Spontaneous remission in adult-onset cyclic neutropenia.
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Biomedical subjects
Publications and source records attributed to K Someya.
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A 40-year-old man was admitted after 8 months of speech disturbance and locomotive ataxia. He had no seizures, lightning pains, paresthesia, visual loss, bladder disturbance or rectal incontinence. He had never been neurologically or psychiatrically ill and had no history of syphilis. When the patient was admitted, his general physical examination including blood pressure and dermatologic examination was normal. His consciousness was alert. He was found to have a deterioration of mental status such as inability to concentrate, failing memory, amnesia and circumstantiality. His pupils were anisocoric and Achilles jerks were absent. No rigidity of the neck muscles, paralysis and sensory disturbance were recognized. Romberg's sign was absent. The right pupil was 7.0 mm and the left was 6.0 mm in room illumination. The pupils were nonreactive to bright light and both did not constricted to near stimuli. 0.125% pilocarpine eyedrops produced bilateral pupillary constriction. The results indicated bilateral tonic pupils. Laboratory data revealed white cell count of 12,600/mm3 and normal erythrocyte sedimentation rate of 8 mm/hr. Cerebrospinal fluid (CSF) examination revealed the following: opening pressure, 140 mm of water; cell counts, 76/mm2 (mononuclear cells); total protein, 116 mg/dl; glucose, 57 mg/dl. A serum venereal disease research laboratories (VDRL) test was positive in a 1:32 titer confirmed by positive treponema pallidum hemagglutination (TPHA) test in a 1:40,960 titer and positive fluorescent treponemal antibody-absorption (FTA-ABS) test. Serum TPHA-IgM was positive in a 1:320 titer but TPHA-IgG was negative. CSF examination revealed positive TPHA test (titer of 1:2,560) and positive FTA-ABS test.(ABSTRACT TRUNCATED AT 250 WORDS)
We report a case retroperitoneal mature teratoma showing an abnormally high CA19-9 (carbohydrate antigen 19-9) serum level in a 30-year-old woman. She was hospitalized for an episode of left upper abdominal pain. High CA19-9 tissue level and immunohistochemical findings were found in removed tissue. This case is the third report of a CA19-9-producing teratoma and literature was reviewed and discussed.
Antiplatelet and antithrombotic effects of KC-764 (2-methyl-3-(1,4,5,6-tetrahydronicotinoyl)pyrazolo[1,5-a]pyridine, CAS 94457-09-7) were studied. KC-764 inhibited arachidonic acid (AA)- and collagen-induced platelet aggregation with IC50s of 1.0 x 10(-8)-2.8 x 10(-7) mol/l for humans, rabbits, guinea pigs and dogs, and IC50s of 3.9 x 10(-6)-3.7 x 10(-5) mol/l for mice and rats in vitro. KC-764 inhibited AA- and collagen-induced aggregation with ID50s of 0.04-0.09 mg/kg p.o. in rabbits and dogs, and ID50 of 13.0 mg/kg p.o. in rats. These antiaggregatory activities of KC-764 were stronger than those of acetyl-salicylic acid (ASA), indometacin, cilostazol and ticlopidine. KC-764 inhibited the production of thromboxane B2 (TXB2) in rabbit platelet microsomes, washed platelets and reconstituted platelet rich plasma (RPRP) with IC50s of 2.9 x 10(-6) mol/l, 2.8 x 10(-7) mol/l and 4.3 x 10(-8) mol/l, respectively. The in vitro inhibitory activity of KC-764 on AA-induced platelet aggregation was more potent when RPRP was used rather than washed platelet suspension containing 30% rabbit plasma. ASA did not show such an augmentation. KC-764 prevented collagen- and AA-induced thrombosis at more than 1 mg/kg p.o. and more than 0.1 mg/kg i.v. in mice and rabbits. KC-764 showed the wider margin of dose between antiplatelet action and prolongation of bleeding time in rabbits than ASA and indometacin. These results indicated that KC-764 was a potent antithrombotic drug to prevent TXB2 production and less possible to induce untoward actions as compared with ASA or indometacin.
