Autologous peripheral stem-cell transplantation after intensive chemotherapy in a case of CD30 (Ki-1)-positive anaplastic large-cell lymphoma.

Simultaneous treatment with peripheral blood stem-cell (PBSC) transplantation and intensive chemotherapy was evaluated in a case of non-Hodgkin's lymphoma (NHL) with poor prognosis. A 59-year-old male diagnosed with a high-grade, anaplastic large-cell (Ki-1) NHL, involving fractures in the left hip, underwent computed tomography and gallium scintigram surveillance. The patient received chemotherapy with epirubicin hydrochloride, cyclophosphamide, vincristine and prednisolone, and the fractured hip bone was repaired following the first course of chemotherapy. After the second and third courses of chemotherapy, PBSCs were harvested and cryopreserved. The patient then received a further course of chemotherapy and PBSC transplantation was conducted using infused cells consisting of 9.63 x 10(6)/kg CD34 cells and 2.24 x 10(5)/kg granulocyte macrophage colony-forming units. Recovery of platelet and white blood cell counts occurred 10 and 8 days, respectively, after PBSC infusion and the patient remains well.

A therapeutic strategy for isolated plasmacytoma of bone.

Two cases of solitary plasmacytoma of bone (SPB) were diagnosed. The first case was a 41-year-old woman, complaining of fatigue from her lumbar region to her legs. The second case was a 56-year-old man complaining of poor gait and severe lumbago with numbness in the toes of both feet. Magnetic resonance imaging showed the osteolytic lesion in the 12th thoracic bone in the first patient and around the 2nd, 3rd and 4th lumbar bones in the second patient. In both patients serum analysis revealed the monoclonal component of immunoglobulin G (IgG) protein with kappa-light chain, and considering this and other findings SPB was diagnosed. Both patients were first treated with irradiation around the involved bone and then with a course of chemotherapy. In the first patient the tumour region of the bone was surgically removed and replaced with a ceramic spacer. The symptoms of both patients were ameliorated, and the patients remained in good condition for around 3 years without conversion to multiple myeloma. In view of the overall effectiveness of treatments for SPB, our therapeutic strategy deserves careful evaluation.

Treatment of acute myeloblastic leukaemia in a patient with Bombay blood type: a case report.

A 62-year-old female was admitted to our hospital with suspected acute leukaemia and after investigation we diagnosed acute myeloblastic leukaemia (AML-M1). The patient's blood type was found to be the very rare Bombay type and surveillance of her relatives showed the same blood type in her male cousin on her mother's side. Alongside chemotherapy the patient received 4000 ml of frozen Bombay-type red cells, 1400 ml of concentrated red cells in manitol adenine phosphate solutions and 360 units of type O concentrated platelets without marked effects. The anti-H antibody was initially at 128 dilution but for unknown reasons increased to 2048 dilution after remission of AML-M1. About 3 months after hospitalization the patient died of Cryptococcus neoformans pneumonia despite strict precautions against infection. Although AML-M1 is a common adult leukaemia and is chemosensitive to anti-leukaemic drugs, neither AML-M1 in a patient with Bombay-type red cells nor its treatment with chemotherapy and transfusion with type Oh frozen red cells have previously been reported.

The CAG regimen (low-dose cytarabine, aclarubicin hydrochloride and granulocyte colony-stimulating factor) for the treatment of elderly acute myelomonocytic leukaemia: a case study.

