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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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slide1
Introduction to Diseases of Hematopoitic and Lymphoid System

Weiping Liu

Department of Pathology

West China Medical school of Sichuan University

slide2
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 *

slide3
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
slide4
2. Myeloid neoplasm
  • acute myeloblastic ( myelocytic ) leukemia
  • chronic myeloproliferative disorders
  • myelodysplastic syndrome, MDS
slide21
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
slide23
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
slide28
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
slide33
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

slide42
diagnosis

1.biopsy and recognize RS cells carefully

typical RS cells have a value for diagnosis of HL

2.immunohistochemical(IHC) staining

CD15 CD30CD20

slide43
CD15

HL

CD30

immunostaining

slide47
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
slide48
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

slide50
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
slide68
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

slide69
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
slide70
Diagnosis
  • Biopsy
  • Morphologic classification
  • Immunophenotypic detection
  • Ig and TCR gene rearrangement analysis
  • Prognosis and factors related
slide71
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
slide72
Clinical features

generalized

multicentric distribution

progressive

poor response to therapy

poor prognosis

high mortality

slide74
Definition

Myeloid neuplasms

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

slide75
Classification

1.acute myeloid leukemia, AML

2. chronic myeloproliferative diseases, CMPD

3. myelodysplastic syndrome, MDS

slide77
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
slide78
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
slide80
AML

Hyperplasitc marrow

slide81
AML BM smear

AML BM biopsy

slide82
AML

kidney

slide83
Clinical manifestation and diagnosis
  • manifestations
  • diagnosis
  • BM aspiration and hemogram
slide84
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)
slide85
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.
slide86
Classification
  • chronic myelogenous leukemia
  • chronic idiopathic myelofibrosis
  • polycythaemia vera
  • essential thrombocythaemia
slide87
CML blood smear

CML BM biopsy

slide88
CML

hepatosplenomegaly

slide90
CML

philadelphia 1

chromosome

slide91
Treatment, prognosis and etiology

treatment

chemotherapy

bone marrow transplantation

etiology

genetics

viral infection ( HIV )

radiation

chemical materials

slide93
Definition
  • A retroviral infection caused by HIV
  • characterized by profound immunosupprssion
  • leading to the opportunistic infections, secondary
  • neuplasms and neurologic manifestations
slide94
AIDS

Definition

Epidemiology

Etiology

Pathogenesis

Clinical features and diagnosis

Morphology

slide95
Epidemiology
  • Three ways for HIV infection :
  • sexual transmission
  • parenteral transmission
  • mother-to-infant transmission
slide96
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%
slide97
Parenteral transmission
  • intravenous drug abusers
  • the largest group
  • hemophiliacs
  • received large amount of F-Ⅷ or Ⅸ concentrates
  • random recipients of blood transfusion
slide98
Etiology
  • a human retrovirus belonging to the lentivirus family
  • two forms of HIV are isolated from patients with AIDS, HIV-1, HIV-2
slide100
HIV virus

HIV virus

slide101
Clinical features and diagnosis
  • Mild acute illness severe disease
  • opportunistic infections
  • neuplasms
  • CNS involvement
slide102
Opportunistic infections
  • protozoal and Helminthic infections
  • fungal infections
  • Bacterial infections
  • viral infections
slide104
Histology of PCP

Silver staining for

pneumocystis carinii

slide105
Neuplasms
  • Kaposi sarcoma
  • a vascular sarcoma
  • lymphomas
  • NHL
  • highly aggressive
  • large B-cell lymphomas
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