Pel-Ebstein Fever and Cyclical Pancytopenia as the Initial Manifestation of Peripheral T-Cell Lymphoma
Materials and Methods/Case Presentation/Objective:
A previously healthy 34-year-old woman presented with a two-year history of recurrent high fevers, night sweats, nausea, vomiting, abdominal pain, cyclical pancytopenia, 40-pound unintentional weight loss, and intermittent macular rash. Episodes initially lasted three to four days but progressively lengthened to approximately ten days before spontaneous resolution.
On admission, her temperature was 39.5°C, and examination demonstrated marked, nontender splenomegaly. Laboratory testing showed hemoglobin 9.8 g/dL, leukocytes 3.0 x 10^9/L, platelets 81 x 10^9/L, and lactate dehydrogenase 427 U/L. Peripheral smear and flow cytometry were unrevealing. Multidisciplinary infectious, rheumatologic, and hematologic evaluation including testing for HIV, CMV, EBV, tick-borne, fungal, parasitic, and periodic fever disorders were negative. PET/CT demonstrated markedly FDG-avid 22-cm splenomegaly and diffuse bone marrow uptake without bulky adenopathy. Bone marrow biopsy showed no lymphoma involvement. Given persistent diagnostic uncertainty and concern for primary splenic pathology, she underwent diagnostic and therapeutic splenectomy. Histopathologic examination of the spleen established CD30-positive peripheral T-cell lymphoma.
Results/Description/Main Outcome Measure(s): Recognition of the characteristic cyclical fever pattern in conjunction with persistent splenomegaly ultimately led to definitive tissue diagnosis. The patient subsequently received a total of 6 cycles of BV-CHP with a complete response on interval PET-CT with a Deauville Score of 1. She then underwent consolidation therapy with autologous peripheral blood stem cell transplant.
Conclusion(s).:
Pel-Ebstein fever with cyclical pancytopenia is an uncommon presentation of lymphoma. In patients with recurrent fever, unexplained cytopenias, and splenomegaly, recognizing this pattern may expedite tissue diagnosis and avoid prolonged nondiagnostic evaluation.
An earlier version was presented at the 2023 MN-ACP Scientific Meeting and 2024 ACP Internal Medicine Meeting. This encore submission includes new longitudinal treatment and outcome data through allogeneic transplantation, with continued remission. All rights reserved.
Trademarked Items:
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