Disorders of hematopoietic
and lymphoid systems
Department of pathology longjie
Lymphoma 淋巴瘤
Leukemia 白血病
Malignant Lymphoma
恶性淋巴瘤
What is malignant lymphoma?
Classification of ML
Malignant
lymphoma (ML)
In this group of lympho-proliferative
diseases,normal lymphoid tissue is
replaced by abnormal cells of lymphoid
origin forming solid malignant tumors of
the lymph nodes,
Hodgkin’s lymphoma,HL
Classification of ML
Non-Hodgkin’s lymphoma,NHL
Hodgkin’s lymphoma
1,Presence of the distinctive neoplastic R-S cells
and non-neoplastic cells usually greatly
outmumber the tumor cells.
2,Studies show that it is a tumor come of B
lymphomacytes.
Pathological change of HL
Generral features
Grossly
Microscopically
Classification
Clinical course
1,a painless enlargenment of the lymph nodes.
General features
3,Initially localized to a single peripheral lymph node
region but orderly spread by contiguity nodes.
4,Extranodal involvement uncommon.
2.More often in upper half of the body (cervical,
axillary,para-aortic).
Grossly
1,The affected lymph node are enlarged,with a smooth
surface,and the lymph node capsule is rarely breached,
2,Lymph nodes conglutinate to be a fixed,nodular mass.
3,Cut surface,white- gray,fishy,yellowish necrosis.
Microscopically
1,Normal construction of lymph nodes were distroied.
2,Characterized morphologically by the presence of
distinctive neoplastic giant cells called Reed-
Sternberg (RS) cells;
3,The RS cells admixed with a variable infiltrate of
reactive,nonmalignant inflammatory cells;
HL
R-S cell + reactive inflammatory cells + stroma
Majority minority
R-S cell
1.ranges in diameter from 20-50 μm;
2,abundant,slightly eosinophilic
cytoplasm;
3.multilobate nucleus (分叶核) or
being multinucleate(多核)
with large,round,prominent,
eosinophhilic nucleoli;
4,Nuclear membrane is distinct.
Classic R-S cell
Hodgkin cell
Variants of R-S cell
Lacunar cell
Popcorn cell
Polymorphic cell
R-S cell
Diagnostic R-S cell
Mirror image cell
1.Two mirror-image nuclei or nuclear lobes,
each comtaining a large,acidophilic
nucleolus surrounded by a distinctive
clear zone,
classic R-S cell
2,Are common in the mixed cellularity
subtype.
典型 RS细胞
lacunar cell
1,Large;
2,has a single hyperlobate nucleus with multiple
small nucleoli;
3,Abundant pale-staining cytoplasm;
4,In formalin-fixed tissue,the cytoplasm retracts,
giving appearance of cells lying in clear
spaces(lacunae).
陷窝细胞
popcorn cell
L&H cell
爆米花细胞
Have a delicate multilobed,puffy
nucleus that has been likened in
appearance to popcorn.
镜影细胞
爆米花细胞
陷窝细胞
霍奇金细胞
木乃伊细胞
classification
1,nodular sclerosis (结节硬化型 )
2,Mixed cellularity (混合细胞型 )
3,lymphocyte predominance(淋巴细胞为主型 )
4,lymhocyte depletion (淋巴细胞消减型 )
Nodular sclerosis HL
1.the presence of lacunar cell;
2.the presence in most cases of collagen bands
that divide the lymphoid tissue into circumscribed
nodules.
3.cellular infiltrate,varying proportions of Lc,
eosinophilic,histiocytes et.
4,common in women,good prognosis
结节硬化型
结节硬化型
陷窝细胞
Mixed-cellularity HL
1.most common form of HL>50y;
2,Male dorminance;
3.typical RS cells are plentiful
4.distinctive cell infiltrate,small Lc,eosinophils,
plasma cells,histiocytes.
混合细胞型
典型 RS细胞
混合细胞型
Lymphocyte predominance HL
1,large number of lymphocytes admixed with variable
number of histiocytes,often within large,poorly
defined nodules;
2,typical RS cells are difficult to find;
3,more common are,popcorn cell”(L&H cell);
4,Classification Nodular~
Diffused~ mixed HL
淋巴细胞为主型
Lymphocyte depletion
1.least common form of HL,<5%;
2.paucity of lymphocytes,relative abundance of RS
cells or their variants;
3.two forms,diffuse fibrosis
reticular variants
4,Poor prognosis.
淋巴细胞消减型
Staging of HL
stage Distribution of disease
I
II
III
IV
Involvement of a single lymph node region(I) or involvement of a single
exrralymphatic organ or tissue(IE)
Involvement of two or more lymph node region on the same side of the
diaphragm alone(II) or with involvement of limited contiguous
extralymphatic organs or tissue(IIE)
Involvement of lymph node regions on both sides of the
diaphragm(III),which may include the spleen(IIIs),limited contiguous
extralymphatic organs or site(IIIE),or both (IIIES)
Multiple or disseminated foci of involvement of one or more
extralymphatic organs or tissue with or without lymphatic
involvement
Clinical course
1,painless enlargement of the lymph nodes;
2,systemic complaints,fever,unexplained weight loss,
pruritus,anemia.
