Forty-nine cases of histiocytosis X (HX) collected over a period of 20 years in National children's Hospital were retrospectively analyzed and discussed about clinicopathological findings, cytopathological feature and pathogenesis.
Nineteen cases were categorized in Letterer-Siwe (LS) 27 cases in multifocal eosinophilic granuloma (MEG) and 3 cases in unifocal eosinophilic granuloma (UEG). Twelve of 19 LS died whereas one case of MEG died. The prognosis for LS was neither dependent on age nor presence or absence of bone lesion and/or diabetes insipidus but on the extent of the vital organ dysfunction such as lung, bone marrow and intestine. Ominous sings which appeared later were respiratory distress, pancytopenia and hypoproteinemia.
Immunohistochemical and enzymohistochemical studies revealed that polynuclear giand cells in granulomatous lesions had different stainability from mononuclear HX cells. Polynuclear cells were week positive for anti S100, Leu6, HLA-DR, while positive for Leu 3a as well as HX cells, but strong positive for various lysosomal enzymes and PAS stain unlike HX cells. Giant cells are likely derived from HX cells but seem to have a different role from HX cells.
The involevement of the thymus were observed in almost all patients at the autopsy including a congenital case. The thymic involvement is the most conspicuous feature which distiguishes HX from the other histiocytosis and may explain the immunodisturbunce of this disease.

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Clinicopathologic findings of 3 cases of malignant histiocytosis (MH), 8 cases of histiocytic medullary reticulosis (HMR), a case of familial hemophagocytic reticulosis (FHR), 8 cases of lymphoproliferative disorders terminating in HMR-like features (symptomatic HMR) and allied disorders are examined. Although it is problem whether MH is identical with HMR, there are some cases overlapping with each other. Pathologically, HMR has a broad spectrum condition containing etiology unknown, reactive, cataplastic, and neoplastic histiocytosis. Symptomatic HMR, especially the case of mosquitoe allergy is a very important on considering the pathogenesis of HMR. Because, as a result of prolonged mosquitoe allergy, immunodifficiency and abnormal T cell proliferation presented and eventually HMR-like feature progressed. It would consider that FHR is a special case of lymphoproliferative disorders rather than true histiocytosis. So called leukemic reticulosis or malignant reticulosis in Japan were heterogenous disorder including not only histiocytosis, but also T-cell, B-cell malignancy and monocytic leukemia.

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The author has studied nuclear DNA amounts, chromosome numbers and growth fraction of non-Hodgkin's lymphoma cells by cytofluorometry, chromosome analysis and immunostaining with the monoclonal antibody Ki-67 in order to clarify the relation between histologic subtypes of non-Hodgkin's lymphoma based on LSG classification and prognosis of the cases.
A great difference was observed in the mode of nuclear DNA amounts according to histologic subtypes of diffuse lymphoma (e.g. medium-sized cell type, 2.45±0.24 C, mixed cell type 3.3±0.72 C, large cell type, 4.05±0.87 C), but there was little difference among those of follicular lymphoma. The frequency of DNA aneuploidy was higher in large cell type and mixed cell type than that of medium-sized cell type. Nuclear DNA amounts paralleled chromosome numbers. The 50% survival time was shorter in the group with high nuclear DNA amounts (≥3.0 C) than that of low nuclear DNA amounts (<3.0 C).
The percentage of occurrence of Ki-67 positive tumor cells of diffuse B cell lymphomas was higher than that of follicular lymphomas and it increased in order; medium-sized, mixed and large cell type in both follicular and diffuse lymphomas. The 50% survival time was shorter in high percentage group of Ki-67 positive cells (≥36%) than low percentage group (<36%). These results indicate that nuclear DNA amounts and the percentage of occurrence of Ki-67 positive cells exactly correlate to prognosis of the cases according to each histologic sub-types of non-Hodgkin's lymphoma except for T cell lymphomas.
The percentage of occurrence of Ki-67 positive cells of T cell lymphomas was lower than that of B cell lymphomas, although prognosis of T cell lymphomas is usually poorer than that of B cell lymphomas. Adequate answer to this problem is not applied in the present study.

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The formation and distribution of metastatic leprotic lesions in the spleen from cutaneous granulomas of leprosy were experimentally investigated with respect to the intrasplenic microcirculation, cell movement and regional function.
Seventy-seven Wistar strain male rats were intradermally inoculated with 1×10⁷ of Mycobacterium lepraemurium at the sternal region. Granulomas developed in the subcutis from 8 weeks after inoculation in 53 rats. Splenic lesions having bacilli were observed in 21 of these rats. No splenic lesions were observed in the rats in which no subcutaneous granuloma developed at the site of the inoculation. The splenic leprotic lesions were examined in the hematoxylin-eosin and Ziehl-Neelsen stained sections, and classified into the three types of 1) extracellular free bacillus, 2) single macrophage which phagocytosed bacilli and formed no granuloma, and 3) granuloma which was a compact accumulation of macrophages phagocytosing bacilli.
The combined total of the three types of lesions was 543; 327 granulomas, 127 single macrophages and 89 bacilli. Analysis of their localization revealed 58% of the lesions in the periarteriolar lymphocytic sheath (PALS), 8% in the marginal zone (MZ), and 34% in the red pulp (RP). Granulomas were found in 66%, 8% and 26% of these lesions in the PALS, MZ and RP, respectively; macrophages in 50%, 8% and 43% of these in the PALS, MZ, and RP; and extracellular free bacilli in 39%, 10% and 51% of these in the PALS, MZ and RP. No leprotic lesions were present in the follicles.
The results suggest that the free macrophages phagocytosing the bacilli and the extracellular free bacilli move to the PALS from the MZ and RP. Thus, Mycobacterium lepraemurium, as a thymus-dependent antigen, provokes granulomatous inflammation mainly in the PALS, although some granulomas develop. in the MZ and RP.

