January 17, 2007

Pel-Ebstein Fever

Synonyms and related 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


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:

Mortality/Morbidity:

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:

Physical:

Causes:


DIFFERENTIALS

Abdominal Abscess
Amebiasis
Arenaviruses
Bronchitis
Carcinoid Lung Tumors
Carcinoid Tumor, Intestinal
Catscratch Disease
Cellulitis
Chagas Disease (American Trypanosomiasis)
Chronic Bronchitis
Coxsackieviruses
Cryptococcosis
Dengue Fever
Echoviruses
Enterobacter Infections
Enterococcal Infections
Factitious Disorder
Fever of Unknown Origin
Human Herpesvirus Type 6
Hypersensitivity Reactions, Delayed
Hypersensitivity Reactions, Immediate
Inflammatory Bowel Disease


Other Problems to be Considered:

Pancreatic carcinoid tumor


WORKUP

Lab Studies:

Imaging Studies:

Procedures:


TREATMENT

Medical Care:

Surgical Care: Surgery is not indicated.

Consultations: Consulting a hematologist or oncologist for the management of the underlying HL is mandatory.

Diet: Diet modification is ineffective in the therapy for Pel-Ebstein fever.

Activity: Activity restriction does not play a role in treatment of Pel-Ebstein fever.


MEDICATION

Antipyretic therapy and chemotherapy for the underlying lymphoma are the 2 mainstays of medical therapy for Pel-Ebstein fever. Treatment of the underlying lymphoma or Hodgkin lymphoma (HL) is beyond the scope of this discussion (see Hodgkin Disease).

Drug Category: Antipyretic agents -- These agents are used to reduce uncomfortable fevers.
Drug Name
Acetaminophen (Tylenol) -- Reduces fever by acting directly on hypothalamic heat-regulating centers, which increases dissipation of body heat via vasodilation and sweating.
Adult Dose325-1000 mg PO q4h prn; not to exceed 4 g/d
Pediatric Dose10-15 mg/kg/dose PO q4-6h; not to exceed 5 doses/d
ContraindicationsDocumented hypersensitivity
InteractionsRifampin can reduce analgesic effects; barbiturates, carbamazepine, hydantoins, and isoniazid may increase hepatotoxicity
Pregnancy B - Usually safe but benefits must outweigh the risks.
PrecautionsHepatotoxicity possible in chronic alcoholism following various dose levels; severe or recurrent pain or high or continued fever may indicate serious illness; acetaminophen is contained in many OTC products, and combined use with these products may result in cumulative doses exceeding recommended maximum dose


FOLLOW-UP

Further Inpatient Care:

Further Outpatient Care:

In/Out Patient Meds:

Complications:

Prognosis:

Patient Education:


MISCELLANEOUS

Medical/Legal Pitfalls:

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