muramidase and Lymphatic-Diseases

Topics: Acid Phosphatase; Adolescent; Female; Histocytochemistry; Humans; Immunochemistry; Lymphatic Diseases; Male; Microscopy, Electron; Middle Aged; Muramidase; Naphthol AS D Esterase; Protease Inhibitors; Skin Neoplasms

Topics: Aneuploidy; Basophils; Blood Platelets; Cell Transformation, Neoplastic; Child; Clinical Enzyme Tests; Cytogenetics; Eosinophilia; Fetal Hemoglobin; Fever; Hematologic Diseases; Humans; Leukemia, Myeloid; Leukocyte Count; Lymphatic Diseases; Muramidase; Primary Myelofibrosis; Prognosis; Skin Manifestations; Thrombocytosis; Vitamin B 12

Lysozyme amyloidosis (ALys) is a form of hereditary systemic non-neuropathic amyloidosis, which is inherited in an autosomal dominant fashion. Lysozyme, which is the amyloidogenic precursor protein in ALys, is a ubiquitous bacteriolytic enzyme synthesized by hepatocytes, polymorphs and macrophages. The aim of this study is to describe the phenotype and outcome of patients with ALys including the role of solid organ transplantation.. Retrospective evaluation of patients with ALys.. UK National Amyloidosis Centre.. All 16 patients with ALys followed at the centre.. A family history of amyloidosis was present in every affected individual. Although the phenotype was broadly similar amongst those from the same kindred, there were marked phenotypic differences between kindreds who possessed the same amyloidogenic mutation. Symptomatic gastrointestinal (GI) amyloid was prevalent, and macroscopically visible amyloidotic lesions were present in nine of 10 patients who underwent GI endoscopy. All symptomatic ALys individuals had hepatic amyloid. Four patients received orthotopic liver transplants (OLT), three for spontaneous hepatic rupture and one case, who had extensive hepatic amyloid and a strong family history of hepatic rupture, pre-emptively. All of the liver grafts were functioning at censor 1.7, 5.8, 9.0 and 11.0 years after OLT. Five patients had progressive amyloidotic renal dysfunction culminating in end-stage renal failure, three of whom underwent renal transplantation (RTx). There was no evidence of renal allograft dysfunction at censor 6.6, 1.8 and 0.8 years after RTx.. Lysozyme amyloidosis is a disease of the GI tract, liver and kidneys, which has a slow natural history. There was a clear family history in all cases within this cohort, demonstrating a high clinical penetrance in the presence of an amyloidogenic lysozyme mutation. There is currently no amyloid-specific therapy for the condition which is managed symptomatically. OLT and RTx appear to be successful treatments for patients with liver rupture or end-stage renal disease, respectively, with excellent outcomes in terms of medium-term graft function and patient survival.

Topics: Adult; Aged; Amyloidosis, Familial; Child; Female; Gastrointestinal Diseases; Humans; Kidney Failure, Chronic; Kidney Transplantation; Liver Diseases; Liver Transplantation; Lymphatic Diseases; Male; Middle Aged; Muramidase; Mutation; Peptic Ulcer Hemorrhage; Phenotype; Purpura; Radionuclide Imaging; Retrospective Studies; Rupture, Spontaneous; Serum Amyloid P-Component; Sjogren's Syndrome; Survival Analysis; United Kingdom

Rheumatoid arthritis was diagnosed in a 30-year-old woman with erythema nodosum and arthritic symptoms since 1994, and she was treated with anti-rheumatic agents. Mediastinal and bilateral hilar lymphadenopathy and abnormal pulmonary shadows were detected in 1996, and she was admitted to our hospital in 1997. We also recognized the elevation of ACE and lysozyme, and found granulomas in a transbronchial lung biopsy and an arthrosis synovia biopsy. From these findings, sarcoidosis was diagnosed. Sarcoidosis demonstrating erythema nodosum, arthritis, and bilateral hilar lymphadenopathy is called Löfgren's syndrome. In Caucasians, Löfgren's syndrome is frequently encountered, but it is rare in Japanese. Our case had coexisting arthrosis symptoms, and satisfied the diagnosis criteria of rheumatic arthritis. Therefore, the differential diagnosis was important. We emphasize that it is necessary to consider Löfgren's syndrome when diagnosing patients with rheumatic features, even in Japan.

Topics: Adult; Arthritis; Arthritis, Rheumatoid; Biomarkers; Diagnosis, Differential; Erythema Nodosum; Female; Humans; Lung; Lymphatic Diseases; Muramidase; Peptidyl-Dipeptidase A; Sarcoidosis; Syndrome; Synovial Membrane; Tomography, X-Ray Computed

Immunohistochemical phenotyping of 7 cases of histioeosinophilic granulomas of thymus and 4 cases of reactive eosinophilic pleuritis was performed. All 11 cases of these 2 entities reacted identically. This supports the view that these 2 lesions are similar in nature. Both lesions are reactions to the presence of insufflated gas and its resorption.

