[Nephropathy associated with idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL)].
Explore the source record for details and available documents.
Adult↗
Biomedical subjects
K Kaito
Publications and source records attributed to K Kaito.
[Erythropoietin improved anemia in a case of multicentric Castleman's disease].
Severe anemia A 37 year-old male with therapy resistant multicentric Castleman's disease (MCD) anemia was treated by subcutaneous injection of erythropoietin. Although immunoglobulin and CRP concentration increased, anemia obviously improved with hemoglobin levels increasing from 4.8 g/dl to 8.5 g/dl without any side effects. Colony assay revealed that the bone marrow mononuclear cells responded to erythropoietin in a dose dependent manner. The mechanism of anemia of MCD is not clearly understood, and treatment is sometimes very difficult. There is no other previous report concerning erythropoietin as a treatment for anemia in MCD.
Adult↗
[Impaired neutrophil function in chronic renal failure--dysregulation of surface adhesion molecule expression and phagocytosis].
To elucidate the impaired neutrophil function in patients with chronic renal failure, we analyzed the expression of the adhesion molecules, LAM-1, LFA-1, Mac-1, gp150/95 and phagocytosis activity of neutrophils in predialysis and hemodialysis patients by flow cytometry. Further, the response to granulocyte colony-stimulating factor (G-CSF), N-formyl-methionyl-leucyl-phenylalanine (fMLP) and tumor necrosis factor alpha (TNF alpha) were investigated. In hemodialysis patients, the expression of LAM-1 was decreased and that of MAC-1 was increased, indicating the activation of neutrophils. Also in predialysis patients, the same condition of "low LAM-1, high MAC-1" was observed, but to a lesser degree. Phagocytosis activity was significantly decreased in hemodialysis patients, whereas the neutrophils of predialysis patients showed almost the same phagocytosis activity compared to the controls. The responses to G-CSF, fMLP, TNF alpha were significantly reduced both in hemodialysis and predialysis patients. The inadequate activation of neutrophils and impaired response to stimulation may play an important role in uremic patients with regard to increased susceptibility to infections.
Adult↗
Cholesterol emboli following percutaneous transluminal coronary angioplasty as speculated by toe skin biopsy.
A 55-year-old man required hemodialysis for acute renal failure 3 days after repeat percutaneous transluminal coronary angioplasty (PTCA). Bilateral acrocyanosis and necrotic lesions of the toes occurred 10 days after PTCA. Skin biopsy samples revealed needle-shaped cholesterol crystals of the intraluminal clefts in his small arteries. He was diagnosed with cholesterol emboli presenting as blue toe syndrome. The conditions improved with anticoagulation and vasodilation, but improvement in renal function was temporary. He later required maintenance hemodialysis. Cholesterol emboli following PTCA are life threatening because they are difficult to diagnose and can cause severe complications.
Angioplasty, Balloon, Coronary↗
[RAEB in T with monosomy 7 after treatment of severe aplastic anemia with long term G-CSF].
A 19-year-old male who suffered from severe aplastic anemia had been treated with granulocyte colony stimulating factor (G-CSF) from September 1991. Marked increase of hematopoietic cells in his bone marrow was observed, and maintenance administration of G-CSF was continued. 15 months later, myeloblasts with nuclear abnormality increased, and 22 months later, myeloblasts with chromosomal abnormality presenting 46, XY, -7, +21 exceeded 20%, and aplastic anemia seemed to be transformed into refractory anemia with excess of blasts in transformation (RAEB in T). The usefulness of G-CSF in the treatment of aplastic anemia is now established, but there are some reports questioning the effect of long-term administration, especially transformation to MDS with monosomy 7. Leukemic transformation from aplastic anemia is very complex, but in some cases, long term administration of G-CSF may affect the natural course and may lead to the earlier development of leukemia.
Adult↗
[Acute myelomonocytic leukemia complicated with syndrome of inappropriate secretion of antidiuretic hormone, nephrotic syndrome, and hemophagocytic syndrome].
