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

histology and non-neoplastic disorders of lymph nodes

TB

cryptococcosis

leishmaniosis

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

Hodgkin lymphoma,HL

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 predominance Hodgkin lymphoma, NLPHL 2. classical Hodgkin lymphoma,CHL lymphocyte-rich, LR mixed cellularity, MC lymphocyte depletion, LD nodular sclerosis, NS

CD20

diagnosis 1.biopsy and recognize RS cells carefully typical RS cells have a value for diagnosis of HL 2.immunohistochemical(IHC) staining CD15 CD30 CD20

CD15 CD30 HL immunostaining

Non-Hodgkin lymphoma, NHL

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 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 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

Acute lymphoblastic leukemia, BM smear

follicular lymphoma, FL 滤泡型淋巴瘤

CD20

Chronic lymphocytic leukemia (CLL)

Burkitt lymphoma

large B-cell lymphoma, spleen

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

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

Diagnosis Biopsy Morphologic classification Immunophenotypic detection Ig and TCR gene rearrangement analysis Prognosis and factors related

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

Clinical features generalized multicentric distribution progressive poor response to therapy poor prognosis high mortality

myeloid neoplasms

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

Classification 1.acute myeloid leukemia, AML 2. chronic myeloproliferative diseases, CMPD 3. myelodysplastic syndrome, MDS

hyperplastic marrow

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

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

AML Hyperplasitc marrow

AML BM smear AML BM biopsy

AML kidney

Clinical manifestation and diagnosis manifestations diagnosis BM aspiration and hemogram

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)

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.

Classification chronic myelogenous leukemia chronic idiopathic myelofibrosis polycythaemia vera essential thrombocythaemia

CML blood smear CML BM biopsy

CML hepatosplenomegaly

CML philadelphia 1 chromosome

Treatment, prognosis and etiology treatment chemotherapy bone marrow transplantation etiology genetics viral infection ( HIV ) radiation chemical materials

Acquired immunodeficiency syndrome AIDS

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

AIDS Definition Epidemiology Etiology Pathogenesis Clinical features and diagnosis Morphology

Epidemiology Three ways for HIV infection : sexual transmission parenteral transmission mother-to-infant transmission

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%

Parenteral transmission intravenous drug abusers the largest group hemophiliacs received large amount of F- Ⅷ or Ⅸ concentrates random recipients of blood transfusion

Etiology a human retrovirus belonging to the lentivirus family two forms of HIV are isolated from patients with AIDS, HIV-1, HIV-2

Human immunod eficiency virus (HIV-1)

HIV virus

Clinical features and diagnosis Mild acute illness severe disease opportunistic infections neuplasms CNS involvement

Opportunistic infections protozoal and Helminthic infections fungal infections Bacterial infections viral infections

Pneumocystis carinii pneumonia, PCP

Histology of PCP Silver staining for pneumocystis carinii

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

Kaposi sacoma of skin

Primary centrol nervous system lymphoma, PCNSL