Indeterminate cell histiocytosis in association with later occurrence of acute myeloblastic leukaemia

Abstract

Indeterminate cell histiocytosis (ICH) is a proliferation of indeterminate CD1a+, CD68+, S100+ and CD207- dermal dendritic cells. We describe a 39-year-old man who developed diffuse ICH and, 6 years later, acute myeloblastic leukaemia (AML). He was treated with cyclophosphamide, etoposide and vinblastine until 2003. In August 2004, he presented dyspnoea, hyperpyrexia and infiltration of the lung parenchyma, compatible with an AML invasion, and died after a course of induction chemotherapy. Cytomorphology and immunophenotype analyses suggested an ICH clonal evolution. The leukaemogenic role of etoposide is discussed. ICH has previously been reported in association with B-cell malignancy, but only one case has shown systemic progression.

Indeterminate Cell Histiocytosis in Association with Acute Myeloid Leukemia

“…An association between ICH and malignancies has been described in four patients: twice with low grade B-cell lymphomas [2], once with mast cell leukemia [8], and another with myelomonocitic leukemia [1]. Our patient presented a monocytic leukemia 7 months after the onset of the skin lesions.…”

“…Indeterminate cells are defined to be closely related to Langerhans cells, histologically and immunocytochemically, but do not contain Birbeck granules. Some authors have reported that indeterminate cells may represent precursors of Langerhans cells which acquire Birbeck granules as they transit from dermal to epidermal sites, possibly the result of the interaction between their cell receptors and epidermis-specific ligands [1, 2, 5]. Others suggest that indeterminate cells represent members of the dermal/epidermal dendritic cell system that migrate from the skin to the regional lymph nodes [1, 2, 5].…”

“…Some authors have reported that indeterminate cells may represent precursors of Langerhans cells which acquire Birbeck granules as they transit from dermal to epidermal sites, possibly the result of the interaction between their cell receptors and epidermis-specific ligands [1, 2, 5]. Others suggest that indeterminate cells represent members of the dermal/epidermal dendritic cell system that migrate from the skin to the regional lymph nodes [1, 2, 5]. Hence ICH seems to be a disorder of proliferating indeterminate cells which have been locally arrested before they can leave the skin travelling as veiled cells via the lymphatics to the T-cell-dependent paracortical areas of the regional lymph nodes [1, 2, 5].…”

“…Others suggest that indeterminate cells represent members of the dermal/epidermal dendritic cell system that migrate from the skin to the regional lymph nodes [1, 2, 5]. Hence ICH seems to be a disorder of proliferating indeterminate cells which have been locally arrested before they can leave the skin travelling as veiled cells via the lymphatics to the T-cell-dependent paracortical areas of the regional lymph nodes [1, 2, 5]. …”

“…Solitary lesions may also occur like soft nodules mainly on the trunk and arms. ICH lesions are usually restricted to the skin [1]. The major part of these cases have an indolent and benign course, not requiring aggressive treatment.…”