Pel-Ebstein Fever Diagnosis and Patterns

Pel-Ebstein is a high-grade fever that keeps rising and falling every 7-10 days or so. The fever rises abruptly, stays high for a week or so and then falls close to normal abruptly again, staying low for about a week. Then this rise and fall pattern is repeated again. Thus, doctors need to know what a person’s temperature has been doing over several weeks in order to identify Pel-Ebstein fever.

Not all patients with Hodgkin disease have this pattern of fever. In fact, only a minority has this exact pattern. However, when it’s present, it can be a strong clue that investigation for Hodgkin lymphoma may be appropriate.

Fever is one of several important symptoms of Hodgkin lymphoma, also known as Hodgkin disease. This particular pattern of fever was named after two European doctors, Pieter Pel and Wilhelm Ebstein, who noticed and reported the pattern more than a century back.

Pel-Ebstein Fever and Hodgkin Lymphoma

A plain old intermittent fever is seen in about 35 percent of people with Hodgkin lymphoma; however Pel-Ebstein fever—high fever for 1-2 weeks, followed by no fever for 1-2 weeks—is seen infrequently in Hodgkin lymphoma.

William Ebstein was a German physician and pathologist described something called “remittent pyrexia occurring in lymphadenoma” in 1887. Pieter Klazes Pel had described this phenomenon occurring with Hodgkin lymphoma a few months earlier in the same journal, thus the name Pel-Ebstein fever.

Since that time, some controversy has emerged regarding how often Pel-Ebstein fever actually occurs in Hodgkin lymphoma. Some have suggested that, because the fever’s name is based in history, students of medicine may be more familiar Pel-Ebstein's link to Hodgkin lymphoma than is deserved, based on the relative weakness of the association.

Nonetheless, periodic fevers in lymphomas are well documented in the medical literature. Here is a case adapted from a 1995 report in the "New England Journal of Medicine" that originally showed a graph of this man’s temperature, spiking high temperatures and normalizing in cycles, over weeks:

Case Report:

A 50-year-old man had a fever, night sweats, and a nonproductive cough for 10 weeks. He took anti-fever medications during the febrile periods. His wife recorded his temperatures on 56 of the 71 days. Biopsy of a rapidly enlarging cervical lymph node revealed nodular sclerosing Hodgkin's lymphoma. The patient's fevers and other symptoms promptly disappeared after the first cycle of chemotherapy.

Fever Patterns

Fever patterns are described in all kinds of different terms, which can be a source of confusion—e.g., intermittent fevers, remittent fevers, sustained fevers, recurrent fevers, etc. In many cases, these patterns can be suggestive but are not specific to any particular disease. However, here are some patterns that doctors sometimes consider:

Relapsing fevers with periods during which patients have no fever for one or more days between fever episodes may be seen with malaria, rat-bite fever, Borrelia infection — of Lyme disease fame, and lymphoma.

Recurrent episodes of fever over periods of six months can suggest a different set of illnesses, including immunodeficiency states.

Finding what is causing a persisting fever is in some cases a very complex process. You can learn more about this topic at fever of unknown origin, of FUO.