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PubMed1989

Virus-associated hemophagocytic syndrome: the diagnostic usefulness of immature histiocytes with benign features in the bone marrow.

A 2-year-old girl developed fever, hepatosplenomegaly, jaundice, lymphadenopathy and pancytopenia. Bone marrow examination revealed increased immature histiocytes (5.5%) and mature histiocytes with hemophagocytosis. All the abnormalities were normalized in one month without any chemotherapy. It was suggested that the presence of immature histiocytes with benign features, even if their number exceeds that of mature histiocytes, does not favor the diagnosis of malignant histiocytosis.

Bone Marrow↗

PubMedDate not supplied

Distribution pattern of concanavalin A on carcinoma cells, histiocytes and mesothelial cells from effusions.

Fixed and unfixed cancer cells, mesothelial cells and histiocytes were exposed to fluorescein-labeled concanavalin A (ConA-FITC). Cancer cells, whether fixed or unfixed, showed a similar pattern of fluorescence, as a continuous layer over the whole periphery of the cell. This pattern of ConA-FITC distribution was also obtained on fixed mesothelial cells and fixed histiocytes. Redistribution of ConA-FITC in form of "caps" and "patches" was recorded on unfixed mesothelial cells and unfixed histiocytes. Of the three cell types studied, only the histiocytes were lysed by the incubation with ConA-FITC.

Ascitic Fluid↗

PubMed1988

Expression of prostaglandin H synthase (cyclooxygenase) in Hodgkin's mononuclear and Reed-Sternberg cells. Functional resemblance between H-RS cells and histiocytes or interdigitating reticulum cells.

The synthesis of prostaglandin, prostacyclin, and thromboxane, which requires the enzyme prostaglandin H (PGH) synthase (cyclooxygenase), is a general property of histiocytes, monocytes, and Langerhans cells. Previously the authors reported the production of prostaglandin E2 in a Hodgkin's cell line, KM-H2, and suggested that these cells therefore have a functional similarity to histiocyte-related cells. The present study confirms that the Hodgkin's neoplastic (Reed-Sternberg, H-RS) cells in tissue are also capable of producing prostaglandins by demonstrating the presence of PGH synthase in these cells in B5-fixed, paraffin-embedded tissue sections. It was found that the H-RS cells from seven of ten patients with Hodgkin's disease were stained variously with anti-PGH synthase antibodies. In normal and reactive lymphoid tissues, anti-PGH synthase staining was restricted to histiocytes, endothelial cells, and interdigitating reticulum cells. Thus, this study provides further evidence for a possible relationship between H-RS cells and histiocytes or interdigitating reticulum cells; this relationship has also been supported by information obtained in extensive immunologic, biochemical, and cell-differentiation studies. The secretion of PGH-synthase products, especially prostaglandin E2, in H-RS cells may play a major role in the regulation of cellular immunity in patients with Hodgkin's disease.

Cell Line↗

PubMed1984

Heterogeneity of histiocytes in primary lung cancer stained with anti-S100 protein, lysozyme and OKT 6 antibodies.

The heterogeneity of histiocytes in primary lung cancer was investigated by immunohistochemical methods using anti-S100 protein and anti-lysozyme antibodies on paraffin sections and OKT-6 monoclonal antibody on frozen sections. T-zone histiocytes (Langerhans cells and their precursors stained by anti-S100 protein and/or OKT-6 monoclonal antibodies) heavily infiltrated tumor tissues and regional lymph nodes in cases of moderately or well-differentiated adenocarcinoma, especially in areas of papillary growth or bronchiolo-alveolar pattern. These cells were interspersed amongst tumor cells showing dendritic figures and were occasionally present in squamous cell carcinoma. However, they were seldom found in other histological types, particularly in small cell carcinoma and carcinoid tumor. The distribution of these cells was different from that of lysozyme-positive macrophages. In this connection, T-zone histiocytes were thought to have a different immunological function from that of the monocyte-macrophage series against lung cancer. The closely similar reactivity of T-zone histiocytes with anti-S100 protein and OKT-6 monoclonal antibodies indicated that these two markers can be employed for paraffin and frozen sections, respectively.

Adenocarcinoma↗

PubMed1980

Sea-blue histiocyte syndrome. A secondary degenerative process of macrophages?

