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Hairycellleukemiapast,present,furturebyYehonghui1Hairycellleukemiapast,prese1Introduce?extremelyrareformofleukemia

middleagedmen?

?pancytopeniaandsplenomegaly

?longlifespan

2Introduce?extremelyrareform2historyofHCL(1923-1953)?Edwardin1923describedsplenomegalywithoutlymphadenopathypancytopeniawithlymphocytosisandmonocytopenia

?Gosselinin1944-19533distinctivesubtypebonelesioncutaneousmanifestation

3historyofHCL(1923-1953)?Edw3444555historyofHCL(1958-1974)?namedashairycellleukemia

microscopicsign?

medianmaturelyphocyte

cytoplasmpseudopodsprotruding

serratedborder

?lymphoproliferativedisorder

6historyofHCL(1958-1974)?nam6777?ACPandTRAP

?biopsy

?elctronmicroscope8?ACPandTRAP?biopsy8BonemarrowbiopsyinHCLreticulinstain9BonemarrowbiopsyinHCLreti9101010?predictclinicoutcome

?splenectomy67%remainedHCRafter6month5yearsOS61%

?chlorambucil

11?predictclinicoutcome?spl11in80s?interferon-alfa

300mu/m2

3timeperweekandlastedforoneyear

side-effect

2-4-8

ORR70%CR8%

12in80s?interferon-alfa12in90s

?AetiologyHTLVEBVHPV-B+5del(5q13)

?OriginofHCCD19+CD20+CD22+SIg+

CD10-

PCA-1

?ScretionTNF-alfaIL-613

in90s?AetiologyHTLV13in90s?purinenucleosideanalogs

?Pentostatin4mg/m2/2Wtotal8times

ORR79%CR76%

?Cladribine0.1mg/Kg/dayfor7days

ORR97%CR85%

?notidenticaltherapy

14in90s?purinenucleosideanal14151515Cladribine:recurrencerate26%,mediantime29months

Sideeffect:progressivelyworseresponsecumulativemyelotoxiceffectsecondtumor16Cladribine:recurrencerate16InTheNewEra?Multi-coloredFlowCytometry

?GenemutationBRAF-MEK-ERKpathway

?Immunotherapyortargetedtherapy

17InTheNewEra?Multi-colored17181818ExpertconsensusondiagnosisofBcellchroniclymphoproligerativedisordersinChina201419Expertconsensusondiagnosis19202020Hairycellleukemiapast,present,furturebyYehonghui1Hairycellleukemiapast,prese21Introduce?extremelyrareformofleukemia

middleagedmen?

?pancytopeniaandsplenomegaly

?longlifespan

2Introduce?extremelyrareform22historyofHCL(1923-1953)?Edwardin1923describedsplenomegalywithoutlymphadenopathypancytopeniawithlymphocytosisandmonocytopenia

?Gosselinin1944-19533distinctivesubtypebonelesioncutaneousmanifestation

3historyofHCL(1923-1953)?Edw2344245525historyofHCL(1958-1974)?namedashairycellleukemia

microscopicsign?

medianmaturelyphocyte

cytoplasmpseudopodsprotruding

serratedborder

?lymphoproliferativedisorder

6historyofHCL(1958-1974)?nam267727?ACPandTRAP

?biopsy

?elctronmicroscope8?ACPandTRAP?biopsy28BonemarrowbiopsyinHCLreticulinstain9BonemarrowbiopsyinHCLreti29101030?predictclinicoutcome

?splenectomy67%remainedHCRafter6month5yearsOS61%

?chlorambucil

11?predictclinicoutcome?spl31in80s?interferon-alfa

300mu/m2

3timeperweekandlastedforoneyear

side-effect

2-4-8

ORR70%CR8%

12in80s?interferon-alfa32in90s

?AetiologyHTLVEBVHPV-B+5del(5q13)

?OriginofHCCD19+CD20+CD22+SIg+

CD10-

PCA-1

?ScretionTNF-alfaIL-613

in90s?AetiologyHTLV33in90s?purinenucleosideanalogs

?Pentostatin4mg/m2/2Wtotal8times

ORR79%CR76%

?Cladribine0.1mg/Kg/dayfor7days

ORR97%CR85%

?notidenticaltherapy

14in90s?purinenucleosideanal34151535Cladribine:recurrencerate26%,mediantime29months

Sideeffect:progressivelyworseresponsecumulativemyelotoxiceffectsecondtumor16Cladribine:recurrencerate36InTheNewEra?Multi-coloredFlowCytometry

?GenemutationBRAF-

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