Histiocytic Sarcoma of the Cervical Spinal Cord with Secondary Involvement of the Skin / 대한피부과학회지

Histiocytic sarcoma is a rare, lymphohematopoietic malignant tumor comprised of tumor cells with the morphological and immunophenotypic features of mature histiocytes. A 35-year-old man presented with a disseminated histiocytic sarcoma that first occurred in the spinal cord and metastasized to the skin and lymph nodes. The tumor cells of the primary histiocytic sarcoma of the spinal cord were very large epithelioid cells with abundant eosinophilic cytoplasms and large, round-to-oval nuclei. In contrast, the metastatic histiocytic sarcoma of the skin was composed of relatively small polygonal cells with well-defined cell borders and high N/C (nucleus/cytoplasm) ratios. Immunohistochemically, both tumors were diffusely positive for histiocyte-associated antigens; but negative for epithelial, melanocyte, lymphoid, and dendritic cell antigens. It is important to recognize the morphological features and immunohistochemical characteristics of metastatic cells in order to ensure accurate diagnoses.

A Case with Disseminated Macrophage-related Histiocytic Sarcoma Diagnosed by Positive Histiocytic Markers / 대한혈액학회지

Malignant histiocytosis (MH) is characterized by an invasive, progressive proliferation of neoplastic histiocytes associated with jaundice, lymphadenopathy, refractory anemia, leukopenia, and often hepatic and splenic enlargement. As lymphoblastic leukemia and lymphoma are regarded as neoplasms of lymphoid cells, MH is thought to represent a malignant transformation of the macrophage and dendritic cells. A classification of malignant histiocytic disorders was oriented by cell lineage in the Histiocyte Society's 1987 version. So dendritic cell-related histiocytic sarcoma (localized or disseminated) and macrophage-related histiocytic sarcoma (localized or disseminated) are the recommended nosology. To establish the correct diagnosis, the major challenge seems to distinguish lymphoid from histiocytic cells. M-CSF receptor, lysozyme, Ki-M8, and S-100 protein, etc are useful markers for histiocytes and T-cell and B-cell lineage markers, such as CD3, CD20, and CD79, etc, for lymphocytes. We have experienced a patient with disseminated histiocytic sarcoma diagnosed by positive istiocytic markers, such as lysozyme, S-100 protein, and CD68.

A Clinical Study of Childhood Histiocytosis Syndrome / 대한소아혈액종양학회지

PURPOSE: We surveyed this study to find the factors related to clinical aspects of patients with histiocytosis syndrome. METHODS: We analyzed the clinical data of thirty patients retrospectively who were diagnosed as histiocytosis syndrome from January 1992 to December 1997 at Keimyung University Dong San Hospital. RESULTS: There were nine cases of Class I patients, twenty cases of Class II patients and one case of Class III patient. Male patients were eighteen, and female patients were twelve. Mean age at diagnosis was 4 years. The most common clinical manifestation was fever, and others were hepatosplenomegaly, pallor, respiratory symptom, and lymphadenopathy in order. Bone was involved in seven cases out of nine Class I patients. Single organ involvement happened in five cases out of Class I patients, two organ involvement happened in two patients, three or four organ involvement happened in one case of Class I patient respectively. Etiology of Class II were EBV in four patients, bacterial infection in four patients, and the others were candida, mycoplasma, mycobacterium tuberculosis. There were pancytopenia, coagulation defect, abnormal liver function tests on laboratory examinations. Most common histologic finding of Class I was proliferation and infilteration of histiocytes. Hemophagocytosis was common in bone marrow examination of Class II patients. Chemotherapy was undergone for seven patients out of nine Class I patients. Six of them showed complete remission. One of them died during chemotherapy. Thirteen patients out of twenty Class II patients are on complete remission, and five of them died. One Class III patient died during chmotherapy. CONCLUSION: The survival rate depends on age, Lahey's organ dysfunction score, severity, and sites of involved organ. One year survival rate by Kaplan-Meier method of ClassI and II patients was 87.5% and 72.2% respectively. In this study, Class II patients showed high mortality rate, so early diagnosis and treatment will be important.

