Introduction to Diseases of Hematopoitic and Lymphoid System
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Introduction to Diseases of Hematopoitic and Lymphoid System Weiping Liu Department of Pathology West China Medical school of Sichuan University. disorders of hematopoitic and lymphoid system red cell disorders anemia tumors white cell disorders* myeloid cells

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Introduction to Diseases of Hematopoitic and Lymphoid System

Weiping Liu

Department of Pathology

West China Medical school of Sichuan University


disorders of hematopoitic and lymphoid system

red cell disorders

anemia

tumors

white cell disorders*

myeloid cells

reactive : leukopenia, leukocytosis*

tumors:

lymphoid cells

reactive

tumors: lymphomas *


  • Contents

  • 1.Diseases of lymphoid tissue

  • Inflammation

  • non-specific lymphadenitis

  • special infection: becteria fungus

  • parasites virus

  • Lymphoproliferative disorders, unknown causes

  • giant lymph node hyperplasia(Castleman’s disease)

  • sinus histiocytosis with massive lymphadenopathy

  • (Rosai-Dorfman disease)

  • Tumors*:

  • primary: lymphoid neoplasm*

  • secondary: metastatic tumors


  • 2. Myeloid neoplasm

  • acute myeloblastic ( myelocytic ) leukemia

  • chronic myeloproliferative disorders

  • myelodysplastic syndrome, MDS







  • lymphoid neoplasms

  • Definition

  • malignant tumors of immune system, including

  • lymphomas, lymphocytic leukemias and plasma cell

  • neoplasms.

  • involved organs and tissues

  • lymph nodes and extralymphocytic organs

  • bone marrow and peripheral blood

  • tumor cells lymphocytes – B, T and NK cell


  • Classification

  • Hodgkin lymphoma, HL

  • Hodgkin Disease, HD

  • Non-Hodgkin lymphoma,NHL

  • B-cell neoplasms

  • T and NK-cell neoplasms

  • tumors of histiocyte and dendratic cells



  • Pathologic changes

  • gross appearance: almost same as that of sarcoma

  • Histologic morphology:

  • archetecture of lymph node

  • partially or completely demolished

  • cells

  • tumor cells --- R-S cell and its variants

  • reactive inflammatory cells: lymphocytes

  • eosenophils

  • histocytes

  • fibrocytes and fibroblasts


Subtypes of Hodgkin lymphoma

1. nodular lymphocyte predominanceHodgkin

lymphoma, NLPHL

2. classical Hodgkin lymphoma,CHL

lymphocyte-rich, LR

mixed cellularity, MC

lymphocyte depletion, LD

nodular sclerosis, NS



diagnosis

1.biopsy and recognize RS cells carefully

typical RS cells have a value for diagnosis of HL

2.immunohistochemical(IHC) staining

CD15 CD30CD20


CD15

HL

CD30

immunostaining



  • The classification on the tumors of hematopoitic and lymphoid tissues by WHO

  • B cell neoplasms

  • precusor B cell neoplasms

  • acute lymphoblastic leukemia / lymphoblasitc lymphoma

  • mature(peripheral) B cell neuplasms

  • T and NK cell neoplasmas

  • precusor T cell neoplasms

  • acute lymphoblastic leukemia / lymphoblasitc lymphoma

  • mature(peripheral) T and NK cell neuplasms


key words lymphoid tissues by WHO

1.archetecture:Diffuse,D

Follicular,F

2.cell size:large cell

small cell

blast

3.immunophenotype: T; B; NK;

4.sites related:

mycosis fungoides, MF

intestinal T-cell lymphoma, ITCL

NK/T-cell lymphoma, nasal type


  • Basic pathologic changes lymphoid tissues by WHO

  • Architecture of LN is partially or completely

  • demolished

  • relatively momnmorphic lymphoid tumor cell

  • infiltrated diffusely or follicularlly

  • the lymphoid tumor cells infiltrate the

  • capsule of LN and extrnodal soft tissues



follicular lymphoma, FL lymphoid tissues by WHO滤泡型淋巴瘤


CD20 lymphoid tissues by WHO


Chronic lymphocytic leukemia lymphoid tissues by WHO

(CLL)


