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Biomedical subjects

K Usuki

Publications and source records attributed to K Usuki.

At least 19 recordsLinked to original sources

Sarcoidosis associated with psoriasis vulgaris.

We report a 70-year-old patient with sarcoidosis associated with psoriasis vulgaris. He had a nodule on the medial lower lid of his right eye. Oral corticosteroid for the sarcoid lesions and oral PUVA for psoriasis were employed. The cutaneous lesion disappeared within two months after starting the therapy. No relapse of sarcoidosis has been seen for eight years. The association of sarcoidosis with psoriasis has been previously reported; however, it is still unclear whether this association coincidental or meaningful.

Aged↗

[Successful combined therapy with ATG, cyclosporin and G-CSF for both liver dysfunction and bone marrow failure in hepatitis-associated aplastic anemia].

A 28-year-old man developed cryptogenic hepatitis in January 1999, and treatment with glycyrrhizic acid improved his liver function. From June, however, pancytopenia began to develop gradually. The patient received G-CSF against leukocytopenia (WBC 1,100/microliter, neutrophils 590/microliter) and was transferred to our hospital in August 1999. A diagnosis of hepatitis-associated aplastic anemia was made on the basis of liver dysfunction (AST 156 IU/l, ALT 386 IU/l), hypoplastic bone marrow, and pancytopenia (WBC 4,400/microliter, neutrophils 3,340/microliter under G-CSF administration, Hb 9.8 g/dl, platelets 2.4 x 10(4)/microliter, reticulocytes 4.7 x 10(4)/microliter). Immediately after starting combined therapy with ATG, cyclosporin, and G-CSF, his liver function began to improve and was normalized on day 7. Pancytopenia began to ameliorate on day 9, and blood parameters on day 60 were WBC 4,200/microliter (without G-CSF administration), Hb 12.0 g/dl, platelets 9.0 x 10(4)/microliter, and reticulocytes 4.1 x 10(4)/microliter. Although the prognosis of hepatitis-associated aplastic anemia is generally poor, immunosuppressive therapy was markedly effective for both pancytopenia and hepatic dysfunction in the present case.

Adult↗

[Successful treatment of idiopathic pure red cell aplasia with antithymocyte globulin].

An 18-year-old woman was admitted to our hospital because of severe anemia on October 16, 1999. Laboratory data included hemoglobin 3.5 g/dl, reticulocytes 2,200/microliter, WBC 3,500/microliter, and Plt 38.5 x 10(4)/microliter. Bone marrow aspiration showed a normocellular marrow with severe erythroid hypoplasia, suggesting a diagnosis of pure red cell aplasia. Methylprednisolone pulse therapy was started on October 20, but there was no response. Administration of cyclosporine A (CyA; 400-450 mg) was begun on November 1, but again there was no response. Antithymocyte globulin (ATG; 800 mg/day for 5 days, 15 mg/kg) was started from December 1 in addition to prednisolone (60 mg/day) and CyA (450 mg/day). On day 7 of ATG therapy, the reticulocyte count began to increase, and reached a peak of 32.6 x 10(4)/microliter on day 20. The patient's hemoglobin level started to increase on day 13, and reached 8.5 g/dl on day 27. A complete response has been maintained up to the time of writing, and the hemoglobin level was 11.9 g/dl on December 14, 2000. This is the first detailed Japanese case report of successful treatment of pure red cell aplasia using ATG.

Adolescent↗

A case of pseudogout.

We report a 56-year-old Japanese male with a gout-like acute attack of synovitis and panniculitis in his right wrist without hyperuricemia. Calcium pyrophosphate dihydrate (CPPD) crystals were found in the synovial fluid of the affected joint. A diagnosis of pseudogout was made. The attack subsided two days after medication with 30 mg of predonisolon. Although this is a relatively common disease in elderly people, we rarely encounter such case reports in dermatological journals.

Calcium Pyrophosphate↗

Three cases of polyarteritis nodosa cutanea and a review of the literature.

We describe three cases of polyarteritis nodosa cutanea (PNC) showing necrotizing arteritis and only cutaneous lesions without systemic symptoms or visceral involvement for eleven, six, and three years after the onset of the disease. Since it was first described, there has been continuous controversy as to whether PNC progresses to systemic PN. Some cases have been described which had begun with a cutaneous lesion and progressed to the systemic form 19 and 18 years after the onset of the disease, so we believe that long term follow-up of this disease is essential.

Adrenal Cortex Hormones↗

Three cases of pityriasis rubra pilaris successfully treated with cyclosporin A.

BACKGROUND: Pityriasis rubra pilaris (PRP) is reported to respond poorly to cyclosporin A (CsA). OBJECTIVE: We attempted to determine the efficacy of CsA in the treatment of classic adult-type PRP. METHODS: Three patients with classic adult-type PRP were treated with 5 mg/kg/day CsA. RESULTS: A sustained clinical response was achieved within 2-4 weeks of therapy. Relapses were noted when the CsA dose was decreased to 1.2 mg/kg/day. CONCLUSION: CsA should be considered in the treatment of classical adult-type PRP.

