Lymphoma Shofik EDITED BY SAYAM 8.53PM

Lymphoma
BY
DR. SAFIKUL ISLAM

PHASE-B RESIDENT
DEPT. OF PAEDIATRIC SURGERY, MMCH
Introduction
Lymphoma are a result of chromosomal alterations resulting in the uncontrolled
growth of cells of lymphoid origin.
Incidence: 3rd most common type of cancer in children, accounting for 11-21% of
paediatric cancer.
Hodgkin Lymphoma accounts for 6% of all pediatric malignancies,
Bimodal distribution : adolescence (15-19) and after 55 years.
HL is exceedingly rare in children below 5 years.
Types:
1.Hodgkin Lymphoma
2.Non-Hodgkin Lymphoma.
HODGKIN LYMPHOMA
 Hodgkin Lymphoma: wide spectrum of histopathologic and clinical presentation.
Cure rate : 90-95%.
Despite these excellent rates of cure, treatment can result in significant short term
and long term morbidity.
WHO classification :
WHO classified HL into two major types:

 1.Classic Hodgkin lymphoma.
 2.Nodular lymphocyte predominant Hodgkin lymphoma (LPHL)
Classic Hodgkin lymphoma is further divided into four subtypes :

 1.Nodular sclerosis (most common in children)
 2.Mixed cellularity (MC).
 3.Lymphocyte rich(LR).
 4.Lymphocyte depleted (LD).

Classic cell include :
-Reed sternberg cell.
-Lymphocyte.
-Histiocyte.
Immune system dysfunction is one of the causes of HL .
In childhood - its Immune immaturity. In adult- Immune dysregulation.
Risk factor:-Increasing family size.
-Lower socioeconomic status.
-Exposure to Epstein barr virus.
The adolescent young adult (AYA) has no gender predilection and the most
common form is nodular sclerosis.
Risk factor:
-Higher socioeconomic status.
-Early birth order.
-Smaller family size.
-Epstein barr virus.
 Hodgkin Lymphoma is derived from a single transformed B cell that has
undergone monoclonal expansion.
CLINICAL PRESENTATION
Classical presentation:-
Painless rubbery and fixed lymph node-
Tumor lysis syndrome.
Mediastinal disease and other constitutional symptoms known as B symptoms
may appear with significant respiratory compromise due to compression of
trachea, carina or both including major bronchi.
Exertional dyspnoea, persistent cough or stridor
Sign of superior venacava obstruction including oedema and cyanosis of the face,
jugular venous distension.
Extralymphatic involvement in liver,lungs,bone,bone marrow and skin.
About one fourth patient will have one or more B symptoms like:
a) Unintentional weight loss- 10% in the previous 6 month.
b) b)Unexplained fever greater than 38°c.
c) c) Drenching or soaking night sweats-Pruritus,fatigue and anorexia also

common.
STAGING
The Ann Arbor staging system and its costeolds modification remain the standard
for adult and Pediatric HL.
Clinical staging requires complete history, physical examination.
Basic test should include :
*Blood cell count with differential.
*Lactate dehydrogenase.
*Alkaline phosphatase.
*ESR or CRP.
*Baseline hepatic and renal function test.
*Electrolytes.
*Chest radiograph.
*CT scan of neck,chest,abdomen and pelvis.
*Bone marrow biopsy reserved for patient with B symptoms and stage lll IV
disease.
* FDG-PET scan replacing Gallium scan and CT scan.
*MRI provides a more accurate evaluation of disease in the abdomen compared
with CT, with better visualization of fat-encased retroperitoneal nodes.
Radiotherapy was reported the first curative treatment.
Single agent chemotherapy was used to treat HL in 1946.
Multi agent treatment with
MOPP(Mechlorethamine,Oncovin,Procarbazine,Prednisone) was reported
in1967.
Currently, biologically based therapies,both immunotherapy and small molecule
are being investigated for use as primary and release therapy.
TREATMENT
Risk classification : 3 catagories

-Low risk disease: Defined as classical Hodgkin lymphoma patient, with clinical
stage l or ll disease showing no B symptoms or bulky nodal involvement (>10cm)
and disease in fewer than three nodal region.
- Intermediate risk disease : Includes stage l,ll and sometimes lllA disease. Some
trials have included B symptoms, Bulky disease,a large number of involved nodal
region and extranodal involvement of disease.
- High risk disease : Are those with stage lllB and lVA/B disease.
 Chemotherapy and radiation therapy are the mainstay treatment
 SURGERY
 The role of surgery in the initial diagnosis and staging for HL has been reduced,
Primary role is to obtain tissue for diagnosis.
 Due to wide application of chemotherapy in all stage of HL,surgical staging has
become irrelevant.
 Biopsies should be taken from most easily accessible site and sent fresh to
pathology for immunohistochemistry, Immunophenotyping, Cytogenetics and
flow cytometry.
TREATMENT TOXICITIES
*Decreased stature.
*Cardiopulmonary dysfunction.
*Thyroid disease.
*Infertility.
*Second Malignancies.
*Impaired Psychosocial functioning.
*Decrease in health related quality of life.
Non hodgkins lymphoma
Non Hodgkin Lymphomas(NHLs) comprise a heterogeneous group of tumors.
Types
On cell of origin On clinical behaviour :

