THE LYMPHOMAS

Divisi Hematologi-Onkologi
DEPARTEMEN I.KESEHATAN
ANAK
FK-USU

Lymphoma : malignant proliferation

of lymphoid cells arising lymphoid
tissues ( lymph node , thymus ,
spleen )

Leukemia : malignancy arising from

the bone marrow, which may include
lymphoid cells.

Lymphoproliferative disorders
(LPDs): polyclonal,nonmalignant
accumulations of lymphocytes that
occur when the immune system fails to
control virally transformed lymphocytes.

Lymphomas of childhood

The Malignant lymphomas:

1. Hodgkins disease
2. Non-Hodgkins lymphoma
(NHL)

.. The malignant lymphoma

Approximately 10% of all cancer in

children < 15 yr., the third most
frequently after leukemia and brain
tumors.
Male : female = 3 : 1
NHL : HD = 1.5 : 1

HodgkinS Disease
Insiden 0 15 tahun
Male predominance
Children have a better response
to treatment
Survival rate > 20 years for
75%

Symptoms and signs

Painless cervical adenopathy (7080%)

Supraclavicular adenopathy (25%)
Mediastinal mass
Node > 2 cm,Ruberry texture,
discrete or matted together , are
not fixed to surrounding tissue
Lymphadenopathy increasing in
size after 2weeks or failing to
resolve within 4-8 weeks.

symptoms and signs

Fatigue , anorexia, weight loss ,

fever , night sweats , pruritus ,
cough
Half of patients : asymptomatic
mediastinal disease (adenopathy
or anterior mediastinal mass)
Splenomegaly or hepatomegaly :
associated advanced disease

Diagnostic investigations and

staging
1.
2.
3.
4.

5.

6.

Biopsy
Imaging
Laparotomy
Bone marrow aspirates and
trephines
Blood tests:CBC,ESR,viral titers
for EBV,CMV,measles,chicken
pox,rubella,renal and liver
function
Ann Arbor Staging

Ann Arbor staging system for

Hodgkins disease
Stage I : involvement of 1 lymph node region(1) or a
single extralymphatic organ or site (IE)
Stage II :involvement of 2 or more lymph node regions on
the same side of the diaphragma (II) or solitary
involvement of an extralymphatic organ or site and of
1 or more lymph node regions on the same site of
diaphragma (IIE)
Stage III : involvement of lymph node regions on both
sides of the diaphragm (III) which may be
accompanied by localized involvement of extralymphatic organ or site (IIIE) or by involvement of the
spleen (IIIS) or both of the these (IIISE)
Stage IV: Diffuse or disseminated involvement of 1 or
more extralymphatic organs or tissues with or without
associated lymph node enlargement

Ann Arbor Staging

RECOMMENDED STAGING
PROCEDURES IN CHILDREN WITH
HODGKINS DISEASE

All patients

Hystory and physical examination

Lymph node biopsy
Laboratorium studies: CBC,,platelet
count,Erythrocyte sedimentation rate,
renal and liver function studies
Radiologic studies

Selected indications
1.
2.

3.
4.
5.

6.

Bone marrow biopsy

Exploratory laparotomy with
splenectomy,retroperitoneal lymph
node biopsies,liver biopsy
Oophoropexy
Bone scan
CT head and necksuspicion of
waldeyers ring involvement
Gallium scan

Treatment
1.
2.
3.

Radiotherapy
Chemotherapy
Combined modality

Complications of therapy of
Hodgkins Disease
Minor treatment complications
1.
Chemical or clinical hypothyroidism
2.
Long term alteration of immune function
Serious treatment complications
3.
Psychosocial problems
4.
Cardiopulmonary dysfunction
5.
Sterility
6.
Soft tissue and bone growth abnormalities
7.
Opportunistic and viral infections

..Complication of therapy of hodgkins Disease

Potentially fatal complications

1.

2.

Second malignant
neoplasmas:leukemia,lymphoma,solid
tumor
Overwhelming bacterial
septicaemia/meningitis
postsplenectomy or postsplenic
irradiation

Long-term sequelae of
lymphoma therapy
1.
2.
3.
4.
5.
6.

7.

