EpistemisPel-Ebstein fever
Specialty: Hematology.
Why it occurs
- Hodgkin's lymphoma (classic variant, most frequently in subtypes of mixed cellularity or lymphocyte depletion; cyclical fever is mediated by the periodic and massive release of pyrogenic cytokines such as interleukin-1, interleukin-6 and tumor necrosis factor alpha produced by Reed-Sternberg cells and the inflammatory tumor microenvironment)
- Peripheral T-cell non-Hodgkin lymphoma
- Nodal or miliary tuberculosis (infectious simulator with hectic fever patterns that are difficult to control)
- Brucellosis (undulating fever pattern that can mimic hematological cyclicity)
- Multicentric Castleman disease (non-clonal lymphoproliferative disorder associated with extreme levels of IL-6).
Initial workup
Positron emission tomography combined with CT (PET-CT) of the whole body to identify foci of carbohydrate hypermetabolism in lymph nodes and extranodal organs (spleen, liver, bone); biopsy of suspicious lymph node by complete excision for histopathological study, immunohistochemistry (characteristic marking for CD15, CD30 and PAX5) and in situ hybridization for Epstein-Barr virus (LMP-1); complete blood count and peripheral blood smear; general laboratory analysis with acute phase reactants (C-reactive protein, very high ESR), transaminases, lactate dehydrogenase (LDH) and kidney function; Repeat blood cultures and urine cultures to systematically rule out a bacterial, fungal, or mycobacterial infectious origin of the fever.
red flags
Coexistence with strident inspiratory dyspnea, facial cyanosis, plethora and distension of the jugular veins and collaterals of the thoracic wall (superior vena cava syndrome due to progressive extrinsic mediastinal compression); neurological signs of acute spinal cord compression (paresthesias in the lower extremities, progressive paraparesis, incontinence or urinary retention of sudden onset in a patient with paravertebral lymph node involvement); profound pancytopenia with imminent risk of neutropenic sepsis or active intracranial hemorrhage.
Standard management
- Brentuximab vedotin — anti-CD30 conjugated antibody that induces apoptosis of neoplastic cells and reduces the secretion of pyrogenic cytokines in selected patients
- ABVD chemotherapy regimen — first choice systemic oncological treatment; The fever usually subsides permanently after the first therapeutic cycle
- Prednisone or dexamethasone — high-potency systemic corticosteroids with a potent antipyretic effect mediated by the inhibition of the synthesis of inflammatory cytokines, used for initial or palliative symptomatic control
- Ibuprofen / Naproxen (non-steroidal anti-inflammatories that block the synthesis of prostaglandin E2 in the hypothalamus, useful to control symptomatic febrile oscillations before definitive diagnosis, after exclusion of platelet contraindications).
Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.
- Area
- Hematology
- Listed causes
- 5
- Treatment options
- 4