Topics: Antibodies, Monoclonal; Biomarkers; Eosinophilic Granuloma; Humans; Immunohistochemistry; Lymphatic Diseases; Muramidase; Myasthenia Gravis; Phenotype; Pleurisy; Receptors, Cholinergic; Thymus Gland

Whereas Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is well described in lymph nodes and other organs, it is frequently not recognized in soft tissue. We studied the clinical and histologic features of 23 previously unreported soft tissue lesions from 17 patients (13 females, 4 males) who were 24 to 66 years of age (mean, 46 years). These lesions involved the extremities (12, 52%), trunk (6, 26%), head and neck (3, 13%), and the retroperitoneum (2, 9%). Associated lymph node involvement was present in four cases; most patients were asymptomatic. RDD of soft tissue had more subtle histologic features than its lymph node counterpart. Emperipolesis was less conspicuous and proliferating histiocytes were frequently spindled, associated with collagen deposition, and arranged in a vague storiform pattern with scattered lymphoplasmacytic aggregates. These features led to a variety of diagnoses, including benign inflammatory and fibrohistiocytic lesions (13 cases) as well as lymphoma and malignant fibrous histiocytoma (three cases). RDD was correctly diagnosed in only one case. Diagnosis was confirmed in 16 of 18 lesions by detection of S-100 protein and histiocytic markers KP1 (12 of 13) and lysozyme (eight of 11) in the characteristic histiocytes. Recognition that RDD of soft tissue occurs in an older patient population than does nodal RDD and that it mimics fibrous and inflammatory lesions of soft tissue is important.

Topics: Adipose Tissue; Adult; Aged; Connective Tissue; Connective Tissue Diseases; Female; Histiocytosis; Histiocytosis, Sinus; Humans; Immunohistochemistry; Lymphatic Diseases; Male; Middle Aged; Muramidase; S100 Proteins

The content of lysozyme in the phagocytosing peripheral blood cells was carried out in 50 patients with active pulmonary sarcoidosis depending on the clinical form, extension and duration of the pathological processes. The function of these cells showed essential changes in patients with widely spread old processes and those of long duration. The changes of lysozyme secretion by neutrophil granulocytes and monocytes were diverse that may be explained by different mechanisms of this process in the cells.

Topics: Chronic Disease; Lung Diseases; Lymphatic Diseases; Monocytes; Muramidase; Neutrophils; Phagocytosis; Sarcoidosis

The number and distribution of lysozyme and S100 immunoreactive cells were analyzed in ten cases of lymph node sarcoidosis. Three phases of granuloma development could be differentiated, each showing a typical immunohistological pattern. The early small granulomas consisted of lys- or very weakly lys+ mononuclear phagocytes and developed within an area of focalized accumulation of S100+ antigen-presenting cells. The mature large granulomas were composed of polygonal epithelioid cells with abundant cytoplasm showing very strong granular lysozyme positivity. S100+ cells could still be observed, mainly around the granulomas, but in diminished number. In the final fibrozing phase the epithelioid cells lost their lysozyme immunoreactivity and no S100+ antigen-presenting cells were present within or around the granulomas. In one patient granulomas with central necrosis and palisading, lys- epithelioid cells were also observed, possibly representing a different microenvironment (antigen/antibody equilibrium?). The change in the pattern and number of lysozyme and S100 immunoreactive cells probably reflects the development of the granulomas and is related to the activity of the disease.

Topics: Epithelium; Granuloma; Histocytochemistry; Humans; Lymph Nodes; Lymphatic Diseases; Muramidase; Necrosis; S100 Proteins; Sarcoidosis; Staining and Labeling

Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. Our results suggested that xanthomatous lesions of the skin to be composed of the histiocytic proliferation of two different cell lineages, i.e. S100+lyso-NCA- T-zone histiocytes and S100-lyso+NCA+ tissue macrophages. Only lesions of histiocytosis X were composed of the former cells. It is suggested that these markers will be useful in determining the delineation of the histiocytic system on the basis of functional heterogeneity.

Topics: alpha 1-Antichymotrypsin; alpha 1-Antitrypsin; Biopsy; Bone and Bones; Carcinoembryonic Antigen; Eosinophilic Granuloma; Histiocytoma, Benign Fibrous; Histiocytosis, Langerhans-Cell; Humans; Immunohistochemistry; Lymphatic Diseases; Muramidase; S100 Proteins; Skin; Skin Neoplasms; Xanthogranuloma, Juvenile

Topics: Arthritis; Female; Fingers; Histiocytes; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Middle Aged; Muramidase; Osteolysis; Radiography; Skin; Skin Neoplasms

A patient with immunohistochemically confirmed nasal T-cell lymphoma is reported. He developed systemic histiocytosis with marked hemophagocytosis, simulating malignant histiocytosis. The differential diagnosis from the latter is discussed.

