Effectiveness and Safety of Avacopan as a Combination Therapy with Glucocorticoids or Monotherapy in Patients with Microscopic Polyangiitis.

We herein report three cases of microscopic polyangiitis (MPA). Two patients were administered avacopan in combination with glucocorticoid (GC), whereas one patient was treated with avacopan monotherapy; none of the patients were co-administered either rituximab or cyclophosphamide. The doses of GC were successfully reduced after the introduction of avacopan in the two patients, and the serum C-reactive protein levels decreased in the patient treated with avacopan monotherapy.

Surface Markers and Chemokines/Cytokines of Tumor-Associated Macrophages in Osteosarcoma and Other Carcinoma Microenviornments-Contradictions and Comparisons.

Osteosarcoma (OS) is the most common primary bone tumor in children and adolescents. Prognosis is improving with advances in multidisciplinary treatment strategies, but the development of new anticancer agents has not, and improvement in prognosis for patients with pulmonary metastases has stalled. In recent years, the tumor microenvironment (TME) has gained attention as a therapeutic target for cancer.

Case report: Characteristics and nature of primary cardiac synovial sarcoma.

Primary malignant cardiac tumors rarely occur, and cardiac synovial sarcoma (SS) is especially rare among such tumors. Herein, we present the case of a 35-year-old female with primary cardiac SS treated with surgery, chemotherapy, and radiotherapy. She presented with chest symptoms and underwent imaging examinations.

Case Report: Angiomatoid fibrous histiocytoma in the hand: a rare clinical presentation and diagnostic challenge.

Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of mesenchymal origin occurring in young adults. Based on its clinical course, it is said to have an intermediate potential. We present a case of a 59-year-old woman with AFH in the hand that was difficult to diagnose.

Takayasu's arteritis after a prolonged course of hypertrophic osteoarthropathy.

A 71-year-old male with hypertrophic osteoarthropathy was referred to our hospital because of a nocturnal fever and tenderness stretching from the left parotid region to the left front neck, in which antibacterials were ineffective. He was diagnosed with Takayasu's arteritis following findings of contrast-enhanced computed tomography and neck ultrasound. This is the first report to describe the development of Takayasu's arteritis after a prolonged course of typical hypertrophic osteoarthropathy, and the proposed hypertrophic osteoarthropathy and Takayasu's arteritis have common underlying pathophysiological factors.

Intraosseous Tophaceous Gout in Hallux Mimicking a Bone Tumor in a Young Patient: A Case Report.

Case: A 17-year-old man experienced night pain in his right hallux. Radiographs revealed a lytic lesion in the proximal phalanx of the right hallux. Magnetic resonance imaging showed a low-signal intensity mass on T1-weighted sequences and isointense-high-signal intensity on T2-weighted sequences.

Two cases of adult T-cell leukemia/lymphoma involving the terminal ileum.

We describe two cases of adult T-cell leukemia/lymphoma (ATLL) with terminal ileal involvement. The first case, a 71-year-old man with lymphoma subtype of ATLL, had a polypoid lesion in the terminal ileum, in addition to a duodenal mass. The second case, a 58-year-old woman with lymphoma subtype of ATLL, had an irregular ulcerative lesion in the terminal ileum and multiple ulcers throughout the stomach.

Systemic mastocytosis with extensive polypoid lesions in the intestines; successful treatment with interferon-alpha.

A 35-year-old female presented in 1989 with hepatosplenomegaly, but no conclusive diagnosis was established. From 1992, she experienced transient episodes of facial flushing and palpitations. Osteosclerotic change was detected radiologically.

A unique case of t(15;17) acute promyelocytic leukaemia (M3) developing into acute myeloblastic leukaemia (M1) with t(7;21) at relapse.

This report describes a case of t(15;17) acute promyelcytic leukaemia (APL, FAB subtype M3) with dysgranulopoiesis at diagnosis in a patient who developed myelodysplasia (MDS) and then a second phenotype of t(7;21) acute myeloblastic leukaemia (AML, FAB subtype M1) at the time of relapse. To our knowledge, there is no report of a second phenotype of AML occurring after complete remission (CR) of APL. Furthermore, this is the first report of chromosomal abnormality t(7;21) in a case of AML.

Characteristics of chemotherapy-induced clinical remission in long survivors with aggressive adult T-cell leukemia/lymphoma.

The acute and lymphoma types of adult T-cell leukemia/lymphoma (ATL) usually have a very poor prognosis, although some patients achieve long survival after chemotherapy. A total of 114 patients with these aggressive types of ATL were newly diagnosed at our institution from 1975 to 1989. By multivariate analysis, poor performance status and high serum creatine levels were associated with shortened survival.

