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HODGKIN’S
LYMPHOMA
Medrockets.com
Hodgkin disease
 Definition:
A neoplastic transformation of lymphocytes particularly in lymph nodes.
Characterized by:
1) the presence of Reed-Sternberg cells on histology
2) spreading in an orderly fashion to contagious lymph nodes
( For example, Hodgkin lymphoma that starts in the cervical lymph nodes
may spread first to the supraclavicular nodes then to the axillary nodes )
 6% of childhood cancer
 5% of cancer in < 14 yr
 15% in person 15-19 yr
 Rare < 10 yr
Large cells ( >45um in diameter) with classically binucleate or
bilobed central nucleus each with a large acidophilic central
nucleoli surrounded by a clear halo. “owl’s eye appearance”
Red-Sternberg cells
Medrockets.com
Epidemiology of Hodgkin’s lymphoma
 Hodgkin disease has bimodal age distribution--one peak in the 20s
and 60s.
 Early peak middle to late 20s
 Second peak after 50 yr
 Sex Male : Female
4: 1 for 3-7 yr
3: 1 for 7-9 yr
1-3: 1 for > 10 yr
 100 folds risk for unaffected monozygotic twin of affected twin
 Associated with specific HLA antigen
 Infectious agents
Human herpes virus 6
CMV
Epstein – Barr virus
 Immunodeficiency
Medrockets.com
Etiology/Risk Factors
Doctors seldom know why one person develops
Hodgkin lymphoma and another does not. But
research shows that certain risk factors increase the
chance that a person will develop this disease.
Having one or more risk factors does not mean that a
person will develop Hodgkin lymphoma. Most people
who have risk factors never develop cancer.
Medrockets.com
Risk Factors
1) Certain viruses:
 Epstein-Barr virus (EBV)
 Human immunodeficiency virus (HIV)
2) Weakened immune system:
 inherited condition
 certain drugs used after an organ transplant
3) Age:
 Hodgkin lymphoma is most common among teens and adults aged 15 to
35 years and adults aged 55 years and older.
4) Family history:
 Family members, especially brothers and sisters, of a person with
Hodgkin lymphoma or other lymphomas may have an increased chance
of developing this disease.
Medrockets.com
Lymphocyte Predominant
10-15% of patients
More common in male
Younger patients
Localized disease
Has best prognosis
Mixed cellularity
30% of patients
< 10 yr of age
Advanced disease
Extranodal extension
Lymphocyte depletion
Rare in children
Common with HIV
Has worst prognosis
Nodular sclerosis
Most common
40% of younger patients
70% of adolescents
Classification
Rye Classification System
Medrockets.com
REAL Classification
( Revised European – American Classification of Lymphoid Neoplasms )
 Nodular lymphocyte predominance
 Classical Hodgkin lymphoma
 Lymphocyte rich
 Mix cellularity
 Nodular sclerosis
 Lymphocyte depletion
 Anaplastic large cell lymphoma Hodgkin like
Medrockets.com
 Enlarged, painless, rubbery, non- erythematous, nontender lymph nodes are
the hallmark of the disease.
 May become painful after drinking alcohol
 Hepatosplenomegaly
 Cough, dyspnea, hypoxia
 Pleural or pericardial effusion
 Heptocellular dysfunction
 B.M infiltration(Anemia, neutropenia, thrombocytopenia)
 25% have ''B'' symptoms
 Although pruritus is common in the disease it is not one of the ‘’B’’ symptoms.
 Cervical, supraclavicular and axillary lymphadenopathy are the most common
initial signs of the disease.
 Disease below diaphragm is rare (only3%)
Clinical presentation
Medrockets.com
Systemic Symptoms (B symptoms)
 Important in staging
 Unexplained fever > 390C
 Weight loss > 10% in 3m
 Drenching night sweats
Immune System abnormalities
 Anergy to delayed-hypersensitivity skin test
 Abnormal cellular immune response
 Decreased CD4:CD8 ratio
 Reduce natural killer cell cytotoxicity
Medrockets.com
Extralymphatic sites may be involved such as:
# Spleen
# Liver
# Bone marrow
# Lung
# CNS
Extralymphatic involvement is more common with
non-hodgkin lymphoma.