A case of sea-blue histiocyte syndrome occurred in a 5-year-old boy. Associated laboratory findings include increased levels of hepatic phospholipids and glycosphingolipids, increased serum alkaline phosphatase level, and increased 24-hour urine mucopolysaccharide value. Bone marrow and liver biopsies and excision of chronically enlarged tonsillar tissue were performed. Macrophages that stained sea-blue with Wright-Giemsa stain were found in all tissues. Electron microscopic studies showed degenerating histiocytes packed with abundant loosely arranged myelin figures, some containing fingerprint-like cores formed by concentrically arranged lamellae with a periodicity of 45 A. These ultrastructural findings are compared with those obtained in other reported cases of the sea-blue histiocyte syndrome, as well as with those found in other clinical conditions in which degenerative macrophages are present. We conclude that the sea-blue histiocyte syndrome is a clinical entity that is associated with a variety of disease states; this entity is characterized by the presence of degenerating macrophages in various organs.

Bone Marrow↗

PubMed1982

Reticulum cell sarcomas (large cell lymphomas) presenting in the skin. High frequency of true histiocytic lymphoma.

The clinical and histologic data from 12 patients with "reticulum cell sarcoma" (large cell lymphoma) presenting in the skin were reviewed. Moreover, when appropriate material was available additional immunological, cytochemical and ultrastructural techniques were used to define the nature of the neoplastic cells. Eight tumors were found to be of true histiocytic origin (histiocytic sarcoma), three of B-cell origin (two B-immunoblastic lymphomas and one centroblastic or large noncleaved follicle center cell lymphoma) and one case could not be classified. Possible explanations for the discrepancy between the current report and other studies as to the frequency of true histiocytic tumors will be discussed. The differentiation into T-cell, B-cell and true histiocytic lymphomas appears to be important, not only because of different clinical behaviour, but possibly also from a therapeutical point of view.

Adult↗

PubMed1984

A case of large "thymic granuloma". Neoplasm of T-zone histiocyte.

An unusual case of classic thymic granuloma is reported. A 16-year-old boy presented with a huge mediastinal mass and the superior vena caval syndrome. Irradiation and combination chemotherapy induced complete remission, despite several episodes of lymph node recurrence. Examination of the first biopsy specimen was considered to indicate a histiocytic tumor or malignant form of eosinophilic granuloma, because of the diffuse proliferation of histiocytes and marked infiltration of eosinophils. Late involvement in the lymph node appeared to have the same histologic characteristics as the mediastinal tumor. Immunohistochemically, the proliferating histiocytes were stained with anti-S100 protein IgG but not with anti-lysozyme or anti-NCA (nonspecific cross-reacting antigen with carcinoembryonic antigen) antibody. These characteristics were similar to those of interdigitating cells in the lymph nodes, thymus, and other lymphoid tissues. The relationship of this case to mediastinal Hodgkin's disease or proliferative disorders of histiocytes is discussed.

Adolescent↗

PubMed1992

Malignant fibrous histiocytoma. A tumor of facultative histiocytes showing mesenchymal differentiation in cultured cell lines.

The histogenesis of malignant fibrous histiocytoma (MFH) is controversial. To elucidate the cellular origin and characteristics of this neoplasm, the authors analyzed cell lines grown from 17 patients (15 soft tissue MFH and 2 bone MFH) by using light and electron microscopy, immunocytochemistry, enzyme cytochemistry, and functional tests for receptors for the Fc portion of immunoglobulin (Fc receptors) and immunophagocytosis. Each culture exhibited a storiform/pleomorphic pattern with mixed cellular populations consisting of spindle cells, polygonal cells, and bizarre giant cells; these morphologic features corresponded to the histologic characteristics of the primary tumors. The cells in each MFH line displayed histiocytic functional markers such as lysosomal enzymes, Fc receptors and immunophagocytosis. However, these cells differed from monocyte-derived macrophages (histiocytes) in immunoreactivity; the MFH cells expressed a mesenchymal antigen (FU3) distributed among perivascular cells and fibroblasts but demonstrated no positive reactions with Leu-M1 (CD15) and Leu-M3 (CD14), which recognize the cells of the monocyte-macrophage lineage. In conclusion, these findings suggest that MFH is not a tumor of true histiocytes but of facultative histiocytes showing mesenchymal differentiation in vitro. Chromosomal analysis performed in one MFH line demonstrated abnormal karyotypes; the modal chromosome number was 58, with 5 marker chromosomes.

