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Chronic Myeloid Leukemia.doc

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Chronic Myeloid Leukemia Also called: Chronic granulocytic leukemia, Chronic myelogenous leukemia, CML Share on facebook Share on twitter Bookmark & Share Printerfriendly !ersion Subscribe to SS Leukemia is cancer of the white blood cells# $hite blood cells hel% your body fight infection# &our blood cells form in your bone marrow# 'n leukemia, the bone marrow %roduces abnormal white blood cells# (hese cells crowd out the healthy blood cells, making it hard for blood to do its work# '
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  Chronic Myeloid Leukemia Also called: Chronic granulocytic leukemia, Chronic myelogenous leukemia, CML Share on facebook Share on twitter Bookmark & Share Printerfriendly !ersion Subscribe to SS Leukemia is cancer of the white blood cells# $hite blood cells hel% your body fight infection# our blood cells form in your bone marrow# 'n leukemia, the bone marrow  %roduces abnormal white blood cells# (hese cells crowd out the healthy blood cells, makingit hard for blood to do its work# 'n chronic myeloid leukemia )CML*, there are too many granulocytes, a ty%e of white blood cell#Most %eo%le with CML ha!e a gene mutation )change* called the Philadel%hia chromosome#Sometimes CML does not cause any sym%toms# 'f you ha!e sym%toms, they may include: ã +atigue ã $eight loss ã  ight sweats ã +e!er  ã Pain or a feeling of fullness below the ribs on the left side(ests that e-amine the blood and bone marrow diagnose CML# (reatments include chemothera%y, stem cell trans%lants, infusion of donated white blood cells following stem cell trans%lants, surgery to remo!e the s%leen, and biologic and targeted thera%ies# Biologic thera%y boosts your body.s own ability to fight cancer# (argeted thera%y uses substances that attack cancer cells without harming normal cells# '/: ational Cancer 'nstitute 0et Chronic Myeloid Leukemia u%dates by email   $hat.s this1  ã Start Here o Chronic Myelogenous Leukemia )P23*: (reatment)ational Cancer 'nstitute* o Also a!ailable in S%anish  o $hat 's Chronic Myeloid Leukemia1)American Cancer Society*  o Also a!ailable in S%anish  Patient information: Chronic myeloid leukemia (CML) in adults (Beyond the Basics) Authors obert S egrin, M2 Charles A Schiffer, M2Section 4ditor ichard A Larson, M22e%uty 4ditor ebecca + Connor, M2  Ad by BlockTheAdApp. More Info | Hide These Ads C/ 5'C M4L5'2 L4674M'A 584 8'4$Chronic myeloid leukemia )also called CML or chronic myelogenous leukemia* is a chronic )longterm* disorder of the bone marrow# Bone marrow is the s%ongy, red tissue that fills the large bones# All of the blood cells are %roduced in the bone marrow#Peo%le with CML ha!e ac9uired an abnormality that causes a section of one chromosome )a strand of genes* to break off and attach to another chromosome this results in an abnormally short chromosome, known as the Philadel%hia chromosome# (his e-change of genetic information causes two genes, BC and ABL, to fuse into one gene, called BC ABL#(he BC ABL gene causes bone marrow cells to %roduce an abnormal en;yme )the BC ABL tyrosine kinase* this en;yme stimulates white blood cells to grow out of control, resulting in ele!ations of the white blood cell count and an increase in the si;e of the s%leen# 4!entually, the disease can transform into a more aggressi!e disease, called acute leukemia#Peo%le with acute leukemia ha!e an increased number of immature white blood cells )called blast cells*# (he o!ergrowth of blast cells leads to an inade9uate number of mature white blood cells, which limits %roduction of other !ital blood cells, including red blood cells and %latelets# /a!ing a decreased number of blood cells and %latelets can increase the risk of de!elo%ing infections or bleeding e-cessi!ely#P/AS4S 5+ C/ 5'C M4L5'2 L4674M'A(here are three %hases of CML:Chronic %hase < 'n the chronic %hase, there are less than = %ercent immature blast cells in the bone marrow# A%%ro-imately >= %ercent of %eo%le are in the chronic %hase when they are initially diagnosed# (his %hase generally lasts se!eral years and is readily controlled with oral medications#  Accelerated %hase < 2uring the accelerated %hase, maturation of white blood cells  becomes %rogressi!ely im%aired, and there are between ?