Topics: Antigens, Surface; Diagnosis, Differential; Erythrocytes; Frozen Sections; Histiocytes; Histocytochemistry; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Lymphatic Metastasis; Lymphoma; Male; Middle Aged; Muramidase; Nasal Cavity; Nose Neoplasms; Phagocytosis; Staining and Labeling

Familial erythrophagocytic lymphohistiocytosis (FEL), a rare, rapidly fatal childhood disease, is characterized by fever, hepatosplenomegaly, pancytopenia, and widely disseminated lymphohistiocytic infiltrates with prominent erythrophagocytosis. Immunophenotypic, immunohistochemical, and ultrastructural studies of two siblings with FEL were performed in an effort to determine the nature of the proliferating histiocyte of FEL. These studies demonstrated that the FEL histiocytes lack S-100 protein, T6, and Birbeck granules, which are found in Langerhans and interdigitating dendritic cells. The FEL histiocytes express alpha 1-antichymotrypsin, Leu-M3, HLA-DR, and, variably, lysozyme and Leu-M1. Thus, the proliferating histiocyte of FEL is a member of the mononuclear phagocytic system and has a phenotype similar to that of the histiocytes that normally populate the sinuses of benign and reactive lymph nodes. These studies suggest that FEL may represent uncontrolled proliferation of sinusoidal histiocytes.

Topics: alpha 1-Antichymotrypsin; Antigens, Differentiation, T-Lymphocyte; Antigens, Surface; Bone Marrow; Chymotrypsin; Erythrocytes; Female; Histiocytes; Histocompatibility Antigens Class II; Histocytochemistry; HLA-DR Antigens; Humans; Immunologic Techniques; Infant; Infant, Newborn; Liver; Lymph Nodes; Lymphatic Diseases; Lymphocytes; Microscopy, Electron; Muramidase; Phagocytosis; Phenotype

We have studied the possible origin of histiocytic cells, present in fibrous histiocytomas (MFH) by using immunohistochemistry to demonstrate lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and receptors for peanut and soy bean agglutinin in tumour cells of MFH compared with their presence in tumour cells of malignant histiocytosis (MH) ('true' histiocytic lymphoma, 'true' histiocytic sarcoma). We included in this study a number of other soft tissue tumours (STT). Lysozyme was detected in half of the cases of malignant histiocytosis (n = 16) but in only two out of 77 MFH. alpha 1-Antitrypsin and alpha 1-antichymotrypsin usually occurred together although the latter was seen in more cases. Both markers were present in majority of cases of MH whereas they were detected in a minority of cases of MFH. MFH cases of the storiform subtype were less frequently stained than the pleomorphic or giant cell subtypes. Receptors for peanut or soy bean agglutinin were detected in nearly all MH cases, whereas their presence was only detected in a small number of MFH. Lysozyme was not detectable in other STT. alpha 1-Antitrypsin and alpha 1-antichymotrypsin were uncommonly present in other STT, except in osteosarcoma and rhabdomyosarcoma. These markers therefore have a limited value as indicators of a possible histiocytic origin of MFH. Lectins showed weak affinity for other STT. In accordance with others, we therefore conclude that the progenitor cell of MFH has to be sought within the undifferentiated mesenchymal cells and that histiocytes themselves probably do not give rise to MFH.

Topics: alpha 1-Antichymotrypsin; alpha 1-Antitrypsin; Chymotrypsin; Histiocytoma, Benign Fibrous; Humans; Immunoenzyme Techniques; Lectins; Lymphatic Diseases; Muramidase; Plant Lectins; Receptors, Mitogen; Soft Tissue Neoplasms; Soybean Proteins

The presence of lysozyme, alpha 1-antitrypsin (AT), alpha 1-antichymotrypsin (ACT), and cytoplasmic receptors for peanut and soy bean agglutinin and for concanavalin A (PNA, SBA, and ConA, respectively) was investigated in formalin-fixed, paraffin-embedded material from 16 cases of malignant histiocytosis. The tumors in these cases did not show phenotypic characteristics of T or B cells. Lysozyme and AT especially were found less frequently in tumor cells from malignant histiocytosis than in normal histiocytes, whereas ACT and binding sites for the lectins were maintained during malignancy. Specimens from 44 per cent of the cases were positive for lysozyme, 56 per cent for AT, 82 per cent for ACT, 88 per cent for PNA receptors, 94 per cent for SBA receptors, and 100 per cent for ConA receptors. Tumor cells from B- and T-cell lymphomas were negative for these markers. Plasma cells, granulocytes, and fibroblasts sometimes bound ConA, but not PNA or SBA. The cases of malignant histiocytosis were subdivided into three groups on the basis of grade of differentiation. The tumor cells from the cases in group 1 showed the highest degree of differentiation, those from group 2 an intermediate degree, and those from group 3 the lowest degree. Mitotic activity was present mainly in groups 1 and 2. Lysozyme was present most frequently in groups 1 and 3 and in cases with the least mitotic activity. Expression of AT was decreased in groups 2 and 3. The presence of phagocytosis, which is not obligatory for the diagnosis, was always correlated with ACT staining. The presence of binding sites for these lectins can be considered a useful marker for malignant histiocytes.