A 59-year-old man was admitted to our hospital because of fever in August 1991. Bone marrow showed normocellularity with 41.5% of CD13, 14, 33 positive blasts, and a diagnosis of AMMoL was made. Laboratory investigation revealed hyponatremia and elevated serum ADH level, indicating the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Intensive chemotherapy successfully induced hematological complete remission and his serum sodium level became normal. In February 1992, he developed proteinuria and findings were consistent with nephrotic syndrome (NS). Renal biopsy specimen showed membranous proliferative glomerulonephritis and massive infiltration of macrophages, and his serum interleukin 6 level was elevated. Five months later, he suffered from pancytopenia and elevation of biliary enzymes with increase of hemophagocytic histiocytes in his bone marrow (hemophagocytic syndrome). He transiently responded to low dose chemotherapy but he died due to severe infection. It is interesting that association between macrophages and/or cytokines with these various complications was suggested in AMMoL.
Fatal Outcome↗
[Aplastic anemia with giant splenomegaly and myelofibrosis successfully treated with antilymphocyte globulin].
Severe aplastic anemia was diagnosed in a 58-year-old female because of pancytopenia with leukocyte count 700/microliters, hemoglobin 3.4 g/dl, platelet count 4.2 x 10(4)/microliters and fatty hypoplastic bone marrow in August 1992. In January 1993, she was admitted with an abdominal skin infection caused by pseudomonas aeruginosa. After treatment of the infection, antilymphocyte globulin was given at a dose of 2,000 mg/day for four consecutive days in July 1993. This resulted in a gradual but steady improvement in her hematological data. In February 1995, her leukocyte count increased to 2,000/microliters, hemoglobin to 15.2 g/dl and platelet count to 11.0 x 10(4)/microliters. Although no splenomegaly or myelofibrosis was found previously, from April 1993, the spleen enlarged and was palpable 10 cm below the costal margin. Her bone marrow biopsy in June 1993 revealed prominent myelofibrosis. Thereafter no changes were found in these features. Splenomegaly and myelofibrosis are characteristic of primary myelofibrosis and although the relationship is uncertain, there is no previous report on aplastic anemia with splenomegaly and myelofibrosis.
Anemia, Aplastic↗
[A case of nephrotic syndrome mimicking membranoproliferative glomerulonephritis (MPGN) and associated with reactive hemophagocytic syndrome after renal death].
We report a case of nephrotic syndrome which mimicked membranoproliferative glomerulonephritis (MPGN) and was associated with hemophagocytic syndrome after renal death. A 41-year-old Japanese man was referred to our hospital because of nephrotic syndrome in February 1979. He had no signs, symptoms nor laboratory data suggestive of liver damage. He was diagnosed as idiopathic MPGN and administered prednisolone and cyclophosphamide (total dose of about 50,000mg). He developed end-stage renal disease, and dialysis therapy was initiated in February 1992. Simultaneously, he was diagnosed as hepatitis C virus (HCV)-positive liver cirrhosis. In August 1994, he died because of reactive homophagocytic syndrome, which occurred in the setting of immunosuppression due to chronic renal failure, liver cirrhosis, and sesecondary diabetes. In this case, we can not deny the possibility that radical therapeutic intervention against "idiopathic MPGN" had a negative effect on the clinical course of chronic HCV infection.
Cyclophosphamide↗
[Bilateral ankle ulcers associated with hydroxyurea therapy for chronic myelogenous leukemia].
Bilateral ankle skin ulcers developed in a 61-year-old man in the chronic phase of chronic myelogenous leukemia receiving hydroxyurea therapy. The circulating immune complex (anti-C3d antibody) was high in this case, but vasculitis was not observed in the pathological findings of biopsied skin materials. This association has been reported in patients who had chronic myelogenous leukemia or other myeloproliferative disorders and were treated with hydroxyurea. It is likely that skin ulcers are caused by hydroxyurea.
Foot Ulcer↗
[Autoimmune hemolytic anemia reactivated by alpha-interferon therapy in a case of chronic active C-type hepatitis].
A case with autoimmune hemolytic anemia (AIHA) induced by interferon-alpha (IFN-alpha) is presented. A 40-year-old male who had a previous history of autoimmune hemolytic anemia, agranulocytosis and thrombocytopenia was admitted to our hospital because of chronic C type hepatitis. Liver biopsy was performed, which diagnosed chronic active hepatitis and IFN-alpha was administrated at a dose of 3 Meg unit per day. 11 days after the initiation of the therapy he developed hemolytic anemia, but Coombs tests were negative. Although IFN was withdrawn 15 days later, anemia became progressively more serious. 20 days later, both direct and indirect Coombs tests became positive. He was diagnosed as AIHA and treated with methylprednisolone pulse therapy, then he recovered soon afterward. Further analysis of Coombs tests revealed that he had both cold type and warm type (IgG) autoantibodies which was the same type of antibodies for AIHA he suffered 10 years ago. In conclusion, latent AIHA may be reactivated by the treatment with IFN-alpha.