@ and ? %ercent blast cells in the  blood or bone marrow# (he number of abnormal cells in the body is more difficult to control with medications, likely because of new mutations that de!elo% in the blast cells#Blast %hase < 'n blast crisis )blast %hase*, there are more than @ to @ %ercent blast cells in the blood or bone marrow# Before recent ad!ances in treatment, blast crisis ty%ically occurred within four to fi!e years after diagnosis and was often unres%onsi!e to treatment#C/ 5'C M4L5'2 L4674M'A ( 4A(M4( 5P('5S(reatment decisions for %eo%le with chronic myeloid leukemia )CML* are com%le- due to the !ariety of a!ailable o%tions# Currently, the most fre9uently used treatment o%tions include:D2isease control with oral tyrosine kinase inhibitors )(7's* such as imatinib )brand name: 0lee!ec*, dasatinib )brand name: S%rycel*, or nilotinib )brand name: (asigna*DPotential cure with hemato%oietic cell trans%lantation )also called bone marrow trans%lantation*, usually after the disease sto%s res%onding or rela%ses during treatment witha (7'D(reatment to reduce sym%toms with chemothera%y )hydro-yurea, busulfan, or interferon al%ha with or without cytarabine*(he choice of thera%y de%ends u%on the %hase of CML, the a!ailability of a stem cell donor, the %atient.s candidacy for stem cell trans%lantation, and the %atient.s %reference# es%onse to treatment < (he %rimary goal of treatment is to reduce or eliminate the cells with the abnormal Philadel%hia chromosome# (his is measured as the cytogenetic response # Such treatment, if effecti!e, will also return the blood counts to normal# (his is measured as the hematologic response #$hile achie!ing a hematologic res%onse will reduce the se!erity of sym%toms associated with CML, %rogression to the accelerated or blast %hase will continue unless a cytogenetic res%onse is achie!ed# Achie!ing a hematologic res%onse is im%ortant, but does not ensure that the disease is ade9uately controlled#Another way to determine how well the disease is controlled is to %erform sensiti!e molecular testing# A %erson is said to ha!e a com%lete molecular response  when there is noe!idence of the BC ABL gene# (he goal of hemato%oietic cell trans%lantation is to achie!e this le!el of res%onse# A molecular res%onse is sometimes seen during longer term follow u% of %eo%le treated with (7's# Chemothera%y rarely, if e!er, %roduces such a res%onse#( 5S'4 7'AS4 '/'B'(5 S )(7's*  (he Philadel%hia chromosome, characteristic of chronic myeloid leukemia )CML*, gi!es rise to the formation of a uni9ue gene %roduct, an abnormal en;yme called the BC ABL tyrosine kinase# esearchers directed their efforts at de!elo%ing com%ounds that could selecti!ely inhibit this abnormal en;yme, resulting in the de!elo%ment of a class of medications known as tyrosine kinase inhibitors )(7's*# (7's slow or sto% the actions of BC ABL, which leads to the ra%id death of cells containing the abnormal Philadel%hia chromosome# ormal cells suffer fewer to-ic effects from (7's com%ared with traditional chemothera%y treatments#Although they ha!e not been %ro!en to cure the disease, (7's are able to achie!e longtermcontrol of CML in the maEority of %eo%le thus, they ha!e become the initial treatment of choice for almost all %eo%le who are newly diagnosed with CML# All of the a!ailable (7's are able to induce hematologic and cytogenetic res%onses in all stages of the disease F?G# As a result, a choice among these medications is usually based u%on the %atient.s medical history and the %otential side effects of each medication )table ?*#Many %rescri%tion and non%rescri%tion medications can interact with (7's, %otentially making the treatment less effecti!e or dangerously increasing the amount of drug in the  bloodstream# (wo non%rescri%tion medications that should be a!oided are acetamino%hen )brand name: (ylenol* and St# Hohn.s wort )also called hy%ericum %erforatum*# 0ra%efruit  Euice should also be a!oided#'matinib )brand name: 0lee!ec* < 'matinib mesylate is a (7' that can be used in %eo%le with all %hases of CML# 't is %ro!en to ha!e significant benefits# 5ne study com%aring imatinib !ersus interferon %lus cytarabine )a form of chemothera%y* for %eo%le with newly diagnosed, chronic %hase CML found that I %ercent of %eo%le who were gi!en imatinib had a com%lete hematologic res%onse rate, and IJ %ercent achie!ed a com%lete cytogenetic res%onse F?G#+urther followu% is needed to determine how long res%onses will last, although the rela%se rate has been remarkably low in %eo%le followed for se!en or more years who achie!ed a com%lete cytogenetic res%onse# At %resent, e-%erts recommend continuing imatinib treatment indefinitely because the disease recurs, often within months, in the maEority of  %eo%le who sto% taking it# Progression to blast crisis can occur des%ite imatinib treatment in %eo%le with accelerated %hase disease and in those who ac9uire new genetic mutations#(he medication should be taken by mouth once daily, with a meal and a large glass of water#'t is e-tremely im%ortant to take e!ery single scheduled dose of your imatinib# Ski%%ing  %ills can seriously Eeo%ardi;e your chances of ha!ing a good res%onse# 5ne study showed that you need to take o!er @ %ercent of your %ills to ha!e a chance of a sustained com%lete res%onse FKG# )See (reatment of chronic myeloid leukemia in chronic %hase after failure of initial thera%y, section on . esistance.#*

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Jul 23, 2017
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