3,5-year survival rate:
stage I-A,II-A,close to 100%;
stage IV-A,IV-B,50% 5-year disease-free survival
non-Hodgkin’s lymphoma
1.arise usually in the lymph nodes(65%),less
frequently in the lymphoid tissue of parenchymal
organs(35%),
2,All variants have the potential for spread;
Leukemia-like picture;
3,originate in B cells,T cells,or histiocytes;
composed of cells that are arrested at
specific stages along their normal
differentiation pathways.
4,80-85%NHL B cell origin
5,haploid neoplastic tissues
Follicular architecture
Diffused architecture
NHL classification systems
1982 Working Formulation for clinical use(low,
intermediate,high-grade)
1992 Kiel (low,high-grade)
1994 REAL (B,T/NK,HD)
Now new classification of WHO (2001)
Formulated a widely accepted classification that
relies on a combination of clinical,morphologic,
phenotypic,and genotypic features.
Precursor B lymphoblastic leukemia/ lymphoma,( B-LBL/L) 前驱 B细胞性白血病 /淋巴瘤
B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma,( B-CLL/ SLL)
B细胞慢性淋巴细胞性白血病 /小淋巴细胞性淋巴瘤
Mentle cell lymphoma,( MCL ) 套细胞淋巴瘤
Follicular lymphoma,( FL ) 滤泡性淋巴瘤
Diffuse large B-cell lymphoma,(DLBCL) 弥漫大 B细胞性淋巴瘤
Extranodal marginal zone B-cell lymphoma of MALT type,( MALT)
黏膜相关淋巴组织型结外边缘区 B淋巴瘤
Nodal marginal zone B-cell lymphoma,淋巴结边缘区 B细胞淋巴瘤
Splenic marginal zone B-cell lymphoma,脾边缘区 B细胞淋巴瘤
Burkitt’s lymphoma,伯基特淋巴瘤
Lymphophasmacytic lymphoma,(LPL) 淋巴浆细胞性淋巴瘤
B-cell prolymphocytic leukemia,B-PLL B前淋巴细胞性白血病
Plasmacytoma / plasma cell myeloma,浆细胞瘤 /浆细胞骨髓瘤
B-NHL
Precursor T lymphoblastic leukemia/ lymphoma,( T-LBL/L) 前驱 T细胞性白血病 /淋巴瘤
T-cell large granular lymphocytic lymphocytic leukemia,( T-LGL)
T细胞大颗粒淋巴细胞性白血病
NK / T cell lymphoma,nasal and nasal type,( NK / TCL ) NK / T细胞 淋巴瘤,鼻和鼻型
Mycosis fungoides / Sezary syndrome,(MF/ SS) 蕈样肉芽肿 /赛塞利综合征
Angioimmunoblastic T-cell lymphoma,(AITCL) 血管免疫母细胞性 T淋巴瘤
Peripheral T cell lymphoma,unspecified,(PTCL) 周围 T细胞淋巴瘤,非特殊性
Adult T-cell leukemia / lymphoma,(ATLL) 成人 T细胞白血病 /淋巴瘤
Analpastic large cell lymphoma,(ALCL) 间变大细胞淋巴瘤
Subcutaneous panniculits like T-cell lymphoma,皮下脂膜炎样 T细胞淋巴瘤
Enteropathy type intestinal T-cell lymphoma,(ITCL) 肠病型肠道 T细胞 淋巴瘤
Heoatisokebuc γδT –cell lymphoma,肝脾 γδT细胞 淋巴瘤
T-cell prolymphocytic leukemia,(T-PLL ) T前淋巴细胞性白血病
T-NHL
淋巴瘤诊断常用标记
antibody,PCR,TCR
LCA BCL-2
L26( CD20) Ig-VDJ
UCHL1( CD45RO)
MAC387( CD68)
BerH2( CD30)
CD15
B-SLL/CLL
1,4% of all NHL
2,Affects persons >50y
3,Low grade
4,Has no follicular architecture
B-SLL/CLL
morphology
1,Cell,conpact,small,apparently
unstimulated lymphocytes
2,Dark-staining round nuclei,scanty
cytoplasm
3,Little variation in size
4,Rare mitotic figures
5,Usually bone marrow invovled
6,Leukemia – like picture
B-SLL/CLL
immunophenotype
1,B-cell markers (+):
CD19,CD20,CD23,sIg,κ/λ light chains
2,CD5(+)
3,monoclonality
B-SLL/CLL
Clinical features
1.generalized lymphadenopathy
2.mild to moderate enlargement of the
liver and spleen
3,Prolonged survival is usual
4,Autoimmune hemolytic anemia
小淋巴细胞性淋巴瘤
小淋巴细胞性淋巴瘤
Follicular
lymphoma
1,Characterized by a nodular or
follicular architecture
2,40% of adult NHLs in US
morphology
1,Low power,nodular appearance
2,Cells,resemble normal follicular
center cells,
centrocyte-like cells
centroblast-like cells
3,Mitoses are infrequent
4,Single necrotic cells (apoptosis) are
not seen.