Adult↗
[Pure red cell aplasia complicated with polyarthritis, angitis, and acute renal failure].
A case of pure red cell aplasia (PRCA) with various complications polyarthritis, angitis, acute renal failure and DIC was successfully treated with steroid pulse therapy was described. A 55-year-old woman was hospitalized with a 9-month of intermittent but progressive joint pain, morning stiffness, general fatigue, and fever. Her initial laboratory evaluation revealed a hemoglobin of 4.4 g/dl and absence of reticulocyte. Her bone marrow aspirate showed no erythroblast which was compatible with a diagnosis of PRCA. Marked leukocytosis and thrombocytosis, positive antinuclear antigen, elevation of gammaglobulin and C-reactive protein and the presence of polyarthritis and angitis which was confirmed by renal angiography, indicated an underlying autoimmune disorders. Steroid pulse therapy was administered at 500 mg/day for 3 days, resulting in the complete response in both red cell aplasia and above findings. PRCA is known to be associated with systemic lupus erythematosus and rheumatoid arthritis very rarely, but this case did not fulfill the criteria of known collagen diseases, and there is no previous report representing PRCA with various complications such as polyarthritis, angitis and acute renal failure. This case may help us to understand more about the relationship between PRCA and autoimmune disorders.
Acute Kidney Injury↗
[Interferon-alpha therapy in the myeloproliferative variants of hypereosinophilic syndrome].
Two cases of hypereosinophilic syndrome (HES) that seemed to be myeloproliferative disorders were treated with interferon-alpha (IFN-alpha). Case 1 was a 45-year-old man with HES who was unresponsive to prednisolone. Because Flaum's hematologic grading score was 8 points, he was diagnosed as a myeloproliferative variant of HES. Although his condition was well controlled by hydroxyurea (HU), cardio-pulmonary insufficiency with refractory eosinophilia developed after 4 years. Instead of HU, IFN-alpha dramatically decreased eosinophil counts, and the clinical situation has stabilized since. Case 2 was a 30-year-old man with HES whose grading score was 9 points. IFN-alpha therapy was started when an accelerated phase of neutrophil dominant with chromosomal abnormality (8 trisomy) occurred. However, he died because of no response to IFN-alpha and other chemotherapy. In both cases, studies of eosinophil colony formation revealed no excess of eosinophil colony-stimulating factor from their lymphocytes. Flaum's hematologic grading score was useful for the diagnosis of myeloproliferative variants of HES. IFN-alpha successfully induced hematologic remission in one case, and this therapy may be beneficial for these patients as a first line agent.
Adult↗
[Cyclosporin for pure red cell aplasia caused rapid improvement of anemia and increase of CD4/8 ratio].
Ciclosporine (CS) caused rapid improvement of anemia and increase of CD 4/8 ratio in two patients with pure red cell aplasia (PRCA). One case was a 45 year old female who were unresponsive to steroids, plasmapheresis and high dose cyclophosphamide, and another was a 61 years old man with diabetes mellitus (DM) without any treatment for PRCA. In both cases hemoglobin increased soon after the initiation of CS and CD 4/8 ratio also rose from 0.80 to 1.47 and 1.78 to 1.98, respectively. There was no side effects to interrupt the course of the therapy. CS seems to inhibit the production of cytokines such as IL-2 and IFN-gamma, and it damages the activated suppressor/cytotoxic T cells. CS is an effective drug for not only refractory cases but the first step therapy for the untreated patients.
Anemia↗
[A case of hemophagocytic syndrome with multiple myeloma].
A case with multiple myeloma complicated with hemophagocytic syndrome (HS) is presented. Because pancytopenia, liver dysfunction and increase of mature histiocytes in the bone marrow appeared rapidly a diagnosis of HS was made. The patient died of multiple organ failure, despite steroid therapy. Autopsy revealed marked invasion of hemophagocytic histiocytes not only into the bone marrow but also into many other organs such as the liver, lymph nodes and kidneys. HS is a histiocyte proliferative disorders, which is likely to be seen in immunocompromised hosts, but there is no previous report about HS and multiple myeloma.
