Lymphoma Review | Mnemonics And Proven Ways To Memorize for the PANCE, PANRE

Introduction to Lymphoma

Definition: Lymphoma is a form of cancer that originates from lymphocytes, which are a specialized type of white blood cell integral to the immune system. The term "lymphoma" is derived from "lymph," referring to the lymphatic tissues involved, and "oma," which denotes a tumor or neoplasm.

Hodgkin vs. Non-Hodgkin Lymphoma

Key Difference:

• Hodgkin Lymphoma: Characterized by the presence of Reed-Sternberg cells, which are large atypical cells found in lymph nodes.

• Non-Hodgkin Lymphoma: Defined by the absence of Reed-Sternberg cells; encompasses a diverse group of lymphatic cancers that vary widely in presentation and prognosis.

Hodgkin Lymphoma Overview

Type: Classed as a hematologic malignancy primarily affecting mature B cells, Hodgkin lymphoma arises within the lymphatic system, which is part of the body's immune framework.Cell Behavior: Abnormalities in gene rearrangement within B cells thwart the normal process of apoptosis (programmed cell death). This dysfunction causes unchecked cell proliferation, resulting in lymphadenopathy, which is the swelling of the lymph nodes.

Reed-Sternberg Cells

Characteristic Feature: These cells are typically multi-nucleated; they often exhibit a binucleated configuration, presenting an "owl-eye" appearance when viewed under a microscope.Pathognomonic: The identification of Reed-Sternberg cells is crucial for the definitive diagnosis of Hodgkin lymphoma as they serve as a hallmark feature distinguishing it from other lymphoproliferative disorders.Variant: There's a mononuclear variant of these cells, referred to as Hodgkin cells; however, this variant is generally deemed less significant in clinical assessments and examinations.

Epidemiology

Bimodal Age Distribution: Hodgkin lymphoma exhibits two distinct peaks in incidence: one occurring in late adolescence to young adulthood (ages 15-30), and another in older adults (typically over 55 years of age).Contiguous Spread: The disease typically spreads contiguously from one localized lymph node to adjacent nodes rather than via hematogenous spread. This spread pattern is often associated with a more favorable prognosis, as localized disease is generally more amenable to treatment.

Risk Factors

Key Risk Factor: Infection with the Epstein-Barr virus (EBV) is frequently linked with Hodgkin lymphoma, particularly in individuals who are immunocompromised, such as organ transplant recipients or those with HIV/AIDS. Other factors may include family history, and some autoimmune diseases have also been implicated.

Types of Hodgkin Lymphoma

Most Common Type: The nodular sclerosing variant is the predominant form, accounting for approximately 70% of classic Hodgkin lymphoma cases.Other Variants: Less common types include mixed cellularity, lymphocyte-rich, and lymphocyte-depleted Hodgkin lymphoma.

Clinical Manifestations

• Non-tender Lymphadenopathy: This is the most prevalent clinical presentation, where affected lymph nodes become enlarged, firm, and rubbery without tenderness. The cervical region is the most frequently involved area.

• Alcohol-Induced Pain: An unusual and specific symptom associated with Hodgkin lymphoma, patients may experience pain in lymph nodes after the consumption of alcohol.

• Mediastinal Mass: A mediastinal mass might be observed on imaging, potentially causing respiratory symptoms such as cough, shortness of breath, or retrosternal chest pain.

• B Symptoms: These include fever, night sweats, and significant weight loss, which are more prominent in advanced stages of the disease, believed to be due to cytokine release from malignant cells.

Diagnosis

Essential Steps:

• Biopsy: An excisional or incisional biopsy of the affected lymph nodes is critical for confirming the diagnosis by identifying Reed-Sternberg cells. Fine needle aspiration (FNA) is generally inadequate for diagnostic purposes.

• Imaging Studies: A positron emission tomography-computed tomography (PET-CT) scan is commonly utilized in staging the disease and evaluating any involvement outside the lymphatic system.

Treatment

General Approach: Treatment typically involves a combination of chemotherapy and radiation therapy tailored to the stage of the disease.ABVD Regimen: A standard chemotherapy regimen for Hodgkin lymphoma includes adriamycin (doxorubicin), bleomycin, vinblastine, and dacarbazine, which are usually given in cycles of three to four, followed by radiation therapy when indicated. Other regimens may also be employed based on individual patient factors and treatment responses.