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Biomedical subjects

Kenichi Nagai

Publications and source records attributed to Kenichi Nagai.

At least 19 recordsLinked to original sources
PubMed2006

Chronic eosinophilic leukemia with the FIP1L1-PDGFRalpha fusion gene in a patient with a history of combination chemotherapy.

Hypereosinophilic syndrome (HES) was diagnosed in December 2000 in a 43-year-old man on the basis of persistent eosinophilia (11.7 x 10(9)/L) and a normal karyotype of the bone marrow cells. He had developed intra-abdominal non-Hodgkin's lymphoma and in 1992 had received 3 courses of combination chemotherapy with doxorubicin (Adriamycin), cyclophosphamide, vincristine, methotrexate, bleomycin, and prednisolone. The patient was orally given prednisolone (10 mg/day) and cyclophosphamide (50 mg/day) as HES treatment without a subsequent improvement of the eosinophilia. In May 2003, anemia (hemoglobin, 7.9 g/dL) and thrombocytopenia (65 x 10(9)/L) manifested with progressive eosinophilia (21.0 x 10(9)/L) and a small number of blasts. The patient became febrile and was admitted in July 2003. Cytogenetic reexamination of the bone marrow cells disclosed the deletion of 4q12, indicating the presence of a fusion of the Fip1-like 1 (FIP1L1) gene to the plateletderived growth factor receptor alpha (PDGFRalpha) gene and consequently the clonal nature of his hematopoietic cells. DNA sequence analysis demonstrated that the breakpoints of the FIP1L1 and PDGFRalpha genes were present in exon 9 and exon 12, respectively. Treatment with imatinib mesylate (300 mg/day) promptly brought about complete remission. Although a number of similar eosinophilic cases have been reported, our patient may be the first such patient with a history of chemotherapy.

Adult↗
PubMed2006

Successful treatment of minimal residual disease-positive Philadelphia chromosome-positive acute lymphoblastic leukemia with imatinib followed by reduced-intensity unrelated cord blood transplantation after allogeneic peripheral blood stem cell transplantation.

We describe a 35-year-old woman with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) who received allogeneic sibling donor peripheral blood stem cell transplantation (PBSCT) and entered a second complete remission. Upon detection of BCR-ABL transcripts after PBSCT, the patient received imatinib, leading to molecular remission. Following the failure of donor leukocyte infusions, she underwent reduced-intensity unrelated cord blood transplantation (RI-UCBT), and has continued durable molecular remission for more than 30 months without substantial graft-versus-host disease. Because of a lack of adverse effects of imatinib on transplantation outcome, a treatment strategy consisting of molecular monitoring-guided initiation of imatinib followed by RI-UCBT may be promising in the management of Ph+ ALL after allogeneic SCT.

Adult↗
PubMed2006

Multiple granulocytic sarcomas in essential thrombocythemia.

A 59-year-old woman was diagnosed with essential thrombocythemia in 1988 and had been treated with hydroxyurea, mitobronitol, busulfan, and ranimustine, in that order. Hepatosplenomegaly, low-grade fever, and body weight loss manifested, and a few blasts were noted in the peripheral blood studied in March 2002. A biopsied specimen of the bone marrow showed myelofibrosis but not a leukemia in August 2004. An abnormal karyotype with der(1; 13) appeared for the first time. She was treated with low-dose prednisolone. In January 2005, she experienced left hip joint pain, and magnetic resonance scanning showed a tumoral lesion in the femoral head. Histological diagnosis of the biopsied mass revealed that it was a granulocytic sarcoma, and radiotherapy was performed. In April 2005, bone scintigraphy showed multiple lesions. She became febrile and red blood cell transfusion-dependent with hepatosplenomegaly and a small number of circulating blasts. Intravenous cytarabine (low dose) and etoposide relieved the fever and hepatosplenomegaly; however, she developed a pathologic fracture of the right humerus. An additional karyotypic abnormality (7q22 deletion) was noted. She subsequently died of infection. Granulocytic sarcoma is very rare in essential thrombocythemia, and this patient may be the first reported case of essential thrombocythemia that developed multiple lesions and a pathologic fracture without transformation to overt leukemia.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Excellent response of chemotherapy-resistant B-cell-type chronic lymphocytic leukemia with meningeal involvement to rituximab.

