General Pathology

Histogenetic Classification
of Neoplasms

Lymphomas & Leukemias

(Hemoblastomas & Hemoblastoses)

Jaroslava Dukov
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague

Leukemia
def.
- diffuse neoplastic proliferation
of the hemopoietic marrow cell

granulocytic
lymphocytic

Lymphoma
def.
malignant neoplasms of cells native to
lymphoid tissue (lymphocytes,
histiocytes) and their precursors and
derivatives.

non Hodgkin (B,T)

Hodgkin

NEOPLASIA classification
HISTOGENETIC (cell of origin)

mesenchymal
epithelial
neuroectodermal
mixed, teratoma
choriocarcinoma
mesotelioma

Main functions of the bone

marrow and lymph nodes:
hematopoetry
immune response
Hematopoetry

gr. haima- blood, poitria - art of

composition characterized by great beauty
of expression

Lymphomas and Leukemias clinical symptomathology

LYMPHOMA
painless
lymphadenomegaly
infiltrated

organs
hepatosplenomegaly
bone marrow
involvement (&
leukemia)

LEUKEMIA
anaemia / fatigue
immunodepression
infections, fever
haemorrhagic diathesis
epistaxis, ecchymoses
bone pain
hepatosplenomegaly
CNS meningeal (ALL)

Pathology & Genetics

Tumours of Haemopoietic
and Lymphoid Tissues
WHO 2001

Principles of Classification

primarily according to lineage

myeloid
lymphoid
histiocytic/dendritic
mast cell

within each category

morphology
immunophenotype
genetic features
clinical syndromes

cell

NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID

TISSUE
(WHO 2001, abbreviated)
Myeloid neoplasms
Chronic myeloproliferative disorders
Myeloproliferative / myelodysplastic diseases
Myelodysplastic syndromes
Acute myeloid leukemias
Lymphoid neoplasms
Precursor B and T cell neoplasms
Most of
Mature B cell neoplasms
them
Mature T and NK cell neoplasms
originate
/ may
Hodgkin lymphoma
originate
Histiocytic and dendritic cell neoplasms
in lymph
Histiocytic sarcoma
nodes
Dendritic cell neoplasms
Mastocytosis

Lymphomas and Leukemias general macroscopy features

LYMPH NODES
enlargement (painless)

HEMOPOETRY
HISTOHOMOLOGOUS
ORGANS
enlargement

BONE MARROW
pyoid (yellowish) or
gray infiltration
activation of reserve
zones
OTHER ORGANS
diffuse

or tumorous
infiltration

Lymphomas and Leukemias general histology features

LYMPH NODES
architecture partly /
fully obscurred with the
neoplastic population
subcapsullar

sinus

defunct
nodular / diffuse
transcapsullar spread

BONE MARROW
hypercellular
architecture partly /
fully obscurred with the
neoplastic population
haemopoetry activation
in the formerly fatty
(reserve) marrow

HISTOHOMOLOGOUS ORGANS colonised

liver, spleen, nodes

NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID

TISSUE
(WHO 2001, abbreviated)
Myeloid neoplasms
Chronic myeloproliferative disorders
Myeloproliferative / myelodysplastic diseases
Myelodysplastic syndromes
Acute myeloid leukemias
Lymphoid neoplasms
Precursor B and T cell neoplasms
Most of
Mature B cell neoplasms
them
Mature T and NK cell neoplasms
originate
/ may
Hodgkin lymphoma
originate
Histiocytic and dendritic cell neoplasms
in lymph
Histiocytic sarcoma
nodes
Dendritic cell neoplasms
Mastocytosis

Myeloid Diseases

Chronic myeloproliferative diseases CMPD

Myelodysplastic/myeloproliferative diseases
MDS/MPD
Myelodysplastic syndromes MDS
Acute myeloid leukaemias AML

