8年制病理学英文课件（2版）：12淋巴造血系统

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1、Chapter 12 The diseases of Hematopoietic and Lymphoid SystemLiu weipingDepartment of PathologyWest China School of MedicineSichuan UniversityCharacteristics The organs and tissues are not confined to a single anatomic site.The cells are constantly in“traffic”.The diseases often appear as systemic in

2、volvements.Diseases of hematopoitic and lymphoid system Red cell disorders White cell disorders*Bleeding disorders Disorders that affect the spleen and thymus White cell disorders Non-Neoplastic Disorders -Reactive lymphoid hyperplasia(lymphadenitis)-Reactive leukocytosis Neoplastic Disorders -lymph

3、omas -myeloid neoplasms -Histiocytic and dendritic-cell neoplasms Lymph nodeBenign lymphoid hyperplasiaBenign lymphoid hyperplasia Non-specific lymphadenitis -acute non-specific lymphadenitis -chronic non-specific lymphadenitis follicular hyperplasiaparafollicular hyperplasiasinus histiocytosis Dist

4、inctive infection Non-specific lymphadenitisTBTuberculosis lymph node Nonspecific inflammation distinctive infectionInfection Tumor metastatic tumorslymphoid neoplasmsLymphoproliferative disorders with unknown reasonLymphomas淋巴瘤淋巴瘤Lymphomas Definition Lymphocyte differentiation WHO Classification Cl

5、inical manifestation and staging Diagnosis Genitics Epidemiology流行病学流行病学 and etiology病因学病因学Lymphomas-Definition Clonal tumors of mature and immature lymphocyte including B-cells,T-cells and Natural Killer(NK)cells and so on.Malignant tumors of immune system.Involved organs and tissues:-Lymphoid orga

6、nsand tissues -Extranodal tissues -Bone marrow -Peripheral bloodLymphomas-Lymphocyte differentiationPhenotypic and genotypic changes in differentiation of B cells and T cells Hodgkin lymphoma,HL Non-Hodgkin lymphoma,NHL B-cell neoplasms T-cell and NK cell neoplasmsLymphomas-WHO Classification Lympho

7、mas-staging systemHodgkin lymphoma,liver,gross This is a liver that is involved with Hodgkin lymphoma.The staging of Hodgkins disease is very important in determining therapy.Thus,it is important to determine whether the patient has only a single lymph node region involved,multiple node regions,or e

8、xtranodal involvement.This picture could probably suffice满足 for non-Hodgkin lymphomatous淋巴瘤的 hepatic disease as well.Lymphoma-Diagnosis Clinical manifestations Histopathology Immunophenotypes GeneticsLymphoma-Diagnosis Biopsy Histologic classification Immunophenotype analysis Genetic analysis:-Ig an

9、d/or TCR Gene rearrangement analysis -Karyotype analysis染色体组型分析 -Detection of chromosome translocation or fusion genes by FISH or PCR etcSome lymphomas with distinctive genetic abnormalities Types Cytogenetic finding Genetic alteration FL滤泡性淋巴瘤滤泡性淋巴瘤 t(14;18)(q32;q21)bcl-2 rearranged*MCL套细胞淋巴瘤套细胞淋巴瘤

10、 t(11;14)(q13;32)cyclin D1 BLBurkitt 淋巴瘤淋巴瘤 t(8;14)(q24;q32)myc*MALTOMA t(11;18)(q21;q21)MALT/API2 trisomy 3ALCL间变性大细胞淋巴瘤 t(2;5)(p23;35)NPM-ALKLymphoma-Epidemiology and etiology Chromosome translocation and oncogenes Inherited genetic factors Viruses -Human T-cell leukemia virus-1,HTLV-1 -Epstein-Ba

11、rr virus,EBV -Kaposi sarcoma herpesvirus/human herpesvirus-8,KSHV/HHV-8 -Hepatitis C virus Bacteria -Helicobacter Pylori,HP-Chlamydia psittaci鹦鹉热衣原体;C.pneumoniae肺炎衣原体;C.trachomatis沙眼衣原体 Environment factors Hodgkin lymphoma Hodgkin lymphomaHodgkin lymphoma-characteristics A special entity实体 of lympho