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Clinicopathological and immunohistochemical features of 50 thymomas were studied with speial reference to their correlation to histological typing. Like as previous reports, most of thymomas with myasthenia gravis were of polygonal cell type, those with pure red cell aplasia or hypogammaglobulinemia were of mixed or spindle cell type, and most of invasive cases were of polygonal cell type with scant to moderate lymphocytes. Leu7 positivity for epithelial component of thymomas was shown in 77% of the cases and the cortical origin of thymomas was suggested because of localized expression of Leu7 on subcapsular epithelium of normal thymus. Each of 9 thymomas inwhich immunophenotypical analysis of the associated lymphocytes was performed contained immature lymphocytes with cortical phenotype, i.e., differentiated toward cortical epithelium. 4 of them including polygonal cell type with scant to moderate lymphocytes and mixed cell type with moderate lymphocytes contained mature lymphocytes with peripheral phenotypes as well as immature lymphocytes. S-100⁺ interdigitating cells were frequent in thymomas of similar histological types with those containing mature lymphocytes. Foci of so-called medullary differentiation were consisted of keratin⁺Leu7^- epithelium and mature lymphocytes with peripheral phenotype and were considered to be remnants of nonneoplastic thymi medulla.

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Immunological characteristics of eight cases of lymphoblastic lymphoma (LBL) were studied. When LBLs were divided into T-cell and B-cell type according to marker expression of neoplastic cells, six of eight cases displayed T-cell markers and other cases expressed B-cell markers. Based on nuclear shape, the cases were morphologically divided into convoluted (two cases) and non-convoluted type (six cases).
Expression of B-cell markers in one of two cases with convoluted nucleus suggested that convoluted nucleus was not limited to T-cell type. In T-LBL, some cases expressed CALLA (J5) or HLA-DR (Ia-like antigen). In five of six cases of T-LBL, Leu-8 positive neoplastic cells were observed. In non-neoplastic thymus, Leu-8 positive cells were found in medulla and subcapsular region. Leu-8 antigen may give a clue to clarify the origin of T-LBL.

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Three anti-macrophage monoclonal antibodies designated TRPM-1, TRPM-2 and TRPM-3 were produced, using thioglycollate-elicited rat peritoneal macrophages as imunogen. By the immunoperoxidase method, TRPM-1 reacted with most macrophages in the thymus, spleen and lymph nodes, and alveolar macrophages, Kupffer cells of the liver and blood monocytes. Moreover, TRPM-1 recognized Langerhans cells in the epidermis and IDCs in the thymus-dependent area. This antibody is thus thought to be an anti-pan-macrophage antibody. TRPM-2 exhibited a similar reaction pattern as TRPM-1 did; however, it recognized fewer macrophages than TRPM-1 did, and failed to react with Langerhans cells in the epidermis. On the other hand, TRPM-3 selectively reacted with splenic marginal zone macrophages and marginal metallophils, lymph node sinus macrophages, and spindle-shaped macrophages in the omentum. Tingible body macrophages in the lymphatic follicles and interdigitating cells in the thymus-dependent areas were not stained with TRPM-3. On immunoelectron microscopy, reaction products for TRPM-1 and TRPM-2 were observed in the cytoplasm of the macrophages, whereas reaction to TRPM-3 was clearly demonstrated on the plasma membrane of splenic marginal zone macrophages and marginal metallophils, as well as lymphatic sinus macrophages. As TRPM-1, TRPM-2, TRPM-3 recognize different macrophage populations, they are considered to be very useful tools to analyze the heterogeneity of macrophages.

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Ultrastrutural localization of lysozyme in human blood cells which were obtained from 10 nonmal volunteers was investigated by electron microscopy applying an immunogold staining method.
The presence of lysozyme was demonstrated as the deposition of gold particles by this staining. Lysozyme was detected in neutrophils, monocytes, eosinophils, reticlum cells and mast cells. On the other hand, no lysozyme was detected in basophils, megakaryocytes, platelets, lymphocytes, plasma cells and erythroid cells.
Generally, lysozyme was preferentially contained in the granules of lysozyme-positive blood cells. In contrast, no lysozyme was detected in the nucleus, perinuclear space, rough endoplasmic reticulum and mitochondria. In neutrophils, both primary and secondary granules contained abundant lysozyme. In monocytes, the granules were remarkably positive for lysozyme. In eosinophils, the granules were positive for lysozyme, but crystalloids negative. In some reticulum cells, phagolysosomes contained small amount of lysozyme. In some mast cells, abundant lysozyme was present in the granules.
Electron microscopic immunogold staining is a useful method to know the exact localization of lysozyme in blood cells.

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