Follicular
lymphoma
滤泡性淋巴瘤 (FL)
Centrocyte-like cells (CC)
1.slightly large than resting lymphocytes
2.cleaved nuclear, indentations and linear
infoldings
3,Chromatin is coarse,condensed; nucleoli
are indistinct.
中心细胞
Centroblast-like cells (CB)
1,3-4 times the size of resting lymphocytes;
2,Vesicular chromatin,several nucleoli,
modest amounts of cytoplasm;
3,Usually a minor component of the overall
cellularity in FL.
中心母细胞
immunophenotype
1,B-cell markers (+):
CD19,CD20,CD10,sIg,κ/λ light chains
2,Neoplastic cell,Bcl-2 (+)
Follicular
lymphoma
3,Translocation t(14;18)
Clinical features
1,Occur predominantly in older
persons (rarely <20y)
2,Affect Males and females equally
3,Painless lymphadenopathy,
4,Involvement,BM common;
extranodal sites uncommon;
5,Indolent course,not easily curable;
6,40%FL DLBCL
Follicular
lymphoma
DLBCL
1,B-cell phenotype,a diffuse
growth pattern,an aggressive
clinical history.
2,50% of all adult NHLs.
morphology
1,The nulei of the neoplastic B cell
are large (3-4 times the resting
lymphocyte)
2,Neoplastic cells resemble,
immunoblasts
centroblasts
DLBCL
Immunoblasts
1.have a round or multilobulated large
vesicular nucleus with one or two centrally
placed prominent nucleoli,
2.abundant cytoplasm,deeply staining,
pyroninophilic or clear
DLBCL(免疫母细胞 )
immunophenotype
1,B-cell markers (+):
CD19,CD20,CD79a,sIg,κ/λ
light chains
2,30%, T(14,18)
rearrangement of Bcl-2 gene
rearrangement of Bcl-6 gene
DLBCL
Clinical features
1,Mainly in older persons (rarely <20y);
15% of childhood lymphoma;
2,Rapidly enlarging,symptomatic
mass at a single nodal or extranodal
site;
3,Common involvement,
gastrointestinal tract,skin,
bone,brain.
4,Aggressive tumors
DLBCL
1,Endemic in some parts of Africa
and sporadic in other areas.
2,Has closed relationship with EBV.
Burkitt’s
lymphoma
morphology
Burkitt’s
lymphoma
1,Intermediate in size,round or oval
nuclei comtaining 2-5 nucleoli;
2,Moderate amount of faintly basophilic
or amphophilic cytoplasm;
3,High mitotic rate;
4.,starry sky” pattern,macrophages
with ingested nuclear debris.
immunophenotype
1,B-cell markers (+):
CD19,CD10,sIg,Burkitt’s lymphoma
Clinical features
1,Affect children or young adults,
30% of childhood NHLs in US;
2,Rarely arises in lymphnode:
3,high grade
Burkitt’s
lymphoma African,maxilla,mandible
involvement
North American,abdominal tumors
involvement
伯基特淋巴瘤
leukemia
Malignant neoplasms of the
hematopoietic stem cells characterized by
diffuse replaement of the bone marrow by
neoplastic cells.
leukemia
The leukemic cells can spill over
into the blood and infiltrate the
liver,spleen,lymph nodes and other
tissues throughout the body.
leukemia
1,Anemia
2,Infection
3,Hemorrhage
Classification of leukemia
Acute
Chronic
Lymphocytic (ALL)
Myelocytic (AML)
Lymphocytic (CLL)
Myelocytic (CML)
FAB classification
ALL(急淋)
FAB分类法, 依据骨髓和外周血涂片细胞形
态学和细胞化学而定。
L1:小细胞为主,预后好;
L2:细胞大小不等,20%大细胞,预后较
好;
L3:细胞大小一致,大细胞为主,预后较差。
FAB分型
M0 急性未分化型白血病
M1 急粒未成熟型(原粒 ≥ 90%)
M2 急粒部分成熟型(原粒 30~90%)
M3 急早幼粒
M4 急粒单(原始> 30%,单 20~80%,余粒
M5 急单
M6 急红白血病(红> 50%)
M7 急巨核细胞白血病
急性淋巴细胞性白血病
慢性淋巴细胞性白血病
急性粒细胞性白血病
慢性粒细胞性白血病