Lymphoma PDF

Lymphoma Treatment Diagnostic criteria Clinical manifestations Pathophysiology  Lymph...

Lymphoma Treatment Diagnostic criteria Clinical manifestations Pathophysiology  Lymphoma Pathophysiology 1. Lymphomas (like some leukemias) have lymphoid stem cell origin 2. Form solid organ tumors in the lymph tissue and later in the bone marrow 3. Diverse group of solid tumors Lymphoma can be present in the spleen and liver Classified as either: Hodgkin lymphoma (less prevalent) Non-Hodgkin lymphoma (more prevalent) Porth C. Pathophysiology : concepts of altered health states. 7th ed. Philadelphia, Lippincott Williams & Wilkins; 2005.  Lymphoma Pathophysiology: Hodgkin Painless, progressive enlargement of cervical lymph nodes Exact cause unknown Risk factors: Exposure to viruses such as Ebstein-Barr Genetic factors Immunosuppression Childhood form of HL is similar to the adult onset 82% of children do not relapse within 5 years 5-year survival rate is 95% Adult mortality has declined most among malignancies Improved radiation Improved chemotherapy  Lymphoma Pathophysiology: Hodgkin HL characterized by the presence of Binucleated/multinucleated giant cells (macrophages): Reed-Sternberg cells Mononuclear giant cells: Hodgkin cells Other inflammatory cells Pathogenesis is still unclear Reed-Sternberg or Hodgkin cell is the neoplastic origin with clonal selection capability HL arises in B-cells that cannot synthesize immunoglobulin & resistant to apoptosis After the primary tumor established Spread to contiguous lymph nodes and can infiltrate vascular system HL is organ tropic to lung, liver, bones and bone marrow  Lymphoma Pathophysiology: Hodgkin 2005. Staging ranges from I (involvement of single lymph node) to IV (disseminated involvement of one or more organs outside the lymphatic system) These stages are further divided: A: absence of specific system manifestations B: presence of specific system manifestations Specific systemic manifestations are: Unexplained weight loss/Unexplained fever/ Drenching night sweats Porth C. Pathophysiology : concepts of altered health states. 7th ed. Philadelphia, Lippincott Williams & Wilkins;  Lymphoma Clinical Manifestations:Hodgkin 80% of patients with HL: nontender enlargement of cervical (neck) lymph node(s) Lymph node(s) are form and rubbery in texture Reed-Stenberg or Hodgkin cells release inflammatory mediators lymphokines and cytokines Low grade fever, night sweats, pruritus, weight loss fatigue 20% of patients have mass at the mediastinum Greater than 1/3 of chest diameter Splenomegaly, hepatomegaly may also be present © Patton, Thibodeau: Anatomy and Phsyiology, Elsevier, 2015  Lymphoma Diagnostic Criteria:Hodgkin Diagnosis and staging is based on: History taking and physical examination Laboratory studies Thoracic and abdominal CT scans Most significant: presence of Reed-Sternberg cells Classic Reed-Sternberg cells Derived from B-lymphocyte Cell is large and binucleated or multi nucleated  Lymphoma Treatment:Hodgkin Treatment is based on clinical staging 77% relapse free survival in all patients newly diagnosed with HL Patients with Stage IA or IIA are clinical early-stage: Chemotherapy Chemotherapy-radiation therapy Radiation therapy alone Patients with Stage III and IV Combination therapy with /without adjunct radiation therapy Reduced doses are used for children High risk patients (poor prognosis): 42-51% 5-year survival rate  Lymphoma :Non-Hodgkin Generic classification of B-cell and T-cell malignancies within immune system NHL occurs more frequently than HL Does not exhibit Reed-Sternberg or Hodgkin cells More likely to affect noncontiguous lymph nodes Etiology is unknown Risk factors Virus Immunodeficiency Genetic factors  Lymphoma Pathophysiology: Non-Hodgkin Genetic susceptibility is highly variable Depends on specific cell affected by neoplasia Follicular lymphoma: mutations of BCL-2 gene is present in 90% of patients Overexpression of BCL-2 protein Inability to control apoptosis Overproliferation of lymph tissue NHL can spread via lymphatic and vascular system NHL organ tropic to liver, spleen, bone marrow  Lymphoma Pathophysiology: Non-Hodgkin Staging is similar to HL The 3 major categories of lymphoid malignancies based on morphology and cell lineage: Non-Hodgkin Lymphomas 1. B-cell neoplasms 2. T-cell/Natural killer (NK)-cell neoplasms Hodgkin lymphoma 3. Hodgkin lymphoma Porth C. Pathophysiology : concepts of altered health states. 7th ed. Philadelphia, Lippincott Williams & Wilkins; 2005.  Lymphoma Clinical Manifestations: Non-Hodgkin Depends on tumor type and extent of disease Most common occurrence (as with HL) Painless enlargement of lymph nodes Other lymph nodes can also be affected Specific systemic manifestations are related to rapid growth of abnormal lymphoid cells and tissues: Unexplained weight loss/Unexplained fever/Drenching night sweats Increased risk of infection Paraneoplastic syndromes  Lymphoma Diagnostic Criteria:Non-Hodgkin Patient history and physical examination Lymph node biopsy to confirm NHL Histopathologic analysis/immunophenotyping to determine lineage Chest and abdominal CT/MRI/PET scan to visualize tumor size and location Cerebrospinal fluid check for metastases with aggressive NHL Staging is important to select treatment  Lymphoma Treatment: Non-Hodgkin Treatment is based on categorizing NHLs into 2 prognostic groups: 1. Indolent (passive, painless) lymphomas 2. Aggressive lymphomas Early stage (Stage I, II) indolent NHL: can be treated effectively with radiation therapy alone Later stage indolent and aggressive NHL: require intensive combination chemotherapy with or without radiation therapy Remission rate for aggressive forms: 60-80% Overall long-term disease free survival: 30-50%