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Dr. (Major) Jahangir Alam
MBBS, DCH, FCPS
Classified child specialist
CMH Dhaka, Bangladesh.
 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 5th ed
 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 LN/ 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
◦ Drenching night sweats
◦ Mild itching may be present in 15-25% of cases but
it is not considered as B symptoms
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
◦ Low dose involved field radiation
 Intensity of chemotherapy & volume of
radiation depends on
◦ Presence of B symptoms
◦ Initial disease staging
◦ Presence of bulky disease
Considered
Standard
therapy
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
(females
Vincristine (Oncovin), prednisone,
procarbazine, doxorubicin
(Adriamycin),
OEPA ± COPP
(males)
Vincristine (Oncovin), etoposide,
prednisone,
doxorubicin (Adriamycin),
BEACOPP
(advanced stage)
Bleomycin, etoposide, doxorubicin
(Adriamycin), cyclophosphamide,
vincristine
(Oncovin), prednisone,
procarbazine
 Most relapse occurs in 1st 3year after
diagnosis, but relapse after 10 year have
been reported.
 Treatment of relapse
Nature of relapse treatment
Relapse with
favorable at diagnosis
Chemotherapy + LD-IFRT
Relapse with high risk
disease
Chemotherapy +
Autologous HSCT
Relapse with in 12
month of diagnosis
Chemotherapy +
Autologous HSCT
+ radiotherapy
 With the use of current therapeutic regimens,
 With dose intense chemotherapy OS has
approached to 100%
Disease stage event-free
survival
(EFS)
Overall
survival (OS)
Early-stage disease +
favorable prognostic
factors
85-90% >95%
Advanced-stage
disease
80-85% 90%
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/dl
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
Hodgkin Disease
Hodgkin Disease

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Hodgkin Disease

  • 1. Dr. (Major) Jahangir Alam MBBS, DCH, FCPS Classified child specialist CMH Dhaka, Bangladesh.
  • 2.  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.
  • 3.  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 5th ed
  • 4.  EBV (mixed cellularity subtype)  Family history of HL  Low socioeconomic status
  • 5.  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).
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  • 8.  Lymphadenopathy ( 90% cases) ◦ Usually painless ◦ Cervical LN/ supraclavicular LN are involved in 60- 80% ◦ Discrete, elastic/rubbery, nontender ◦ Spreads mostly by contiguity from one chain to another
  • 9.  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
  • 10.  Splenomegaly  Systemic symptoms: ◦ Pel-Ebstein fever ◦ Weight loss >10% in 6 months ◦ Drenching night sweats ◦ Mild itching may be present in 15-25% of cases but it is not considered as B symptoms B symptoms
  • 11.  Other less common manifestations are ◦ Pulmonary manifestation (17%) ◦ Neurological manifestation (late presentation) ◦ Bone disease(2%) ◦ Bone marrow infiltration(5%) ◦ Liver disease (2%) ◦ Renal manifestation
  • 12.  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
  • 13. Note: Can be further subclassified as A catagories- Asypmtomatic B catagories- presence of B symptoms
  • 14. 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
  • 16. 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
  • 17. 1) Liver function test 2) Renal function test 3) S. electrolyte 4) S. uric acid 5) S. inorganic PO4 6) S. calcium 7) DAT
  • 18.  In general ◦ Combined Chemotherapy ◦ Low dose involved field radiation  Intensity of chemotherapy & volume of radiation depends on ◦ Presence of B symptoms ◦ Initial disease staging ◦ Presence of bulky disease Considered Standard therapy
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  • 20. 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 (females Vincristine (Oncovin), prednisone, procarbazine, doxorubicin (Adriamycin), OEPA ± COPP (males) Vincristine (Oncovin), etoposide, prednisone, doxorubicin (Adriamycin), BEACOPP (advanced stage) Bleomycin, etoposide, doxorubicin (Adriamycin), cyclophosphamide, vincristine (Oncovin), prednisone, procarbazine
  • 21.  Most relapse occurs in 1st 3year after diagnosis, but relapse after 10 year have been reported.  Treatment of relapse Nature of relapse treatment Relapse with favorable at diagnosis Chemotherapy + LD-IFRT Relapse with high risk disease Chemotherapy + Autologous HSCT Relapse with in 12 month of diagnosis Chemotherapy + Autologous HSCT + radiotherapy
  • 22.  With the use of current therapeutic regimens,  With dose intense chemotherapy OS has approached to 100% Disease stage event-free survival (EFS) Overall survival (OS) Early-stage disease + favorable prognostic factors 85-90% >95% Advanced-stage disease 80-85% 90%
  • 23. 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/dl Histology : classical HL Initially not respond to chemotherapy
  • 24.  Secondary malignancy  Cardiac toxicity  Pulmonary dysfunction  Thyroid dysfunction  Gonadal dysfunction & infertility  Growth retardation  Psychosocial problem
  • 25.  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

Editor's Notes

  1. eosinophili