Adult↗

[Treatment of adult acute lymphoblastic leukemia by KHALL-93--long-term outcome].

Twelve previously untreated adult patients with acute lymphoblastic leukemia were treated with a KHALL-93 regimen. The mean age was 46.9 years. Four patients (1/3) were over 60 years old, and 5 were Ph1 positive (42%). The complete remission rate was 100%. The 5-year survival was 50% (6/12). The 5-year event-free survival was 50% (6/12) in total, 71% (5/7) in Ph1 negative patients, and 63% (5/8) in patients younger than 60 years old. These results indicate that a KHALL-93 regimen is an effective therapy for adult acute lymphoblastic leukemia.

Adult↗

Increased serum soluble Fas ligand associated with recurrent B-cell non-Hodgkin's lymphoma after autologous peripheral blood stem cell transplantation.

Fas-ligand (FasL) is a member of the tumor necrosis factor family and transmits apoptotic cell death signal by binding to its receptor, Fas. FasL is expressed on the cell surface of activated T-cell and natural killer (NK) cell. It has been shown that the FasL can be released from the cell surface by metalloproteinase. The serum soluble FasL (sFasL) is increased in some patients with NK cell lymphoma/large granular lymphocytic leukemia. We have recently seen a patient with recurrent B-cell lymphoma accompanied with an increased serum sFasL level after autologous peripheral blood stem cell transplantation. The sFasL was markedly decreased with the tumor regression induced by the chemotherapy. We present here the first case of an elevated serum sFasL level associated with B-cell lineage malignancy and discuss the possible clinical value of sFasL.

Adult↗

Characterization of native human thrombopoietin in the blood of normal individuals and of patients with haematologic disorders.

Thrombopoietin (TPO) isolated from thrombocytopenic plasma of various animal species has previously been shown to comprise only truncated forms of the molecule, presumably generated by proteolysis. Native TPO has now been partially purified from normal human plasma by immunoaffinity chromatography and was confirmed to be biologically active. Gel filtration in the presence of SDS revealed that TPO eluted in two peaks: a major peak corresponding to the elution position of fully glycosylated recombinant human TPO (rhTPO) consisting of 332 amino acid residues, and a minor peak corresponding to a smaller molecular size. Immunoblot analysis also revealed that most plasma-derived TPO migrated at the same position as fully glycosylated rhTPO, corresponding to a molecular size of approximately 80 to 100 kDa. Furthermore, the size distribution of circulating TPO in patients with various haematologic disorders did not differ markedly from that of plasma-derived TPO from healthy individuals. These results indicate that the truncation of circulating TPO is not related to disease pathophysiology, and that the predominant form of TPO in blood is a biologically active approximately 80- to 100-kDa species. The size distribution of TPO extracted from normal platelets was similar to that of TPO in plasma; the proportion of truncated TPO was decreased by prior incubation of platelets with hirudin. indicating that the endogenous truncated TPO, at least in platelet extract, was generated by thrombin-mediated cleavage.

Hematologic Diseases↗

[X-linked sideroblastic anemia].

A 20-year-old man presented with microcytic hypochromic anemia (hemoglobin: 9.3 g/dl, MCV: 82.0 fl, MCHC: 29.5 g/dl) and dimorphism RBCs in circulating blood (RDW: 26.8%). Ringed sideroblasts accounted for 29.5% of bone marrow erythroblasts. Iron overload was also observed. Because the patient had a clear family history of anemia, he was given a diagnosis of X-linked sideroblastic anemia. The activity of delta-aminolevulinic acid synthase (ALAS) in bone marrow erythroblasts was low. However, we did not detect mutation of the gene for ALAS. The patient has responded well to a treatment regimen consisting of oral vitamin B6, Fe-chelation therapy, and phlebotomy.

Adult↗

[Successful treatment with splenic irradiation for idiopathic thrombocytopenic purpura associated with primary immunodeficiency syndrome].

A 50-year-old man was admitted to our hospital because of intracranial hemorrhage and thrombocytopenia (platelet count: 3,000/microliter). Low levels of IgG (76 mg/dl) and IgA (30 mg/dl) and a normal pattern of peripheral blood T and B cell subsets yielded a diagnosis of common variable immunodeficiency (CVID). The number of megakaryocytes in bone marrow was within normal limits (64/microliter), and a diagnosis of idiopathic thrombocytopenic purpura was made. Both high-dose intravenous gamma-globulin and prednisolone were ineffective. Because of the coexistence of CVID, splenic irradiation (total 15 Gy) was performed instead of splenectomy. The platelet number began to increase 5 days after the initiation of irradiation, had increased to 8.7 x 10(4)/microliter at the end of irradiation, and was 22.9 x 10(4)/microliter 2 weeks later.

Common Variable Immunodeficiency↗

[Successful pregnancy and delivery during alpha-interferon therapy for essential thrombocythemia].