Bone Marrow↗
[Interferon therapy of idiopathic thrombocytopenic purpura].
The authors evaluated the efficacy of a daily administration of recombinant human alpha 2a interferon (IFN), given at a dose of 300MU for 12 consecutive days, in patients with steroid-nonresponsive or -dependent idiopathic thrombocytopenic purpura (ITP). Nine patients received courses of IFN therapy. Mean platelet counts rose from 1.39 to 10.9 x 10(4)/microliters and PAIgG decreased from 151.7 to 59.7 ng/10(7) cells. The maximum rise in platelet counts occurred from 10 to 42 days (mean 19.1) after the initiation of IFN. Complete response (CR) was achieved in 3 of 13 courses (23.1%), and partial response (PR) in 8 (61.5%). One CR case continued for longer than 20 months without further treatment, but intermittent IFN therapy was necessary for the other. The increment of the platelet counts was transient in all of the partial responders. No severe side effect requiring interruption of the course of IFN was experienced. Both serum IgG and PAIgG significantly correlated with the increment of platelet counts, therefore the mechanism of IFN on ITP was presumed to be associated with the inhibition of autoantibody production. Daily administration of IFN appears to be an effective and safe treatment protocol for refractory ITP.
Adult↗
[High-dose cepharanthin therapy of idiopathic thrombocytopenic purpura].
Clinical efficacy of oral high-dose cepharanthin (40-60 mg/day) was evaluated in nine patients with idiopathic thrombocytopenic purpura who were unable to discontinue the administration of adrenocorticosteroids or immunosuppressive drugs. Mean platelet counts significantly (p less than 0.05) rose from 4.5 +/- 0.9 x 10(4)/microliters to 8.9 +/- 4.2 x 10(4)/microliters without any side effects. Two to five months after the initiation of this therapy, 4 patients, including 3 who could discontinue adrenocorticosteroids, kept their platelet counts over 10 x 10(4)/microliters. It was suggested that the oral administration of cepharanthin could be a beneficial and safe strategy for ITP.
Administration, Oral↗
[Successful low-dose etoposide therapy for a case of myelofibrosis with chronic myelogenous leukemia megakaryocytic predominance type].
A 38-year-old woman presented with ear pain and vertigo. No hepatosplenomegaly or lymphadenopathy were found, but her platelet counts markedly rose to 414 x 10(4)/microliters with an increase of megakaryocytes in the bone marrow (859/microliters). Cytogenetic assay revealed positive Ph1 chromosome and rearrangement of the break point cluster region (bcr). Although platelet counts remained under 100 x 10(4)/microliters after the administration of carboquone, a high fever and pancytopenia appeared 31 months later. Bone marrow biopsy showed marked myelofibrosis which was improved by low dose etoposide. This case was thought to be Ph1 positive ET, but it was more compatible with CML megakaryocytic predominance type according to the newly proposed "Hannover criteria for myeloproliferative disorders" and cytogenetic assay.
Adult↗
[Pharmacokinetics of BHAC, VP-16 and Ara-C derived from BHAC in a hemodialysed patient with AMMoL].
Although recently the occurrence of a malignant neoplasma as a complication of uremia is becoming more frequently, pharmacokinetics of antitumor agents are not precisely studied in patients with impaired renal function. In this report we investigated pharmacokinetics of enositabine (BHAC), arabinosylcytosine (Ara-C) and etoposide (VP-16) in a patient on maintenance hemodialysis who suffered from acute myelomonocytic leukemia and treated by BHAC-EV regimen. Pharmacokinetic parameters of BHAC in uremia were not different from that in patients with normal renal function, and hemodialysis did not affect on the plasma level of BHAC. No significant accumulation of Ara-C was seen in uremia, but there remained the possibility that Ara-C could be removed by hemodialysis. So BHAC was able to be used in uremic patients safely. Since VP-16 was proved to be not a hemodialyzable but an accumulative substance and prolongation of plasma half life was prompt in uremia, VP-16 should be administered to uremic patients very cautiously. From these results BHAC-EV regimen was presumed to be a safely, well tolerated and beneficial regimen in uremic patients.
Adult↗