Emergency presentation:
Infections
SVC obstruction ( facial edema, increased JVP
and Dyspnea)
Medrockets.com
The doctor considers the following to determine the
stage of Hodgkin lymphoma:
The number of lymph nodes affected.
Whether these lymph nodes are on one or both sides of
the diaphragm.
Whether the disease has spread to the bone marrow,
spleen, liver, or lung.
Each stage is divided into A or B symptoms according to
the presence of systemic symptoms.
Staging of Hodgkin’s Lymphoma
Medrockets.com
Ann Arbor Staging Classification for Hodgkin
Disease
 Stage I
Involvement of a single lymph node (1)
or of a single extra lymphatic site or organ(1f)
 Stage II
Involvement of two or more lymph node regions on the same side of
the diaphragm(II)
or localised involvement of an extra lymphatic site
or organ and one or more lymph node regions on the same side of the
diaphragm (IIf)
Medrockets.com
Stage III
Involvement of lymph node regions on both sides of the
diaphragm (III) which may be accompanied by the involvement of
spleen (IIIS) or by localized involvement of an extra lymphatic site or
organ ( IIIf) or both ( IIIsf)
Stage IV
Diffuse or disseminated involvement of one or more
extra lymphatic organs or tissues with or without associated lymph node
involvement.
The absence or presence of fever > 38C for three consecutive days ,
drenching night sweats , or unexplained loss of > 10% body weight in
the 6 months preceding admission are to be denoted in all cases by
the suffice letters A & B respectively.
Medrockets.com
Medrockets.com
DIAGNOSIS
 An excisional lymph node biopsy is the essential first step in diagnosis.
 A biopsy is the only sure way to diagnose Hodgkin lymphoma.
 Excisional Biopsy
 Light Microscopy
 Immunocytochemistry
 Molecular Studies
 Chest X – Ray
 Mediastinal Mass
 CT Scan
 Chest
 Abdomen
 Pelvis
 Blood CP & ESR
 LFT’s
 Bone Marrow Aspiration
 Serum Copper & Ferritin
 Bone Scan
 Gallium 67 Scan / FDG/PET
Medrockets.com
TREATMENT
 Treatment depends on :
 Stage of the disease
 Age at diagnosis
 Presence / absence of B symptoms
 Presence of hilar lymphadenopathy
 Presence of bulky nodal disease
 Current Treatment Regimen
 Combined chemotherapy with or without low dose involved
field radiation therapy
Medrockets.com
Chemotherapy Regimens
 MOPP
(Mechlorethamine , Vincristine , Procarbazine ,
Prednisolone)
 COPP
(Cyclophosphamide , Vincristine , Procarbazine ,
Prednisolone)
 ABVD
(Adriamycin , Bleomycin , Vinblastine , Dacarbazine)
 BEACOPP ( For advanced stage disease )
(Bleomycin , Etoposide , Doxorubicin , Cyclophosphamide
, Vincristine , Procarbazine , Prednisolone)
TREATMENT
Medrockets.com
 Therapy is entirely based on the stage.
 Localized disease ( stage IA and IIA ) is managed predominantly with
radiation.
 All patients with evidence of ‘’B’’ symptoms as well as stage III and IV
are managed with chemotherapy.
 The most effective combination chemotherapeutic regimen for
Hodgkin lymphoma is ABVD ( adriamycin, bleomycin, vinblastin and
dacarbazine).