Prognostic significance of the proportion of Ki-67-positive cells in adult T-cell leukemia.

The authors examined peripheral blood samples from patients with adult T-cell leukemia (ATL) using the monoclonal antibody Ki-67 which detects a nuclear antigen present in actively proliferating cells. In patients with chronic ATL, the percentage of Ki-67-positive cells was significantly lower than in acute ATL patients (median values, 3.3% versus 18.

Cytogenetic studies of human T-cell leukemia virus type I carriers. A family study.

Recently, the chromosome 14q11 anomaly has been reported to be specific to adult T-cell leukemia (ATL), and this anomaly has also been confirmed in the preleukemic state of adult T-cell leukemia (pre-ATL) patients. Because the cytogenetic abnormality at the stage of human T-cell leukemia virus type I (HTLV-I) carrier remains uncertain, we performed cytogenetic studies of lymphocytes stimulated with phytohemagglutinin in three HTLV-I carriers and three non-HTLV-I carriers in an ATL family. As a result, in three HTLV-I carriers, four of 311 cells examined (1.

[Trisomy of chromosome 11 in a case of common ALL antigen-positive acute myeloblastic leukemia (FAB-M1)].

In February 1986, a 68-year-old woman was diagnosed as having acute myeloblastic leukemia (FAB-M1). At the time of diagnosis, 86.0% of the bone marrow cells were myeloblastoid, and 15% of these myeloblastoid cells were positive to myeloperoxidase.

[Cytogenetic studies of healthy HTLV-I carriers (Report 1): Cytogenetic studies of an ATL family].

The chromosome 14q11 anomaly has been reported to be specific to adult T-cell leukemia (ATL) and this anomaly has also been confirmed in preleukemic state of ATL (pre-ATL) patients though the frequency is low. In an attempt to clarify if the same chromosome aberrations could be found also at the stage of HTLV-I carrier and if there is any cytogenetic difference from non-HTLV-I carriers, a cytogenetic study of lymphocytes stimulated with phytohemagglutinin in three HTLV-I healthy carriers and three non-HTLV-I carriers in an ATL family was performed. The results were as follows.

[A trial for prolongation of chronic phase of chronic myeloid leukemia--maintenance of leukocyte counts within normal range with busulfan].

To prolong the survival of patients with chronic myeloid leukemia (CML), 19 patients were treated with busulfan to keep their leukocyte counts within normal range by controlling bone marrow hyperplasia. The duration of chronic phase in these patients was significantly longer than that in historical controls who were treated conventionally with busulfan. This prolongation was not ascribable to the difference in such prognostic factors between the two therapy groups as splenomegaly, leukocyte count and percentage of peripheral blasts.

Chromosomes and surface markers in lymph node cells from 2 patients with AILD and IBL-like T-cell lymphoma.

Chromosomes and surface markers in lymph node cells from 2 patients with angioimmunoblastic lymphadenopathy associated with dysproteinemia (AILD) or immunoblastic lymphadenopathy (IBL) and with IBL-like T-cell lymphoma, respectively, were examined before treatment. In the patient with AILD, a small clone with chromosome abnormality was found in lymph node cells although a surface marker study failed to demonstrate monoclonality. In this patient, the clinical and cytogenetic findings suggested a subtype of lymphoma with a mild clinical course and a benign histological appearance.

[Epidemiological studies on malignant lymphoma in Nagasaki City, especially in relation to atomic bomb exposure].

One hundred and eighty-two (182) cases of malignant lymphoma registered at the Nagasaki Tumor Registry from 1973 to the end of 1977 were studied in relation to atomic bomb exposure. No significant difference in the incidence of malignant lymphoma was found between the exposed and nonexposed groups. One hundred and thirty-one (131) cases excluding Hodgkin's disease and mycosis fungoides were histologically reviewed and classified according to Lymphoma Study Group (L.

Prognostic significance of the morphological dysplastic changes in chronic myelogenous leukemia.

Various morphological dysplastic changes were observed in patients with chronic myelogenous leukemia, especially in the acute crisis. To clarify their significance, we divided 45 patients in the acute crisis into two groups by our scoring system, the dysplastic group and the non-dysplastic group. Five of 25 subjects in the non-dysplastic group entered complete remission.

Primary pancreatic carcinoma in childhood. Pancreatoblastoma.

A case of primary pancreatic carcinoma confirmed by postmortem examination in a 15-year-old girl is presented. The tumor was studied by light and electron microscopy and an indirect immunoperoxidase technique. Some of the tumor cells contained eosinophilic, PAS-positive, diastase-resistant granules.