Topics: alpha 1-Antitrypsin; Chymotrypsin; Concanavalin A; Histiocytes; Histocytochemistry; Humans; Immunoenzyme Techniques; Lectins; Lymphatic Diseases; Mitosis; Muramidase; Peanut Agglutinin; Phagocytosis; Plant Lectins; Receptors, Mitogen; Soybean Proteins

Seven patients with Hodgkin's disease were studied for the presence of lysozyme and alpha-1-antitrypsin activity by immunoelectron microscopy. As a result, Reed-Sternberg cells, Hodgkin's cells, and atypical cells were distinctly positive for lysozyme in four cases and weakly positive in the remaining three cases. These cells were also positive for alpha-1-antitrypsin in all cases. Because the cells of the monocyte-macrophage lineage also bore lysozyme and alpha-1-antitrypsin, it is suggested that Reed-Sternberg cells, Hodgkin's cells, and the atypical cells are derived from the monocyte-macrophage lineage.

Topics: alpha 1-Antitrypsin; Histocytochemistry; Hodgkin Disease; Humans; Immunoenzyme Techniques; Leukemia, Myeloid; Lymph Nodes; Lymphatic Diseases; Macrophages; Microscopy, Electron; Monocytes; Muramidase; Sarcoidosis; Tuberculosis

Topics: alpha 1-Antitrypsin; Histiocytes; Humans; Lymphatic Diseases; Lymphoma, Large B-Cell, Diffuse; Muramidase; Phenotype; S100 Proteins; T-Lymphocytes; Terminology as Topic

A case of pagetoid reticulosis is presented. Histopathology showed infiltration of the epidermis by mononuclear cells. Twenty percent of the mononuclear cells showed the presence of lysozyme indicating a histiocytic origin. Electron microscopy confirmed the presence of lymphocytes and histiocytes but these cells were outnumbered by Sézary cells. The presence of large numbers of Sézary cells indicates that pagetoid reticulosis is a cutaneous T-cell lymphoma closely related to mycosis fungoides.

Topics: Adult; Histiocytes; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Male; Microscopy, Electron; Muramidase; Skin; Skin Neoplasms; Syndrome

Two Chinese patients with sinus histiocytosis and massive lymphadenopathy are reported. The results of enzyme and immunohistochemical studies are presented.

Topics: Acid Phosphatase; Adult; alpha 1-Antitrypsin; Asian People; Biopsy; Child; China; Esterases; Female; Histiocytes; Humans; Immunoenzyme Techniques; Immunoglobulins; Lymph Nodes; Lymphatic Diseases; Male; Muramidase; Otitis Media with Effusion; S100 Proteins

It was shown recently that monocytoid cells express B-cell-restricted antigens and polyclonal surface immunoglobulins, and the term monocytoid B lymphocytes (MBL) has thus been offered as a more appropriate designation. Although most commonly seen in toxoplasmic lymphadenitis, MBL have been observed in a variety of reactive and neoplastic conditions involving lymph nodes. In the present study MBL were found in 17 of 22 lymph nodes from 20 patients with the acquired immunodeficiency syndrome (AIDS) and AIDS-related lymphadenopathy. In all 17 samples, the MBL were found in lymph nodes with florid reactive follicular hyperplasia, and they were geographically close to the hyperplastic lymphoid follicles. However, MBL were not detected in lymph nodes showing involuted follicles or lymphocyte depletion. The disappearance of MBL apparently parallels the progressive involution of secondary follicles. Leu-3+/Leu-2+ (T-helper/T-suppressor) ratios were studied in 14 lymph node cell suspension samples and ten peripheral blood samples. The lymph node Leu-3+/Leu-2+ ratios were significantly lower in AIDS-related lymphadenopathy than in non-AIDS-related reactive follicular hyperplasia (P less than 0.001); the peripheral blood ratios were decreased in nine of the ten cases. The diminished T-helper status in patients with AIDS and AIDS-related lymphadenopathy may be relevant to the immunopathogenesis of follicular involution and, indirectly, to the disappearance of MBL.

Topics: Acquired Immunodeficiency Syndrome; Adolescent; Adult; Antigens, Differentiation, B-Lymphocyte; Antigens, Surface; B-Lymphocytes; Humans; Leukocyte Count; Lymph Nodes; Lymphatic Diseases; Male; Middle Aged; Muramidase; Receptors, Antigen, B-Cell; T-Lymphocytes

Fifty-seven cases of juvenile xanthogranuloma that fulfilled the classic description of histologic findings of the disease were analyzed clinicopathologically and immunohistochemically. Two forms could be distinguished: 47 cases of the infantile form and 10 of the adolescent and young adult form. The infantile lesion was found at birth in 8 patients (17%), and was noted within 1 year after birth in 33 (70%). Twenty-two had multiple lesions and five of the six for whom follow-up was feasible had spontaneous involution. About half of the lesions were located on the head and neck, 30% on the trunk, and 20% on the extremities. All six adolescents had a solitary tumor located in the head and neck region. Comparison of the latter form with reticulohistiocytoma and cutaneous fibrous histiocytoma was established from a differential point of view. Immunohistochemically, most lesions of juvenile xanthogranuloma displayed a positive reaction for lysozyme and alpha-1-antichymotrypsin and were negative for S-100 protein, thereby suggesting that the essential constituents of this lesion would derive from the mononuclear phagocyte system.

