Download Hodgkin`s Disease (HD)

Hodgkin’s Disease (HD)
A disease of lymph nodes with a
predictable pattern of spread
Epidemiology
• Age: bimodal peak age
–Third decade
–After 50
• Gender: male to female = 1.3 to 1.0
Etiology and Risk Factors
• Unknown
• Possible etiologic factor
–1) Familial factor
–2) Viruses - EBV
Signs and Symptoms
•
•
•
•
HD is a lymph node-based malignancy
Common = asymptomatic lymphadenopathy
Systemic symptoms
Extranodal involvement
Location of Lymphadenopathy
• 80% LN above the diaphragm
–Anterior mediastinum
–Cervical, supraclavicular, axillary
Systemic Symptoms
• 40% of patients -> systemic
symptoms
– B-symptom => Fever, Night sweat,
Weight loss
– Chronic pruritus
Extranodal Involvement
• “E” lesion by direct invasion
• Hematogenous metastasis (Stage
IV): spleen, lungs, liver, bone
marrow
Diagnosis
• Biopsy
• Pathology : “Reed - Sternberg Cell”
– Diagnostic tumor cell
– Must be identified
– Large size, binucleated, large eosinophilic nucleoli
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
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)
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
Prognostic Factors
• Stage of disease: the most important prognostic
factor
• Number of sites of involvement
• Bulky disease ( particularly in the mediastinum)
• B symptoms
• Age
•
•
•
•
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
Treatment
• Stage I-II = Radiotherapy
• Stage III-IV = Chemotherapy
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
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
Non-Hodgkin’s Lymphoma
Heterogeneous group of
lymphoproliferative malignancies
Epidemiology
• The incidence has been increasing
worldwide.
• This increase is more marked for older
persons.
Etiology and Causative Factors
• The origin is UNCERTAIN.
• Causative factors with a predisposition
– Immunosuppression
– Infectious agents (EBV, HTLV-1, Herpes type 8,
H.pylori)
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
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
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
Histology: 2 prognostic groups
• Indolent lymphoma
• Aggressive lymphoma
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
Designations applicable to any stage
• A: No symptoms
• B: Weight loss, unexplained fever, night sweats
• E: Localized involvement of extralymphatic
tissue
• S: Spleen involvement
Treatment
• Indolent 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)
Radiotherapy
• Involve-field RT:
– Involved region + first-echelon adjacent LN
region
• Extended field RT:
– involve field + second-echelon adjacent LN region
Example
Radiation Dose:
• Indolent: 2,500 - 3,500 cGy
• Aggressive: 3,500 - 4,500 cGy
Late effects of treatment
• Normal tissue toxicity
• Secondary cancers