Hemophagocytic Lymphohistiocytosis in Association with Primary Cutaneous Anaplastic Large Cell Lymphoma

Abstract

Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)−) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors.

Primary cutaneous anaplastic large cell lymphoma

“…Disease and medication associations may offer insight into PC‐ALCL pathogenesis. PC‐ALCL has been linked to atopic dermatitis, mosquito bite hypersensitivity, hemophagocytic lymphohistiocytosis and a paraneoplastic syndrome of inappropriate antidiuretic hormone with tumor cells showing antidiuretic hormone expression . Other disease associations include case reports of chronic myelogenous leukemia in remission, keratosis lichenoides chronica and squamous cell carcinoma .…”

ALK-Positive Anaplastic Large Cell Lymphoma Associated With Hemophagocytic Lymphohistiocytosis

“…A literature review of ALCL-associated HLH in adults is presented in Table 2. Of the 14 cases found in the literature, eight patients improved and six patients died [6][7][8][9][10][11][12][13][14][15][16][17][18]. Seven patients were ALK-positive, five ALKnegative, and two ALK unknown.…”

Primary cutaneous CD30+ anaplastic large T cell lymphoma. A series of nine cases

“…There are conditions that have been associated with PALCL, such as atopic dermatitis, hypersensitivity to mosquito bites, hemophagocytic lymphohistiocytosis and paraneoplastic syndrome of inappropriate antidiuretic hormone secretion, chronic myelogenous leukemia, chronic lichenoid keratosis and squamous cell carcinoma. [26][27][28][29][30][31][32][33][34][35] Patient 5 in our series concomitantly suffered from advanced prostate cancer, and patient 8, mycosis fungoides prior to the PALCL diagnosis.…”