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Hematopatologie

Limfom= cancer al tesutului limfoid


Leucemie= cancer al celulelor
circulante sangvine (sange si
maduva)

Leucemie

Ce este leucemia?
Cancer al celulelor sangvine
Acuta sau cronica
Este afectata capacitatea de maturare a celulelor
sangvine
La nivelul maduvei osoase hematogene se
formeaza numeroase celule tinere, imature
(blasti)

Tipuri principale
Leucemie limfocitara acuta (LLA)
Leucemie mieloida acuta (LAM)
Leucemie limfocitara cronica (LLC)
Leucemie mieloida acuta(LMC)

Demographics of Leukemia
Patients (2001 Data)
CLL=Chronic
Lymphocytic
ALL=Acute
Lymphocytic

ALL
11%

others
17%

CML=Chronic
Mylogenous
AML=Acute
Mylogenous

CML
15%

CLL
26%

AML
31%

Sources from Leukemia, Lyphoma,


Myeloma Facts 2001

Total Reported Cases = 31,500

Simptome
leucopenie --> infectii
anemie --> paloare
trombocitopenie --> sangerare

DX
Frotiu din pulpa degetului
Hemoleucograma
Biopsie medulara
Analiza LCR

Pictures Of Blood
Platelet
White Cell

Platelet
Red Cell

Normal human blood


Sources from Arginine.umdnj.edu

White Cell

Red Cell

Blasts

Blood with leukemia


Sources from beyond2000.com

Development of Leukemia in the


Bloodstream

Stage 1- Normal

Stage 2- Symptoms

Stage 3- Diagnosis

Legend
White Cell
Red Cell
Platelet
Blast
Germ

Stage 5a- Anemia

Stage 4- Worsening

Stage 5b- Infection

Sources from Leukemia, by D. Newton and D. Siegel

Cauze
Expunere ridicata la radiatii sau substante
toxice
Virusuri
Modficari genice
Frecvent necunoscut

Tratament
Chemoterapie
Terapie monoclonala
Radioterapie
Imunoterapie
Transplant medular

Corticala

Paracorticala

Medulara

Centrul germinal in hiperplazia foliculara este caracterizat de


prezenta de macrofage cu corpi tingibili, expandare folicul

In general, lymph nodes in a benign reactive process


are more likely to enlarge quickly and be tender.

Marginal zone

Mantle zone

germinal center

HODGKIN VS NON -HODGKIN ?


NK/T?
B?
B-Cell lymphomas (80%)
T-Cell lymphomas (15%)

Clasificarea se realizeaza din punct de vedere


microscopic, molecular si genetic
Limfoame agresive
Limfoame indolente

B-cell development memory


LLC
LMa

stem
cell

B-cell
mature
naive
B-cell

germinal
center
B-cell

lymphoid
precursor
progenitor-B

LLA

pre-B
immature
B-cell

LMz
LLC
MM

DLBCL,
LF, LB, LH

plasma cell

Limfoame B (80%)

Limfocitele B pot produce Ac prin activarea si


transformarea lo in plasmocite
Sunt mai multe tipuri de limfoame
.

Precursor B cell neoplasms:


Precursor B lymphoblastic leukemia/lymphoma
Mature (peripheral) B cell neoplasms:
Chronic lymphocytic leukemia/small lymphocytic lymphoma
B cell prolymphocytic leukemia
Splenic marginal zone lymphoma
Hairy cell leukemia
Splenic lymphoma/leukemia, unclassifiable
Splenic diffuse red pulp small B-cell lymphoma*
Hairy cell leukemia-variant*
Lymphoplasmacytic lymphoma
Waldenstrom macroglobulinemia
Heavy chain diseases (alpha, gamma, mu)
Plasma cell myeloma
Solitary plasmacytoma of bone
Extraosseous plasmacytoma
Extranodal marginal zone B-cell lymphoma of muocsa associated lymphoid tissue (MALT
lymphoma)
Nodal marginal zone B-cell lymphoma (MZL)
Pediatric type nodal MZL
Follicular lymphoma
Pediatric type follicular lymphoma
Primary cutaneous follicle center lymphoma
Mantle cell lymphoma
Diffuse large B cell lymphoma (DLBCL), not otherwise specified
T cell/histiocyte rich large B-cell lymphoma
DLBCL associated with chronic inflammation
Epstein-Barr virus (EBV)+ DLBCL of the elderly
Lymphomatoid granulomatosis
Primary mediastinal (thymic) large B cell lymphoma
Intravascular large B cell lymphoma
Primary cutaneous DLBCL, leg type
ALK+ large B-cell lymphoma
Plasmablastic lymphoma
Primary effusion lymphoma
Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
Burkitt lymphoma
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma
and Burkitt lymphoma
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell
lymphoma and classical Hodgkin lymphoma WHO 2008