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The effect of 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) and its analogue 22-oxacalcitriol (OCT), which was reported to have very weak bone resorbing activity, on the production of tumor necrosis factor (TNF)-alpha was investigated. Mononuclear cells (MNC; 10(6)/ml) were incubated in 5% FCS/RPMI-1640 medium containing 1 microgram/ml lipopolysaccharide (LPS) in the presence or absence of 10(-8) M 1,25(OH)2D3 or 10(-8) M OCT for up to 96 h. Both 1,25(OH)2D3 and OCT suppressed TNF-alpha release by LPS-stimulated mononuclear cells, from the early to late stage of the incubation period, while neither 1,25(OH)2D3 nor OCT shifted the peak time point of TNF-alpha release clearly. MNC (10(6)/ml) were incubated with 1 microgram of LPS in the presence of various concentrations of 1,25(OH)2D3 or 10(-8) M OCT for 48 h. 1,25(OH)2D3 reduced TNF-alpha levels of LPS-stimulated MNC culture supernatant at 48 h in a dose-dependent manner. The half-maximal dose (ED50) for this suppressive effect was 3.7 x 10(-9) M. OCT decreased TNF-alpha levels of culture supernatant at 48 h with a half-maximal dose of 7.8 x 10(-11) M, which indicates that it is approximately 50 times more potent than that of 1,25(OH)2D3. These results indicate that OCT may be applicable as an immunosuppressive agent with limited calcium metabolic activity.
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The influence of sustained absorption on the oral availability of propranolol (P) and the metabolic disposition of P were investigated by obtaining the partial metabolic clearances (CLm) following long-acting P (LA) dosing in comparison with the conventional propranolol tablet (CP). Ten healthy volunteers were given a single oral dose of an LA capsule (60 mg) and CP (20 mg x 3) using a crossover design. Blood and urine samples were collected over 24- and 48-h postdose periods, respectively. Concentrations of P, propranolol glucuronide (PG), 4-hydroxypropranolol (4P), 4-hydroxypropranolol glucuronide (4PG), 4-hydroxypropranolol sulfate (4PS), and naphthoxylactic acid (NLA) were determined by HPLC with fluorescence and UV detection. Significant differences were observed between LA and CP in the area under the plasma concentration-time curves (AUCs) for P, PG, and NLA and in the amounts excreted into urine (Ae) for all measured metabolites (i.e., PG, 4P, 4PG, 4PS, and NLA). The parallel decrease of the AUC for P and the excreted amounts of all measured metabolites following LA dosing resulted in partial metabolic clearances (CLm) and renal clearances (CL) for P and its metabolites that were similar to those observed for CP. Therefore, the hepatic metabolism of P would not be affected by the slower absorption at a single oral dose of 60 mg. These results indicate that the poor absorption of P from the gastrointestinal tract might be one of the factors causing the low bioavailability of P observed after administration of the sustained-release formulation.
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A housewife, 40 years of age, was admitted with dysesthesia of the extremities, muscle weakness, and attacks of adynamia and thirst. She had been taking a laxative for more than 20 years. On physical examination, blood pressure was 94/56 mmHg. Laboratory tests revealed thrombocytosis, low serum K and marked increases in both plasma renin activity and serum aldosterone. Serum TBG was increased. Serum gastrin was also markedly increased and could not be enhanced by exogenous secretin. Both angiotensin 11 loading test and noradrenalin loading test failed to increase blood pressure. Ammonium chloride loading to examine the disturbance of urinary acidification was abnormal in the short term test and borderline in the long term test. Following a diagnosis of pseudo-Bartter's syndrome induced by long term intake of laxative and repeated diarrhea, the administration of laxative was interrupted and potassium, indomethacin and spironolactone were administered. However, serum K remained low. Hypergastrinemia, thrombocytosis and a high serum TBG level also persisted, the causes of which remain unknown. This is the first reported case of pseudo-Bartter's syndrome associated with hypergastrinemia, thrombocytosis and increased serum TBG.
In this study, we have investigated the in vitro effects of the immunomodulators lobenzarit and traxanox and a newly synthesized immunosuppressant, mizoribine, as well as cyclosporin A, on bone resorption using neonatal mouse calvariae labelled with 45Ca. As stimulators of bone resorption, bovine parathyroid hormone (PTH), lipopolysaccharide (LPS), interleukin 1 beta (IL-1 beta) and tumor necrosis factor-alpha (TNF-alpha) were used. Lobenzarit, traxanox, mizoribine and cyclosporin A inhibited or tended to inhibit bone resorption stimulated by PTH, LPS, IL-1 beta or TNF-alpha in a dose-dependent manner. Basal bone resorption was inhibited by immunosuppressant cyclosporin A or mizoribine, while immunomodulators lobenzarit and traxanox failed to inhibit basal bone resorption. Removal of lobenzarit from the culture medium resulted in the recovery of bone resorptive activity. These results suggest that the inhibitory effect of immunomodulators on bone resorption is reversible and nonselective. Also, it raises the possibility that immunomodulators and immunosuppressants may affect bone resorption by different mechanisms.
