CONTEXT Pulmonary Langerhans cell histiocytosis is the most common and best known pulmonary histiocytic lesion; however, the realm of pulmonary histiocytic lesions also includes an assortment of uncommon diseases that may exhibit pulmonary involvement. OBJECTIVE To review pulmonary Langerhans cell histiocytosis and other pulmonary histiocytoses to better ensure correct diagnosis and optimal a...

Myeloid sarcomas are extramedullary accumulations of immature myeloid cells that may present with or without evidence of pathologic involvement of the bone marrow or peripheral blood, and often coincide with or precede a diagnosis of acute myeloid leukemia (AML). A dearth of experimental models has hampered the study of myeloid sarcomas and led us to establish a new system in which tumor induct...

BACKGROUND Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, ...

Histiocytic necrotizing lymphadenitis (HNL) or Kikuchi's disease is a distinctive, self-limited disorder characterized by necrotizing cervical lymphadenopathy in young individuals. HNL is more prevalent among Asians and is a relatively common disorder among Koreans. A preceding fever, lymphopenia, and occasional skin rashes suggest a viral etiology and there have been sporadic reports of viral ...

Haematophagocytic activity is a salient feature of malignant histiocytic medullary reticulosis. This potency has also been observed as an aberrant property of non-histiocytic malignant cells or as a benign histiocytic process in response to infections or malignancies. We report the case of a patient who presented simultaneously with a limited stage small cell lung carcinoma and a fatal reactive...

CRDD: cutaneous Rosai-Dorfman disease RDD: Rosai-Dorfman disease INTRODUCTION Rosai-Dorfman disease (RDD) is a benign nonLangerhans cell histiocytic disorder often involving the cervical lymph nodes. Extranodal disease was reported in 43% of patients with RDD in a review of the literature, with the skin reported to be the most common extranodal site. In the absence of lymph node involvement, RD...

A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.

By BERTHA OTTENSTEIN, Ph.D., GERHARD SCHMIDT, M.D., AND S. J. THANNHAUSER, M.D. I N 1882. Charles Ernst Gaucher’ described as “epithelioma primitif de Ia rate” the disease which now bears his name. In his case he found the splenic pulp entirely replaced by large cells and attributed this condition to a primary tumorous growth, epithelioma of the spleen. Collier2 (1895) in England, and Picou and...

Erdheim- Chester disease is a rare non- Langerhans cell histiocytosis that usually involves the bones, heart, central nervous system, retroperitoneum, eyes, kidneys, skin and adrenals. Lungs are affected in up to one-half cases; at CT scan various patterns are described: interstitial disease, consolidations, micronodules and microcysts, with or without pleural involvement. We presented a case o...