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Updated: Mar 28, 2026
Flow Cytometry to Estimate Leukemia Stem Cells in Primary Acute Myeloid Leukemia and in Patient-derived-xenografts, at Diagnosis and Follow Up
Published on: March 26, 2018
[Langerhans cell sarcoma developing acute myeloid leukemia after achieving complete response by THP-COP]
Kota Hamaguchi1, Akari Hashimoto, Akihito Fujimi
1Department of Hematology and Oncology, Oji General Hospital.
Insights
This case study presents the first documented instance of Langerhans cell sarcoma (LCS) in a patient with idiopathic cytopenia of undetermined significance (ICUS), who later developed acute myeloid leukemia (AML). It highlights the aggressive nature of LCS and its rare association with other hematological malignancies.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Langerhans cell sarcoma (LCS) is an exceptionally rare and aggressive dendritic cell neoplasm, with fewer than 70 cases reported globally.
- Co-occurrence of LCS with other hematological malignancies is infrequently documented.
- Idiopathic cytopenia of undetermined significance (ICUS) represents a state of unexplained low blood cell counts.
Abstract:
An 86-year-old man presented with enlarged left submandibular, left inguinal, and superficial femoral lymph nodes. He was diagnosed with Langerhans cell sarcoma (LCS) on the basis of the histopathological findings of the left inguinal lymph node biopsy. In addition, laboratory examinations revealed normocytic normochromic anemia, and bone marrow aspiration and biopsy led to a diagnosis of idiopathic cytopenia of undetermined significance (ICUS). Because of the patient's age, he was administered a regimen of cyclophosphamide, pirarubicin, vincristine, and prednisolone (THP-COP), and achieved a partial response after six courses. However, he developed acute myeloid leukemia (AML) 11 months after completion of the THP-COP therapy, and received only supportive care until his death. LCS is an extremely rare and aggressive dendritic cell neoplasm. To the best of our knowledge, only 67 cases have been reported in the literature. There are case reports describing the concurrence of hematological malignancies. Herein, we report the first documented development of LCS in a patient with ICUS who progressed to AML, and summarize the published data on the epidemiology of and therapeutic options for LCS.
