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Indeterminate cell histiocytosis in association with acute myeloid leukemia
Filipa Ventura1, Teresa Pereira, Maria da Luz Duarte
1Dermatology and Venereology Department, Braga Hospital, Apartado 2242, 4701-965 Braga, Portugal.
Insights
Indeterminate cell histiocytosis (ICH), a rare disorder, showed lesion regression with thalidomide. However, this case highlights its association with acute monocytic leukemia.
Area of Science:
- Dermatology
- Hematology
- Oncology
Background:
- Indeterminate cell histiocytosis (ICH) is a rare histiocytic disorder.
- ICH cells exhibit features of both Langerhans and non-Langerhans histiocytosis.
- This case presents a unique clinical scenario of ICH.
Observation:
- A 62-year-old man presented with firm nodular lesions on his upper lip.
- Histopathology, immunohistochemistry, and ultrastructural analysis confirmed ICH.
- The patient developed acute myeloid leukemia, subtype monocytic leukemia (M5), post-remission.
Findings:
- Thalidomide treatment led to near-complete regression of ICH lesions within 7 months.
- The patient experienced remission of ICH but subsequently developed acute monocytic leukemia.
- The case demonstrates a potential association between ICH and acute myeloid leukemia.
Implications:
- This case underscores the effectiveness of thalidomide in treating indeterminate cell histiocytosis.
- It highlights a rare but significant association between ICH and acute monocytic leukemia.
- Further research may elucidate the link between these conditions and inform treatment strategies.
Abstract:
Indeterminate cell histiocytosis (ICH) is a rare proliferative disorder, in which the predominant cells share morphologic and immunophenotypic features from both Langerhans and non-Langerhans cell histiocytosis. We describe a 62-year-old man presenting a 2-month history of firm nodular lesions on the upper lip. Histopathology, immunohistochemical, and ultrastructural analysis showed typical findings of ICH. The patient was treated with thalidomide and almost complete regression of the lesions was reached within 7 months. Nevertheless, one month after remission, he developed an acute myeloid leukemia of the subtype monocytic leukemia (M5). The patient's condition rapidly worsened and he died due to a respiratory failure four weeks later. We present this case because apart of being rare it joins the effectiveness of thalidomide and the association with an acute monocytic leukemia. A review of the literature is made.
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