Late recurrence
Second tumors
Growth impairment
Endocrine dysfuntion
Fertility
Educational and psychologic
functioning
Organ dysfunction

NON HODGKINS LYMPHOMA

Divers group of cancers , 5 15%

of malignancy in children < 15 yr.
Childhood Lymphoma are rapidly
proliferating, high grade , diffus
malignancy
Hystologically
classified:lymphoblastic lymphoma
(LL) ,small non-cleaved cell
lymphoma (Burkit lymphoma
(BFM)

Burkitt-like lymphoma (BLL) , Large

B-cell lymphoma (LBCL) ,
Anaplastic large cell lymphoma
( ALCL)

Symptoms and signs

Childhood NHL: any site of

lymphoid tissue, lymph nodes,
thymus, liver , spleen.

Extralymphatic sites : bone, bone

marrow, CNS, skin, testes.

Location of tumor

Intra abdominal primary tumors :

31%
Mediastinal
:
27%
Head and neck primary sites
:
29%
Tissue of Waldeyers ring
Cervical lymph nodes
Peripheral lymph nodes,skin,

Lymphoma Sites

Initial symptoms

Constitutional
Cough
symptoms :
Sore throat
fever,night
Abdominal pain
sweats ,weight
Vomiting
loss uncommon
Adenopathy
in NHL , more
Painless,rapidly
suggestive of
enlarging
Hodgkins
lymphadenopathy
Disease

Most rapidly growing human tumors

Airways compression
(cough,dyspnea,orthopnea)
Superior v.cava obstruction ( facial
edema, facial plethora,venous
engorgement) result of mediastinal
NHL .These symptoms are a true
emergencyrapid diagnosis and
treatment

Pleural or pericardial effusions

CNS and bone marrow involvement
Bone marrow contains more
than 25% lymphoblast-
patients are diagnosed with
ALL

Abdominal pain, distention, a right

lower quadrant mass,
intussusception a child > 5 y

Many children present: fever,

weight loss, irritability

Bone pain metastatic disease

Diagnostic

Physical examination
CBC,peripheral blood smear,renal
and liver fuction,electrolyte
Biopsi: histopathologic ,
,immunophenotyping, cytogenetics
Bone marrow

St.Jude Children s Research Hospital Staging System for

Non-Hodgkins Lymphoma

Stage 1: NHL is limited to one lymph node group (e.g., neck,

underarm, groin, etc.) or tumor outside of the abdomen or
mediastinum (middle chest)

Stage 2: NHL is limited to one tumor with local lymph node

involvement, or NHL is limited to two or more tumors or lymph
node groups on the same side of the diaphragm, or NHL is
limited to a primary tumor of the gastrointestinal tract
with/without involvement of local lymph nodes.

Stage 3: NHL includes tumors or lymph node groups on both

sides of the diaphragm, or any primary NHL tumor within the
thorax (trunk) or extensive NHL within the abdomen, or any NHL
around the spine or the outermost membrane of the brain and
spinal cord (dura mater).

Stage 4: NHL is in the bone marrow or central nervous system

(CNS), with/without other sites of involvement. Bone marrow NHL
is defined as 5% malignant cells in an otherwise normal bone
marrow with normal blood counts and smears. By contrast,
lymphoblastic lymphoma that produces more than 25%
malignant cells in the bone marrow is defined as leukemia.

Lymphadenopathy

Enlargement of lymph nodes

results from antigen-stimulated
proliferation of lymphocytes and
other cells intrinsic to the lymph
node or from infiltration of nodes
by extrinsic cells such as
polymorphonuclear leukocytes or
metastatic malignant cells

Causes of lymphadenopathy
1.Infection
1.Bacterial:
Streptococcus,st
aphylococcus
,mycobacteriosis
,brucellocis,tular
emia,syphillis

2.Viral :Epstein-Barr
virus,cytomegalov
irus, HIV , Rubella
3. Fungal:
histoplasmosis,
Coccidioidomycos
is
4.Protozoa:
Toxoplasmosis,
Malaria

2.Autoimmune
Disease
Rheumatoid
3.Storage
arthritis,SLE,
Disease
serum sickness,
Autoimmune
Niemann-Pick
hemolytic
and Gauchers
anemia
Disease

4.Drug reaction
Phenytoin and
others
5.Malignancy
Lymphoma ,
Leukemia,Metasta
tic
Rhabdomyosarco
ma,
Neuroblastoma,

Histiocytosis :
LCH , Malignant
histiocytosis,
sinus
histiocytosis
with massive
lymphadenopat
hy

6.Miscellaneou
s
Sarcoidosis
Kawasaki
syndrome
Cat-scratch
fever