A 70-year-old woman was diagnosed with B-cell-type chronic lymphocytic leukemia (B-CLL) in May 2001. Initial white blood cell (WBC) count was 37 x 10(9)/l and most of the cells were mature small lymphocytes. Surface antigen analysis of these lymphocytes revealed positive reactions for CD19, 20, 25, 5, and lambda-light chain. Despite her Rai stage-0 status, various treatments were ineffective, including cyclophosphamide; fludarabine; 6-mercaptopurine; a combination of vincristine, cyclophosphamide, prednisolone, and adriamycin; and etoposide. Her WBC count increased, ranging from 150 to 450 x 10(9)/l, with marked splenomegaly, and symptoms of meningitis, such as headache, ophthalmalgia, hearing disturbance, and abnormal behavior, being manifested. The WBC count in the cerebrospinal fluid was elevated to 134/microl. The surface phenotype of these cells was identical to that of circulating lymphocytes, indicating meningeal involvement of leukemia, a rare complication in B-CLL. At the time of this WBC elevation, 24% of circulating lymphocytes had prominent nucleoli, indicating progression of the disease to CLL/prolymphocytic leukemia. Her symptoms disappeared after repeated intrathecal injections of methotrexate and dexamethazone. After four courses of treatment of the refractory B-CLL with rituximab, an anti-CD20 monoclonal antibody, the WBC count returned to normal levels and the splenomegaly disappeared. She is currently well, with sustained remission, as of April 2004.

Aged↗
PubMed2005

[A case of T4 esophageal squamous cell carcinoma in esophagogastric junction effectively treated by neoadjuvant FAP therapy].

A 59-year-old man was admitted to our hospital because of vomiting and body weight loss. Upper GI series and upper digestive tract endoscopy revealed type 3 cancer lesion in the area from the lower thoracic esophagus to the middle gastric body. In the biopsy specimen squamous cell cancer was shown. We perfomed neoadjuvant chemotherapy because the computed tomography revealed invasion to the diaphragm and aorta. After chemotherapy a 65% reduction was obtained, and we performed total gastrectomy with lower esophagectomy and lower mediastinal and abdominal lymph node dissection and Roux-en Y reconstruction in the left thoracoabdominal serial incision. In the histological examination, Grade 1 response was obtained in the primary specimen and Grade 3 response in the lymph node specimens. In the 44 months since surgery, the patient has remained well and disease free. It was suggested that a combination of neoadjuvant chemotherapy and surgery was successful treatment for this case.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Usefulness of sequential automated analysis of fragmented red blood cells for the differential diagnosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic hematopoietic cell transplantation.

Differentiating thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) from other complications following allogeneic hematopoietic cell transplantation (HPCT) requires objective, reliable markers. To this purpose, we assessed the clinical usefulness of sequential quantified analysis of fragmented red blood cells (FRC) with the Sysmex XE-2100 automated hematology analyzer. The correlation between manual and automated counting was significant (r = 0.917; P < .0001). Of 25 cases, the peak FRC percentage (FRC%) exceeded 1.3% after allogeneic HPCT in 11 cases, and lactate dehydrogenase levels were elevated in 5 of these 11 cases. Two patients received diagnoses of TTP-HUS following allogeneic HPCT, and both had initial diagnoses of acute graft-versus-host disease. In both cases, the sharp increase in the FRC% to >3% simultaneously with clinical exacerbation was helpful for differentiating TTP-HUS following allogeneic HPCT from other complications. We conclude that FRC% data sequentially obtained by an automated count seem to be useful as an objective marker of TTP-HUS following allogeneic HPCT.

Acute Disease↗
PubMed2005

[Renal insufficiency caused by leukemic cell infiltration in Sézary syndrome].

A 77-year-old woman was admitted to our hospital because of pneumonia and heart failure in July 2002. She had been diagnosed as having with Sézary syndrome in 1993, and had been treated with a combination of prednisolone, methotrexate, and cyclosporin A with subsequent stable disease but persistent generalized erythroderma. On admission, the white blood count was 23.9 X 10(9)/L with 28% Sézary cells, and serum creatinine levels were within normal limits. One month after admission, the pneumonia and heart failure improved remarkably with antibiotics and diuretics. However, at the same time, her renal function deteriorated with increasingly high serum creatinine levels. She died of anuria in September, 2002. An autopsy showed marked perivascular and peritubular infiltration of abnormal lymphocytes with degenerative nephrotubuli in the kidneys. This patient may be the first reported case of Sézary syndrome with renal failure caused by leukemic infiltration.

Aged↗
PubMed2004

Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report.