Adult↗

PubMed1975

1,3-bis(2-chloroethyl)-1-nitrosourea(BCNU), cyclophosphamide, vincristine- and prednisone-(BCOP). A new therapeutic regimen for diffuse histiocytic lymphoma.

Sixty-seven evaluable patients with mixed cellular and histiocytic lymphomas, both nodular and diffuse, were treated with a combined drug regimen of BCNU, cyclophosphamide, vincristine, and prednisone administered for six monthly courses. Of 28 previously untreated patients with diffuse histiocytic lymphoma, 14 (50%) achieved a complete remission, and an additional 7 (25%) had a good partial response for an overall remission rate of 75%. Complete remissions were also obtained among the small number of mixed and nodular histiocytic lymphomas which were treated. The median survival for previously untreated patients with diffuse histiocytic lymphoma who achieved a complete remission is nearly two years and 42% (8/19) of these patients remain in their initial unmaintained remission (range 2-119 weeks). Hematologic toxicity, although acceptable, was the limiting factor. Granulocytes were more often and more severely depressed than platelets.

Carmustine↗

PubMed2001

Cytologic clue of so-called nodular histiocytic hyperplasia of the pleura.

So-called "nodular histiocytic hyperplasia" (NHH) is a benign histiocytic lesion caused by mechanical irritation, inflammation, and tumor. Frequently, it has been confused with mesothelial lesions and other malignant neoplasms. The diagnostic clue is proliferating cells in the lesion showing diffuse, strong immunoreactivity against the histiocytic marker, CD68. Recently, we encountered a case of so-called NHH of the pleura and confused it with various malignant neoplasms on histologic examination. An 80-yr-old Korean female presented with ascites, pleural effusions, and nodules on the pleural base. Both ascites and pleural effusion tapping smears displayed moderate cellularity, vaguely nodular cellular aggregates mainly composed of mononuclear cells with bland morphology, entrapped mesothelial cells, and background lymphocytes. Pleural biopsy demonstrated vaguely nodular, compact cellular aggregates of reactive histiocytes which were immunoreactive against CD68. Based on our case, cytologic examination as well as immunohistochemical study should be stressed in the case of so-called NHH. They can provide us more credible morphologic clues to reach a more accurate diagnosis than histologic examination alone, and we can avoid invasive procedures or unnecessary therapies to patients. To our best knowledge, this is the first report describing the cytologic features of so-called NHH in the English-language literature.

Aged↗

PubMed1993

Malacoplakia and Rosai-Dorfman disease: two entities of histiocytic origin infrequently localized in the female breast--the cytologic aspect in aspirates obtained via fine-needle aspiration cytology.

We describe one case of Rosai-Dorfman disease (RDD) localized in the breast and one case of breast malacoplakia (MPK) both diagnosed by fine-needle aspiration cytology (FNAC). In RDD the cytologic features were lymphocytophagocytosis, large histiocytes with slight nuclear atypia, and lymphohistiocytic aggregates in a polymorphous background which included frequent plasma cells. In MPK the key diagnostic feature was the presence of Michaelis-Gutmann bodies (M-G bodies), in various stages of evolution, intermingled with vacuolized medium-sized histiocytes, other inflammatory cells, and debris. Pap staining is the technique of choice to show lymphocytophagocytosis and M-G bodies and there is no need to resort to special staining methods. RDD and MPK share a common cytologic background: the presence of numerous benign reactive histiocytes, either engulfing apparently intact lymphocytes, or phagocytizing M-G bodies. The cytologic findings seen in both cases suggest that the presence of histiocytes in a breast aspirate is far from being nonspecific and can represent an important lead to disparate diseases such as RDD and MPK.

Adult↗

PubMed2002

[Bone marrow manifestation of Niemann-Pick disease. A review of histiocytic proliferations of the bone marrow].

