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Review
. 2016 Dec 20;10(1):365.
doi: 10.1186/s13256-016-1143-9.

Rituximab, Etoposide, Methylprednisolone, High-Dose Cytarabine, and Cisplatin in the Treatment of Secondary Hemophagocytic Lymphohistiocytosis With Classical Hodgkin Lymphoma: A Case Report and Review of the Literature

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Free PMC article
Review

Rituximab, Etoposide, Methylprednisolone, High-Dose Cytarabine, and Cisplatin in the Treatment of Secondary Hemophagocytic Lymphohistiocytosis With Classical Hodgkin Lymphoma: A Case Report and Review of the Literature

Steve Hu et al. J Med Case Rep. .
Free PMC article

Abstract

Background: Hemophagocytic lymphohistiocytosis is becoming an increasingly recognized disorder in adults. Classical Hodgkin lymphoma is a relatively uncommon etiology of hemophagocytic lymphohistiocytosis and may complicate treatment options. Rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin are discussed here as a treatment regimen.

Case presentation: A 66-year-old Hispanic man previously in good health presented with a 1-month history of recurrent fevers, chills, and night sweats and a 3-week history of new onset jaundice. A bone marrow biopsy revealed a normocellular bone marrow with increased histiocytes with areas of hemophagocytic activity. He met five out of eight criteria for hemophagocytic lymphohistiocytosis diagnosis including fevers, pancytopenia, hemophagocytosis, ferritin of 23,292 ng/mL (>500 ng/mL), and soluble-CD25 of 15,330 pg/mL (>1033 pg/mL). A right cervical lymph node biopsy revealed CD15, CD30, MUM-1, and Epstein-Barr virus-encoded small ribonucleic acid-positive cells with morphologic findings of classical Hodgkin lymphoma, lymphocyte-rich subtype. He completed 2 weeks of hemophagocytic lymphohistiocytosis-directed therapy with etoposide and dexamethasone, but then was switched to rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin due to minimal improvement in his pancytopenia and hepatic impairment. He completed one full cycle of rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin with notable improvement in serial hepatic function panels and had an undetectable Epstein-Barr virus viral load. However, he eventually died due to complications of Enterococcus faecalis bacteremia and colonic microperforation in the setting of persistent pancytopenia.

Conclusions: This case discusses the challenges facing treatment of adult malignancy-associated hemophagocytic lymphohistiocytosis. Rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin may be a viable option for patients with secondary hemophagocytic lymphohistiocytosis and Hodgkin lymphoma who cannot tolerate standard therapies due to hepatic impairment. Targeted therapy and immunotherapy are promising new areas of developing treatments.

Keywords: Case report; EBER; ESHAP; Epstein–Barr virus; Hemophagocytic lymphohistiocytosis; Hodgkin lymphoma; Pancytopenia; Rituximab.

Figures

Fig. 1
Fig. 1
Hemophagocytic activity was present as evidenced by scattered histiocytes containing abundant red blood cells on the bone marrow aspirate. Wright-Giemsa, 1000× oil immersion
Fig. 2
Fig. 2
The bone marrow clot section showed focal areas contained rare large atypical cells (center of image) in a mixed background consisting of granulomatous inflammation, lymphocytes, eosinophils, and plasma cells. An atypical mitotic figure is present in the lower right corner. Hematoxylin and eosin, 400×
Fig. 3
Fig. 3
Areas with granulomatous inflammation in the bone marrow were positive for Epstein–Barr virus-encoded small RNA by in situ hybridization (200×)
Fig. 4
Fig. 4
The subsequent cervical lymph node biopsy shows large Reed–Sternberg cells and variants in a background of mixed inflammation, diagnostic of classical Hodgkin lymphoma. Hematoxylin and eosin, 400×

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