Lymphoma

Lymphomas
Definitions:

Malignant disease of lymphatic tissues.

common malignant disease in childhood
(3rd )
2 types:
- Hodgkin’s lymphoma.
- Non Hodgkin lymphoma (more Common
in Pediatrics).
 Hodgkin’s Disease

One of malignant lymphomas.

Affect males > females 3:1.
Age: 5-15Ys.
It is unicentric in origin.
 Etiology

Unknown But there are

theories. e.g Related
to Epstein-Barr Virus
(EBV) infection which
can cause infectious
mononucleosis.
•Some countries have very high
prevalence of EBV positivity in HD

e.g. Iran, Greece, Kenya 92-94%

Nodal mass
≥ 6Cm
Clinical Picture
(Bulky)
Most common presentation is “peripheral L.N
Enlargement”. (90% of cases).
Most common:
- Cervical 60-80%
- Axillary 20%
- Inguinal 5%.
Characters of L.N.:
- Consistancy → Rubbery.
- Painless.
- Not tender.
Mediastinal syndrome → as it can be presented by
mediastinal L.N enlargement (60% of cases).
Bulky mediasinal
mass
Mass/thoracic
ratio ≥ 0.33%
Bulky mediasinal mass
Mass/thoracic ratio ≥ 0.33%

Internal transverse diameter of

a thorax between T5 and T6
--Maximum inspiration
--Upright position
 Clinical Picture
Splenomegaly → stage of ds is at least stage III.
Hepatomegaly & presence of Enlarged L.N at porta
hepatis → Obstructive jaundice.
Bone marrow involvement which may be presented by:
◼Pallor → due to Anemia
◼Bleeding manif.→due to thrombocytopenia.

Systemic symptoms: 30%(Bsymptoms)

◼Intermittent fever (pel-Epstien).
◼Night sweats.

◼Loss of weight >10% during last 6 months.

Clinical Picture
Itching

--15-25% of patients

--Severe ------- advanced stage

--B-symptoms

--Return -------- Relapse

Clinical Picture
CNS manifestation
Intra-cranial infiltration
Metabolic ↑ Ca, ↓ Na, ↓ glucose

Infection (Herpes, Meningitis, abscess)

Chemotherapy-related (oncovin-steroid)

Radiotherapy related.

Paraneoplasmic manifestations
Clinical Picture
Jaundice

--Hepatic involvement.
--Hemolytic anemia
--Hepatitis (viral-Toxic-Toxoplasma)
--Enlarged L.Ns in the porta hepatis
--Blood transfusion
Clinical Picture
Hematologic

--Immune hemolytic anemia:

--Immune thrombocytopenia
--Immune neutropenia

--Bone marrow suppression / infiltration

--Eosinophilia (15% of patients)

 Diagnosis
Ultrasound Grey scale evaluation of
lymphomatous nodes:
1. Size: LN in lymphoma tend to be
larger in size (progressive nodal
enlargement on serial follow up is more
important)
2. Shape: round in shape
1. Borders: well-defined (except if
infiltrating adjacent structure)
2. Echogenicity: Hypoechoic without
echogenic hilus
Suspicoius LN Reactive LN
 Diagnosis
I- L.N Biopsy:
⚫The most important diagnostic test.
⚫Biopsy should be taken from the large L.N Bec. it is the
pathologic one.
 Diagnosis
Presence of Reed-sternberg
giant cells surrounded by benign
inflammatory cells is diagnostic
for Hodgkin’s disease.
Accordingly the disease is
histopatholgically classified into
4 types (Rye classification):

◼Lymphocyte predominance: Best prognosis.

◼Lymphocyte depletion: Worst prognosis.

◼Mixed cellularity: Most common in children.

◼Nodular sclerosis: Most common in adults.

 Clinical staging of the disease:
Ann Arbor classification
Stage Description

I Single lymph node region

or
of a single extralymphatic organ or site (IE).
II Two or more lymph node regions on the same side of the diaphragm
or
localized involvement of an extralymphatic organ or site and of one more
lymph node regions on the same side of the diaphragm (IIE).

III Lymph node regions on both sides of the diaphragm

or
spleen, splenic hilar node, celiac node, porta hepatis node (IIIS).

IV Diffuse or disseminated involvement of one or more extralymphatic

organs or tissues with or without associated lymph node enlargement.