A Case of Malignant Histiocytosis Showing Papulosquamous Skin Lesions and Fever as Initial Manifestations

We report a case of malignant histiocytosis which began with intermittent fever and scaly skin lesions. A 3-year-old girl presented with erythematous scaly papules on the face and the trunk, and high fever for 3 months. The cutaneous lesions consisted of widespread coin-sized erythematous scaly papules with marginal brownish pigmentation. She was anemic and thrombocytopenic and had impairment of the liver function. Histopathologic study of the skin lesions showed non-specfic findings except for hyperkeratosis. However, bone marrow examination revealed an increased number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide and vincristine. She died the next day after the treatment had begun.

A Case of Malignant Histiocytosis

Malignant histiocytosis is a rare, usually fatal malignant neoplasm of reticuloendothelial systems. The disease is associated with fever, malaise, weight loss, hepatosplenomegaly, lymphadenopathy, pancytopenia, jaundice, and purpura. A 44-year-old female patient is described who had multiple, purple crusted nodules and plaques in the skin. In the laboratory study, pancytopenia was noted on the peripheral blood. In addition many atypical histiocytes were seen on the bone marrow aspiration. A lesional biopsy showed nodular infiltrations of atypical histiocytes in the dermis and some erythrophagocytosis was seen. Immunohistochemically, the histiocytes were weakly stained for lysozyme and α-l-antichymotrypsin, but were unstained for S-100 protein, cytokeratin, CEA(carcinoembryonic antigen), pan T/B marker CD30(ki-1), UCHL-1 LCA(leukocyte common antigen), and α-l-antitrypsin.

A case of malignant histiocytosis with various skin manifestations / 대한피부과학회지

Malignant histiocytosis(MH) is a rapidly progressive and usally fatal disorder characterized clinically fever, generalized weakness, lymphadeno athy, hepatosplenomegaly accompanied by jaundice, and purpura. The frequency of skin manifestations has been repoterd about 15% of MH patients. Papules, plaques and niidules are commonly met. Histopathologically MH shows infiltrations of atypical histiocyte; with uarious maturity in many organs including skin. We present a case of a 32-year-old female with MH involving not only lymph node, liver, spleen and bone marrow but also skin with vaorious features ; erythematous papules, annular purpuric patches, subcutaneous nodules and hemomagic nodules. Immunohist,ochemically the majority of patients atypical histiocytes in lesional skin shows a-1 antitrysin positive, but lysozyme negative. It may suggest that these are poorly differentiated atypical histiocytes rather than well differentiated ones.

Clinical Analysis on 30 Cases of Histiocytosis / 昆明医科大学学报

The pathogenesis and clinical manifestation of histiocytosis are veriant, and misdiagnosis is relatively high. We reviewed a total of cases of this disease and related literature, and the possibility pathogenesis and its clinical features. were discussed.

Malignant histiocytosis: Clinicopathologic review of 18 cases with immunohistochemical study

We reviewed clinical and pathologic characteristics of 18 cases of malignant histiocytosis including 2 autopsy cases with special interest on their immunohistochemical characteristics. We report 3 cases of unusual immunohistochemical finding and postulate these cases may be a supportive evidence of the view that dendritic cells share common precursor with those of mononuclear phagocytic system. More accumulated cases in the future and careful analysis would be needed to answer the basic question about the origin of malignant histiocytosis.

CLINICAL ANALYSIS OF MALIGNANT HISTIOCYTOSIS (MH) IN 26 CASES / 重庆医科大学学报

The clinical manifestation of MH is variable, induding fever, anemia, hemorrhage, hepatosplenomegaly, enlarged lymphonodes and aggressive emaciation, so that the diagnosis of MH cannot be made clinically multiple sites and repeated bone marrow aspirations are very important diagnostic ways. The subjeat to explore efficacious treatment is importent in future