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Hodgkin’s Disease (HD)
A disease of lymph nodes with a predictable pattern of spread
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Epidemiology Age: bimodal peak age Gender: male to female = 1.3 to 1.0
Third decade After 50 Gender: male to female = 1.3 to 1.0
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Etiology and Risk Factors
Unknown Possible etiologic factor 1) Familial factor 2) Viruses - EBV
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Signs and Symptoms HD is a lymph node-based malignancy
Common = asymptomatic lymphadenopathy Systemic symptoms Extranodal involvement
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Location of Lymphadenopathy
80% LN above the diaphragm Anterior mediastinum Cervical, supraclavicular, axillary
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Systemic Symptoms 40% of patients -> systemic symptoms
B-symptom => Fever, Night sweat, Weight loss Chronic pruritus
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Extranodal Involvement
“E” lesion by direct invasion Hematogenous metastasis (Stage IV): spleen, lungs, liver, bone marrow
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Diagnosis Biopsy Pathology : “Reed - Sternberg Cell”
Diagnostic tumor cell Must be identified Large size, binucleated, large eosinophilic nucleoli
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Histology 1. Lymphocyte predominant (LP) = few RS cells, good prognosis 2. Nodular sclerosis (NS) = the most common, young adult, female 3. Mixed cellularity (MC) = generalized lymphadenopathy 4. Lymphocyte depletion (LD) = numerous RS cells, poor prognosis
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Staging : The Coltswolds Classification for HD
I: A single LN region or a lymphoid structure (eg., spleen, thymus, Waldeyer’s ring) II: Two or more LN regions on the same side of the diaphragm III: LN regions or structures on both sides of the diaphragm 1: with/without involement of splenic, hilar, celiac, or portal nodes 2: with involvement of paraaortic, iliac, or mesenteric nodes IV: Extranodal site(s) beyond that designated E ( a single extranodal site, contiguous or proximal to a known nodal site)
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Designations applicable to any disease stage
A: No symptoms B: Fever, drenching sweats, weight loss X: Bulky disease > 1/3 the width of the mediastinum > 10 cm maximal dimension of nodal mass E: Involvement of a single extranodal site, contiguous or proximal to a know nodal site
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Prognostic Factors Stage of disease: the most important prognostic factor Number of sites of involvement Bulky disease ( particularly in the mediastinum) B symptoms Age
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Investigations 1. Imaging : CT scan of the chest/ abdomen
2. Lab tests: CBC, ESR, LDH 3. Bone marrow biopsy: B-symptoms 4. Staging laparotomy : consist of Splenectomy LN sampling: celiac/splenic/hilar/paraaortic/paracaval/iliac nodes Wedge/needle biopsy of the liver open iliac crest Bone Marrow Biopsy Oophoropexy
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Treatment Stage I-II = Radiotherapy Stage III-IV = Chemotherapy
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Radiotherapy Subtotal lymphoid irradiation = Mantle + Paraaortic field
Mantle field = base of mandible to diaphragm Cover LN above diaphragm -> submandibular, cervical, supraclavicular, infraclavicular, axillary, mediastinum, hilar Dose = 3,600 cGy
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Side Effects of Radiotherapy
Acute N/V, Dermatitis, Fatigue Delay Pneumonitis Herpes zoster infection Subclinical hypothyroidism Infertility Secondary malignancies secondary solid tumors ( lung, breast) chemotherapy -> most common = leukemia
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Non-Hodgkin’s Lymphoma
Heterogeneous group of lymphoproliferative malignancies
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Epidemiology The incidence has been increasing worldwide.
This increase is more marked for older persons.
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Etiology and Causative Factors
The origin is UNCERTAIN. Causative factors with a predisposition Immunosuppression Infectious agents (EBV, HTLV-1, Herpes type 8, H.pylori)
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NHL Usually originates in lymphoid tissues Can spread to other organs
Prognosis depends on the histologic type, stage and extent of disease, age, and treatment
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Clinical Manifestations
Asymptomatic LN enlargement (most common presentation) 1) Location of lymphadenopathy most common = neck, inguinal, and abdominal LNs 2) Systemic symptoms fever, weight loss, night sweats ( usually heralding more advanced disease) 3) Primary extranodal lymphoma depend on the site of origin
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Staging Evaluation Pathologic diagnosed by an experienced hematopathologist. Staging Evaluation includes: History + Physical Exam Lab : CBC, LDH Bone marrow biopsy Chest X-Ray CT abdomen and pelvis/chest Gallium scan
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Histology: 2 prognostic groups
Indolent lymphoma Aggressive lymphoma
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Staging: Ann Arbor System (commonly used)
I: A single nodal region (I) or single extralymphatic site (IE) II: 2 or more nodal regions on the same side of diaphragm(II) or a single localized extralymphatic site and its regional nodes on the same side of diaphragm (IIE) III: Nodal regions on both sides of the diaphragm IV: Diffuse or disseminated involvement of 1 or more extralymphatic organs with/without associated nodal involvement
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Designations applicable to any stage
A: No symptoms B: Weight loss, unexplained fever, night sweats E: Localized involvement of extralymphatic tissue S: Spleen involvement
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Treatment Indolent Lymphoma Aggressive Lymphoma Stage I + II = RT
Stage III + IV = Chemotherapy + RT Aggressive Lymphoma Stage I + II = combined modality ( Chemotherapy + RT) Stage III + IV = Chemotherapy ( Doxorubicin based)
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Radiotherapy Involve-field RT: Extended field RT:
Involved region + first-echelon adjacent LN region Extended field RT: involve field + second-echelon adjacent LN region
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Example
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Radiation Dose: Indolent: 2,500 - 3,500 cGy
Aggressive: 3, ,500 cGy
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Late effects of treatment
Normal tissue toxicity Secondary cancers
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