Topics: Adolescent; Adult; Child; Female; Histocytochemistry; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Male; Muramidase; S100 Proteins; Xanthogranuloma, Juvenile

A 71-year-old male presenting high fever, pancytopenia, liver dysfunction and jaundice died without a confirmed diagnosis. Microscopically, histiocytes with marked atypia and erythrophagocytosis had infiltrated the spleen, enlarged lymph nodes, liver, and bone marrow. From the above features this case was diagnosed as malignant histiocytosis. The infiltrating histiocytes were classified into three categories: 1) phagocytic cells, 2) atypical, mostly non-phagocytic cells, and 3) bizarre cells including multinucleated giant cells. An immunohistochemical study of histiocyte markers demonstrated that lysozyme was positive in the atypical cells and some phagocytic cells, whereas alpha-l-antitrypsin tended to be localized in the phagocytic cells. Bizarre cells were negative for both markers. No S-100 protein was demonstrated in the neoplastic cells. These immunohistochemical features suggested monocyte-phagocytic origin of the tumor in this case.

Topics: Aged; alpha 1-Antitrypsin; Bone Marrow; Histiocytes; Humans; Immunoglobulins; Liver Neoplasms; Lymph Nodes; Lymphatic Diseases; Male; Muramidase; Phagocytes; S100 Proteins; Splenic Neoplasms

Using the peroxidase antiperoxidase (PAP) method, lysozyme (LZM) was shown to exist in normal, reactive and neoplastic cells belonging to the mononuclear phagocyte system (MPS), but was not detected in histiocytosis X cells. Immunostaining for cytoplasmic LZM by the PAP method is useful for identification of mononuclear phagocytes and for diagnosis of the diseases in which these cells participate.

Topics: Granulomatosis with Polyangiitis; Histiocytes; Hodgkin Disease; Humans; Immunoenzyme Techniques; Inflammation; Leukemia, Myeloid; Lymphadenitis; Lymphatic Diseases; Lymphoma, Large B-Cell, Diffuse; Mononuclear Phagocyte System; Muramidase; Neoplasms

Topics: Antigens; Antigens, Neoplasm; Cell Adhesion Molecules; Glycoproteins; Histiocytes; Histiocytosis, Langerhans-Cell; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Muramidase; S100 Proteins; Skin; Skin Diseases; Skin Neoplasms; Xanthogranuloma, Juvenile

A 37-year-old Japanese man was admitted to our hospital because of high fever. The peripheral blood showed pancytopenia with a few erythrophagocytic histiocytes. A bone marrow aspirate revealed 12% immature histiocytes and 7% leukoerythrophagocytic histiocytes. A postmortem examination showed an infiltrative proliferation of immature histiocytes and erythrophagocytic histiocytes in the sinusoid of the liver, the urinary bladder, etc. These findings were consistent with malignant histiocytosis. In addition, these abnormal histiocytes showed a phenotype of S 100 protein +lysozyme.- In agreement with Watanabe's report, our findings suggest that malignant histiocytosis is the neoplasm of T-zone histiocytes.

Topics: Adult; Histiocytes; Humans; Lymphatic Diseases; Male; Muramidase; S100 Proteins

Epidermal mononuclear cell infiltrate from three patients with pagetoid reticulosis was examined for the presence of the cytoplasmic markers lysozyme, alpha 1-antitrypsin and alpha 1-antichymotrypsin, using specific antisera and a peroxidase-antiperoxidase technique. Many of the infiltrating cells possessed these markers, indicating that they belonged to the monocyte-macrophage-histiocyte series.

Topics: Adult; alpha 1-Antitrypsin; Chymotrypsin; Histiocytes; Histocytochemistry; Humans; Lymphatic Diseases; Macrophages; Male; Middle Aged; Monocytes; Muramidase; Skin Neoplasms

Topics: Adult; Humans; Lymphatic Diseases; Muramidase; Peptidyl-Dipeptidase A

Topics: Female; Humans; Infant; Isocitrate Dehydrogenase; Leukemia, Monocytic, Acute; Lymphatic Diseases; Male; Muramidase; Peptidyl-Dipeptidase A

Eight cases with malignant histiocytosis (MH), two cases with systemic Letterer-Siwe disease, and one case with sarcomatous variant of MH were studied clinicopathologically. Characterization of neoplastic histiocytes was performed by immunohistochemical staining for S100 protein, lysozyme, and nonspecific cross reacting antigen with carcinoembryonic antigen (NCA). The immunohistochemical characteristics of histiocytes were S100+lys-NCA- in eight MH and two Letterer-Siwe disease cases and S100-lys+NCA+ in the sarcomatous variant of MH. MH and Letterer-Siwe disease were considered to have derived from a specific S100+ histiocytic cell lineage (T-zone histiocyte with S100 protein) independent of the monocyte--macrophage system, from which a sarcomatous variant was derived. Leukemic change of MH was discussed with special reference to the maturation and differentiation of T-zone histiocytes.

