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 Hodgkin lymphoma (HL) is characterized by

progressive enlargement of the lymph nodes.



It is considered unicentric in origin and has
a predictable pattern of spread by extension
to contiguous nodes.
 Etiology is unknown.

 worldwide incidence of HL is approximately


2 - 4 new cases/100,000 population/yr

 HL accounts for approximately 5% of cancers


in persons 14 yr of age or younger;
 it accounts for approximately 15% of cancers

in adolescents (15-19 yr of age)

Ref: Nelson textbook of


Pediatrics 20th ed.
paediatric hematology- philip Lanzkowsky
 EBV (mixed cellularity subtype)
 Family history of HL
 Low socioeconomic status
 Reed-sternberg (RS) cell is the hallmark of
Hodgkin lymphoma.
 It is a large cell (15-45 µm) with multiple or

multilobulated nuclei.
 It is neoplastic clone cell originating from B

lymphocyte in lymphnode germinal centers.


 It can’t synthesize immunoglobulin due to

dysregulation of nuclear of nuclear factor


kappa B (NFĸB).
 Lymphadenopathy ( 90% cases)
◦ Usually painless
◦ Cervical L N/ supraclavicular LN are involved in
60- 80%
◦ Discrete, elastic/rubbery, nontender
◦ Spreads mostly by contiguity from one chain to
another
 Mediastinal adenopathy (60%):
◦ 20% of patient have bulky mediastinal disease.
◦ Persistent nonproductive cough
◦ Superior vena caval symptoms
 Enlargement of neck vessels
 Hoarseness of voice
 Dyspnoea
 Dysphagia
 Splenomegaly

 Systemic symptoms:

◦ Pel-Ebstein fever
◦ Weight loss >10% in 6 months B
◦ Drenching night sweats symptoms

◦ Mild itching may be present in 15-25% of cases but


it is not considered as B symptoms
 Other less common manifestations are
◦ Pulmonary manifestation (17%)
◦ Neurological manifestation (late presentation)
◦ Bone disease(2%)
◦ Bone marrow infiltration(5%)
◦ Liver disease (2%)
◦ Renal manifestation
 Haematological manifestation:
◦ Anemia
◦ Neutropenia(50%)
◦ Lymphocytopenia-Due to hypersplenism or BM
infiltration
◦ Eosinophilia (50%) – due to IL-5 production
◦ In advance stage DAT test frequently positive with
hemolysis
◦ Immune thrombocytopenia may be present in 1-
2%
cases
Note:
Can be further subclassified as
A catagories- Asypmtomatic
B catagories- presence of B symptoms
1) CBC: Normocytic normochromic anemia
Neutrophilia in 50% cases Eosinophilia
in 50% cases
Lymphocytopenia
ESR: raiesd
2) S. ferritin: raised
3) CXR: both PA & Lateral view
Mediastinal lymphadenopathy
4) Lymphnode biopsy:
Presence of RS cell with diffuse infiltration of
lymphocyte,histiocyte and many eosinophil &
plasma cell
CXR showing
mediastinal mass
For staging:
1) CT scan of neck, chest, abdomen, pelvis

2) Positron emission tomography (PET) scan

3) Technitium-99 bone scintography

For classification:
1) Immunohistochemistry
1) Liver function test
2) Renal function test
3) S. electrolyte
4) S. uric acid
5) S. inorganic PO4
6) S. calcium
7) DAT
 In general
◦ Combined Chemotherapy Considere
◦ Low dose involved field radiation d Standard
therapy

 Intensity of chemotherapy & volume of


radiation depends on
◦ Presence of B symptoms
◦ Initial disease staging
◦ Presence of bulky disease
Chemotherapy regimen Corresponding agents
ADVD Doxorubicin (Adriamycin),
bleomycin,
vinblastine, dacarbazine

ABVD-Rituxan Doxorubicin (Adriamycin),


bleomycin,
vinblastine, dacarbazine,
rituximab

COPP Cyclophosphamide, vincristine


(Oncovin),
prednisone, procarbazine

OPPA ± COPP Vincristine (Oncovin), prednisone,


procarbazine, doxorubicin
(females (Adriamycin),

OEPA ± COPP Vincristine (Oncovin), etoposide,


prednisone,
(males) doxorubicin (Adriamycin),

BEACOPP Bleomycin, etoposide, doxorubicin


(Adriamycin), cyclophosphamide,
(advanced stage) vincristine
(Oncovin), prednisone,
procarbazine
 Most relapse occurs in 1 s t 3year after
diagnosis, but relapse after 10 year have
been reported.
Treatment of relapse
Nature of relapse treatment
Relapse with Chemotherapy + LD-IFRT
favorable at diagnosis
Relapse with high risk Chemotherapy +
disease Autologous
HSCT
Relapse with in 12 Chemotherapy +
month of diagnosis Autologous
HSCT
+ radiotherapy
 With the use of current therapeutic regimens,
Disease stage event-free Overall
survival survival (OS)
(EFS)
Early-stage disease + 85-90% >95%
favorable prognostic
factors
Advanced-stage 80-85% 90%
disease

 With dose intense chemotherapy OS has


approached to 100%
 Advanced stage of disease (Stage IIB, IIIB, or IV)
 The presence of B symptoms
 The presence of bulky disease
 Extranodal extension (liver)
 Male sex
 Elevated erythrocyte sedimentation rate
 White blood cell count 11,500/mm3 or higher
 Hemoglobin less than 11.0 g / d l
 Histology : classical HL
 Initially not respond to chemotherapy
 Secondary malignancy
 Cardiac toxicity
 Pulmonary dysfunction
 Thyroid dysfunction
 Gonadal dysfunction & infertility
 Growth retardation
 Psychosocial problem
 During therapy
◦ Physical exam (LN, Liver, spleen)
◦ Lab: CBC, ESR, LFT
◦ Imaging : CT scan, PET
◦ Organ toxicity monitoring: cardiac function test,
Pulmonary function test
 Disease monitoring after treatment: by CXR,
CT scan
 Long term sequelae monitoring: life long

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