Burkitt lymphoma lymphoid tissues by WHO


large B-cell lymphoma, spleen lymphoid tissues by WHO


HD NHL lymphoid tissues by WHO

patients children/youth people of any age

stages I / II III / IV

symptoms 40% 15%

site LN, 90% LN, 70%

neck/mediastinum extranodal,30%

dissemination predictable random

extranodal uncommon common

leukemia no yes

tumor cell R-S cells lymphocytes


  • Clinical manifestations and stages lymphoid tissues by WHO

  • Manifestations

  • Localized lymphadenopathy painlessly and progressively

  • Invasion and destroy the surrounding tissues

  • pressing the surrounding organs and tissues

  • disturbance of metabolism and functions in

  • involved organs

  • staging system

  • Ann Arbor Classification, (1971) I-IV


  • Diagnosis lymphoid tissues by WHO

  • Biopsy

  • Morphologic classification

  • Immunophenotypic detection

  • Ig and TCR gene rearrangement analysis

  • Prognosis and factors related


  • heterogeneous lymphoproliferative disorders

  • 1. T-cell lymphomas

  • 2. NK/T-cell lymphomas

  • 3. Large B-cell lymphomas

  • 4. anaplastic large cell lymphomas

  • 5. true histiocytic sarcomas


  • Clinical features lymphoid tissues by WHO

    generalized

    multicentric distribution

    progressive

    poor response to therapy

    poor prognosis

    high mortality


    myeloid neoplasms lymphoid tissues by WHO


    Definition lymphoid tissues by WHO

    Myeloid neuplasms

    arise within hematopoietic stem cells and typically give rise to monoclonal proliferations that diffusely replace normal bone marrow cells


    Classification lymphoid tissues by WHO

    1.acute myeloid leukemia, AML

    2. chronic myeloproliferative diseases, CMPD

    3. myelodysplastic syndrome, MDS


    hyperplastic marrow lymphoid tissues by WHO


    • acute myeloblastic (myeloid) leukemia, lymphoid tissues by WHO

    • ( AML )

    • Definition

    • malignant tumor of hematopoitic stem cells

    • tumor cells proliferate diffusely inside Bm

    • tumor cells extensively infiltrate outside Bm

    • usually do not form a mass or masses


    • Classification lymphoid tissues by WHO

    • short disease course

    • the number of WBC in peripheral blood:

    • leukemic / non-leukemic

    • cell involved:

    • FAB classification of AML: M1-M7

    • The classification of AML by WHO *

    • cytogenetic changes

    • with or without multicellular dysplasia

    • treatment related


    AML lymphoid tissues by WHO

    Hyperplasitc marrow


    AML lymphoid tissues by WHO BM smear

    AML BM biopsy


    AML lymphoid tissues by WHO

    kidney




    • c leukemiashronic myeloproliferative diseases(CMPD)

    • Definition

    • CMPDs are clonal haematopoitic stem cell disorders characterised by proliferation in the bone marrow of one or more of the myeloid lineages.


    • Classification leukemias

    • chronic myelogenous leukemia

    • chronic idiopathic myelofibrosis

    • polycythaemia vera

    • essential thrombocythaemia


    CML leukemiasblood smear

    CML BM biopsy


    CML leukemias

    hepatosplenomegaly


    CML leukemias

    philadelphia 1

    chromosome


    Treatment, prognosis and etiology leukemias

    treatment

    chemotherapy

    bone marrow transplantation

    etiology

    genetics

    viral infection ( HIV )

    radiation

    chemical materials



    • Definition leukemias

    • A retroviral infection caused by HIV

    • characterized by profound immunosupprssion

    • leading to the opportunistic infections, secondary

    • neuplasms and neurologic manifestations


    AIDS leukemias

    Definition

    Epidemiology

    Etiology

    Pathogenesis

    Clinical features and diagnosis

    Morphology


    • Epidemiology leukemias

    • Three ways for HIV infection :

    • sexual transmission

    • parenteral transmission

    • mother-to-infant transmission


    • Sexual transmission leukemias

    • the major way of HIV infection

    • homosexual or bisexual male predominant

    • 46% in HIV infected people

    • 56% in HIV infected men

    • heterosexual contact of members 11%


    • Parenteral transmission leukemias

    • intravenous drug abusers

    • the largest group

    • hemophiliacs

    • received large amount of F-Ⅷ or Ⅸ concentrates

    • random recipients of blood transfusion


    • Etiology leukemias

    • a human retrovirus belonging to the lentivirus family

    • two forms of HIV are isolated from patients with AIDS, HIV-1, HIV-2


    Human immunodeficiency virus leukemias

    (HIV-1)


    HIV virus leukemias

    HIV virus



    • Opportunistic infections leukemias

    • protozoal and Helminthic infections

    • fungal infections

    • Bacterial infections

    • viral infections



    Histology of PCP leukemias

    Silver staining for

    pneumocystis carinii


    • Neuplasms leukemias

    • Kaposi sarcoma

    • a vascular sarcoma

    • lymphomas

    • NHL

    • highly aggressive

    • large B-cell lymphomas


    Kaposi sacoma of leukemiasskin



    The end leukemias


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