Chronic Myeloproliferative Diseases CMPD

def.:
clonal proliferation of one or more of the myeloid
lineages (granulocytic, erythroid, megakaryocytic)
hemopoietic stem cells in the bone marrow

hepatomegaly, splenomegaly

development into myelofibrosis or acute blast phase

Myeloid Diseases
Chronic

myeloproliferative diseases CMPD

CML (Philadelphia chromosome t(9,22)(q34;q11)

Chronic neutrophilic leukaemia
Chronic eosinophilic leukaemia
Polycythemia vera
Chronic idiopathic myelofibrosis
Essential thrombocythemia

Myeloid Diseases

Myelodysplastic/myeloproliferative diseases
MDS/MPD
Chronic myelomonocytic leukaemia
Atypical chronic myeloid leukaemia
Juvenile myelomonocytic leukaemia
MDS/MPD - unclassifiable

Myelodysplastic syndromes - MDS

def.:

bone marrow failure and dysplasia in one or more

myeloid cell lineages

the number of blasts is in the blood or marrow < 20%

(xAML)

development to acute leukaemia or death of bone marrow

failure

Myeloid Diseases
Myelodysplastic

syndromes -

MDS

Refractory anaemia
Refractory anaemia with ringed sideroblasts
Refractory anaemia with multilineage dysplasia
Refractory anaemia with excess blasts
MDS associated with isolated del(5q)
chromosome abnormality
MDS - unclassifiable

Myeloid Diseases
Acute myeloid leukaemias -

AML

clonal expansion of myeloid blasts in bone

marrow blood or other tissue

Leukemia
Acute
morphology:

AML ALL -

aplastic anemia
agranulocytosis,
thrombocytopenia

adults
children

Myeloid Diseases
Acute myeloid leukaemias -

AML

AML with recurrent cytogenetic abnormalities

AML with multilineage dysplasia
AML and MDS therapy- related

AML not otherwise categorised

19 nosology units

NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID

TISSUE
(WHO 2001, abbreviated)
Myeloid neoplasms
Chronic myeloproliferative disorders
Myeloproliferative / myelodysplastic diseases
Myelodysplastic syndromes
Acute myeloid leukemias
Lymphoid neoplasms
Precursor B and T cell neoplasms
Most of
Mature B cell neoplasms
them
Mature T and NK cell neoplasms
originate
/ may
Hodgkin lymphoma
originate
Histiocytic and dendritic cell neoplasms
in lymph
Histiocytic sarcoma
nodes
Dendritic cell neoplasms
Mastocytosis

Lymphoma
def.
malignant neoplasms of cells native to
lymphoid tissue (lymphocytes,
histiocytes) and their precursors and
derivatives.

non Hodgkin (B,T)

Hodgkin

Lymphoid Neoplasms

B cell

T and NK cell

Hodgkin lymphomas

(lymphomas & leukaemias)

--

Lymphoid Neoplasms
B cell

lymphomas & leukaemias

(17 nosology units in 2001 WHO classif.)

Precursor B-cell neoplasm

Mature B-cell neoplasms

(85% nH ML)

B-cell proliferations of uncertain malignant

potential

Small Lymphocytic Lymhpoma (SLL) /

Chronic Lymhocytic Leukemia (CLL)
Small B-lymphocytes, proliferating cells (prolymphocyte, paraimmunoblast)

peripheral blood lymphocytes >10x109/ l,

bone marrow lymphocytic infiltration

splenomegaly, hepatomegaly, lymphadenopathy

immunodeficiency, bleeding, disordered healing

Clinical behaviour

INDOLENT

leukemisation common

occasional transformation to aggressive lymphoma /

leukemia

Dg: Morphology confusing - immunophenotyping necessary

CD23+, CD5+, cyclin D1

Leukemia
Chronic
morphology: bone marrow infiltration
splenomegaly
hepatomegaly
enlarged lymph nodes

clinic: may remain silent for a long time

CML related to myeloproliferative disorders
CLL close to nH ML (95%B)
Hairy cell leukemia tricholeukemia
small B lymphoid cells

