Histiocytic sarcoma progressing from follicular lymphoma and mimicking acquired hemophagocytic lymphohistiocytosis
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LETTER TO THE EDITOR
Histiocytic sarcoma progressing from follicular lymphoma and mimicking acquired hemophagocytic lymphohistiocytosis Christoph Schünemann 1 & Gudrun Göhring 2 & Yvonne Lisa Behrens 2 & Hans-Heinrich Kreipe 3 & Arnold Ganser 1 & Felicitas Thol 1 Received: 13 July 2020 / Accepted: 15 July 2020 / Published online: 17 August 2020 # The Author(s) 2020
Dear Editor, Histiocytic sarcoma (HS) is a rare and aggressive nonLangerhans cell histiocytosis with a poor prognosis [1]. In a subset of cases, HS is clonally related to concurrent hematological malignancies, especially lymphomas [2]. Acquired hemophagocytic lymphohistiocytosis (HLH) is a systemic reactive process that includes macrophage activation with hemophagocytosis in the bone marrow [3]. A 57-year-old woman presented with a progressive hypopharyngeal tumor. B symptoms were absent, and laboratory results were unremarkable. Histologic examination showed a blastic infiltration with monocytoid/histiocytic morphology with CD68, CD163, CD14, and lysozyme expression (CD1a, CD3, CD20, CD34, CD56, CD117, MPO and S100 negative, immunohistochemistry (IHC)) (Fig. 1a). Staging revealed mediastinal, mesenterial, and retroperitoneal lymphadenopathy as well as an incidental follicular lymphoma (FL) grade 1 in the bone marrow with otherwise unaffected hematopoiesis. Histiocyte infiltration of the bone marrow was excluded. Fluorescence in situ hybridization (FISH) revealed the presence of translocation t(14;18) within the cervical HS as well as the FL of the bone marrow (Fig. 1b). Immunoglobulin heavy chain (IgH) rearrangement was present in the FL but absent in the cervical HS (PCR amplification). We diagnosed HS probably arising from follicular lymphoma.
* Felicitas Thol [email protected] 1
Department of Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Carl-Neuberg-Str.1, 30625 Hannover, Germany
2
Department of Human Genetics, Hannover Medical School, Carl-Neuberg-Str.1, 30625 Hannover, Germany
3
Institute of Pathology, Hannover Medical School, Carl-Neuberg-Str.1, 30625 Hannover, Germany
We initiated a therapy with R-CHOEP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone with etoposide) and documented a stable disease after two cycles of therapy. Subsequently, therapy continuation was delayed due to persisting fever. Extensive diagnostics and empiric antimicrobial therapies failed. The patient’s general condition markedly worsened with onset of a sepsis-like syndrome with a high fever, tachycardia, and hypotension as well as an unexplained progressive pancytopenia. Another bone marrow aspirate showed slightly increased cellularity, normal hematopoiesis, and extensive hemophagocytosis (Fig. 1c). Because of the clinical presentation and positive diagnostic criteria for HLH according to the HLH-2004 protocol [4] (5 out of 8 criteria, including rising ferritin of 7370 μg/L), we suspected malignancy-associated HLH (mal-HLH). We initiated corticosteroids promptly (dexamethasone 10 mg/m2) on
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