Topics: Adult; Antigens, Neoplasm; Cell Adhesion Molecules; Child; Child, Preschool; Female; Glycoproteins; Histiocytes; Histiocytosis, Langerhans-Cell; Histocytochemistry; Humans; Infant; Infant, Newborn; Lymphatic Diseases; Male; Middle Aged; Muramidase; S100 Proteins

Since lysozyme and alpha 1-anti-chymotrypsin are constituents of normal histiocytes, their value as tumor cell markers in histiocytes neoplasias has been investigated using the indirect immunoperoxidase method and commercially available specific antisera on formaldehyde-fixed, paraffin-embedded 5 micrometers sections after pretreatment with pronase. The distribution of both markers was determined in 35 cases of malignant fibrous histiocytoma (MFH) and in 13 cases of malignant histiocytosis (MH). In 12 cases of MH both markers were found whereas in MFH alpha 1-antichymotrypsin was demonstrated in 26 and lysozyme in 16 cases only. In general, the staining for alpha 1-anti-chymotrypsin was more intense than the staining for lysozyme. A negative reaction does not exclude the possibility of MH or MFH. The presence of both constituents in tumours, however, can be considered as indicative of histiocytogenic origin and both can be useful markers for distinguishing histiocytic neoplasias from other tumours.

Topics: alpha 1-Antichymotrypsin; Chymotrypsin; Diagnosis, Differential; Histiocytoma, Benign Fibrous; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Muramidase; Protease Inhibitors; Trypsin Inhibitors

Topics: Humans; Immunoenzyme Techniques; Lymphatic Diseases; Muramidase

Previously we reported that malignant histiocytosis presenting as lethal midline granuloma (MH-LMG) showed rapidly progressive course (mean survival 14 months). Because of the same biopsy findings of polymorphic cellular infiltrates (PCI), we considered that MH-LMG and midline malignant reticulosis (MMR) are similar disease. In this paper, we studied 5 cases with MH-LMG showing slow clinical course with more than 5 year survivals, and a similarity found in MH-LMG and MMR in which disseminated and localized disease are present, is discussed. Among clinical data, no abnormality in hematologic findings in MH-LMG with slow clinical course was the only different point from that with progressive course. Biopsy findings of PCI in which atypical histiocytes with positive reactions for lysozyme by immunoperoxidase procedures were present, were identical with those in MH-LMG with progressive course. Autopsy findings justified a diagnosis of MH. MH with slow clinical course seems to be identical with MMR showing localized lesion. Therefore a term MMR should be replaced by a term MH-LMG in the classification of LMG.

Topics: Adult; Biopsy; Child; Female; Histiocytes; Humans; Lymphatic Diseases; Male; Middle Aged; Muramidase; Prognosis; Time Factors

Malignant histiocytosis (MH) is a true histiocytic disorder, whose identification is still based on too broad morphologic criteria. Using routine histology, cytochemical and immunohistochemical techniques on involved lymph nodes, 15 cases of MH have been investigated. Pleomorphism and cellular atypia, phagocytosis, lack of cohesiveness between proliferating cells, sinusoidal involvement, and plasmacytic infiltrate were the most common histologic features. MGG-stained imprints from 14 cases showed a composite tumor population mainly consisting of histiocyte-appearing cells, poorly differentiated atypical cells, and multinucleated giant cells. These cells, irrespective of cytologic features, revealed a diffuse, moderately to strongly positive reaction with acid phosphatase and nonspecific esterase. Naphthol-AS-D-chloroacetate esterase, Sudan black B, alkaline phosphatase, and beta-glucuronidase reactions were completely negative. Immunoperoxidase studies in 11 cases demonstrated that tumor cells stained positively for both kappa and lambda chains. These cells were also positive for albumin. Polytypic staining for IgG was observed in two cases, and a weak staining for lysozyme was found in two other nodes. Global results confirm the value of these studies for functional profile determination of MH proliferating cells. A combined approach using a variety of cytochemical and immunohistochemical techniques should be routinely considered in MH as useful additional studies for a more precise diagnostic definition of the disease.

Topics: Adolescent; Adult; Albumins; Biopsy; Child; Child, Preschool; Female; Histiocytes; Humans; Immunoglobulin G; Immunoglobulin kappa-Chains; Immunoglobulin lambda-Chains; Lymph Nodes; Lymphatic Diseases; Male; Muramidase; Phagocytosis; Staining and Labeling

Histiocytosis X, multicentric reticulohistiocytosis, juvenile xanthogranuloma, the "fibrous" type of dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma are all characterized by dermal and/or subcutaneous infiltrates composed at least partially of cells having morphologic features suggestive of histiocytes. Paraffin-embedded tissues representing these conditions were stained for lysozyme (muramidase) with a peroxidase-antiperoxidase technic. The cells of juvenile xanthogranuloma were rich in lysozyme. Some of the cells of histiocytosis X showed a positive pattern, and the cells of the other three conditions were essentially negative. This study confirmed the histiocytic nature of juvenile xanthogranuloma and multicentric reticulohistiocytosis, supported the interpretation that there is a histiocytic component in the lesions of histiocytosis X, and cast some doubt on the alleged histiocytic nature of "fibrous" dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma.

