alpha-chymotrypsin and Lymphatic-Diseases

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

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

Total hemolytic activity of serum (CH50), complement components C3 and C4, alpha 1antitrypsin (alpha 1AT), alpha 1antichymotrypsin (alpha 1X), antithrombin III (AT III), alpha 2 macroglobulin (alpha 2M), and inter-alpha-inhibitor (I-alpha-I) were measured in 23 Japanese and 19 European children with the Mucocutaneous Lymph node Syndrome (MCLS) during the acute phase of disease. Second sera, obtained after day 20 were available from 18 Japanese and 10 European children. In 28 out of 31 children with mild disease, as assessed by the coronary risk score of Asai and Kusakawa, complement was normal or elevated during the acute phase. In 10 out of 11 children with high risk scores, CH50 was below the normal range. One child in this group had ECG changes during the acute phase, one patient died and two others developed coronary aneurysms. C1I was elevated in all 42 cases, alpha 1AT in 40, and alpha 1X in 38 patients. alpha 1AT was depressed in two children, one of whom developed an aneurysm. One of the four children with depression of alpha 1X died of myocardial infarction. Decreased concentrations of AT III, alpha 2M and I-alpha-I were frequent and tended to mark the more severe courses of the disease. A third group of 20 children was evaluated 5 weeks to 6 months after the acute illness. Mean concentrations of all five protease inhibitors were completely normalized in this group. The results of this study indicate that consumption of complement and of protease inhibitors occurs in many cases of MCLS during the acute phase. Determination of CH50 appears to be useful to identify high risk patients early in the course of their illness. Transient deficiency of substances for control of inflammation may in part be responsible for the severe vascular lesions seen in some patients.

Topics: alpha 1-Antichymotrypsin; alpha 1-Antitrypsin; Alpha-Globulins; alpha-Macroglobulins; Antithrombin III; Child; Child, Preschool; Chymotrypsin; Complement C3; Complement C4; Complement System Proteins; Female; Humans; Infant; Lymphatic Diseases; Male; Mucocutaneous Lymph Node Syndrome; Prognosis; Protease Inhibitors

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

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