SRI KARTIKA SARI

Penyakit limfoproliferatif
maligna
 Konsep dasar :
 Proliferasi sel bersifat neoplastik
 Proliferasi maligna bersifat “klonal” timbul dari tipe
sel tunggal
 Sel limfoid dpt dikenali dgn petanda tertentu sbg T, B
atau nol
 Sel2 B mempunyai imunoglobulin permukaan.
Sel T menarik eritrosit domba in vitro dgn
pembentukan
E rossete.
Sel nol kekurangan petanda B dan T yg biasa
 Salah satu konsep dari limfoma - - - → sel yg
predominan menunjukkan tahap dlm diferensiasi
atau maturasi limfosit
 Sel2 predominan tdk selalu mirip dgn morfologi
limfosit normal - - → mengalami
transformasi
 Penyakit sel B yg tersering dan menunjukkan derajat
deferensiasi/transformasi - - → menimbulkan
leukemia limfositik kronik, sbg besar limfoma Hodgkin
dan penyakit imunoproliferatif.
 Bbrp menunjukkan patologik dan klinik cukup jelas
Leukemia limfositik kronik
( CLL )
25% kasus leukemia
Imunitas humoral dan seluler 
Leukemia limfositik kronik ( CLL
)Klinis :
 Pembesaran kelenjar limfe superfisial, simetris, terpisah
 Splenomegali, hepatomegali
 Anemia , pucat , dyspneu
 Bila disertai trombositopenia dapat terjadi perdarahan abnormal
 Leukositosis ( sebagian besar bervariasi antara
30.000– 300.000 / cmm dan di darah tepi 70 – 99% limfosit matur )

 Anemia normokrom normositer. Anemia hemolitik dgn coomb

positif (10-20%)
 Trombositopenia ( sering dijumpai )
 Sutul : 25 – 95% limfosit
Sedian apus
darah : sel kecil dengan kromatin inti padat yg menggumpal
 Sel
dan sitoplasma yg sempit disekitarnya
 Sel sel lebih besar dgn sitoplasma lebih jernih
 Sel basket atau smear----inti limfosit telanjang
Stadium CLL
 0 : Limfositosis absolut :  15.000 / cmm
 I : Stadium 0 + pembesaran kelenjar limfe
 II : Stadium I + hepatosplenomegali
 III : Stadium II + anemia ( Hb  11 g / dl )
 IV : Stadium III + trombositopenia (
100.000 /
cmm )
Prognosi
s
 Bervariasi
Stage dan respon pengobatan mrpkn indikator
prognostik
 Prognosis scr umum - - → lebih baik , lebih dr
separuh bisa bertahan hidup > 5 th
Haematopoietic
Malignancies
Myeloproliferative Malignant
diseases Leukaemias lymphomas

chronic myeloid Acute myeloid Hodgkin’s

leukaemia leukaemia
(CML) (AML) lymphoma
Polycythemia Chronic myeloid Non-
vera leukaemia hodgkin’s
(PV) (CML) lymphoma
(NHL)
Idiopathic Acute lymphatic
myelofibrosis leukaemia Burkitt's lymphoma
(MF) (ALL)
cutaneous T-cell
Essential Chronic lymphatic lymphoma (CTCL)
thrombocythemia leukaemia
(ET) (CLL)

hairy cell
leukaemia
(HCL)
Haematopoietic
Malignancies
Myeloproliferative Malignant
Leukaemias
diseases lymphomas
 Family of chronic  Neoplastic disease  Neoplastic
neoplastic of a disease of
diseases haematopoietic lymphatic tissue
 Due to a clonal precursor cell  Originates in
disorder arising  Characterised by lymph node
at the level of replacement of or spleen
the pluripotent normal bone  Hodgkin’s (15%)
stem cell marrow
 non-Hodgkin’s
 Characterised by  Often infiltration
(85%)
abnormal into other
proliferation of 1 organs
or more blood  Malignant clones
cell lines suppress normal
cell formation
Hairy – Cell
Leukemia
 Keganasan limfoid B atipik (jarang)
 Patologi :
 Sel patognomonik - - → k a r a k t e r i s t i k
limfositik
 Infiltrasi - - - → sutul, limpa dan hati
 Fgs sutul terganggu
 Hipersplenism
hematolog
i
 Sitopenia / pasitopenia
Sediaan apus darah : sel-sel berambut
 Sutul : sering kering (dry tap)
 Biokimiawi :
Kadar lisosim serum normal /rendah
Gambaran
klinik
 Penyakit di usia pertengahan dan lanjut
 Pria lebih banyak
 Splenomegali
 Hepatomegali
 Jarang limfadenopati
prognosi
sAngka harapan hidup : sekitar 3 tahun
Limfoma ( Limfoma
maligna )
 Jaringan limfoid normal diganti jaringan
limfoid abnormal