Essential thrombocythemia (ET) was diagnosed in a 31-year-old woman who had a miscarriage in the first trimester of her first pregnancy. Because of her wish for child, the patient was given alpha interferon (alpha-IFN) instead of hydroxyurea or aspirin to lower her platelet count to about 80 x 10(4)/microliter. She also had a miscarriage in her second pregnancy. Thereafter, the platelet count was kept at 60-70 x 10(4)/microliter by dose escalation of alpha-IFN. In the 35th week of her third pregnancy, the patient delivered a healthy baby with a normal blood count. Several small infarctions were observed in the placenta. Control of platelet count by alpha-IFN administration was effective in preventing recurrent miscarriage associated with ET, and had no adverse effect on patient fertility or fetal development.

Adult↗

Effects of nicotine on peripheral cutaneous blood flow and skin temperature.

We hypothesized that if nicotine was used in a form that was not adulterated with other hazardous substances found in tobacco, it would increase cutaneous blood flow (CBF) resulting in an increase in skin temperature. The effects of nicotine on CBF was investigated in 80 healthy volunteers and 6 patients with peripheral circulation disturbances. Each subject was required to chew nicotine gum (containing 2 mg nicotine) for 15 min and the CBF was then measured with laser blood flowmetry. Skin temperature of 35 volunteers was measured with thermography before and after chewing the gum for 15 min. A control study was performed using ordinary gum without nicotine. Increased CBF (> or = + 1 ml/min/100 g) was observed in 55 of 86 subjects (64%, 33.7-38.6 ml/min/100 g, P < 0.01). An elevation in skin temperature (> + 0.1 degree C) was also observed with nicotine gum in 26 of 35 healthy subjects (74%, + 0.62 +/- 0.96 degree C, P < 0.001). The increase in CBF was greater in subjects in which the initial CBF was lower than in others (P < 0.01). Nicotine gum was found to increase CBF (55/86) and elevate skin temperature (26/35). The smaller the initial CBF value, the greater was the increase in CBF. Nicotine or nicotine derivatives might prove to be useful agents for the treatment of peripheral circulation disturbances.

Adult↗

Chronic neutrophilic leukemia associated with chronic lymphocytic leukemia.

We report on an 83-year-old male with chronic neutrophilic leukemia (CNL) associated initially with IgM monoclonal gammopathy and later with B cell chronic lymphocytic leukemia (CLL), in which the clone differed from that of the preceding monoclonal gammopathy. At initial presentation, the patient had hepatosplenomegaly, leukocytosis (29100 x 10(6)/l) with an increase of mature neutrophils (83%), 20q- chromosomal abnormality, an increased leukocyte alkaline phosphatase score, elevated serum levels of vitamin B12 and uric acid, a low serum level of granulocyte colony-stimulating factor, and high serum IgM (1015 mg/dl: lambda type M protein). Thereafter, lymphocytosis developed gradually. Three years after the initial presentation, the patient had no serum M protein, but showed evidence of leukocytosis (36600 x 10(6)/l) with 20q- chromosomal abnormality and an increase of mature neutrophils (51%) and small lymphocytes (43.5%), CD5+/19+/20+/HLA-DR+ and surface membrane IgM+/D+/kappa+. Gene rearrangements of the immunoglobulin heavy and kappa light chains were also present. To our knowledge, this is the first reported case of CNL associated with CLL.

Aged↗

Regulation of hepatic thrombopoietin production by portal hemodynamics in liver cirrhosis.

OBJECTIVE: This study was designed to clarify how thrombopoietin (TPO) functions in and, to some extent, causes thrombocytopenia complicating liver cirrhosis and portal hypertension. METHODS: Our study population consisted of 19 cirrhotic and six noncirrhotic patients who underwent percutaneous transhepatic portography (PTP) and hepatic venography. RESULTS: The platelet counts of the cirrhotic patients were significantly lower than those of the noncirrhotic patients (8.7 +/- 4.1 vs 17.4 +/- 7 x 10(4)/microl; p < 0.01). The flow direction in the splenic vein was confirmed by PTP. Ten hepatofugal and nine hepatopetal flow directions in the splenic vein were noted among the cirrhotics. The hepatofugal group showed lower portal venous pressure (20 +/- 10 vs 32 +/- 4 cm H2O; p < 0.01) than the hepatopetal group and had a higher incidence of hepatic encephalopathy (six of 10 vs zero of nine; p < 0.01). The hepatic vein-portal difference in TPO did not differ substantially between the cirrhotics and noncirrhotics (0.12 +/- 0.04 vs 0.24 +/- 0.07 fmol/ml). Comparisons of this value among the three groups showed the TPO difference to be lowest in the hepatofugal group (hepatofugal: 0.04 +/- 0.03, hepatopetal: 0.21 +/- 0.07, noncirrhotic: 0.24 +/- 0.07; p < 0.05). CONCLUSIONS: Our findings suggest that TPO production in the cirrhotic liver is regulated by the portal blood supply to the liver. Thus, portal hemodynamics may play a critical role in the development of thrombocytopenia.

Aged↗