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Lymphoma: cancers of the lymphatic system

Lymphoma is a group of cancers that arise in the lymphatic system. This article explains types, signs, diagnosis, history, and common approaches to treatment and follow-up care.

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Overview

Lymphoma denotes a set of cancers that originate in the lymphatic system, the network of vessels and organs that help defend the body against infection. It affects lymphocytes, a class of white blood cell. For a basic explanation of the affected anatomy see the lymphatic system. Lymphoma may be limited to a single lymph node or widespread.

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Key characteristics

Most lymphomas are classified by the subtype of lymphocyte involved and by their behaviour. The two broad categories are Hodgkin lymphoma and non-Hodgkin lymphoma. Within non-Hodgkin types there are many distinct entities, including B-cell and T-cell lymphomas and rarer forms such as hepatosplenic lymphoma. Certain cancers can appear primarily in the brain and eye region and are referred to as primary central nervous system lymphoma.

Symptoms and diagnosis

Common warning signs include painless swollen lymph nodes, unexplained fevers, night sweats, and weight loss. Diagnosis typically requires medical history, physical exam and tissue evaluation by biopsy. Imaging and laboratory tests help with staging and planning treatment.

Classification and examples

  • Non-Hodgkin lymphoma: a large, varied group with many subtypes differing in speed of growth and response to therapy.
  • Hodgkin lymphoma: defined by characteristic Reed–Sternberg cells and a generally distinct clinical course.
  • Other specific types: B‑cell and T‑cell lymphomas, primary central nervous system lymphoma, hepatosplenic lymphoma.

History, treatment and outlook

Descriptions of what is now called Hodgkin lymphoma date back to the 19th century and led to modern pathological classification. Treatment depends on type and stage and may combine chemotherapy, radiotherapy, targeted immunotherapies and, in selected cases, stem cell transplantation. Prognosis varies widely: some lymphomas are highly curable while others require long‑term management.

Importance and further reading

Because lymphomas encompass many distinct diseases, accurate diagnosis is essential for appropriate care. For more clinical and patient information consult specialist sources and guidelines via the links above and trusted medical services. Early evaluation of persistent lymph node swelling or systemic symptoms is recommended.

Causes

The disease is based on a malignant transformation of lymphoid cells of different maturation and differentiation stages. Depending on which cell type is affected, different types of lymphoma develop, which differ in manifestation, course, therapeutic influenceability and prognosis. Traditionally, a distinction is made between Hodgkin's lymphoma and the heterogeneous group of non-Hodgkin's lymphomas (NHL). Today, the classification of malignant lymphomas by the World Health Organization is common. In addition to the cell of origin (B-lymphocyte or T-lymphocyte), this distinguishes between

  1. Indolent lymphomas (dolor = lat. pain; low grade, meaning: slow-growing lymphomas that do not cause any symptoms for a long time)
  2. Aggressive lymphomas (high grade, meaning fast-growing lymphomas that cause rapid symptoms)
  3. Hodgkin's disease.

Due to their origin in the lymphatic system, some malignant lymphomas are caused by disorders of the immune system. With the increasing number of organ transplantations and the subsequent need for immunosuppression as well as immunodeficiencies in HIV, the incidence (number of new cases) of so-called post-transplant lymphoproliferative disorders has increased. Congenital immune system disorders such as Wiskott-Aldrich syndrome or severe combined immunodeficiency (SCID) have also been reported to increase the incidence of lymphoma. The age peaks in Europe and the USA (not in the rest of the world) are around 30 and 60 years of age, men are affected slightly more often than women.

In the treatment of malignant lymphomas, therapy with cytostatics (chemotherapy) plays a decisive role, sometimes supplemented by radiotherapy. This means that many lymphomas can be treated well. Some lymphomas are curable today.

Classification

Lymphomas are basically divided into Hodgkin's lymphomas, which account for about 25% of all lymphomas, and non-Hodgkin's lymphomas, which account for about 75% of all lymphomas.

Hodgkin's Lymphoma

Hodgkin's lymphoma (HL, Hodgkin's disease) is a malignant, monoclonal B-cell lymphoma. In histology, mostly Sternberg-Reed cells (multinucleated giant cells) and mononuclear Hodgkin cells are detectable.

Non-Hodgkin's Lymphoma

Non-Hodgkin's lymphomas (NHL) are a heterogeneous group of monoclonal neoplasms derived from precursor B or T lymphocytes.

B-cell lymphomas

  • Chronic lymphocytic leukemia
  • Plasmocytoma (multiple myeloma)
  • Lymphoplasmocytic lymphoma (including Waldenström's disease)
  • Follicular lymphoma
  • MALT lymphoma
  • diffuse large B-cell lymphoma (DLCBL)
  • Burkitt's Lymphoma

T-cell lymphomas

  • T-/NK-cell lymphoma
  • NK cell leukemia
  • T-cell prolymphocytic leukemia
  • mycosis fungoides
  • Sézary Syndrome

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