 ABVD is superior to MOP (meclorethamine, vincristin(oncovin) ,
prednisolone and procarbazine) because ABVD has fewer side effects
such as:
1) Permanent sterility
2) Secondary cancer formation
3) Aplastic anemia
4) Peripheral neuropathy
TREATMENT
Medrockets.com
International Prognostic Index
 The International Prognostic Index (IPI) was first developed to help
doctors determine the prognosis for people with fast-growing
lymphomas. The index depends on 5 factors:
1) The patient’s age
2) The stage of the lymphoma
3) Whether or not the lymphoma is in organs outside the lymph system
4) Performance status (PS) – how well a person can complete normal
daily activities
5) The blood (serum) level of (LDH)
Medrockets.com
Medrockets.com
LONG TERM COMPLICATIONS
 Secondary malignancy
 Acute Myelogenous Leukemia
 Non Hodgkin lymphoma
 Carcinomas of breast , lungs & thyroid
 Short stature
 Hypothyroidism
 Sterility
 Dental caries
 Sub clinical pulmonary dysfunction
 Ischemic heart disease
Medrockets.com

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Hodgkin's lymphoma

  • 2. Hodgkin disease  Definition: A neoplastic transformation of lymphocytes particularly in lymph nodes. Characterized by: 1) the presence of Reed-Sternberg cells on histology 2) spreading in an orderly fashion to contagious lymph nodes ( For example, Hodgkin lymphoma that starts in the cervical lymph nodes may spread first to the supraclavicular nodes then to the axillary nodes )  6% of childhood cancer  5% of cancer in < 14 yr  15% in person 15-19 yr  Rare < 10 yr
  • 3. Large cells ( >45um in diameter) with classically binucleate or bilobed central nucleus each with a large acidophilic central nucleoli surrounded by a clear halo. “owl’s eye appearance” Red-Sternberg cells Medrockets.com
  • 4. Epidemiology of Hodgkin’s lymphoma  Hodgkin disease has bimodal age distribution--one peak in the 20s and 60s.  Early peak middle to late 20s  Second peak after 50 yr  Sex Male : Female 4: 1 for 3-7 yr 3: 1 for 7-9 yr 1-3: 1 for > 10 yr  100 folds risk for unaffected monozygotic twin of affected twin  Associated with specific HLA antigen  Infectious agents Human herpes virus 6 CMV Epstein – Barr virus  Immunodeficiency Medrockets.com
  • 5. Etiology/Risk Factors Doctors seldom know why one person develops Hodgkin lymphoma and another does not. But research shows that certain risk factors increase the chance that a person will develop this disease. Having one or more risk factors does not mean that a person will develop Hodgkin lymphoma. Most people who have risk factors never develop cancer. Medrockets.com
  • 6. Risk Factors 1) Certain viruses:  Epstein-Barr virus (EBV)  Human immunodeficiency virus (HIV) 2) Weakened immune system:  inherited condition  certain drugs used after an organ transplant 3) Age:  Hodgkin lymphoma is most common among teens and adults aged 15 to 35 years and adults aged 55 years and older. 4) Family history:  Family members, especially brothers and sisters, of a person with Hodgkin lymphoma or other lymphomas may have an increased chance of developing this disease. Medrockets.com
  • 7. Lymphocyte Predominant 10-15% of patients More common in male Younger patients Localized disease Has best prognosis Mixed cellularity 30% of patients < 10 yr of age Advanced disease Extranodal extension Lymphocyte depletion Rare in children Common with HIV Has worst prognosis Nodular sclerosis Most common 40% of younger patients 70% of adolescents Classification Rye Classification System Medrockets.com
  • 8. REAL Classification ( Revised European – American Classification of Lymphoid Neoplasms )  Nodular lymphocyte predominance  Classical Hodgkin lymphoma  Lymphocyte rich  Mix cellularity  Nodular sclerosis  Lymphocyte depletion  Anaplastic large cell lymphoma Hodgkin like Medrockets.com
  • 9.  Enlarged, painless, rubbery, non- erythematous, nontender lymph nodes are the hallmark of the disease.  May become painful after drinking alcohol  Hepatosplenomegaly  Cough, dyspnea, hypoxia  Pleural or pericardial effusion  Heptocellular dysfunction  B.M infiltration(Anemia, neutropenia, thrombocytopenia)  25% have ''B'' symptoms  Although pruritus is common in the disease it is not one of the ‘’B’’ symptoms.  Cervical, supraclavicular and axillary lymphadenopathy are the most common initial signs of the disease.  Disease below diaphragm is rare (only3%) Clinical presentation Medrockets.com