12、ma and occupies up to 20%of all lymphomas.Children and young adults are more commonly involved.The tumor arises in a single node or chain of nodes spreads to the anatomically contiguous nodes.the neoplastic cells:Reed-Sternberg(R-S)cell and its variants.Hodgkin lymphoma-Subtypes Nodular lymphocyte p

13、redominance Hodgkin lymphoma,NLPHL （结节性淋巴细胞为主型（结节性淋巴细胞为主型HL）Classical Hodgkin lymphoma,CHL（经典霍奇金淋巴瘤）Nodular sclerosis结节硬化型,NS Mixed cellularity混合细胞型,MC Lymphocyte rich富于淋巴细胞型,LR Lymphocyte deplete淋巴细胞减少型,LD Hodgkin Lymphoma-Classification Type Histologic Features Frequency Prognosis预后Nodular scleros

14、is Bands of fibrosis,lacunar cells陷窝细胞Most frequent type(60-80%),more common in women Good,most are stage I or II Mixed cellularity Composed of many different cells Most frequent in older persons,second most frequent overall(15-30%)Fair,most are stage III Lymphocyte rich Mostly reactive lymphocytes

15、and many Reed-Sternberg cells Uncommon(5%).Older adults Good to excellent Lymphocyte depletion Many Reed-Sternberg cells and variants变异型Rare(1%)Poor,most are stage III or IV Nodular Lymphocyte predominance Mostly B-cells and few Reed-Sternberg variant cells Uncommon(5%)Good,most are stage I or II Ho

16、dgkin lymphoma,nodular sclerosis type,low power microscopic This is the most frequent type that often has a low stage and therefore a good prognosis.Note the bands of pink collagenous tissue(the sclerosis)dividing the field in this lymph node.Hodgkin lymphoma,nodular sclerosis type,medium power micr

17、oscopic At medium power,nodular sclerosing Hodgkin lymphoma has prominent bands of fibrosis.Staging of Hodgkin lymphoma is important to try and determine therapy and the prognosis.Staging is often done by radiographic射线 means,with CT scans used to determine where lymphadenopathy淋巴腺肿大 is located,ultr

18、asonography超声波检查法 to determine size and lesions of liver and spleen,and chest radiograph.Histologic diagnosis is typically made from biopsy of an involved lymph node.A bone marrow biopsy may be performed as well.Staging laparotomy手术分期 is less commonly used nowadays because the radiographic procedure

19、s are excellent.Hodgkin lymphoma,lacunar cells陷窝细胞,high power microscopic At high power,there are scattered large cells with a surrounding prominent clear space,an artefact人工造成的 of formalin fixation.These are the lacunar cells characteristic for the nodular sclerosis type of Hodgkin lymphoma.Lucunar

20、 cellCHLNodular sclerosisHodgkin lymphoma,Reed-Sternberg cells,lymph node,high power microscopic Note the large cells with large,pale nuclei containing large purple nucleoli at the arrowheads.These are Reed-Sternberg cells that are indicative of Hodgkins disease.Most of the cellular content of foci

21、of Hodgkin lymphoma consists of reactive lymphoid cells.There are four main subtypes of classic Hodgkin lymphoma with CD15+Reed-Sternberg cells and variants:lymphocyte rich,nodular sclerosis,mixed cellularity,and lymphocyte depletion.The lymphocyte predominance subtype with CD15-Reed-Sternberg varia

22、nt cells acts more like a low-grade B-cell lymphoma.porphyry spleen Proposed signals mediated“cross talk”between R-S cells and surrounding normal cells in Classical Hodgkin lymphoma Non Hodgkin LymphomasNon-Hodgkin lymphoma-characteristics 80%to 90%of all lymphomas are NHLs Extranodal lymphoma occup

23、ies about 1/3 of all NHLs.Histopathologic classification is more complex.Clinical manifestations are closely related to the sites involved.A spectrum from lymphomas to leukemias.WHO classification for tumors of lymphoid tissues-NHLB cell neoplasms precursor B cell neoplasms mature(peripheral)B cell