 Limfoma Hodgkin
khas : sel Reed Sternberg
 Limfoma non Hodgkin
noduler / difus

 Diagnosa pasti limfoma dengan

pemeriksaan jaringan ( patologi
anatomi )
The
Lymphati
c System
Lymphatic
Tissue
 Lymph nodes, spleen, liver, skin and the
respiratory, GI and GTU tract
 Lymphocytes undergo further proliferation
and differentiation in lymphoid tissue
 B-lymphocytes
 tend to reside in lymph nodes & spleen
 T-lymphocytes
 tend to circulate throughout the lymphatic system
Lymph Node - normal
histology
afferent lymphatic vessel capsule

follicle (mainly B-
cells)
- germinal centre
- mantle zone

artery

efferent lymphatic vessel

vein
LIMFOMA
HODGKIN
 Etiologi : tdk diketahui
 Patologi :
 Proliferasi selular heterogen
 Sel patognomonik : sel Reed-Sternberg - - → sel
besar
dgn 2 inti serta nukleoli yg besar
 Gangguan imunitas seluler
Gambaran klinis limfoma Hodgkin
 Semua umur ( anak jarang, dewasa muda lebih
sering )
 Pria  wanita
 Pembesaran kelenjar limfe :
tidak nyeri , tidak lunak, asimetris
dan terpisah
leher : 60 – 70% kasus
aksila : 10 – 15%
kasus inguinal : 6 – 12 %
kasus retroperitoneal :
sering mediastinum : 6 –
11 %
 Anemia normokrom normositer ( sering )
 Bila infiltrasi di sutul ( + ) dapat sebabkan kegaglan
sutul disertai gambaran anemia dengan lekoeritroblas
 Lekositosis : 1/3 kasus
 Lekocyte alkaline phosphatase ( ALP ) : 
 Eosinofilia
 Stadium lanjut : limfopenia
 Jumlah trombosit: permulaan normal, pada stadium
lanjut
dapat 
 LED 
 Hiperurikemia
 Cell mediated immunity dan antibodi  mudah
infeksi
Hodgkin’s
Lymphoma
 15% of lymphomas
 First described by Thomas Hodgkin in 1832
 Originally had a very poor
prognosis (<10% survival at 5
years)
 Improved staging techniques and understanding
of the pattern of spread helps direct
management
 Now curable in over 70% of cases through the
use of radiotherapy and chemotherapy
Non-Hodgkin’s Lymphoma
(NHL):
Definition and Indication
A heterogeneous group of B- and T-cell
malignancies that are diverse in cellular origin,
morphology, cytogenetic abnormalities, response
to treatment, and prognosis
Non-Hodgkin’s Lymphoma
(NHL)
 85% of lymphomas
 6th major cause of cancer deaths yearly
Heterogeneous group of malignant diseases
arising from lymphoid tissue
 lymph nodes, spleen
 Various immune cell types
 principally B-cells
derivation (>85%)
 T-cells derivation
 Histiocytes (very rarely)
 Various stages of
differentiation and
NHL
Incidence
 Incidence of 13.3/100,000 per year (Aust)
 Predominates in the 40-70 years age group
 most common neoplasm in the 20-40 age
group
 Incidence is rising
 150% growth over the past 30 years
 increasing by 4% annually since 1970’s
 Mortality rate is also rising
 2% rise per year
 third highest rise, exceeded only by lung cancer in women
and malignant melanoma
NHL
Incidence
 Increases with age
 implications
 Slight male
predominance
overall
 Striking male
predominance for
several subtypes
 Incidence of certain subtypes varies greatly
around the world
 Burkitt’s Lymphoma in African children