lymphoma2018 200204215635

U LYMPHO ÁC TÍNH


ALL

CLL

Lymphomas

MM

naïve

B-lymphocytes
Lymphoid
progenitor

AML
Hematopoietic
stem cell

Myeloid
progenitor

Plasma
cells
T-lymphocytes

Myeloproliferative disorders
Neutrophils
Eosinophils

Basophils
Monocytes
Platelets
Red cells


1. Bãûnh hc
a. L bãûnh ạc tênh dng lympho, phạt sinh
tổỡ caùc g/ õoaỷn muọỹn trong q/trỗnh bióỷt
hoaù lympho bỗnh thỉåìng.
b. Âàûc âiãøm gii pháùu v chỉïc nàng
+ U lympho tãú bo trỉåíng thnh cọ âàûc
tênh lan to, kh nàng tàng sinh tháúp. Tãú
bo ạc tênh thỉåìng lan ra cạc vng xa,
âãún tu xỉång. Tãú bo tàng sinh cháûm
v cọ thãø âỉåüc khäúng chãú båíi cạc cå
chãú âiãưu ho tàng trổồớng bỗnh thổồỡng.
+ ULPKH tóỳ baỡo lồùn hồn vaỡ ờt trổồớng
thaỡnh thổồỡng cheỡn eùp tọứ chổùc lympho
bỗnh thổồỡng do tng sinh cao v tãú bo
thỉåìng lỉu hnh trong mạu.
+ U lympho tãú bo B trỉåíng thnh cọ thãø
tảo ra cạc paraprotein. U lympho tãú baìo T


c. Dỉûa vo âàûc âiãøm täø chỉïc (nang/
näút hồûc lan to) v loải tãú bo (tãú
bo nh, trỉåíng thnh cọ hồûc khäng
cọ nhán ch hồûc tãú bo låïn cọ nhán
“chuøn dảng”) âãø phán loải u lympho

thnh cạc thãø cọ âäü aùc tờnh thỏỳp, trung
bỗnh vaỡ cao theo tióỳn trióứn lỏm sng nãúu
khäng âiãưu trë.
+ Ung thỉ tiãưm áøn gäưm cạc tãú bo nh,
trỉåíng thnh v thỉåìng åí dảng nang.
Nhán tỉång âäúi nh, cháút nhiãùm sàõc
tảo củc v lỉåüng bo tỉång êt.
+ Ung thỉ táún cäng cọ tênh lan to. Tãú
bo låïn hồûc êt biãût hoạ.


d. Tiãún triãøn vãư täø chỉïc hc
+ Mäüt säú bãûnh nhán ULPKH âäü ạc tênh
tháúp s tiãún triãøn âãún u lympho âäü ạc
tênh trung gian hồûc cao tuy váùn mang
cạc dáúu áún ca âäü ạc tênh tháúp.
+ U lympho âäü ạc tênh cao, sau khi âiãưu trë
cọ thãø tråí thnh u cọ âäü ạc tênh tháúp.
Âiãưu ny cọ l phn ạnh sỉû “lnh
bãûnh” ca qưn thãø tãú bo to.


d. Tiãún triãøn vãư täø chỉïc hc
+ Mäüt säú bãûnh nhán ULPKH âäü ạc tênh
tháúp s tiãún triãøn âãún u lympho âäü ạc
tênh trung gian hồûc cao tuy váùn mang
cạc dáúu áún ca âäü ạc tênh tháúp.
+ U lympho âäü ạc tênh cao, sau khi âiãưu trë
cọ thãø tråí thnh u cọ âäü ạc tênh tháúp.
Âiãưu ny cọ l phn ạnh sỉû “lnh

bãûnh” ca qưn thãø tãú bo to.