24、neoplasmsT and NK cell neoplasms precursor T cell neoplasms mature(peripheral)T and NK cell neoplasmsNHL Examples Lymphoblastic leukemia/lymphoma Chronic lymphocytic leukemia/small lymphocytic lymphoma，CLL/SLL Follicular lymphoma,FL Diffuse large B-cell lymphoma，DLBCL Burkitt lymphoma,BL Plasma cell

25、 myeloma Extranodal NK/T-cell lymphoma,nasal type Lymphoblastic leukemia/lymphomaTdTChronic lymphocytic leukemia/small lymphocytic lymphoma Follicular lymphomaBcl-2 Diffuse large B-cell lymphoma Burkitt lymphoma Plasma cell myeloma Extranodal NK/T-cell lymphoma,nasal typeClinicopathologic difference

26、s between HL and NHL HL NHL Patient children/young adults any aged peopleStage I/II III/IVSymptoms 40%15%Site LN,90%LN,70%neck/mediastinum extranodal,30%Dissemination predictable random Extranodal uncommon commonLeukemia no yesTumor cell R-S cells lymphocytes Myeloid neoplasmsMyeloid neoplasms-Defin

27、ition Myeloid neoplasms arise within hematopoietic stem cells and typically give rise to monoclonal proliferations that diffusely replace normal bone marrow cells.Myeloid neoplasms:Classification Myeloproliferative neoplasms,MPN Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of P

28、DGFRA,PDGFRB or FGFR1 Myelodysplastic/myeloproliferative neoplasms,MDS/MPN Myelodysplastic syndromes,MDS Acute myeloid leukemia(AML)and related precursor neoplasms Acute leukemia of ambiguous lineage Acute myeloid leukemia (AML)AML are tumors marked by a blockage in the differentiation of early myel

29、oid cells.Immature myeloid cells accumulate in the marrow,replacing normal elements,and frequently circulate in the peripheral blood.AML-clinical manifestations Anemia Hemorrhage Bone pain Fatigue Fever Hepatosplenomegaly LymphadenopathyAML-DiagnosisLaboratory findings:Peripheral blood increased num

30、ber of white cells immature white cells BM aspiration immature white cellsPeripheral blood Bone marrow biopsyMyeloid sarcoma A myeloid sarcoma is a tumour mass consisting of myeloid blasts with or without maturation occurring at an anatomical site other than the bone marrow.Granulocytic sarcoma,so c

31、alled choloroma,is the most common type of myeloid sarcoma.Leukemoid reactions Reactive leukocytosis Many immature white cells appear in the blood Response to severe infection,malignant tumor,toxic events or emergencies Reversible Granulocyte sarcomaMPOMyeloproliferative neoplasms,MPNDefinition:MPN

32、are clonal haematopoitic stem cell neoplasms characterized by proliferation in the bone marrow of one or more of the myeloid lineages.MPN is characterized by hypercellularity of the BM with effective haematopoietic maturation and increased number of granulocytes,red blood cells and/or platelets in t

33、he PB.Myeloproliferative neoplasms:Classification Chronic myelogenous leukemia*BCR-ABL1 positive Chronic neutrophilic leukemia Polycythaemia vera Primary myelofibrosis Essential thrombocythaemia Chronic eosinophilic leukemia Mastcytosis Myeloproliferative neoplasm,unclassifiable Chronic myelogenous

34、leukemiaCML Blood smear CML BM biopsyCML-Detection of t(9;21)and ABL/BCR fusion gene Myeloid neoplasms-Etiology Genetic abnormalities -chromosome translocation -gene mutation -gene deletion and gain Radiation exposure Environment factors Treatment Chemotherapy Radiotherapy Antibiotic therapy Bone ma

35、rrow transplantation Biological therapySummary Both AML and MPN are all clonal haematopoitic stem cell disorders.Lab findings are important for establishing diagnosis of the tumors.Characteristic genetic abnormalities were found in most of cases.No alternation presented between AML and MPN.Histiocyt

36、ic and dendritic cell neoplasmsHistiocytic and dendritic cell neoplasms -Classification Histiocytic sarcoma Dendritic cell sarcoma Tumors derived from Langerhans cells Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma Other rare dendritic cell tumors disseminated juvenile xanthogranuloma Tumors derived from langerhans cells Langerhans cell histiocytosis Langerhans cell sarcoma Langerhans cell histiocytosis