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KEGANASAN DARAH
Prof. dr. Rismawati Yaswir, Sp.PK(K)
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KEGANASAN DARAH
I. KELAINAN MIELOPROLIFERATIF PROLIFERASI SEL-SEL MIELOID YANG TIDAK
TERKONTROL
1. AKUT : LEUKEMIA MIELOSITIK AKUT (LMA)2. KRONIS
LEUKEMIA MIELOSITIK KRONISPOLISITEMIA VERAMIELOFIBROSIS DENGAN METAPLASIA MIELOIDTROMBOSITOPENIA ESSENSIAL
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KEGANASAN DARAH
II. KELAINAN LIMFOPROLIFERATIF PROFILERASI SEL-SEL LIMFOID YANG TIDAK
TERKONTROL (LIMFOSIT SEL PLASMA)
1. LEUKEMIA LIMFOSTIK AKUT & KRONIS2. LIMFOMA PA3. DISKRASIA SEL PLASMA Ig ABNORMAL
– MIELOMA MULTIPLE– MAKROGLOBULINEMIA WALDENSTROM
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PEMATANGAN SEL
BLASTMIELO – MONO EOS, BAS, NEUT MONOSIT
MEGAKARIOBLAST - TROMBOSITRUBRILAST - ERITROSITLIMFOBLAST - LIMFOSIT
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ASAL SEL DARAHTEORI MONOFILETIK
SEL INDUK TOTIPOTEN
SEL INDUK PLURIPOTEN SEL INDUK PLURIPOTEN
MIELOID LIMFOID
GRAN MONO MEGAKAR ERITROID BURSA TIMUS
EOS NEUT BAS MONO TROMBO ERI LIMFOSIT B LIMFOSIT T
SEL PLASMA (Ig)
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ETIOLOGI
– BELUM PASTI– DUGAAN :
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LEUKEMIA AKUT
LEUKEMIA LIMFOSITIK AKUT (ALL) ALL : ANAK 75 %, USIA 3 – 4 TAHUN ORANG TUA > 40 %
FAB: LEUKEMIA AKUT
ANLL (LNLA)
ALL (LLA)
GAMBARAN KLINISPUCAT, LEMAH …O.K ANEMIADEMAM, INFEKSI …L.ABNORMALPERDARAHAN (GUSI, KULIT)
……TROMBOSITOPENIA KEGAGALAN SST
INFILTRASI NYERI TULANGORGANOMEGALI
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PEMERIKSAAN LABORATORIUM
DARAH TEPIHb, Ht, Jumlah EriLeukosit …AleukemikLeukosit N subleukemik
Leukosit Leukemik …. TrombositLED
SEDIAAN APUSE : Normositik Normokrom Eri Berinti (+)L : Hiatus Leukemicus (Blast > 50 %)Auer Rod (+) :
Mieloblast/Monoblast/Promielosit LMA (ANLL)…..(+)/(-) LLA Selalu (-)
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LEUKEMIA
PROLIFERASI SEL LEUKOPOETIK TAK TERKONTROL DALAM SUMSUM TULANG
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SUMSUM TULANG– HIPERSELULER– GBR MONOTON BLAST ...> 30 %– ERITROPETIK & TROMBOPOETIK TERTEKAN
MEMBEDAKAN : M DAN L SITOKIMIA
SITOKIMIA LGA LLA LMOA
PEROKSIDASE +/++ - +/-
SUDAN BLACK B +/++ - +/-
PERIODIK ACID SCHIFF
-/+ hls +ksr -
DIAGNOSIS * SUMSUM TULANG * SITOKIMIA
PEMERIKSAAN LABORATORIUM
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LEUKEMIA LIMFOSITIK AKUT (ALL)
KATEGORI KLASIFIKASI ALL
L1 UKURAN SEL KECIL, BLAST HOMOGEN BENTUK INTI TERATUR, BULAT ANAK INTI SAMAR/ TIDAK JELAS SITOPLASMA SEDIKIT
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KATAGORI KLASIFIKASI ALL
L2 UKURAN SEL BESAR, BLAST HETEROGEN BENTUK INTI TAK TERATUR,
MELEKUK ANAK INTI SATU/LEBIH JELAS/BESAR SITOPLASMA BANYAK
LEUKEMIA LIMFOSITIK AKUT (ALL)
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KATAGORI KLASIFIKASI ALL
L3UKURAN SEL BESAR,BLAST HOMOGENBENTUK INTI TERATUR,BULAT/LONJONGANAK INTI SATU/LEBIH, SANGAT JLSSITOPLASMA BANYAK BIRU,
BERVAKUOLE
LEUKEMIA LIMFOSITIK AKUT (ALL)
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PEMERIKSAAN LABOR PADA LEUKEMIA NON LIMFOBLASTIK
INSIDEN
LMA (ANLL) … DEWASA 80 %
GAMBARAN KLINIS• PUCAT, LEMAH …O.K ANEMIA• DEMAM, INFEKSI …L.ABNORMAL• PERDARAHAN (GUSI, KULIT) ……
TROMBOSITOPENIA KEGAGALAN SST INFILTRASI
• NYERI TULANG• ORGANOMEGALI
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• DARAH TEPI Hb, Ht, Jumlah Eri
Leukosit …Aleukemik
Leukosit N subleukemik
Leukosit Leukemik …. Trombosit LED
PEMERIKSAAN LABOR PADA LEUKEMIA NON LIMFOBLASTIK
• SEDIAAN APUS E : Normositik Normokrom Eri Berinti (+) L : Hiatus Leukemicus (Blast >
50 %)Auer Rod (+) :
Mieloblast/Monoblast/Promielosit
LMA (ANLL) …..(+)/(-)
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M1 = BLAST TANPA MATURASI BLAST >>>, PRO < 3 %
M2 = BLAST DENGAN MATURASI BLAST + PRO >>
M3 = PROMIELOSIT DENGAN GRANUL KASAR/HALUS, KADANG DITEMUKAN
MULTIPLE AUER ROD M4 = MIELO-MONOSITIK .. 4 : 1
AKUT NON LIMFOSITIK LEUKEMIA (ANLL …. LMA)
FAB MEMBAGI ATAS :
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AKUT NON LIMFOSITIK LEUKEMIA (ANLL …. LMA)
FAB MEMBAGI ATAS :
M5 = MONOSITIK
M5a : MONOBLAST >>>
M5b : PROMONO – MONOSIT >> M6 = ERITROLEUKEMIA
RUBRIBLAST + MIELOBLAST M7 = MEGAKARIOSITIK
MEGAKARIOSIT > 30 %
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AML M1
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LEUKEMIA MIELOBLASTIK AKUT (M1)(Sediaan apus darah tepi)
Auer body
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AML M2
Auer Rod
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Auer rod
LEUKEMIA MIELOBLASTIK AKUT (M2)(Sediaan apus sumsum tulang)
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AML M3
Auer Rods
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AML M4
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AML M5
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AML M6
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