Lymphoproliferative disorders

by
Prof.Dr Shaza Abdel Wahab

Clinical case :
A 30 years old man referred for evaluation of p
ainless cervical and axillary lymph nodes enlar
gement with fever ,sweating and loss of weight
. O/E : hepatosplenomegaly was elicited .
What is the possible diagnosis ?

Definition of Lymphopriferative diseases:

They are clonal (neoplastic ) proliferation of

the lymphocytes which reside in the lymphoid
tissues .

Lymphomas :
Clonal disease arises from malignant transformation of
mature and immature lymphocytes (B,T,and NK natural
killer cells .
It comprises 30 subtypes according to the WHO classi
fication.
Broadly can be classified into Hodgkin and Non
Hodgkin lymphoma .

 Hodgkin Lymphoma (HL ) :

-It is a clonal neoplasm derived from B lymph
ocytes and characterized by the presence of R
eed-Stern (RS)cells.
-has bimodal age distribution with a peak
between 15-35y.and a second peak in the 4-6 th
decades of life.

 Risk factors :
1-EBV
2-HIV: predisposes to EBV associated HL

WHO classification of HL :
I Classic Hodgkin Lymphoma (cHL) :
Nodular sclerosis
Mixed cellularity
Lymphocyte rich
Lymphocyte deplete

II-Nodular lymphocyte- predominant :

(NLPHL ) previously called atypical
NB: in classic HL : the characteristic RS cell e
xpress CD30 and CD 15 surface antigens.
while NLPHL express CD45,CD20,CD79a

Clinical features :
typical presents with painless lymph node enla
rgement starts in 1 group and spread in a conti
guous pattern .
o The cervical lymph node commonly involved.

Diagnosis :
I Clinical features above
II-diagnostic testing :
1- excisional LN biopsy with morphological ,immunohistochemical staining to
differentiate cHL from NLPHL.
2-BM examination is essential for staging
3-Complete Blood picture with differential and
ESR and S.uric acid ,LDH and albumin level.

 Positive emission tomography (PET/CT-scan )

is preferred over CT-scan in assessment of trea
tment response.
Pulmonary function tests should be performed
in all patients going to receive chemotherapy c
ontaining Bleomycin.

 International prognostic score (IPS) for cHL :

International prognostic factors :
1-s.albumin 4g/dl
2- Hemoglobin 10.5g/dl
3-Male gender
4-age 45y.
5-stage IV
6-WBCs15000/cm
7-lymphocytes8% of WBCs

Treatment :
Early HL ( stage Ia, IIa )
Advanced HL (stage I b ,IIb ,stage III and IV )
or bulky disease.
Early stages have a good prognosis and surviv
al with combined chemo-radiotherapy.
Advanced disease : chemotherapy (ABVD)
6 cycles radiotherapy for bulky disease.

Relapsed HL :
o Stage I or localized relapse radiotherapy
o Advanced relapse intensive high dose salvage che
motherapy followed by ASCT.
NLPHL :
a distinct type of HL behaves as indolent B Lympho
ma.
Treatment of stage Iradiotherapy or rituximab
For advanced disease chemotherapy.

Non Hodgkin Lymphoma :

General principles :
NHL is a group of disorders which encompass
es all lymphomas which dont meet the HL crit
eria.

Classifications :
I. The WHO classifications subdivide NHL bas
ed on morphological,immunophenotyping
genetic and clinical features.
II. The subtypes are generally divided into precu
rsor lymphoid neoplasms , mature B neoplas
m ,mature T and NK neoplasm based on their
resemblance to normal stage of maturation .

III. Mature B neoplasm (FL and DLBCL ) account

for 90% of all lymphoid neoplasm.
Risk factors for NHL :
. HIV infections ,immunosuppressed and primar
y immunodefiency ,Hashimotos thyroiditis ,ot
her viral infections as EBV,human herpesvirus
8 ,human Tcell leukemia virus I,HCV, H.pylori
infection associated with gastric MALT lymph
oma.

Clinical picture :
1) Painless lymphadenopathy
2) B-symptoms
3) Extranodal involvement in bone marrow ,skin
GIT,tests ,liver ,spleen,involvement of Waldy
ers ring and CNS involvement .

Ann Arbor staging for lymphoma

International prognostic index (IPI ) :

1) Age60y
2) LDHnormal
3) ECOG PS 2-4
4) Stage III or IV
5) 2 extranodal sites

Diagnosis :
Lab: CBC if BM involved cytopenia
complete metabolic profile as s.uric acid,
s.K ,Ca ,ph and s.LDH.
Imaging : CT-scan neck ,chest and abdomen
for staging .
PET/CT-scan for monitoring disease
response.
LN biopsy : with morphology and immunohistochemistry.

Treatment Overview :
Depends on stage and grade (type ) of NHL.
In indolent lymphoma and aggressive lympho
ma ,limited stage combined modality RT(ra
diotherapy +chemotherapy ).
Advanced stage indolent lymphoma (asympt
omatic ) wait and watch . For symptomatic
chemotherapy.

In highly aggressive lymphoma as lymphoblas

tic lymphoma or burkit lymphoma need aggres
sive chemotherapy similar to Acute lymphobla
stic leukemia.
Relapsed lymphomas :2nd line high dose chem
otherapy followed by auto stem cell transplant
ation.