Pel–Ebstein fever is a rarely seen condition noted in patients with

Hodgkin's lymphoma Hodgkin lymphoma (HL) is a type of lymphoma, in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the patient's lymph nodes. The condition w ...

in which the patient experiences

fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...

s which cyclically increase then decrease over an average period of one or two weeks. A cyclic fever may also be associated with other conditions, but it is not called "Pel–Ebstein fever" unless the fever is associated with Hodgkin's.

Signs and symptoms

Causes

The cause is currently unknown although speculation centers on host immune response – particularly the cyclical release of

cytokines Cytokines are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are peptides and cannot cross the lipid bilayer of cells to enter the cytoplasm. Cytokines have been shown to be involved in autocrin ...

, lymph node

necrosis Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

, and damaged stromal cells.

Diagnosis

Cyclical fevers normally require periodic temperature monitoring to detect, though it is possible one could sense subjective changes in body temperature as well. To count as Pel-Ebstein fever diagnostic workup for Hodgkin's lymphoma would be required as well if that diagnosis was not already made.

Treatment

Treatment with

non-steroidal anti-inflammatory agents Non-steroidal anti-inflammatory drugs (NSAID) are members of a therapeutic drug class which reduces pain, decreases inflammation, decreases fever, and prevents blood clots. Side effects depend on the specific drug, its dose and duration of ...

or treatment of the underlying Hodgkin's (usually with

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...

) will help the symptoms.

Eponym

The condition is named after

Wilhelm Ebstein Wilhelm Ebstein (27 November 1836, Jawor, Jauer, Prussian Silesia – 22 October 1912) was a German physician. He proposed a Low-carbohydrate diet, low-carbohydrate high-fat diet to treat obesity. Ebstein's anomaly is named for him. Biography ...

and P. K. Pel who both published papers in 1887 noting the phenomenon. Both doctors published in the same journal, though Pel published first by several months. A long-term dispute persisted between Pel and Ebstein on the

etiology Etiology (pronounced ; alternatively: aetiology or ætiology) is the study of causation or origination. The word is derived from the Greek (''aitiología'') "giving a reason for" (, ''aitía'', "cause"); and ('' -logía''). More completely, e ...

of the condition.

Controversy

Researchers have speculated whether this condition truly exists, since some authorities anecdotally estimate only a 5–10% occurrence rate. In his Lettsomian Lecture ''Making Sense'', delivered to the Medical Society of London in 1959,

Richard Asher Richard Alan John Asher, FRCP (3 April 1912 – 25 April 1969) was an eminent British endocrinologist and haematologist. As the senior physician responsible for the mental observation ward at the Central Middlesex Hospital he described and n ...

refers to Pel–Ebstein fever as an example of a condition that exists only because it has a name. "Every student and every doctor knows that cases of Hodgkin's disease may show a fever that is high for one week and low for the next week and so on. Does this phenomenon really exist at all?...". They cite Richard Asher's lecture ''Making Sense'' (Lancet, 1959, 2, 359)

References

External links

{{DEFAULTSORT:Pel-Ebstein fever Fever Hematologic malignant neoplasms