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

Introduction to Diseases of Hematopoitic and Lymphoid System

Weiping Liu

Department of Pathology

West China Medical school of Sichuan University


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

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 *


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • 2. Myeloid neoplasm

  • acute myeloblastic ( myelocytic ) leukemia

  • chronic myeloproliferative disorders

  • myelodysplastic syndrome, MDS


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

histology and non-neoplastic disorders of lymph nodes


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

TB


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

cryptococcosis


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

leishmaniosis


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

lymphoid neoplasms


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

Hodgkin lymphoma,HL


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

CD20


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

diagnosis

1.biopsy and recognize RS cells carefully

typical RS cells have a value for diagnosis of HL

2.immunohistochemical(IHC) staining

CD15 CD30CD20


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

CD15

HL

CD30

immunostaining


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • Non-Hodgkin lymphoma, NHL


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

key words

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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • Basic pathologic changes

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

Acute lymphoblastic leukemia, BM smear


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

follicular lymphoma, FL滤泡型淋巴瘤


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

CD20


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

Chronic lymphocytic leukemia

(CLL)


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

Burkitt lymphoma


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

large B-cell lymphoma, spleen


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

HD NHL

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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • Clinical manifestations and stages

  • 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


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • Diagnosis

  • Biopsy

  • Morphologic classification

  • Immunophenotypic detection

  • Ig and TCR gene rearrangement analysis

  • Prognosis and factors related


Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

  • Malignant histiocytosis, MH

  • 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


  • Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Clinical features

    generalized

    multicentric distribution

    progressive

    poor response to therapy

    poor prognosis

    high mortality


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    myeloid neoplasms


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Definition

    Myeloid neuplasms

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


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Classification

    1.acute myeloid leukemia, AML

    2. chronic myeloproliferative diseases, CMPD

    3. myelodysplastic syndrome, MDS


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    hyperplastic marrow


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • acute myeloblastic (myeloid) leukemia,

    • ( 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


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Classification

    • 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


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    AML

    Hyperplasitc marrow


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    AML BM smear

    AML BM biopsy


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    AML

    kidney


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Clinical manifestation and diagnosis

    • manifestations

    • diagnosis

    • BM aspiration and hemogram


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Bone marrow biopsy: the value in diagnosis and treatment of leukemias

    • 1.to evaluate the degree of BM hyperplasia

    • 2. to find the residual leukemic cells

    • 3. to diagnose extra-mydullary invasion of leukemia (chloroma / granulocytic sarcoma)


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • chronic 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.


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Classification

    • chronic myelogenous leukemia

    • chronic idiopathic myelofibrosis

    • polycythaemia vera

    • essential thrombocythaemia


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    CML blood smear

    CML BM biopsy


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    CML

    hepatosplenomegaly


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    CML

    philadelphia 1

    chromosome


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Treatment, prognosis and etiology

    treatment

    chemotherapy

    bone marrow transplantation

    etiology

    genetics

    viral infection ( HIV )

    radiation

    chemical materials


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Acquired immunodeficiency syndrome

    AIDS


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Definition

    • A retroviral infection caused by HIV

    • characterized by profound immunosupprssion

    • leading to the opportunistic infections, secondary

    • neuplasms and neurologic manifestations


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    AIDS

    Definition

    Epidemiology

    Etiology

    Pathogenesis

    Clinical features and diagnosis

    Morphology


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Epidemiology

    • Three ways for HIV infection :

    • sexual transmission

    • parenteral transmission

    • mother-to-infant transmission


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Sexual transmission

    • 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%


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Parenteral transmission

    • intravenous drug abusers

    • the largest group

    • hemophiliacs

    • received large amount of F-Ⅷ or Ⅸ concentrates

    • random recipients of blood transfusion


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Etiology

    • a human retrovirus belonging to the lentivirus family

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


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Human immunodeficiency virus

    (HIV-1)


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    HIV virus

    HIV virus


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Clinical features and diagnosis

    • Mild acute illness severe disease

    • opportunistic infections

    • neuplasms

    • CNS involvement


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Opportunistic infections

    • protozoal and Helminthic infections

    • fungal infections

    • Bacterial infections

    • viral infections


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Pneumocystis carinii pneumonia, PCP


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Histology of PCP

    Silver staining for

    pneumocystis carinii


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    • Neuplasms

    • Kaposi sarcoma

    • a vascular sarcoma

    • lymphomas

    • NHL

    • highly aggressive

    • large B-cell lymphomas


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Kaposi sacoma of skin


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    Primary centrol nervous system lymphoma, PCNSL


    Introduction to diseases of hematopoitic and lymphoid system weiping liu department of pathology

    The end


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