Biomedical subjects
Publications and source records attributed to T Kanoh.
The association between amyloid and systemic lupus erythematosus is rarely reported. Our patient, a 26-year-old woman with systemic lupus erythematosus, developed steroid-resistant exertional dyspnea and pulmonary fibrosis, without proteinuria or the nephrotic syndrome. Our studies showed that she had pulmonary amyloidosis.
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A patient with pyoderma gangrenosum (PG) and ulcerative colitis (UC) is described. He had melena and systemic skin lesions. The skin lesions consisted of small discrete ulcers on the back and head and large punched-out ulcers on the legs. He was successfully treated with prednisolone and salazosulfapyridine. He became asymptomatic after two weeks' treatment. Although the association of PG with UC is well documented among Caucasians, it is very rare among Japanese.
The present investigation was undertaken in order to elucidate the delayed reaction of Bence Jones proteins to sulfosalicylic acid. The author observed this interesting phenomenon in 2 of 32 untreated patients with multiple myeloma who excreted Bence Jones proteins. Their urine samples contained a large amount of Bence Jones proteins with alpha 2 mobility on electrophoresis, which is rarely encountered in urinary Bence Jones proteins. The delayed reaction may be linked, in part at least, to the chemical properties of Bence Jones proteins responsible for alpha 2 mobility.
A significant incidence of neutrophils with ring-shaped nuclei was observed in all of four patients with chronic neutrophilic leukemia. The present data may give further support for the view that chronic neutrophilic leukemia is not a reactive disorder but a rare form of myeloproliferative disorder. It seems possible that this phenomenon can be used as a marker for abnormal granulopoiesis in human diseases.
Abnormal accumulation of Tc-99m MDP in the lungs and stomach was observed in a patient with multiple myeloma and refractory hypercalcemia. There was no roentgenographic evidence of calcification. At postmortem examination, the presence of amyloid deposits was confirmed in both organs, while the corresponding metastatic calcification was demonstrated only in the gastric mucosa. It seems likely that, in our case, soft-tissue localization of bone-seeking agent was attributable to the presence of amyloid deposits rather than metastatic calcification. No cases showing the combined occurrence of amyloid deposition and metastatic calcification in soft-tissues have previously been demonstrated to concentrate bone-seeking agent.
The incidence of selective IgA deficiency (SIgAD) was determined in a healthy adult population of 222,597 Japanese volunteer blood donors. Of the blood donors screened, only 0.007% (1:14,840) were found to be IgA-deficient (less than 10 mg/dl) by means of the double diffusion method, while 0.005% (1:18,500) were less than 5 mg/dl, and 0.003% (1:31,800) were less than 1 mg/dl by means of the single radial immunodiffusion method. Statistical analysis of the results clearly showed that the incidence of SIgAD in Japanese blood donors is very much lower than that in blood donors of European ancestry. The Japanese population may occupy a unique position in the ethnical peculiarities. Anti-IgA antibodies were found in 3 (25.0%) of 12 IgA-deficient blood donors whose IgA levels were less than 5 mg/dl, a prevalence rate comparable to that in donors of European ancestry. Although it is difficult to develop a suitable file of IgA-deficient donors in Japan, the establishment of a Rare Donor Registry System on IgA deficiency is a matter of urgency.
Bone marrow smears from 401 patients with malignant plasma cell disorders (MPCD) were examined to evaluate the incidence of phagocytosis by plasma cells and to elucidate the pathogenesis of this phenomenon. Phagocytizing plasma cells were observed in 6 patients (1.6%), 1 of whom had plasma cell leukaemia, from a pool of 382 patients with multiple myeloma that included 7 with plasma cell leukaemia, but not in those with other types of MPCD. In 1 of the 6 cases, the plasma cells acquired phagocytizing ability in the terminal stage of the illness. The incidence of phagocytizing plasma cells in multiple myeloma was higher than might have been expected, judging from a small number of previous reports concerning phagocytosis by plasma cells. The incidence of phagocytizing plasma cells in plasma cell leukaemia (1:7) was not statistically greater than that in non-leukaemic multiple myeloma (5:375).
Bone marrow aspirates from 10 patients with multiple myeloma (6 IgG myeloma, 3 IgA myeloma, 1 Bence Jones myeloma) were examined for nucleolus-associated localization of J chains. On light microscopy using the PAP method, almost all or a significant number of myeloma cells exhibited intranuclear spots stained with anti-J chains in 3 (IgG myeloma) out of the 8 cases positive for cytoplasmic J chains. In contrast, the nucleus of myeloma cells examined was constantly negative for anti-heavy and -light chains. Immunoelectron microscopically, J chain was identified as dicrete round electron-dense precipitates, corresponding to the whole nucleolus, and as sparsely distributed, small electron-dense deposits in the nucleus. In addition, some connections were found between nuclear and nucleolar electron-dense precipitates. Several possible explanations have been proposed to account for this localization of J chains.
Practically all myeloma cells had prominent vacuoles, around and within which the end product of acid phosphatase (AP) reaction was demonstrated. These vacuoles were considered to be autophagic. In remission, only a few myeloma cells were vacuolated. Serial determinations showed transition from an initial high AP score (3.86) to a low score (1.32) in remission. Moreover, there was a trend towards a lower average percentage of positive cells in remission (72%) as compared to the preinduction phase (100%). Intracellular Bence Jones proteins of the present case may be toxic and capable of causing vacuolation with activation of lysosomal enzymes.
The occurrence of Bowen's disease on unexposed area of the body in a 68-year-old man with multiple myeloma is reported. Not only non-reticular but also reticular malignancy should be taken into consideration as internal malignancy associated with Bowen's disease. The previous reports of Bowen's disease associated with reticular malignancy are also reviewed.
In a 73-year-old man with acute myelomonocytic leukemia (M4 on FAB classification) Auer rods were demonstrated in mature granulocytes and monocytes in the blood as well as the bone marrow. Cytochemically, a significant number of leukemic blasts were positive for both alpha-naphthyl-acetate esterase and naphthol-AS-D-chloroacetate esterase. Auer rods in mature granulocytes were associated with immature cytoplasm and not infrequently with pseudo-Pelger anomaly. The significance of these observations is discussed.
A case of common variable immunodeficiency in a pregnant woman is presented. Lobar pneumonia developed in the sixth month of the pregnancy and her serum immunoglobulin levels were found to be extremely low. She was treated successfully with immune human serum globulin and antibiotics. She delivered a full-term baby without any troubles. Various immunologic studies were done in the peripheral blood of the patient and the neonate. Consequently, functional abnormalities of helper T cells were considered to be responsible for the hypogammaglobulinemia.
The test sera from 50 patients treated with intravenous gammaglobulins (IVG) were examined for LAV/HTLV-III antibody by ELISA tests in order to evaluate the risk of transmission of LAV/HTLV-III virus via administration of IVG. The three ELISA-positive sera were negative for the antibody by both indirect immunofluorescence assay (IFA) and Western blot assay (WBA). Thus, all of the test sera were negative for LAV/HTLV-III antibody. Two of the ELISA-positive/IFA-negative/WBA-negative samples had HLA-DR4 antibodies causing positive ELISA results.
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