Bãûnh Hodgkin

U lympho khäng Hodgkin

- Lan trn tráût tỉû theo
hãû bảch huút
- Hiãúm gàûp täøn thỉång
ngoi hảch
- Triãûu chỉïng ton thãø
v cọ giạ trë tiãn lỉåüng
- Täøn thỉång hảch
trung tám v hảch theo
trủc
- Biãøu hiãûn lám sng
giäúng nhau. Âạp ỉïng
täút våïi trë liãûu åí mi
thãø v mi giai âoản

- Lan trn ngáùu nhiãn,
cọ l qua âỉåìng mạu
- Cọ täøn thỉång ngoi
hảch
- Êt gàûp triãûu chỉïng
ton thãø
- Thỉåìng täøn thỉång
hảch ngoải vi, hảch
mảc treo v mạu.

- Lám sng âa dảng.
Âạp ỉïng âiãưu trë täút åí
bãûnh nhán cọ âäü ạc
tênh tháúp.


2. Di truưn

Âàûc trỉng l chuøn âoản t(14;18)


HL incidence according to geography

Zdroj: UK Cancer Research


Incidence of the HL according to age (UK)

Zdroj: UK Cancer Research


Incidence of NHL by geography

Source: UK Cancer Research


Incidence of NHL by age (UK)

Zdroj: UK Cancer Research

Malignant lymphoproliferation
Def: clonal disease of lymphocyte
in various stage of onthogenesis
WHO 1999:
Precursor

ALL (B,T)

Peripheral

LBL

NHL

HL

HL: Hodgkin´s lymphoma, NHL: nonhodgkin´s lymphoma, ALL: acute lymphoblastic
leukemia, LBL: lymphoblastic lymphoma


Lymphoma Classification
• Revised European-American Lymphoma (REAL):
– (1990s) the REAL classification, based on immunophenotypic
and genetic features, identifying distinct clinicopathologic
NHL entities
• World Health Organization (WHO):
– (2001) classification, based on the major 3 cell types
• WHO:
– (Sep 2008) revised classification became available

• 30 mature B-cell lymphoma subtypes
• 21 T- and NK-cell lymphoma subtypes
• 9 precursor B-cell lymphomas/leukemias
• 5 types of Hodgkin’s lymphoma

Jaffe et al. Blood. 2008;112:4384-4399; Vardiman et al. Blood. 2009;114: 937-951.


Updated WHO Classification: 2008
B-cell neoplasms
I. Precursor B-cell neoplasm: precursor B-acute lymphoblastic
leukemia/lymphoblastic lymphoma (LBL).
II. Peripheral (“Mature”) B-cell neoplasms.
A. B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma.
B. B-cell prolymphocytic leukemia.
C. Lymphoplasmacytic lymphoma/immunocytoma.
D. MCL.
E. FL.
F. Extranodal marginal zone B-cell lymphoma of mucosa-associated
lymphatic tissue (MALT) type.
G. Nodal marginal zone B-cell lymphoma ( monocytoid B-cells).
H. Splenic marginal zone lymphoma ( villous lymphocytes).
I. Hairy cell leukemia.
J. Plasmacytoma/plasma cell myeloma.
K. DLBCL.
L. Burkitt lymphoma.
Jaffe et al. Blood. 2008;112:4384-4399; Vardiman et al. Blood. 2009;114:937-951.


Updated WHO Classification: 2008 (cont’d)

T-cell and putative NK-cell neoplasms
I. Precursor T-cell neoplasm: precursor T-acute lymphoblastic leukemia/LBL.
II. Peripheral T-cell and NK-cell neoplasms.
A. T-cell chronic lymphocytic leukemia/prolymphocytic leukemia.
B. T-cell granular lymphocytic leukemia.
C. Mycosis fungoides/Sézary syndrome.
D. Peripheral T-cell lymphoma, not otherwise characterized.
E. Hepatosplenic gamma/delta T-cell lymphoma.
F. Subcutaneous panniculitis-like T-cell lymphoma.
G. Angioimmunoblastic T-cell lymphoma.
H. Extranodal T-/NK-cell lymphoma, nasal type.
I. Enteropathy-associated T-cell lymphoma.
J. Adult T-cell lymphoma/leukemia (human T-lymphotrophic virus [HTLV]
1+).
K. Anaplastic large cell lymphoma, ALK+.
L. Anaplastic large cell lymphoma, ALK-.
M. Aggressive NK-cell leukemia.
Jaffe et al. Blood. 2008;112:4384-4399.