Lymphoplasmocytic lymphoma LPL

/Waldenstrm macroglobulinemia
small

B lymhocytes lymphoma
bone marrow, LN, spleen
older adults
monoclonal IgM serum paraprotein
hyperviscosity symptoms

Burkitts Lymphoma

highly malignant small B cell lymphoma

EBV (DNA) related

endemic in Africa, sporadic elsewhere

high mitotic rate
starry sky appearance (due to non neoplastic

macrophages admixture)

Lymphoid Neoplasms

B cell

T and NK cell

Hodgkin lymphomas

(lymphomas & leukaemias)

--

Lymphoid Neoplasms
T and NK cell lymphomas & leukaemias
(16 nosology units in 2001 WHO classif.)

Precursor T-cell neoplasm

Mature T-cell neoplasms

(EB virus HTLV-1)

T-cell proliferations of uncertain malignant potential

Mycosis Fungoides and Sezary Syndrome

Def.:
MF: mature T- cell lymphoma presenting in the
skin with patches/plaques and characterized
by epidermal and dermal infiltration of small to
medium size T-cells with cerebriform nuclei

SS: generalized mature T- cell lymphoma

characterized by the presence of erythroderma
, lymphadenopathy and T-cells with
cerebriform nuclei aggresive form of MF

Mycosis fungoides and Sezary Syndrome

adults

M:F 2:1
years lasting course
trunk erruptions
rarely generalization

Lymphoid Neoplasms

B cell

T and NK cell

Hodgkin lymphomas

(lymphomas & leukaemias)

--

Lymphogranuloma Malignum
Hodgkin
def.

malignant lymphoma containing

diagnostic RS or Hodgkin tumorous

cells in a rich cellular background

Lymphoid Neoplasms WHO 2001

Hodgkin

lymphomas

HL

Nodular lymphocyte predominant HL

Classical HL
Nodular sclerosis classical HL
Lymphocyte-rich classical HL
Mixed cellularity classical HL

Lymphocyte-depleted classical HL

Classical HL
85% of HL
Nodular sclerosis CHL (NSHL)
Most frequent, young adults
Very good prognosis with treatment
Lymphocyte-rich CHL (LRCHL)
Rare
Very good prognosis with treatment
Mixed cellularity CHL (MCHL)
Frequent, adults
Medium prognosis
Lymphocyte depleted CHL (LDHL)
Very rare, immunocompromised patients
Poor prognosis

(CHL)

Nodular Lymphocyte Predominant HL

15% of HL
B-lymphoma
Differential diagnosis may be very difficult
Highly atypical CD30-/CD15-/CD20+/CD45+
L/H cells = popcorn cells
Reactive cells
Lymphocytes, histiocytes, plasma cells, no eosinophils
Nodular growth
No fibrosis
Very good prognosis with treatment even in relapsing disease

NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID

TISSUE
(WHO 2001, abbreviated)
Myeloid neoplasms
Chronic myeloproliferative disorders
Myeloproliferative / myelodysplastic diseases
Myelodysplastic syndromes
Acute myeloid leukemias
Lymphoid neoplasms
Precursor B and T cell neoplasms
Most of
Mature B cell neoplasms
them
Mature T and NK cell neoplasms
originate
/ may
Hodgkin lymphoma
originate
Histiocytic and dendritic cell neoplasms
in lymph
Histiocytic sarcoma
nodes
Dendritic cell neoplasms
Mastocytosis

Histiocytic and Dendritic Cell

Neoplasms

Macrophage/histiocytic neoplasm (CD68, CD 1)

Langerhans cells:
m. Hand Schller- Christian HSCH triad : calva defects, diab.insip.,
exophtalmos

eosinophilic granuloma
m. Letterer Sive

(bone)
(skin , hepatosplenomegaly, lymph nodes)

Dendritic cell neoplasms

Mastocytosis