Each stage is further subdivided into A or B according to absence (A) or

presence (B) of systemic symptoms.
Clinical staging of the disease:
Ann Arbor classification
Risk stratification of Hodgkin
lymphoma
Risk group Risk Criteria

Low risk Stage I or II without B

symptoms or bulky disease
(IA,IIA)
Intermediate Stage IA,IIA with bulk disease
risk Or IB,IIB, IIIA and IVA
High risk IIIB and IVB

COG, Children Oncology Group

 Diagnosis
II- Radiologic studies:
⚫Plain X-ray chest: to detect mediastinal L.N or lung
disease.
⚫Abdominal ultrasound.
⚫C.T scan: neck, chest, abdomen, pelvis.
⚫MRI.
⚫PET
scan
 Diagnosis
III- Hematologic studies:
⚫Complete blood picture: anaemia and leucopenia.
⚫Bone marrow exam: for B.M infiltration.
IV- Biochemical studies:
⚫ ESR
⚫ Serum Copper level.
⚫ Serum ferritin.
⚫ Fibrinogen and haptoglobin level.
⚫ Serum lactic dehydrogenase and alkaline phosphatase
levels.
These biochemical changes are indicative of active
disease.
 Prognosis
Good prognosis is suspected with:
▪Young age.
▪Stages I or II.
▪Females.
▪Lymphocytic predominance
▪ type.
▪Absence of systemic
symptoms (B symptoms)
▪Absence of bulk disease
 Treatment
Combined chemotherapy &radiotherapy are the standard
of care)
Radiotherapy: for intermediate and high risk patients
2100cGy
Chemotherapy: ABVD protocol in cycles ( on D1 &D15)
Low risk : 4 cycles ± IFRT
Intermediate risk: 6 cycles + IFRT
High risk: 8 cycles + IFRT
Adriamycin I.V.
Bleomycin I.V.
Vinblastin I.V.
Decarbazine I.V.
Autologus Bone marrow transplantation: for recurrent and
resistant disease.
Non Hodgkin lymphoma (NHL(

Accounts for 60% of all childhood

lymphomas.
Affects males > females. 2.5:1.
Multicentric in origin.
Age: from 3 years old
 Clinical Picture
Abdominal mass (Burkitt lymphoma) is the most common
clinical presentation in our country.
Peripheral L.N enlargement: cervical, axillary,
inguinal…etc. The glands are usually firm (not rubbery),
painless and not tender.
Mediastinal L.N enlargement → mediastinal syndrome.
Hepatosplenomegaly may occur.
Bone marrow infiltration may occur → anemia, leucopenia
and thrombocytopenia.
 Diagnosis

Lymph node biopsy.

Bone marrow examination to
differentiate NHL from leukemia.
Peripheral blood exam.
Radiologic studies as for Hodgkin’s
disease.
 Clinical Staging

As for Hodgkin’s disease but it is

not sub classified to A or B. (because
the disease is more aggressive than
Hodgkin’s disease).
 Treatment
Surgery: for emergency situation (intestinal obstruction).
Chemotherapy:
◼The most important line of treatment (COMP protocol)
Durgs used:
◼Cyclophosphamide.
◼Vincristine.

◼Methotrexate.

◼Prednisone.

Radiotherapy: for emergency situation e.g. SVC or

Mediastinal syndrome
Bone marrow transplantation: for recurrent and resistant
diseases
(Allogeneic).
 T
H
A
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You
Evidence of Relation to EBV?
- Occur in more than one member of the family.
-Increased in twins.
-Characteristic cells of Hodgkin’s lymphoma
contain EBV.
Value of CNS Examination?
-CNS infiltration
-Intracranial haemorrhage: thrombocytopenia
-Effect of chemotherapy
-Menigitis & meningeo-encephalitis
-Corticosteroid therapy
-Malignancy associated psychosis
A child with Hodgkin’s presented
with jaundice, What’s DD?
-L.N in porta-hepatis→ Obstructive jaundice.
-Associated Hepatitis: * Blood transfusion
* EBV
-Auto-immune haemolytic anaemia→ Jaundice
with =ve coomb’s test.
-Toxic hepatitis due to chemotherapy.
-Incompatible blood transfusion.
A child with Hodgkin’s presented
with jaundice, What’s DD?
-Bone marrow involvement→ Aneamia.
-Auto-immune haemolytic anaemia.
-Fe deficiency anaemia : failure of fe
incorporation in haem molecule.
-Effect of chemotherapy.