Niemann-Pick disease is an inherited autosomal recessive lysosomal storage disorder of sphingolipids that results in an accumulation of sphingomyelin in the cells of the reticulo-histiocyte system due to an enzyme defect. Type B of this disorder is characterised by a late onset and a variable manifestation of clinical symptoms. In the presented case a bone marrow biopsy was performed because of a pancytopenia and splenomegaly. A suspicious histiocyte proliferation was found in the bone marrow, showing typically large macrophages with foamy cytoplasm. Enzymatic analysis of a fibroblast culture confirmed the diagnosis of Niemann-Pick disease type B, with reduced activity of acid sphingomyelinase. A broad spectrum of disorders is associated with an increase in histiocytic cells within the bone marrow. In particular the differential diagnosis includes typical storage diseases, such as Gaucher's disease, and other metabolic disorders. Furthermore, reactive conditions with secondary increase in bone marrow macrophages have to be considered. In accordance with the presented patient the most common causes of histiocytic disorders involving the bone marrow are reviewed.

Bone Marrow↗

PubMed1988

Factor XIIIa and the classic histiocytic markers in malignant fibrous histiocytoma: a comparative immunohistochemical study.

Fifteen cases of malignant fibrous histiocytoma (MFH) and 79 cases of differential-diagnostically related soft tissue tumors were evaluated for immunoreactive cells for the subunit A of factor XIII (F-XIIIa) in comparison with the staining obtained by the classic histiocytic markers: lysozyme, alpha 1-antitrypsin (AAT) and alpha 1-antichymotrypsin (AACT). Ubiquitous and focal staining patterns were distinguished. Only three cases of MFH were characterized by an ubiquitous positive reaction for AAT and AACT, in contrast to the obligatory positive staining of MFH for F-XIIIa. This low ratio is probably related to the high proportion of predominantly fibroblastic and myxoid types of MFH (11/15). In the three cases of MFH characterized by ubiquitous positive reactions for both antiproteases and for F-XIIIa, the frequency of positive cells for AAT and AACT exceeded that for F-XIIIa. Thus, the positive cells for antiproteases and those for F-XIIIa represent different levels of fibro-histiocytic differentiation; the F-XIIIa-positive cells are fibro-histiocyte precursors. F-XIIIa-positive stromal cells are present in the normal mesenchyme, but their significance is unknown. The fact that these cells are a constant feature of MFH argues for a histiocytic pathway of their differentiation. The ubiquitous presence of F-XIIIa-positive cells in MFH distinguishes them from the histologically similar soft tissue tumors. However, the focal presence of F-XIIIa-positive cells indicate only a host response to an unspecified tissue injury that may occur in all kinds of soft tissue tumors.

Adult↗

PubMed1995

A case of pleomorphic T-cell lymphoma with a high content of reactive histiocytes presented with hypereosinophilia.

A case of peripheral T-cell lymphoma classified, according to the updated Kiel classification, as a large pleomorphic T-cell lymphoma with a high content of reactive histiocytes and blood hypereosinophilia is reported. Light microscopic examination revealed a diffuse effacement of the lymph node structure by large pleomorphic lymphoma cells mixed with eosinophils and many histiocytes, some of them presenting discrete features of hemophagocytosis. The neoplastic cells were CD3, CD5, CD8 and HLA-DR positive but failed to show CD30 antigen. DNA molecular analysis displayed simultaneous rearrangements of the genes coding for the delta chain of the T-cell receptor and for the Ig heavy chain. Increased serum levels of angiotensin converting enzyme and ferritin were found and probably induced by the reactive histiocytes. Immunoassays (ELISA) with antibodies directed against some cytokines and against the Tac peptide (sIL-2R) were performed. They demonstrated high serum levels of sIL-2R and a slight increase in GM-CSF, but neither IL-5 nor IL-3. The association of blood hypereosinophilia and histiocytic hyperplasia with a peripheral T-cell lymphoma is discussed.

Adult↗

PubMed1970

The two varieties of lymphoid tissue "reticulosarcomas", histiocytic and histioblastic types.