Topics: Fibroma; Granuloma; Histiocytes; Histiocytoma, Benign Fibrous; Histiocytosis, Langerhans-Cell; Humans; Immunoenzyme Techniques; Lipoma; Lymphatic Diseases; Muramidase; Skin Diseases; Skin Neoplasms

Topics: alpha 1-Antichymotrypsin; Chymotrypsin; Diagnosis, Differential; Histiocytes; Histiocytoma, Benign Fibrous; Histiocytosis, Langerhans-Cell; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Muramidase

In two patients who had malignant histiocytosis, renal involvement was present at an early stage of their diseases and consisted clinically of proteinuria and renal failure. The associated renal lesion was characterized by a diffuse and global endocapillary hypercellularity of the glomeruli imputable to atypical cells occluding the capillary loops. Immunoglobulins were absent from this lesion. The atypical cells were positively identified by lysozyme immunoperoxidase study as malignant histiocytes. It is suggested that renal biopsy complemented by marker study could play a role in the diagnosis of malignant histiocytosis.

Topics: Aged; Basement Membrane; Bone Marrow; Erythrocytes; Humans; Immunoenzyme Techniques; Kidney Diseases; Kidney Glomerulus; Lymphatic Diseases; Male; Middle Aged; Muramidase; Phagocytosis; Spleen

Topics: alpha 1-Antichymotrypsin; Chymotrypsin; Histiocytoma, Benign Fibrous; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Muramidase; Trypsin Inhibitors

The immunohistochemical and ultrastructural characteristics of involved lymph nodes from four patients with typical clinical and histopathologic features of malignant histiocytosis are reported. The neoplastic cells in all four cases had numerous irregular surface projections and pseudopodia, as well as lobulated or biolobed nuclei with macronucleoli. The cytoplasm contained many lysosomes, mitochondria, and polyribosomes, and displayed evidence of phagocytosis. Langerhans' granules were not identified. The ultrastructural features in the four cases were similar, effectively excluded an epithelial origin in each instance, and delineated morphological characteristics common to neoplastic macrophages. Intracytoplasmic muramidase (CM), an enzyme marker essentially restricted to cells of myeloid and true histiocytic origin, was identified with the unlabeled immunocytochemical technique and was strongly positive in neoplastic histiocytes at all stages of differentiation. Thus, fine structural features and the presence of CM are useful confirmatory studies in the diagnosis of malignant histiocytosis.

Topics: Cell Membrane; Histiocytes; Humans; Immunoglobulins; Lymph Nodes; Lymphatic Diseases; Muramidase; Neutrophils; Organoids; Phagocytosis

Malignant histiocytosis (MH) is a rare, usually fatal systemic disease considered to be a neoplasm of true histiocytes. Because MH may be difficult to differentiate from non-Hodgkin's lymphomas or carcinoma, we examined surgical and autopsy material from 10 patients with MH using the immunoperoxidase technique to determine if the presence of intracellular lysozyme is helpful in making this distinction. The cases of MH were divided into three groups based on the degree of cytologic atypia and the amount of phagocytic activity of the neoplastic cells: group I--minimal cytologic atypia and rare erythrophagocytosis; group II--minimal cytologic atypia with extensive erythrophagocytosis: group III--moderate to marked cytologic atypia and rare phagocytosis. Moderate to strong staining for lysozyme was observed in the neoplastic cells of group I, weak or absent staining in group II cells, and no staining in group III cells. These findings suggest the loss of detectable enzyme in poorly differentiated or dedifferentiated neoplastic histiocytes. Consideration must be given to these observations in evaluating the use of lysozyme as a possible serum or tissue aid to the diagnosis of MH.

Topics: Adolescent; Adult; Aged; Cell Differentiation; Diagnosis, Differential; Female; Histiocytes; Histocytochemistry; Humans; Lymphatic Diseases; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Muramidase; Phagocytosis

We studied the value of serum lysozyme as a helpful test in distinguising tuberculous involvement of intrathoracic glands from lymphoma. Nineteen of the 28 patients (all Asian immigrants) with intrathoracic glandular tuberculosis had raised serum lysozyme level as compared with 2 of the 29 patients with lymphoma. While a normal serum lysozyme level is unhelpful, a raised level in an Asian immigrant with hilar or mediastinal lymphadenopathy makes tuberculosis a highly probable diagnosis.

Topics: Africa, Eastern; Bronchial Neoplasms; Clinical Enzyme Tests; Diagnosis, Differential; Female; Humans; India; Lymphatic Diseases; Lymphoma; Male; Muramidase; Sarcoidosis; Thoracic Neoplasms; Tuberculosis, Lymph Node; Tuberculosis, Pulmonary; Wales

Serum angiotensin-converting enzyme (ACE) activity was studied in healthy controls, in 57 untreated sarcoidosis patients, and in 164 patients with other chest or lymph node diseases. The serum ACE activity of healthy persons was independent of sex, intake of meals, and smoking habits. There were no diurnal variations. Healthy children had a significantly higher ACE mean value than adults, whose ACE activity was not affected by age. The sarcoidosis patients had the highest ACE mean values, but those of patients with silicosis and asbestosis were also significantly elevated. Pulmonary cancer patients had decreased serum ACE activity, which was probably due to antimitotic treatment. Serum lysozyme (LZM) concentrations did not correlate with normal ACE activity, but the correlation between elevated ACE and LZM was significant in sarcoidosis and silicosis, and the trend was clearly the same for asbestosis. This indicates separate sources for these enzymes when ACE activity is normal, and a common source, i.e. macrophages, when ACE activity is increased. ACE production in certain diseases involving macrophages may be due to the bradykinin inhibiting effect of this enzyme.

