Malignant histiocytosis: A case of generalized histiocytosis with infiltration of Langerhans' granule-containing histiocytes

Abstract

A 9‐year‐old girl with leukemic infiltration of cells characterized by indented nuclei and abundant faintly acidophilic cytoplasm was autopsied. Electron microscopic studies of the cervical lymph nodes and tumor nodules in the liver and kidney (fixed in formalin solution for 3 years) together with the specimens taken from the cutaneous eruptions at autopsy demonstrated that the proliferating cells frequently contained Langerhans' granules in the cytoplasm. Accordingly, this case was thought to be a disease with systemic, progressive, and malignant proliferation of Langerhans' granule‐containing histiocytes (LGH). The intimate relationship between Letterer‐Siwe's disease and LGH has recently been recognized. Inasmuch as the present case satisfied both the clinicopathologic criteria of Letterer‐Siwe's disease and malignant histiocytosis, the proliferating histiocytes in both of them could be the same type of cell (LGH).

Origin and Function of Epidermal Langerhans Cells

“…LC, -as defined by their unique cytoplasmic organelles, -the Langerhans cell (or Birbeck) granules, were detected in various mesodermal tissues (Kondo 1969, Hoshino et al 1970, Breathnach 1977) and, furthermore, LC granules were found to be present in certain histiocytic cells in histiocytosis X (Basset & Turiaf 1965, Cancilla et al 1967) and occasionally, in malignant ceils of patients with malignant histiocytosis (Imamura et al 1971) and leukemic reticuloendotheliosis (Shamoto et al 1976). LC, -as defined by their unique cytoplasmic organelles, -the Langerhans cell (or Birbeck) granules, were detected in various mesodermal tissues (Kondo 1969, Hoshino et al 1970, Breathnach 1977) and, furthermore, LC granules were found to be present in certain histiocytic cells in histiocytosis X (Basset & Turiaf 1965, Cancilla et al 1967) and occasionally, in malignant ceils of patients with malignant histiocytosis (Imamura et al 1971) and leukemic reticuloendotheliosis (Shamoto et al 1976).…”

Langerhans Cell Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report

“…These were 9 cases (7 cases had aggressive clinical course and 2 cases had a benign clinical course.) reported on by Ben-Ezra et al (2), and 9 isolated cases (3-11). Of the 19 cases, including our case, the skin was involved at the time of diagnosis in 9 cases, and the skin was the only organ involved in 3 of the cases.…”

“…The lymph nodes, spleen, liver, bone marrow, thymus, lung and kidney were the other organs frequently involved. The heart was involved in two cases (2,3). The age at the time of diagnosis ranged from newborn (2) to 76 yr (7) with the average age being 37.…”

Leukemic transformation of Langerhans cell sarcoma

“…Imamura et al [10] described a 9-year-old girl with leukemic infiltration of Langerhans' granule-containing histiocytes characterized by indented nuclei and abundant, faintly acidophilic cytoplasm. The initial diagnosis of this patient was acute granulocytic leukemia, because the peripheral WBC count was 69,200 lL -1 , with 62% myeloblast-like, abnormal cells.…”