Pediatric Lymphoma

Paediatric lymphomas
Lymphoma in beginning
• First described in 1832 by

Dr Thomas Hodgkin
• Sternberg(1898) and Reed(1902) are
credited for first definitive description
of histopathology
• During this period lymphoma was
shrunken using exposure to radiation s
in 1896
• In 1970 MOPP drug regimen was found
to be highly effective
• Megavoltage radiotherapy was used
during 1950
DR THOMAS HODGKIN
Lymphomas
• Paediatric lymphoma is highly curable malignancy.

• Paediatric tumours constitute 6-8% of all cancers.
• India has approximately one-fifth of the world’s paediatric cancer
load
Two types of lymphomas
• Hodgkin Lymphoma – 6% of child hood cancers

• Uncommon in children less than 3 years
• Peak Incidence is around 15 to 35 years
• Non Hodgkin Lymphoma :
 3% of all childhood malignancies in children younger than 5 year
8% to 9 .5 % in 5 to 19 years of age
• Sex
• Sex ratio is 10:1 male dominance under 7 years
• Sex ratio 1:1 for children after 12 years
HODKIN LYMPHOMA
Etiology factors
1. Familial aggregation: High incidence in twins, first degree relatives
have 3 fold increase risk
2. Environmental factors/Lifestyle factors: like Epstein Barr Virus and
HIV.
3. Human Leukocytic Antigen Association with Hodgkin's Disease
4. Non viral factors
5. Primary immunodeficiency
Pathophysiology
Lytic infection
Sensitive to antivirals
Latent infection
Reed Sternberg Cells
• Hall mark of HL is All Hodgkin

lymphoma come from germinal centre
B cells.
• Multinucleated / bi lobed nucleus

• Owl eye appearance R-S Cells
• Inflammation occur
WHO classification of Hodgkin's lymphoma
1. Nodular : large cell, multilobed (popcorn cells).localised, nonbulky

seen in mediastinum
2. Classical Hodgkin's lymphoma classified as
• Lymphocytic Predominance
• Nodular Sclerosis
• Mixed Cellularity popcorn cells
• Lymphocytic Depletion
EBV GENE EXPRESSION IN REED STERNBERG CELLS

Clinical Features Cervical
1. Painless enlargement of lymph supraclavi
glands cular
2. one side of cervical region, felt as
firm, rubbery, non tender and
movable .
3. As disease progress deeper glands
are involved
4. Chronic whooping type of cough
mainly due to mediastinal
compression in the form of
respiratory distress
5. General symptoms include fever,
6. Loss of weight ,night sweats,
7. pruritis
Areas of Lymphadenopathy
Modified Ann Arbor Staging System 1
Stage Characteristics
1 Involvement of one lymphatic gland/ single extra lymphatic organ
2 Involvement of two or more lymph gland regions on the same side of
diaphragm / localised involvement of extra lymphatic site
2
3 Involvement of glands in regions on both sides of diaphragm /
localised involvement of extra lymphatic organ or site or spleen.
4 Disseminated involvement of one or more extra lymphatic organ with
or without associated lymph gland involvement 3
4A Asymptomatic
4B Fever ,sweating, weight loss,
4C Bulky tumour more than 10 cm in size ,mediastinal mass
4
Diagnostic Evaluation
1.History And Physical 8.Liver Biopsy

Examination
2. Complete Blood Count 9.Chest X Ray
3. Liver Function Test , 10. Lymph Node Biopsy

Renal Function Test
4 Flurodeoxyglucose PET Scan 11. Echocardiography ,
and CECT Scan Pulmonary function test
5.Bone Marrow Biopsy
12. Lymph angiography
6.Pleural Cytology
Treatment
.
• 3 cycles of chemo
• Stag
Field radiation Stage
• Field irradiation therapy Stage 12 courses of
IIa •Stage
Total gland 3 b chemo therapy
eand
Ia • 6 month
and plus radiation
•&Chemo therapy and chemotherapy
II IIIa
irradiation
Ia IV
b • 9 month chemo
therapy
Chemotherapy : new combination therapies
• MOPP
• COPP
• ABVD+ Rituxan
• BEA COPP
• OPPA+COPP(females)
• OEPA+COPP(males)
• ABVE -PC OR (DBVE-PC )
• ICE + Brentuximab
• IFOS/VINO +Bretuximab
Radiation Therapy
(only for patients with bulky disease)
• Low dose radiation therapy (15

-25Gy)
• Adopt measures to reduce
1.Mantle field
infertility
• Sperm banking
• Testicular shield
2. Para aortic
• In order to protect ovaries
Inverted T
transposition of ovaries to midline.
3. Pelvic
Complications
CHEMOTHERAPY
• Lung • Lung • Sepsis
RADIATION THERAPY
SURGERY
capacity toxicity • Adhesion
restricted • Cardiac • Malignancy
• growth toxicity as
decreases • Sterility secondary
• Sterility
Advances in Hodgkin lymphoma research
1. Targeted therapy :
A protein CD30 is found in HL cells, drug Brentuximab vedotin
target this protein
2. Immunotherapy
Used in recurrent HL
3. Immune checkpoint inhibitors

