Introduction
Background
Hodgkin lymphoma (HL) is an unusual lymphoma that can manifest with constitutional symptoms (eg, fever, weight loss, night sweats) termed B-type symptoms. The periodic fever associated with HL is known as the Pel-Ebstein fever.
The criteria for B-type symptoms include fever (ie, temperature >38°C [>100.4°F]) for 3 consecutive days, weight loss exceeding 10% of body weight in 6 months, and night sweats. Pruritus, previously considered an important systemic symptom, does not by itself have prognostic importance and is not sufficient on its own to classify a patient as possessing B-type symptoms.
Pathophysiology
The pathophysiologic mechanism of the Pel-Ebstein fever is unknown; however, it is presumed to be due to cyclic cytokine release.
Frequency
United States
No reliable objective data are known regarding the occurrence rate of classic Pel-Ebstein fever in a series of patients with HL. Anecdotally, some authorities estimate a 5-10% occurrence rate.
International
US and international rates of Pel-Ebstein fever are not known to differ.
Mortality/Morbidity
- The Pel-Ebstein fever is not an independent cause of either morbidity or mortality, although the high fevers can be quite uncomfortable.
- The episodic hemolysis sometimes associated with Pel-Ebstein fever has been suggested to be due to increased susceptibility of older erythrocytes to temperature-induced hemolysis. Two cases of Pel-Ebstein fever temporally associated with pancytopenia have been reported, which would seem to suggest a cytokine-mediated etiology.
Race
No evidence indicates that Pel-Ebstein fever manifests differently or with different frequencies according to race.
Sex
Pel-Ebstein fever is not known to be sex-linked.
Age
No frequency differences by age group are known.
Clinical
History
- Pel-Ebstein fever was described by 2 physicians in the late 1800s.
- Wilhelm Ebstein, a German internist who was a professor of medicine in Göttingen, described a patient with relapsing fever in "Das chronische Rückfallsfieber, eine neue Infektionskrankheit." Berliner klinische Wochenschrift, 1887, 24: 565-8, 837.
- Pieter Pel was a physician in Amsterdam who noted a similar finding years earlier in "Zur Symptomatologie der sogenannten Pseudo-Leukãmie." Berliner klinische Wochenschrift, 1885, 22: 3-7.
- Pel-Ebstein fever has been used as an example of a disease that exists only because it has a name.
Physical
- The diagnosis of Pel-Ebstein fever is made by examining a patient's temperature over several weeks.
- The classic description is of a relapsing, high-grade fever that can reach 40-40.5°C (105-106°F) and has a periodicity of 7-10 days. The fever spikes are abrupt in onset and resolution. The fever spikes overlie a temperature baseline that never quite reaches normal.
- Multiple, evenly spaced Beau lines in the nailbeds have been described as indicative of relapsing fever.
Causes
- The etiology of Pel-Ebstein fever is thought to be cytokine release. Cytokines known to be released by Reed-Sternberg cells include interleukin 13, macrophage colony-stimulating factor, transforming growth factor-alpha, interleukin 10, and the T cell–directed CC chemokine thymus- and activation-regulated chemokine. Monocyte chemotactic protein 1 and interleukin 8 have also been detected at increased levels in HL tissues.
More on Pel-Ebstein Fever |
 Overview: Pel-Ebstein Fever |
| Differential Diagnoses & Workup: Pel-Ebstein Fever |
| Treatment & Medication: Pel-Ebstein Fever |
| Follow-up: Pel-Ebstein Fever |
| References |
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References
Colvett KL, Patel D, Smith JK. Multiple Beau''s lines in a patient with fever of unknown origin. South Med J. Dec 1993;86(12):1424-6. [Medline].
Dankbaar H, Willemze R, Bieger R. Bone marrow disorders in Hodgkin''s disease: cyclic pancytopenia coinciding with Pel-Ebstein fever. Neth J Med. 1983;26(3):74-6. [Medline].
Good GR, DiNubile MJ. Images in clinical medicine. Cyclic fever in Hodgkin''s disease (Pel- Ebstein fever). N Engl J Med. Feb 16 1995;332(7):436. [Medline].
Hilson AJ. Pel-Ebstein fever [letter; comment]. N Engl J Med. Jul 6 1995;333(1):66-7. [Medline].
Koopmans RP, van den Born BJ, Kersten MJ, Hart W. [Clinical reasoning and decision making in practice. A 41-year old with periodic fever of unknown origin]. Ned Tijdschr Geneeskd. Sep 13 2003;147(37):1778-83. [Medline].
McKenna W, Lampert I, Oakley C, Goldman J. Pel-Ebstein fever coinciding with cyclical haemolytic anaemia and splenomegaly in a patient with Hodgkin''s disease. Scand J Haematol. Nov 1979;23(5):378-80. [Medline].
Molina Boix M, Ortega Gonzalez G, Garcia Perez B, Perez Gracia A. [Pel-Ebstein fever, hemolytic anemia and Hodgkin''s disease (letter)]. An Med Interna. Jul 1989;6(7):388-9. [Medline].
Racchi O, Rapezzi D, Ferraris AM, Gaetani GF. Unusual bone marrow relapse of Hodgkin''s disease with typical Pel- Ebstein fever. Ann Hematol. Jul 1996;73(1):39-40. [Medline].
Reimann HA. Periodic (Pel-Ebstein) fever of lymphomas. Ann Clin Lab Sci. Jan-Feb 1977;7(1):1-5. [Medline].
Storgaard L, Karle H. Fever and haemolysis in Hodgkin''s diseases. Acta Med Scand. Apr 1975;197(4):311-6. [Medline].
Talbot TR. Cases from the Osler Medical Service at Johns Hopkins University. Hodgkin''s disease with Pel-Ebstein fevers. Am J Med. Mar 2002;112(4):312-3. [Medline].
Further Reading
Keywords
Pel-Ebstein fever, periodic fever of lymphomas, Hodgkin disease, Hodgkin's disease, HD, Hodgkin's episodic fever, Hodgkin episodic fever, B symptoms, B-type symptoms, Hodgkin lymphoma, Hodgkin's lymphoma, HL, lymphoma, lymphoma-related fever, relapsing fever, hemolysis, hemolytic anemia, periodic fever spikes
