What is Pel-Ebstein Fever?

Doctor seeing a patient with a history of recurring high fever. Istockphoto.com/Stock Photo©AlexRaths

Question: What is Pel-Ebstein Fever?

Answer:

It’s 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. It was named after two European doctors, Pieter Pel and Wilhelm Ebstein, who noticed and reported the pattern more than a century back.

More on Pel-Ebstein Fever

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 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 fever, night sweats, and 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.

More on 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.

Relapsing fevers with periods during which patients have no fever for one or more days between feverepisodes 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.

Updated February 2016, TI.

Sources:

NCCN Clinical Practice Guidelines in Oncology: Hodgkin Lymphoma. Version 2.2015. National Comprehensive Cancer Network. Available at. Accessed: August 22, 2015.

Ryder RE, Mir MA, Freeman EA. An Aid to the MRCP PACES: Stations 1, 3 and 5. John Wiley & Sons, Apr 13, 2009.

Eichenauer DA, Engert A, André M, Federico M, Illidge T, Hutchings M, et al. Hodgkin's lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014 Sep. 25 Suppl 3:iii70-5

Schattner A, Keshet N. Pel-Ebstein cyclic fever: not just lymphoma. Am J Med. 2010;123(7):e3.

Racchi O, Rapezzi D, Ferraris AM, Gaetani GF. Unusual bone marrow relapse of Hodgkin's disease with typical Pel-Ebstein fever. Ann Hematol. 1996;73:39–40.

Good G, DiNubile M. Cyclic Fever in Hodgkin's Disease (Pel–Ebstein Fever). N Engl J Med. 1995;332:436.

Dall L, Stanford JF. Fever, Chills, and Night Sweats. In: Walker HK, Hall WD, Hurst JW, editors. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd edition. Boston: Butterworths; 1990. Chapter 211.

Continue Reading