he fibrinogen level will be reduced +he prothrombin time prolonged =ibrin degradation products or fibrin 5dimers present.

    7atients with #$$ "especially + cell may have amediastinal mass visible on chest radiograph.Meningeal leu!emia will have blasts present in thespinal fluid, seen in appro(imately 3% of cases atdiagnosis6 it is more common in monocytic types of#M$.

    +he #uer rod, an eosinophilic needle5li!e inclusion inthe cytoplasm, is pathognomonic of #M$

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    Laboratory Findings

    #M$ classification by ;=#B,; "=rench,#merican, British was based of morphologyand histochemistry /

    #cute undifferentiated leu!emia "M0

    #cute myeloblastic leu!emia "M: #cute myeloblastic leu!emia withdifferentiation "M2

    #cute promyelocytic leu!emia "#7$ "M #cute myelomonocytic leu!emia "M> #cute monoblastic leu!emia "M3 1rythroleu!emia "M? Mega!aryoblastic leu!emia "M-

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    Differential Diagnosis

    #M$ must be distinguished from othermyeloproliferative disorders /

    Chronic myeloid leu!emia Myelodysplastic syndromes.

    #$$ must be separated from otherlymphoproliferative disease such as /

    Chronic lymphocytic leu!emia $ymphomas

    'airy cell leu!emia. #typical lymphocytosis of mononucleosis and

    pertussis.

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    Treatment

    +he first step in treatment is to obtain

    complete remission /

    normal peripheral blood with resolution of

    cytopenias normal bone marrow with no e(cess blasts

    normal clinical status.

    +he type of initial chemotherapy dependson the subtype of leu!emia.

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    AML

    #M$ are treated with a combination / anthracycline "daunorubicin or idarubicin plus cytarabine, either alone or in combination with other

    agents.

    +his therapy will produce complete remission in *0%of patients under age ?0 years and in 30@?0% ofolder patients.

    #7$ is treated differently from other forms of #M$.nduction therapy should include anthracycline plusall5trans5retinoic acid.

    Aith this approach 0@3% of patients will achievecomplete remission.

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    ALL

    #dults with #$$ are treated / aunorubicin incristine

    7rednisone #sparaginase +his treatment produces complete

    remissions in 0% of patients.

    emission induction therapy for #$$ is lessmyelosuppressive than treatment for #M$and does not necessarily produce marrowaplasia

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    Prognosis

    #ppro(imately -0@*0% of adults with #M$ under age ?0years achieve complete remission. 'igh5dose postremission chemotherapy leads to cure in

    3@>0% of these patients, and high5dose cytarabine hasbeen shown to be superior to therapy with lower doses.

    #llogeneic bone marrow transplantation is curative in 30@

    ?0% of cases. #utologous bone marrow transplantation may be superior

    to nonablative chemotherapy. Dlder adults with #M$ achieve complete remission in up

    to 30% of instances.

    +he cure rates for older patients with #M$ have been verylow "appro(imately :0@:3% even if they achieveremission and are able to receive postremissionchemotherapy.