Limfom folicular
Cauzat de translocarea genica ce duce la
supraexpresia genei BCL-2 avand ca efect blocarea
apoptozei[t(14;18)]
Origine in centrul germinal
Nu se poate vindeca
cel mai frecvent tip de limfom indolent
asimptomatic

Tratamentul in Limfomul asimptomatic


(watch-and-wait)
Diferite scheme de tratament
Desi indolent mortalitatea poate sa fie mare
Apare transformarea intr-un limfom agresiv

Limfom Burkitt

Forma agresiva de limfom intalnita


frecvent la copii din Africa. Afecteaza
frecvent oasele fetei, ovarele, si gg
limfatici abdominali
Distributia geografica sugereaza
transmiterea prin intepatura de insecta
Cauzata de virusul Epstein_Barr

Celule nediferentiate printre care se gasec


macrofage dand aspectul de cer instelat
Numeroase mitoze

t(8;14) este caracteristica

Limfom difuz cu celula mare B

Cel mai frecvent tip de limfom


agresiv
Frecvent asimptomatic
Afectarea extranodala este
frecventa
vindecare ~ 40%

DLBCL

Celule de talie mare, cu citoplasma


moderata/abundenta. Nucleii prezinta nucleoli
proeminenti, mitoze ocazionale.
Diagnostic diferential cu metastazele de carcinom

Limfom T (15%)
Precursor T-lymphoid neoplasms
T lymphoblastic leukemia/lymphoma,
Mature T cell and NK cell neoplasms
T cell prolymphocytic leukemia,
T cell large granular lymphocytic leukemia,
Chronic lymphoproliferative disorder of NK cells,
Aggressive NK cell leukemia,
Systemic EBV-positive T cell lymphoproliferative disease of childhood,
Hydroa vacciniforme-like lymphoma,
Adult T cell leukemia/lymphoma,
Extranodal NK/T cell lymphoma, nasal type,
Enteropathy-associated T cell lymphoma,
Hepatosplenic T cell lymphoma,
Subcutaneous panniculitis-like T cell lymphoma,
Mycosis fungoides,
Szary syndrome,
Primary cutaneous CD30-positive T cell lymphoproliferative disorders
Lymphomatoid papulosis,
Primary cutaneous anaplastic large cell lymphoma,
Primary cutaneous gamma-delta T cell lymphoma,
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T cell lymphoma,
Primary cutaneous CD4-positive small/medium T cell lymphoma
Peripheral T cell lymphoma, NOS
Angioimmunoblastic T cell lymphoma
Anaplastic large cell lymphoma, ALK-positive
Anaplastic large cell lymphoma, ALK-negative
WHO 2008

Epidemiologia limfoamelor

Al 5 tip de cancer
M>F
LNH in crestere
LH stabil

Simptome
Adenopatii

Febra neexplicabila

Transpiratii nocturne

Oboseala cronica

Pierdere in greutate

Mancarime

DX

X-Rays

CT

RMN

Biopsie=golden standard

Tratament

chemoterapie

Radioterapie

Transplant medular

Tratament chirurgical

Monoclonal (retuximab in
limfoamele CD20+)

LIMFOM HODGKIN

Thomas Hodgkin (1798-1866)

Tipuri
Limfom nodular cu
predominenta limfocitara
Limfom clasic
Celularitate mixta
Scleroza nodulara
Depletie limfocitara
Predominenta limfocitara

Caracteristice histologice

Frecventa

Prognostic

Scleroza
nodulaa

Benzi de fibroza, celule


lacunare

cea mai frecventa forma, mai


frecvent la femei

Bun, dg in std I
si II

Celularitate
mixta

Celule diferite

Mai frecvent la batrani


Al doiale cel mai frecvent tip

Rezonabil,
frecvent dg std
III

Predominen Majoritate limfocite B si rare


ta
celule/ variante Reedlimfocitara
Sternberg

rar

Bun, dg in std I
si II

Depletie
limfocitara

rar

Prost, dg in std
II si IV

Tipuri

numeroase celule/variante
Reed-Sternberg

Stadializare

Un singur grup de gg sau o singura localizare extranodala

II

doua sau mai mute grupuri gg dar localizate de o singura parte a diafragmului sau
afectare limitata extranodala prin extindere de la gg

III

Gg de ambele parti ale diafragmului cu afectare splenica sau afectare limitata


extranodala prin extindere de la gg sau amandoua

IV

Afectare extranodala extinsa cu/fara afectare gg

PROF. DR . G . SIMU

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