The Clinical Presentation of Lymphomas
Lymphomas, blood cell tumors that usually originate in lymphatic tissues (and can spread to other organs), are among the most diverse and most curable of all malignancies. There are two major groups of lymphomas: About 90 percent are non-Hodgkin lymphomas (NHLs) and about 10 percent are Hodgkin lymphomas (HLs).
NON-HODGKIN LYMPHOMAS (NHLs)
Let’s start with NHLs, malignant neoplasms derived from B cell progenitors, T cell progenitors, mature B cells, mature T cells, or (rarely) natural killer cells. NHLs are the seventh most common type of cancer in American men and the sixth most common in American women. The American Cancer Society estimates nearly 72,000 cases and 20,000 deaths in 2015.
Most NHL patients are first seen by their PCPs because they are showing signs of lymphadenopathy, although the enlarged nodes are usually painless. Here’s what your patients might present with:
- Aggressive NHLs usually present acutely or subacutely with a rapidly growing mass, systemic B symptoms (fever, night sweats, weight loss), and/or elevated levels of serum lactate. Treatment in these cases should be treated promptly to manage symptoms, slow or revert progression, and optimize survival.
- Indolent lymphomas often present only with slow-growing lymphadenopathy, hepatomegaly, splenomegaly, or cytopenias.
- Less commons presentations include skin rash, pruritus, hypersensitivity to insect stings and bites, generalized fatigue, malaise, fever of unknown origins, ascites, and effusions.
- Patients with primary GI track lymphoma may present with anorexia, weight loss, nausea and vomiting, chronic pain, abdominal fullness, early satiety, symptoms of visceral obstruction, or even acute perforation and GI hemorrhage.
- Patients with primary CNS lymphoma may have headache, lethargy, facial neurologic symptoms, seizures, paralysis, spinal cord compression or lymphomatous meningitis.
- Getting older is a strong risk factor for lymphoma overall, with most cases occurring in people in their 60s or older
- The risk of NHL is higher in men than in women, but there are certain types of non-Hodgkin lymphoma that are more common in women
- NHL in the United States is more common in Caucasians than African Americans or Asians
- Inordinate contact with chemicals such as benzene, and certain herbicides and insecticides
- Inordinate radiation exposure
- History of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, Sjogren disease, and celiac sprue disease
- History of infections that directly transform lymphocytes such as T-cell leukemia/lymphoma virus (HTLV-1) and the Epstein-Barr virus (EBV)
- History of therapy with immunosuppressant drugs used in organ transplants, autoimmune disease, and HIV
- Inherited immune disorders such as hypogammaglobulinemia and Wiskott-Aldrich syndrome
HODGKIN LYMPHOMAS (HLs)
There are two major types of Hodgkin lymphomas: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Both are marked by the presence of HLs of the Reed-Sternberg cell. Note that this type of cell also can be found in reactive lymphadenopathy (such as infectious mononucleosis) and more rarely in other types of non-Hodgkin lymphomas. Both are marked by the presence of Reed-Sternberg cells.
According to the National Cancer Institute, the five-year survival rate (2004-2010) is 85.3 percent. And the number of deaths has been declining.
Because there are no widely recommended screening tests for Hodgkin lymphomas, you will need to pay attention to your patient’s risk factors in combination with these clinical presentations:
- Asymptomatic lymphadenopathy, above the diaphragm 80% of time but also found under the arm or in the groin
- Intermittent fever is observed in approximately 35% of cases; infrequently, the classic Pel-Ebstein fever is observed (high fever for 1-2 weeks, followed by an afebrile period of 1-2 weeks)
- Chest pain, cough, shortness of breath, or a combination of those may be present due to a large mediastinal mass or lung involvement
- Patients may present with pruritus
- Pain at sites of nodal disease, precipitated by drinking alcohol, occurs in fewer than 10% of patients but is specific for Hodgkin lymphoma
- Back or bone pain may rarely occur
- A family history is also helpful; in particular, nodular sclerosis Hodgkin lymphoma (NSHL) has a strong genetic component and has often previously been diagnosed in the family
- History of infectious mononucleosis caused by Epstein Barr virus (EBV)
- Occurs slightly more often in men than women
- Most common with young adults (peaking at about 20) and older adults (peaking at about 65); and those in the United States, Canada, and Northern Europe
- History of younger siblings with the disease (but only by 5% per the American Cancer Society), and especially so with identical twins
- People from higher socioeconomic backgrounds (though the correlation is still unclear)
- People infected with HIV
- Lymphoma is widely diverse group of diseases that originate in lymphatic tissues
- Because the symptoms caused by lymphomas are often the same as those from infections, patient history, risk factors, and clinical presentation need to be viewed in total
- Presentation most often includes asymptomatic lymphadenopathy, usually found in the neck (above the diaphragm)
- Precise diagnosis and classification requires evaluation of lymph node tissue obtained via adequately sized biopsy
- Hodgkin lymphomas (HLs) and non-Hodgkin lymphomas (NHLs) are curable in the majority of patients
Miguel Islas-Ohlmayer, M.D., is a hematologist and blood and marrow transplant physician. Board certified in internal medicine and hematology, his main interests include hematologic malignancies as well as blood and marrow stem cell transplantation. Dr. Islas-Ohlmayer practices at our OHC Kenwood office and is a clinician at the Blood Cancer Center at The Jewish Hospital-Mercy Health. The Blood Cancer Center, powered by OHC physicians, is a recent recipient of The Joint Commission’s Gold Seal of Approval for Blood & Marrow Transplants.