Topics: Adolescent; Adult; Alveolitis, Extrinsic Allergic; Asbestosis; Bronchitis; Female; Hodgkin Disease; Humans; Lung Diseases; Lung Neoplasms; Lymphatic Diseases; Lymphoma; Male; Middle Aged; Muramidase; Peptidyl-Dipeptidase A; Pneumonia; Pulmonary Fibrosis; Sarcoidosis; Silicosis; Thoracic Neoplasms; Tuberculosis, Lymph Node; Tuberculosis, Pulmonary

Topics: Humans; Immunoenzyme Techniques; Intestinal Neoplasms; Lymphatic Diseases; Muramidase

Serum lysozyme levels were significantly raised in a group of eight patients with malabsorption associated with gastrointestinal lymphomas of a type recently characterised as malignant histiocytosis of the intestine. In four of the cases, levels were markedly raised. In contrast there was no significant difference between groups of patients with uncomplicated adult coeliac disease and healthy controls. The estimation of serum lysozyme is a simple test to perform and may be valuable in the diagnosis of malignant histiocytosis of the intestine, in particular differentiating it from uncomplicated adult coeliac disease.

Topics: Adolescent; Adult; Aged; Celiac Disease; Female; Humans; Intestinal Neoplasms; Lymphatic Diseases; Male; Middle Aged; Muramidase

Topics: Humans; Immunoenzyme Techniques; Lymphatic Diseases; Lymphoma, Non-Hodgkin; Muramidase

Topics: Clinical Enzyme Tests; Humans; Lymphatic Diseases; Muramidase

Topics: Adult; Humans; Lymphatic Diseases; Male; Muramidase

Infusion of cycloheximide i.v., an antibiotic known to inhibit synthesis of protein, at a rate of 0.2 mg/kg/hr, reliably caused lysis of fever in 15 chronically febrile patients with Hodgkin's disease who did not have detectable bacterial, fungal, or viral infection. Antipyretic effects were also seen in some patients with reticulum cell sarcoma, lymphosarcoma, acute leukemia, histiocytic medullary reticulosis, plasma cell myeloma, carcinoma of the lung, and carcinoma of the cervix. The drug failed to produce defervescence in four patients with normal granulocyte reserves, who were febrile due to bacterial infection. When infused at a rate of 0.2 mg/kg/hr, the drug apparently caused an acute alteration of protein metabolism in man in that plasma amino acid nitrogen rose acutely while plasma levels of muramidase and ribonuclease fell during the period of the infusion. The data suggest that continuing synthesis of protein may be involved in nonbacterial fever of neoplastic disease. Mammalian granulocytes and monocytes are known to elaborate a pyrogenic protein following appropriate stimulation; it is suggested that in some types of neoplastic disease, particularly Hodgkin's disease, tumor cells may produce and release a pyrogenic protein and that drug-induced inhibition of its synthesis is responsible for the observed lysis of fever.

Topics: Bacterial Infections; Cycloheximide; Female; Fever; Hodgkin Disease; Humans; Leukemia; Lung Neoplasms; Lymphatic Diseases; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Non-Hodgkin; Multiple Myeloma; Muramidase; Neoplasm Proteins; Neoplasms; Nitrogen; Ribonucleases; Uterine Cervical Neoplasms

Topics: Humans; Leukemia; Lymphatic Diseases; Muramidase

Topics: Cell Line; Granulocytes; Humans; Leukemia; Leukocytes; Lymphatic Diseases; Monocytes; Muramidase; Spectrophotometry

Topics: Adult; Cell Membrane; Cells, Cultured; Fluorescent Antibody Technique; Glucose; Histocytochemistry; Humans; Immunoglobulins; Lectins; Leukocyte Count; Lymphatic Diseases; Male; Microscopy, Electron; Middle Aged; Mitosis; Monocytes; Muramidase; Thymidine; Tritium; Uridine

Topics: Acetates; Alkaline Phosphatase; Biopsy; Blood Cell Count; Blood Proteins; Bone Marrow; Chlorine; Chronic Disease; Clinical Enzyme Tests; Diagnosis, Differential; Esterases; Humans; Leukemia; Lymphatic Diseases; Lymphoma, Large B-Cell, Diffuse; Monocytes; Muramidase; Neutrophils; Primary Myelofibrosis; Reticulocytes

Topics: Adult; Blood Transfusion; Female; gamma-Globulins; Herpes Simplex; Humans; Immunotherapy; Lymphatic Diseases; Muramidase; Recurrence

Topics: Humans; Leukemia, Lymphoid; Leukemia, Myeloid; Lymphatic Diseases; Lymphocytes; Muramidase

Topics: Adult; Anti-Infective Agents, Local; Carcinoma; Hematologic Diseases; Humans; Lymphatic Diseases; Muramidase; Neoplasms

Topics: Disease; Gastrointestinal Diseases; Humans; Lymphatic Diseases; Muramidase; Palatine Tonsil; Pharyngeal Diseases