HL - histologic subtypes WHO classification
1. Nodular sclerosis: HL 50-80%
2. Mixed cellularity: HL 20-30%

3. Classic HL lymphocyte rich
4. Classic HL, lymphocyte depletion
5. Nodular HL lymphocyte predominant
(paragranuloma)

WHO Classification of NHL by Disease Course
Indolent
A. FL (follicular small cleaved cell [Grade 1],
follicular mixed small cleaved and large cell
[Grade 2], diffuse small cleaved cell)
B. CLL/SLL
C. LL (Waldenström’s macroglobulinemia)
D. Extranodal marginal zone B-cell lymphoma
(MALT lymphoma)
E. Nodal marginal zone B-cell lymphoma
(monocytoid B-cell lymphoma)
F. Splenic marginal zone lymphoma (splenic
lymphoma with villous lymphocytes)
G. Hairy cell leukemia
H. Mycosis fungoides/Sézary syndrome (CTCL)
I. T-cell granular lymphocytic leukemia
J. Primary cutaneous anaplastic large cell
lymphoma/lymphomatoid papulosis (CD30+)
K. Nodular lymphocyte predominant Hodgkin's
lymphoma

Aggressive
A. Diffuse large cell lymphoma (includes diffuse mixed
cell, diffuse large cell, immunoblastic,
T-cell rich large B-cell lymphoma)
B. Burkitt lymphoma/Burkitt cell leukemia/Burkittlike lymphoma
C. Precursor B- or T-cell lymphoblastic
lymphoma/leukemia
D. Primary CNS lymphoma
E. Adult T-cell leukemia/lymphoma (HTLV 1+)

F. MCL
G. Polymorphic posttransplantation
lymphoproliferative disorder (PTLD)
H. AIDS-related lymphoma
I. True histiocytic lymphoma
J. Primary effusion lymphoma
K. Aggressive NK-cell leukemia/blastic NK-cell
lymphoma

L. B- or T-cell prolymphocytic leukemia

CTCL=cutaneous T-cell lymphoma; CNS=central nervous system.
Jakić-Razumović et al. Croat Med J. 2002;43:527-534; DeVita et al, eds. Cancer Principles and Practice of Oncology. 8th ed.
Philadelphia, PA: Lippincott Williams & Wilkins;2008.


3. Bãûnh nguyãn
a. Nhiãùm virus
+ Virus HTLV1 (human T lymphotrophic virus 1)
cọ liãn quan tåïi u lympho v leukemia cáúp
lympho T.
+ EBV coï liãn quan tåïi u lympho Burkitt.
b. Räúi loản miãùn dëch
Suy gim miãùn dëch báøm sinh v màõc
phi: nguy cå màõc u lympho cao v tiãún
triãøn thỉåìng nhanh.
c. Di truưn: Nguy cå màõc u lympho cao åí
mäüt säú gia õỗnh hoỷc ồớ anh em sinh õọi.
d. Hoaù chỏỳt trỉì sáu, nhán thåm (Benzen) v
cháút âäüc mu da cam.

3. Lám sng
a. Thỉåìng gàûp mäüt hồûc vi hảch låïn,
khäng âau, åí cäø, bẻn v nạch.
b. Täøn thỉång ngoi hảch (vng Waldayer,
âỉåìng tiãu hoạ, da, tuún nỉåïc bt,
xỉång) åí âäü aïc tênh trung gian vaì cao.
c. Cháøn âoaïn caïc giai âoản mün bàịng
chủp X quang läưng ngỉûc, sinh thiãút tu
xỉång v gan, mäø bủng thàm d.


Classical Hodgkin Lymphoma


How does lymphoma present?
• Patient notices lumps in
neck, under arms, in
groin (lymphadenopathy)
• Lymphadenopathy noted
during examination for
other reason eg. check up
Abnormal blood findings
unusual (cf. leukaemia)