Non Hodgkin Lymphoma
Non Hodgkins Lymphoma
• The malignancy involving lymphoid organ
• it is due secondary to Epstein Barr virus (EBV) , Burkitts lymphoma
(African lymphoma ) arises due to this viral infection
• A mosquito plays a role in its transmission
T lymphoblastic
lymphoma 5 to 20%
Non mature
B lymphoblastic
lymphoma3%
Paediatric non Hodgkin

Types of Burkitt like 35 to 40 %
lymphoma
pediatric NHL DLBCL
(WHO ) B cells Primary mediastinal
Follicular lymphoma (rare)
Mature Paediatric nodal marginal

zone lymphoma
ALCL
T cell
Peripheral T cell
lymphoma
Incidence
0 TO 14 YEARS 15 TO 19 YEARS
6%
19%
1% 17%
29%
21%
38%
37%
FOLLICULAR
BURKITT LYMPHOBLASTIC
DLBCL 10% BURKITT
ALCL DLBCL
OTHERS 20% ALCL
LYMPHOBLASTIC OTHERS
Etiology
• Risk factors are
• Immunodeficiency (acquired immunodeficiency syndrome)
• Post transplant immunosuppression
• Post transplant lymphocytic proliferation
• Previous neoplasm (secondary malignancy )
Burkitt lymphoma
• 50% of childhood NHL are of this type

1.
• It is seen in Waldeyers ring, abdomen
and GIT
• Malignant cells show a mature B cell
structure and tumour appears as
“starry sky “
.2.
1 and 2 showing Typical starry-sky
appearance
of a case of Burkitt lymphoma
Pathophysiology
Predisposing factors Precipitating factor
Differentiation in peripheral lymphoid tissue
Malignant transformation of either T or B cells
T cell proliferate on antigenic stimulation and migrate into follicles
These activated follicles become germinal centres

containing macrophages, follicular dendrite cells T and B cell
Develops in lymphoid tissue
Spread to all
MODIFIED ST JUDE’S CLINICAL STAGING SYSTEM FOR NON-
HODGKIN LYMPHOMA
Stage 1(low risk)
• One single site

extra nodal
• Single anatomic
area nodal
Stage 2(low risk)
Stage II
• Single tumour extra
nodal with regional
node involvement
• 2 or more nodal mass
on same side of Ileocecal
diaphragm ,2 single
extra nodal tumour
• Primary
gastrointestinal
tumour
Stage 3 (advanced)
• Stage III
• all primary intra
thoracic tumour
• Intra abdominal , two
or more nodal or
extra nodal areas on
both side of
diaphragm
Stage 4 (advanced)
Any of the above

with initial
involvement of
bone marrow or
CNS
Clinical features
• Acute abdomen ( site is lymphoid tissue in GIT in ileocecal region)
• Non tender enlargement of lymph nodes
• Manifestations due to compressions , mediastinal compression,
• Burkitt lymphoma
• Tumour in jaw
• Abdominal lumps
• Lymphoblastic Lymphoma
• dyspnea, anterior mediastinal mass, wheezing ,stridor, dysphagia, swelling of
head and neck
Diagnostic Evaluation
1. History Physical Examination
2. Complete Blood count

3. X Ray , CT Scan , Lungs and GI, MRI
4. Biochemistry : BUN, Uric Acid ,LDH, Ca, Serum

Electrolyte, Phosphorus
8. Bone Marrow Aspiration and
5. PEt Scan and USG abdomen
Biopsy
6. Lumbar Puncture
9. Immune Phenotyping
7. Cytogenetic Evaluation And Molecular Studies
Complications
• Superior vene cava syndrome
• Tumour Lysis syndrome
• Respiratory or cardiac compromise
• Paraplegia
• Obstructive jaundice
• GI bleeding
• Airway obstruction
• Superior mediastinal syndrome
Principles of management
1. Chemotherapy
2. Surgery has very limited role
3. Radiation therapy is very rare
Treatment based on severity
• Short intensive pulsed
In localised B NHL - Burkitt NHL chemotherapy for 2 to 3 month
• Cyclophosphamide, Methotrexate,,
Prednisolone , Dexamethasone
• 80 to 90% survival
Disseminated childhood B Cell • Short intensive chemotherapy for 5 to
6 month
non Hodgkin lymphoma
• Multiagent protocols
• Rituximab
Treatment
• 80%survival with chemotherapy
• Drugs
Disseminated Childhood • Prednisolone, Dexamethasone,
Vincristine, Danurubicin, L-
Lymphoblastic Lymphoma asparginase,
cyclophosphamide,
methotrexate,
metacaptopurine
Disseminated Anaplastic • Survival is 60 to 70%