Elderly patients with acute myelomonocytic leukaemia (AMMoL) frequently have a poor quality of life after induction of remission using high-intensity treatment; we seek a more appropriate regimen for such patients. An 86-year-old man was hospitalized with a diagnosis of AMMoL (FAB classification M4), of abnormal karyotype, and complications of diabetes mellitus and complete right bundle branch block. He was treated with CAG therapy (cytarabine 10 mg/m2 subcutaneously every 12 h for 14 consecutive days; aclarubicin hydrochloride 10 mg/m2 per day, bolus intravenously for 4 consecutive days; granulocyte colony-stimulating factor 100 microg/day, subcutaneous injection for 14 consecutive days) every 3 months. White blood cell counts were at their lowest (around 600 - 800/microl) 12 days after the end of therapy, but returned to about 2000 - 2300/microl 30 days after stopping therapy. No symptoms of drug-related toxicity, except slight nausea, were found. Complete remission with a good quality of life was induced and lasted over 2 years suggesting that CAG therapy might prove effective in elderly patients with AMMoL.

Primary small cell carcinoma of the larynx.

Small cell carcinoma is a rare tumor of the larynx. We present such a case in a 78-year-old female. The histopathological diagnosis at the time of laryngomicroscopic biopsy was squamous cell carcinoma, upon which basis we initially chose surgical treatment. The surgical specimen, however, revealed small cell carcinoma. Despite the administration of radiotherapy and chemotherapy, the patient died 9 months after initial presentation. We believe that this case illustrates the need for a sufficiently large biopsy specimen in order to arrive at the correct histopathological diagnosis when small cell carcinoma of the larynx is present, and that immunohistochemistry and electron microscopy should be performed to aid the diagnosis.

Recurrent dislocation of the patella in Kabuki make-up syndrome.

Three patients with Kabuki make-up syndrome (KMS) who had recurrent dislocation of the patella are reported. In all, dislocation of the patella developed in adolescence, and they had generalized ligamentous laxity and dysplasia of the patellofemoral joints. In two of the three patients, surgical intervention was required; the operation was successful in one, but yielded poor results in the other. In the third patient, cardiac complications precluded operation. Orthopedists should be aware of this possible complication when treating patients with KMS.

Differential immunohistochemical staining of cancerous cells with anti-single-stranded DNA antiserum in ordinary pathological paraffin section after DNA-denaturation by acid hydrolysis.

Based on the finding that the nuclear DNA of cancerous cells is much more unstable than that of non-cancerous cells, yielding a larger amount of single-stranded DNA by acid hydrolysis, we developed a new method of cancer cell detection in ordinary pathological sections by immunohistochemical staining with anti-single-stranded DNA antiserum after acid hydrolysis. Methylated bovine serum albumin was conjugated with heat-denatured calf thymus DNA and used as the antigen of single-stranded DNA, and white male rabbits were immunized with the antigen to obtain the polyclonal antiserum. Ordinary paraffin-embedded sections were prepared from the formalin-fixed biopsy specimens taken from 482 malignant tumors and 73 benign tumors of human epithelial and non-epithelial origins. Additional 82 biopsy specimens of borderline malignancy were also examined. The sections were immunohistochemically stained with the antiserum after RNase digestion and DNA denaturation by hydrolysis with 2 N HCl at 30 degrees C. The acid hydrolysis for 20-30 min was optimum for the specimens fixed with 10% buffered formalin at room temperature for 16-24 hrs, and all cancerous cells were specifically stained positive, in sharp contrast to the negative stainability of all non-cancerous cells including inflammatory cells. This new method gives us decisive help in making diagnosis of malignancy in daily pathological examination. The possibility of malignancy of borderline lesion was discussed.

Laryngomicrosurgery with carbon dioxide laser for laryngeal papillomatosis: application of a two-stage operation.