 Indolent.
 Mature B cell Neoplasm.
 Aggressive.
 Mature T cell and NK cell Neoplasm.
 Highly aggressive.
Indolent Lymphoma are slowly progressive but incurable disease with median
survival time of 8-10 years.
Aggressive Lymphomas, such as Burkitt and Burkitt like Lymphomas, are rapidly
progressive at presentation but curable in 70% to 90% of patient.
INCIDENCE EPIDEMIOLOGY AND CLASSIFICATION :
 NHL : 7% of cancer in children and adolescent.
 Rare at less than 5 years
 More common in male 1.1 to 1.4:1 with a higher frequency in whites than in
black.
 A family history of a hematologic malignancy produces an increased risk.
Some DNA and RNA virus also play role in pathogenesis of NHL like :
 -Epstein barr virus.
 -Human lymphotropic virus Type 1(HTLV-1).
 -Human herpes virus 8.
 -HIV virus.
 -Bacterial overgrowth, Helicobacter pylori infection also responsible for

development and proliferation of Lymphomas.
Clinical presentation :
 *NHL must be considered in any children with lymphadenopathy.
 *Most children with B cell lymphoma present with a palpable abdominal or
mediastinal tumor.
 *Present as a mass in right iliac fossa confused with Appendicitis or
Appendiceal abscess.
 May also present as intussusception bleeding ascites or bowel perforation.
 Present as respiratory distress due to mediastinal mass.
 Superior venacava syndrome and respiratory distress are common in NHL.
Many children with NHL will present with advance stage disease including
 Bone marrow involvement.
 Malignant pleural or pericardial effusion.
 Patient may present with B symptoms, which reflects prognostic significance

and occur in up to one third of the children with NHL.
staging
The children oncology group divides

NHL into two categories.
 Limited : Disease corresponds to stage l and stage ll.
 Extensive : Correlates with stage lll and lV.
Most widely used staging system for NHL is st Judes Murphy staging system
HISTOLOGIC SUBTYPE
 *Burkitt lymphoma
 *Lymphoblastic lymphoma
 *Anaplastic large cell lymphoma (ALCL)
 *DLBCL
*Children initially suspected of having NHL should be evaluated immediately
because of the high risk of either metabolic and anatomic complication before
therapy begins.
* Rapid growth of these tumor, may create life threatening complication over
night in a child who seemed healthy the previous day.
PROGNOSTIC RISK FACTOR
*When all patient are treated similarly, the stage of Lymphoma at diagnosis is a
strong prediction of outcome.
* CNS involvement both Burkitt lymphoma and Lymphoblastic lymphoma is
associated with worse prognosis.
* Patients older than 15 years of age.
* An elevated LDH also predicts a worse prognosis
TREATMENT
*NHL is extremely chemo sensitive

*The treatment regimen used for Burkitt Lymphoma and diffuse large B cell
lymphoma (DLBCL) is Cyclophosphamide, Vincristine, Doxorubicin, Etoposide,
prednisone.
* Therapy for Burkitt lymphoma and Diffuse large cell lymphoma (DLCL) dose
of Cytarabine and Methotrexate increased along with above mentioned regimen.
* Humanized monoclonal antibody is also used.
Prognosis
*Over the past several decades considerable improvement has been occured in
disease free survival (DFS) and overall survival(OS).
* Today, more than 90% of children with NHL will achieve complete remission.
*The 5 years survival rates for children ages birth to 14 and 15 to 19 years
diagnosed were 90.7% and 86% respectively .
* Patient with localized disease regardless of histologic subtype event free
survival ( EFS) typically exceeding 80%.
EARLY COMPLICATION
*Patient may initially have a constellation of significant metabolic derangement

known as Tumor Lysis Syndrome.
* Sometimes presents with renal failure in 30% Cases.
*Infection is much greater risk for NHL.
* Bone marrow suppression.
*Chemotherapy itself may cause acute complication including severe chemical
burn due to extravasation of certain vesicant agent (Vincristine, Anthracycline).
LONG TERM SEQUELE
 With current therapy for NHL long term complication include :
 -Cardiac toxicity.
 -Infertility.
 -Secondary Leukemia.
 -Anthracycline related cardiomyopathy.

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Lymphoma

DR. SAFIKUL ISLAM

WHO classified HL into two major types:

 2.Nodular lymphocyte predominant Hodgkin lymphoma (LPHL)

Classic Hodgkin lymphoma is further divided into four subtypes :

 2.Mixed cellularity (MC).

 4.Lymphocyte depleted (LD).

b) b)Unexplained fever greater than 38°c.

c) c) Drenching or soaking night sweats-Pruritus,fatigue and anorexia also

Risk classification : 3 catagories

Non Hodgkin Lymphomas(NHLs) comprise a heterogeneous group of tumors.

On cell of origin On clinical behaviour :

 Rare at less than 5 years

 -Human lymphotropic virus Type 1(HTLV-1).

 -Human herpes virus 8.

 -Bacterial overgrowth, Helicobacter pylori infection also responsible for

 Malignant pleural or pericardial effusion.

 Patient may present with B symptoms, which reflects prognostic significance

The children oncology group divides

 Extensive : Correlates with stage lll and lV.

 *Anaplastic large cell lymphoma (ALCL)

*NHL is extremely chemo sensitive

*Patient may initially have a constellation of significant metabolic derangement

 -Anthracycline related cardiomyopathy.

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