  • 10. Systemic Symptoms (B symptoms)  Important in staging  Unexplained fever > 390C  Weight loss > 10% in 3m  Drenching night sweats Immune System abnormalities  Anergy to delayed-hypersensitivity skin test  Abnormal cellular immune response  Decreased CD4:CD8 ratio  Reduce natural killer cell cytotoxicity Medrockets.com
  • 11. Extralymphatic sites may be involved such as: # Spleen # Liver # Bone marrow # Lung # CNS Extralymphatic involvement is more common with non-hodgkin lymphoma. Emergency presentation: Infections SVC obstruction ( facial edema, increased JVP and Dyspnea) Medrockets.com
  • 12. The doctor considers the following to determine the stage of Hodgkin lymphoma: The number of lymph nodes affected. Whether these lymph nodes are on one or both sides of the diaphragm. Whether the disease has spread to the bone marrow, spleen, liver, or lung. Each stage is divided into A or B symptoms according to the presence of systemic symptoms. Staging of Hodgkin’s Lymphoma Medrockets.com
  • 13. Ann Arbor Staging Classification for Hodgkin Disease  Stage I Involvement of a single lymph node (1) or of a single extra lymphatic site or organ(1f)  Stage II Involvement of two or more lymph node regions on the same side of the diaphragm(II) or localised involvement of an extra lymphatic site or organ and one or more lymph node regions on the same side of the diaphragm (IIf) Medrockets.com
  • 14. Stage III Involvement of lymph node regions on both sides of the diaphragm (III) which may be accompanied by the involvement of spleen (IIIS) or by localized involvement of an extra lymphatic site or organ ( IIIf) or both ( IIIsf) Stage IV Diffuse or disseminated involvement of one or more extra lymphatic organs or tissues with or without associated lymph node involvement. The absence or presence of fever > 38C for three consecutive days , drenching night sweats , or unexplained loss of > 10% body weight in the 6 months preceding admission are to be denoted in all cases by the suffice letters A & B respectively. Medrockets.com
  • 16. DIAGNOSIS  An excisional lymph node biopsy is the essential first step in diagnosis.  A biopsy is the only sure way to diagnose Hodgkin lymphoma.  Excisional Biopsy  Light Microscopy  Immunocytochemistry  Molecular Studies  Chest X – Ray  Mediastinal Mass  CT Scan  Chest  Abdomen  Pelvis  Blood CP & ESR  LFT’s  Bone Marrow Aspiration  Serum Copper & Ferritin  Bone Scan  Gallium 67 Scan / FDG/PET Medrockets.com
  • 17. TREATMENT  Treatment depends on :  Stage of the disease  Age at diagnosis  Presence / absence of B symptoms  Presence of hilar lymphadenopathy  Presence of bulky nodal disease  Current Treatment Regimen  Combined chemotherapy with or without low dose involved field radiation therapy Medrockets.com
  • 18. Chemotherapy Regimens  MOPP (Mechlorethamine , Vincristine , Procarbazine , Prednisolone)  COPP (Cyclophosphamide , Vincristine , Procarbazine , Prednisolone)  ABVD (Adriamycin , Bleomycin , Vinblastine , Dacarbazine)  BEACOPP ( For advanced stage disease ) (Bleomycin , Etoposide , Doxorubicin , Cyclophosphamide , Vincristine , Procarbazine , Prednisolone) TREATMENT Medrockets.com
  • 19.  Therapy is entirely based on the stage.  Localized disease ( stage IA and IIA ) is managed predominantly with radiation.  All patients with evidence of ‘’B’’ symptoms as well as stage III and IV are managed with chemotherapy.  The most effective combination chemotherapeutic regimen for Hodgkin lymphoma is ABVD ( adriamycin, bleomycin, vinblastin and dacarbazine).  ABVD is superior to MOP (meclorethamine, vincristin(oncovin) , prednisolone and procarbazine) because ABVD has fewer side effects such as: 1) Permanent sterility 2) Secondary cancer formation 3) Aplastic anemia 4) Peripheral neuropathy TREATMENT Medrockets.com
  • 20. International Prognostic Index  The International Prognostic Index (IPI) was first developed to help doctors determine the prognosis for people with fast-growing lymphomas. The index depends on 5 factors: 1) The patient’s age 2) The stage of the lymphoma 3) Whether or not the lymphoma is in organs outside the lymph system 4) Performance status (PS) – how well a person can complete normal daily activities 5) The blood (serum) level of (LDH) Medrockets.com
  • 22. LONG TERM COMPLICATIONS  Secondary malignancy  Acute Myelogenous Leukemia  Non Hodgkin lymphoma  Carcinomas of breast , lungs & thyroid  Short stature  Hypothyroidism  Sterility  Dental caries  Sub clinical pulmonary dysfunction  Ischemic heart disease Medrockets.com