We reported a case of Becker type congenital myotonia with myalgia. A 28 year-old woman admitted to our hospital because of right anterior chest pain and back pain. She was diagnosed as myotonic dystrophy by other university hospital when she was 16 years old. Physical examination revealed only myotonia and could not find muscle atrophy or cataracta which were usually found in myotonic dystrophy. The quadriceps muscle biopsy revealed complete absence of type IIB muscle fiber. We diagnosed her Becker type congenital myotonia by the clinical course and family history and the histopathological findings of quadriceps muscle. Myalgia which she complained seemed to have some connection with this disease. But we could not prove the pathogenesis of this myalgia.
Megakaryocytes in the peripheral blood in which leukoerythroblastosis was recognized were studied by electron microscopy on the vertically cut section of the buffy coat of the blood, and percentages of them in 10,000 nucleated cells distributed from the top of the bottom of the buffy coat were counted. In 15 of 31 patients, percentages of peripheral blood megakaryocytes ranging from 0.01% to 0.64% were seen. There was difference of the result among diseases shown peripheral blood megakaryocytes. Namely, in patients with myelofibrosis and CML, in whom extramedullary hematopoiesis was predominant, many cases ascertained peripheral blood megakaryocytes were demonstrated. Because of this result, the extramedullary hematopoiesis appears to play an important role to the presentation of megakaryocytes in the peripheral blood. On the other hand, patients indicating both megakaryocytes and abnormal sideroblasts in the peripheral blood had sideroblastic anemia marrow. This result seems to show that a part of megakaryocytes are directly flowed out from the marrow into the blood simultaneously accompanied with abnormal sideroblasts.
A 44-year-old man, who was seen for further examination of macroscopic hematuria, was found to have black eruptions on the penis and the scrotum. These eruptions appeared benign clinically, but on histological examination they showed the features of Bowen's disease. These eruptions were diagnosed as Bowenoid papulosis and were successfully treated with 5-FU cream. Twenty five cases of Bowenoid papulosis observed in men have been reported in Japan. Although Bowenoid papulosis is characterized by the histopathologic conditions of Bowen's disease, the lesions of Bowenoid papulosis differ clinically from those of Bowen's disease in several respects: onset at earlier average age, multiplicity of lesion, lack of associated symptoms, smaller size of the lesion, clinical morphologic features and possible spontaneous regression.
The clinical usefulness of a I-131 anti CEA F(ab')2 MoAB and CA 19-9 F(ab')2 MoAB cocktail(IMACIS-1) for the localization of a cancer has been studied in 35 patients (Lung Ca. 10, Pancreas Ca. 7, Gastric Ca. 6, Colon Ca. 5, Hepatoma 2, Cholangio Ca. 2, Esophageal Ca. 1, Uterus Ca. 1, and, Retroperitoneal tumor 1). Of these 28 patients (80%) had an abnormal accumulation of radioactivity in the tumor sites (Lung Ca. 5, Pancreas Ca. 7, Gastric Ca. 6, Colon Ca. 5, Hepatoma 2, Esophageal Ca. 1, Uterus Ca. 1, and, Retroperitoneal tumor 1). Eighteen cases (56.3%) in 32 cases in which the primary site was known showed an abnormal radioactive accumulation and, similarly, 20 metastatic sites (71.4%) in 16 cases with 28 metastatic sites also showed an abnormal accumulation of radioimmunoscintigraphy showed no correlation with the serum CEA or CA 19-9 concentration. Further, no side effect was observed.
A 59-year-old-male was admitted in September 1984 with fever and right hypochondrial pain. On computed tomography, a bulky retroperitoneal tumor involving the intestine was detected. Exploratory laparatomy yielded a diagnosis of non-Hodgkin's lymphoma. Although intensive and aggressive treatment combined chemotherapy and radiotherapy was administered, no conclusion could be drawn about its efficiency, and no remission was indicated by followup studies. He refused further treatment because of bone marrow depression. However, 9 months after the last treatment disappearance of the tumor was recognized on computed tomography examination. This phenomenon was confirmed by the second laparatomy performed in order to treat skin-colon fistula caused from diverticulitis of the colon. It was recognized no evidence of malignant lymphoma in the resected colon specimen. He remains in excellent health 26 months after this events. The clinical course of this case was probably compatible with the concept of spontaneous remission of malignant lymphoma.