Among the many treatments reported, CO2 laser surgery has recently been applied in the field of laryngomicrosurgery to treat laryngeal papilloma. It is exceedingly difficult to cure this disease, however, because of its rapid recurrence following surgery. Incomplete removal of papilloma is believed to be caused by the difficulty of precisely identifying residual papillomas during surgery. In an effort to resolve this problem, we have developed a new surgical method which we call the "two-stage operation," in which a series of two operations are performed about 10 days apart. By the time of the second operation, the bleeding, coagulated blood, and carbonized tissue from the first operation have resolved, and the laryngeal image is much clearer. If additional lesions are found, they are removed at the second operation. We applied this method to several patients with recurrent laryngeal papillomatosis and obtained good results. This new method is clearly useful in the treatment of laryngeal papillomatosis, and it promises to decrease the recurrence rate. In this paper we present the case of a 26-year-old male with laryngeal papilloma who underwent surgery 1 month previously at another hospital. Laryngeal endoscopy revealed multiple papillomas especially of the glottis. The first operation with the CO2 laser was performed on April 16, 1990. Visible lesions appeared to have been completely removed. The second operation was carried out 10 days after the first operation, and the small residual lesions were removed with the CO2 laser. The patient has been disease-free for 18 months.

Beneficial effects of a calcium antagonist, nifedipine, on death and cardiovascular calcinosis induced by dietary magnesium deficiency in adult mice.

The effects of nifedipine on the survival rate and the cardiovascular calcinosis were investigated in dietary magnesium (Mg)-deficient mice. Male mice aged 8 to 10 weeks were fed with a diet which was either normal (0.07%) or deficient (0.007%) in Mg for 30 d. Nifedipine was added to the Mg-deficient diet for 30 d in a concentration of 1000 ppm. About 65% of Mg-deficient mice died within 30 d. In contrast, all the nifedipine-treated mice survived for 30 d. Mg-deficient mice showed hypomagnesaemia and hypocalcaemia which were not influenced by nifedipine treatment. Aortic and cardiac calcium contents increased in Mg-deficient mice, and these increments were normalized by nifedipine treatment. These results suggest that the life-saving effect of nifedipine in Mg-deficient mice might relate, at least in part, to the prevention of a deleterious increase in Ca in cardiovascular tissues.

A short-term evaluation of the effectiveness of stabilization-type occlusal splint therapy for specific symptoms of temporomandibular joint dysfunction syndrome.

To clarify the short-term effectiveness of stabilization-type occlusal splint therapy, a specific symptom approach was used toward 30 temporomandibular joint dysfunction patients with more than two major symptoms (temporomandibular joint and/or masticatory muscle pain, temporomandibular joint sounds, and limitation of mandibular movement). Eighty-seven percent of the patients with pain responded with this therapy and more than 50% had complete relief of pain 4 weeks after insertion of the splint. Temporomandibular joint sounds and limitation of mandibular movement responded more slowly than pain. These results suggest that the stabilization-type occlusal splint should be selected as a first choice among several therapies and that temporomandibular joint pain is particularly susceptible to this therapy.

Enhancement of S1 nuclease-susceptibility of negatively superhelical DNA by netropsin.

It was evidenced that the antibiotic netropsin enhances the single-strand-specific nuclease S1-susceptibility of negatively superhelical DNA. In contrast, an intercalating drug inhibited S1 action on the superhelical DNA. Negatively superhelical DNA is known to possess several (or a number of) unbasepaired sites sensitive to S1 cleavage. S1 cleaves generally the DNA once at these sites to result in production of the full-length linear form. However netropsin-bound DNA had a tendency to be cleaved by S1 simultaneously at plural sites producing several species of linear DNAs smaller than full-length size.

Soft tissue collagen in congenital dislocation of the hip. Biochemical studies of the ligamentum teres of the femur and the hip joint capsule.

Collagens of the ligamentum teres of the femur and the hip joint capsule of 14 patients with congenital dislocation of the hip (CDH) were studied biochemically. Collagen was a major component of these tissues. Solubility of collagen was reversely related to age except in two cases. Collagen of both ligamentum teres of the femur and hip joint capsule was composed of type I, III and V collagens except in three cases. In these three cases with nevus or general joint laxity, two additional collagenous components were also found. The ratios of type III collagen to type I collagen were increased in CDH except in one case with short stature. These abnormalities of collagen metabolism could be the underlying cause of CDH and other clinical symptoms in these patients.