On the basis of histological sections and cytological smears in 110 cases, the "reticulosarcoma" (exclusive of Ewing's sarcoma and reticulosarcomas of bone marrow) were divided into two varieties: histiocytic types and histioblastic types.The correlation between the histological and cytological evaluation was excellent in each case; only those tumours classified as histiocytic presented a continuous and abundant network of reticulin.The histioblastic type predominated in the male sex. The difference in the clinical expressions of the two varieties is not statistically significant, except as to the frequency of cutaneous lesions: 27.7% in the histiocytic type and 2.6% in the histioblastic type.While the duration of their evolution is not different, only the histioblastic type is transformed into leukaemia, which is of the "monoblastic" type: this transformation was observed in 17.5% of cases, while it was never observed in histiocytic type.

Diagnosis, Differential↗

PubMed1999

Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation.

Two elderly women with complex medical histories presented with erythematous patches, in one case involving the face and forearms, and in the other both elbows. Punch biopsies from both patients revealed intravascular proliferations of medium-sized and large cells with luminal occlusion typical of angioendotheliomatosis. Immunostaining did not show either lymphocytic or endothelial cell antigens but was consistent with a histiocytic differentiation of the intravascular cells in both cases, and was further substantiated by ultrastructural examination in one case. One patient received a course of cyclophosphamide therapy over 15 days. Skin lesions faded but did not disappear. The patient died 10 months later from cardiac and renal failure, which was most probably unrelated to the skin lesions. In the other case, lesions diminished but did not entirely resolve with treatment with low doses of oral prednisone. Angioendotheliomatosis can be divided into a malignant variant, which is an angiotropic lymphoma mostly of B-cell phenotype, and a benign, reactive variant, which is characterized by a proliferation of cells expressing endothelial cell markers. Only one case of angioendotheliomatosis with cells of histiocytic differentiation has been published previously under the name of intravascular histiocytosis. Our cases are very similar to the latter. The question arises as to whether intravascular histiocytic cell proliferation is a neoplastic proliferation of histiocytes or an early stage of classic reactive angioendotheliomatosis representing the residual cells associated with organization of microthrombi, which will be later followed by endothelial cell proliferation.

Aged↗

PubMed1981

Radiologic manifestation of histiocytic lymphoma in the skeletal and central nervous system.

The radiographic manifestation of histiocytic lymphoma in the skeletal and central nervous system of 112 non-selected patients were reviewed in a retrospective study. Histiocytic lymphoma metastatic to bone and brain are late manifestations of the disease occurring in 15 and 4%, respectively. Involvement of the spinal cord and nerve roots is found in 6% and represents often the initial manifestation of the disease. Histiocytic lymphoma of brain presents angiographically as avascular mass without site predilection and on contrast enhanced computer tomography as hyperdense lesion without necrotic center. On myelography an extradural mass or complete block is found. The most common skeletal locations are spine, pelvis, rib and femur. The ratio of multifocal to unifocal skeletal manifestations is approximately 2 to 1. The radiographic appearance varies from completely lytic, often associated with a soft tissue mass, to osteoblastic, often with Pagetoid features. Early recognition of histiocytic lymphoma manifestations is important to guarantee prompt institution of appropriate therapy since the disease can progress extremely rapidly.

Adolescent↗

PubMed2001

Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease.

Erdheim-Chester disease (ECD) is a rare multisystem histiocytosis syndrome of unknown cause that usually affects adults. Histiocytic infiltration of multiple end organs produces bone pain, xanthelasma and xanthoma, exophthalmos, diabetes insipidus, and interstitial lung disease. Differential diagnosis includes Langerhans cell histiocytosis, metabolic disorders, malignancy, and sarcoidosis. ECD can be diagnosed using a combination of clinical and histopathologic findings. Sites of involvement include lung, bone, skin, retroorbital tissue, central nervous system, pituitary gland, retroperitoneum, and pericardium. Symmetrical long bone pain with associated osteosclerotic lesions, xanthomas around the eyelids, exophthalmos, and/or diabetes insipidus suggest ECD. Approximately 35% of patients have associated lung involvement, characterized by interstitial accumulations of histiocytic cells and fibrosis in a predominantly perilymphangitic and subpleural pattern. This pattern distinguishes ECD from other histiocytic disorders involving the lung. The diagnosis is confirmed by tissue biopsies that contain histiocytes with non-Langerhans cell features. In general, the clinical course of patients with this disease varies, and the prognosis can be poor despite treatment. Clinical trials for treatment of ECD have not been conducted and treatment is based on anecdotal experience.

Adult↗