Lymphoma • Chemotherapy and autologous
allogenous HSCT (long term survivors)
Hematopoietic Stem Cell Transplantation
• In case of recurrent NHL or relapsed patients with Burkit NHL,LL chemotherapy along with HSCT is
having survival rate of 10 to 30 %
• In case of ALCL chemotherapy along with autologous or allogenous HSTC has long term survivors
• EBV Specific cytotoxic T lymphocytes can be generated from allogeneic donors for advanced
Hodgkin .
Bone marrow transplant
• In case of relapse Autologous bone marrow transplant from identical

twins is effective
Novel Therapeutic Approaches
1. Target therapy : Rituximab or

Ibrutinb
2. Immunotherapy : CAR –T cells
3. Immuno modulating drugs
T cell
(red and blue spheres)
attacking cancer cells
T cell
Lymphoma nursing care plan
1. Ineffective breathing pattern related to tracheobronchial obstruction.

2. Ineffective airway clearance related to obstruction due to enlarged
mediastinal masses.
3. Sexual dysfunction related to disturbed body structure and function
due to chemotherapy, radiation therapy.
4. Fatigue related to decreased energy production or chemotherapy.
5. Deficient knowledge related to lack of exposure, unfamiliarity or mis
interpretations
6. Interrupted family process related to situational crisis .
Lymphoma nursing care plan
• Pain related to diagnosis, treatment ,physiologic effects of neoplasiaa
• Imbalanced nutritional status less than body requirement related to
loss of appetite.
• Impaired skin integrity related to administration of chemotherapeutic
agents, radiotherapy.
• Body image disturbance related to loss of hair, side-effect of
chemotherapy .
• Risk for injury related to malignant process.
• Risk of infection related to depressed body defences.
Care of lymphomas
• Care of child undergoing chemotherapy
• Care of child undergoing radiotherapy
• Monitoring lab test , especially blood values
• Parent education in relation to low immunity of child
• Patient and family emotional and psychological support
Conclusion
• Paediatric lymphomas is a most common cancer .
• Advancement in medicine increased the survival rate is more than 80
percent for both Hodgkin and Non Hodgkin lymphoma.
• But ,long term complications like sterility, solid tumour, cardiac ,
pulmonary disease have reported.
• Now efforts are made to reduce relapse by risk stratification
approach.
References
• Parthsarathy A . Piyush Gupta . Agarwal Rohit. IAP Textbook of paediatrics
5th edition’ Newdelhi :Jaypee brothers medical publications :2013
• Gupte Suraj. The Short Textbook Of Paediatrics .12th Edition. New delhi;
The Health Science Publisher :2018
• Hockenberry J Marlin, Wilson David, Rodgers C Cheryl. Essentials of
Paediatric Nursing. Second South Asian Edition. South asia; Elseveir
Publications: 2019
• Marlow D R, Redding Textbook of Paediatric Nursing 4th Edition Singapore
Harwourt ;Brace &Company: 2013.
•
References
• Indian council of medical research consensus document for
management of paediatric lymphomas and solid Tumour : New
Delhi ;2017

Pediatric Lymphoma

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What are the different types of lymphomas discussed?

What are the different types of lymphomas discussed?

What are the stages of lymphoma according to the modified St. Jude's clinical staging system?

What are the stages of lymphoma according to the modified St. Jude's clinical staging system?

Paediatric lymphomas

• First described in 1832 by

• Paediatric lymphoma is highly curable malignancy.

• Hodgkin Lymphoma – 6% of child hood cancers

• Hall mark of HL is All Hodgkin

• Multinucleated / bi lobed nucleus

1. Nodular : large cell, multilobed (popcorn cells).localised, nonbulky

EBV GENE EXPRESSION IN REED STERNBERG CELLS

1.History And Physical 8.Liver Biopsy

3. Liver Function Test , 10. Lymph Node Biopsy

• Low dose radiation therapy (15

3. Immune checkpoint inhibitors

Paediatric non Hodgkin

(WHO ) B cells Primary mediastinal

Follicular lymphoma (rare)

Mature Paediatric nodal marginal

• 50% of childhood NHL are of this type

Differentiation in peripheral lymphoid tissue

Malignant transformation of either T or B cells

T cell proliferate on antigenic stimulation and migrate into follicles

These activated follicles become germinal centres

Develops in lymphoid tissue

Stage 1(low risk)

• One single site

Any of the above

1. History Physical Examination

2. Complete Blood count

4. Biochemistry : BUN, Uric Acid ,LDH, Ca, Serum

Disseminated Anaplastic • Survival is 60 to 70%

• In case of relapse Autologous bone marrow transplant from identical

1. Target therapy : Rituximab or

1. Ineffective breathing pattern related to tracheobronchial obstruction.

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