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Brief Report
July 2018

Hemophagocytic Lymphohistiocytosis in Cutaneous T-Cell Lymphoma

Author Affiliations
  • 1Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois
  • 2Division of Hematology and Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
JAMA Dermatol. 2018;154(7):828-831. doi:10.1001/jamadermatol.2018.1264
Key Points

Question  What are the characteristics of patients with cutaneous T-cell lymphoma and hemophagocytic lymphohistiocytosis with respect to cutaneous T-cell lymphoma subtype, treatment received, and outcome?

Findings  In this case series of 7 patients, cytotoxic cutaneous T-cell lymphoma subtypes that involve the deep dermis and subcutaneous tissue were most commonly associated with hemophagocytic lymphohistiocytosis. Patients receiving allogeneic stem cell transplants had the best outcomes.

Meaning  Hemophagocytic lymphohistiocytosis is a rare, life-threatening complication of cutaneous T-cell lymphoma that is mostly associated with cytotoxic cutaneous T-cell lymphoma involving the deep dermis and subcutaneous tissue; awareness that it may mimic a granulomatous or infectious condition, resulting in delayed diagnosis and incorrect treatment, is critical.

Abstract

Importance  Hemophagocytic lymphohistiocytosis (HLH) has been reported as a serious complication of cutaneous T-cell lymphoma (CTCL). Despite available diagnostic guidelines, it remains a diagnostic and therapeutic challenge in this patient population.

Objectives  To examine the characteristics of CTCL associated with HLH and analyze the presenting signs and symptoms, therapeutic options, and outcome.

Design, Setting, and Participants  In this case series, patients diagnosed with CTCL and HLH who were treated at a single institution from January 1, 2014, through December 31, 2017, were studied.

Exposures  The HLH-2004 trial criteria, HScore, and various clinical and histopathologic variables were applied to and analyzed in the cohort.

Main Outcomes and Measures  Subtype of CTCL, treatment administered for HLH, and patient outcome were assessed.

Results  Seven patients (4 men and 3 women; median age, 50 years; range, 34-77 years) were identified from the database and included in the study. Cytotoxic subtypes of CTCL that involve the deep dermis and subcutaneous tissue were most commonly associated with HLH. Four patients met 5 or more HLH-2004 trial criteria, and 5 had an HScore probability greater than 85% at presentation. Common presenting HLH symptoms were fever and malaise. Cyclosporine, polychemotherapy, and systemic corticosteroids were the most common treatments. Patients receiving allogeneic stem cell transplants had the best outcomes, with all 3 of these patients alive and in complete remission.

Conclusions and Relevance  Hemophagocytic lymphohistiocytosis is a life-threatening complication of CTCL associated with rare cytotoxic CTCL subtypes that primarily involve the subcutaneous tissue. Because these cases may resemble a granulomatous or infectious condition, the diagnosis and appropriate management are often delayed. The results of this study demonstrate the need for high awareness of HLH in patients with panniculitic lymphomas and indicate that allogeneic stem cell transplantation may be the best option for a sustained remission.

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