Heavy chain diseases (HCD) are monoclonal lymphoproliferative disorders of B-cells characterized by the synthesis of truncated heavy chains without associated light chains. In patients with mu-HCD, which is a very rare form of HCD, neoplastic cells produce immunoglobulin M heavy chain. The prognosis for patients with mu-HCD is poor, and there is no specific treatment for mu-HCD. In this report, we present a patient with mu-HCD accompanied by splenomegaly and thrombocytopenia. We treated this patient with the fludarabine monophosphate therapy we use for patients with B-cell chronic lymphocytic leukemia. After 5 courses of fludarabine monophosphate treatment, the splenomegaly and thrombocytopenia improved. Fludarabine monophosphate therapy may be a new strategy to improve the prognosis of patients with mu-HCD.

Antimetabolites, Antineoplastic↗
PubMed2004

Primary myelofibrosis terminated in basophilic leukemia and successful allogeneic bone marrow transplantation.

Transformation of primary myelofibrosis (PMF) to basophilic leukemia is very rare. We report the case of a 44-year-old man who had had PMF for 6 years. His hematopoiesis deteriorated with marked splenomegaly, requiring multiple red blood cell and platelet transfusions. Soon after splenectomy, progressive basophilia (32.3 x 10(9)/L) developed, infiltrating the skin as well as the bone marrow. The patient underwent allogeneic bone marrow transplantation with cells from an HLA-matched sibling. Despite the presence of hyperhistaminemia (99.1 ng/mL) after conditioning with cyclophosphamide, the pregrafting and post-grafting periods were uneventful. Prophylactic administration of both H1 and H2 receptor antagonists and sufficient hydration appeared to be important.

Adult↗
PubMed2004

[Severe hyponatremia with consciousness disturbance caused by hydroxyurea in a patient with chronic myeloid leukemia].

A 79-year-old man was admitted because of consciousness disturbance on August 9, 2002. He had been diagnosed as having chronic myeloid leukemia in 1999, and since then, he had continued to take hydroxyurea (1500 mg/day) orally. On admission, his serum sodium concentration was as low as 119 mEq/L, while urinary sodium excretion was high. Based on the blood picture and lack of hepatosplenomegaly, we considered that the leukemia was still in the chronic phase. Because of normal blood level of the antidiuretic hormone (ADH) concentration and sufficient urine volume, the syndrome of inappropriate ADH secretion (SIADH) was unlikely, and sodium-losing nephropathy was suspected. After discontinuation of hydroxyurea, the urinary sodium excretion decreased and the patient's consciousness became clear concomitantly with improvement in the serum Na level. This patient appears to be the first case of hyponatremia caused by hydroxyurea.

Aged↗
PubMed2004

[Pleural involvement in the course of chronic myelomonocytic leukemia and the development of multiple colonic perforation due to leukemic infiltration in the acute leukemia phase].

A 68-year-old man with chronic myelomonocytic leukemia (CMML) was initially treated with hydroxyurea with subsequent stable disease. In the time course, he developed bilateral pleuritis accompanied by leukocytosis and spiking fever. Cytologic analysis of the pleural effusion revealed abundant mature granulocytes and monocytes. He was treated with intravenous or oral etoposide with consequent resolution of the pleuritis, indicating the pleural involvement of CMML. Three months later, he developed hepatomegaly and became febrile. One month thereafter, the CMML transformed to acute myeloid leukemia, and the patient developed massive bloody stools associated with epigastric pain and leukocytosis. A gastrofiberscopic examination showed multiple bleeding gastric ulcers. The bleeding ulcers were treated with the clipping procedure; however, the bloody stools continued. Although intravenous etoposide was effective for the leukocytosis and hepatomegaly, the treatment did not improve the bloody stools. The patient finally died of panperitonitis. The autopsy showed multiple ulcers of the transverse colon, some of which were perforated. Microscopically, the ulcerated areas were densely infiltrated with leukemic cells predominantly consisting of immature monocytes and granulocytes. This patient may be the first reported case of CMML complicated by colonic perforation due to leukemic infiltration.

Aged↗
PubMed2003

Association of intestinal malignant lymphoma and ulcerative colitis.

A 42-year-old woman with refractory ulcerative colitis (UC) developed ascites, pleural effusion, pretibial edema and severe anemia. Colonofiberscopic examination showed a bulky submucosal tumor in the sigmoid colon, which was histologically diagnosed as malignant lymphoma (diffuse large, B cell type). The lymphoma was resistant to chemotherapy. Autologous peripheral blood stem cell transplantation (PBSCT) was effective; however, she died of severe infection after the second PBSCT. Although the association of intestinal lymphoma with UC is rare, lymphoma should be taken into consideration when the clinical course of UC is atypical or when UC is refractory to therapy.

Adult↗
PubMed2003

[Current status of reduced-intensity stem cell transplantation; survey in the Kinki area].

The status of reduced-intensity stem cell transplantation (RIST) in the Kinki area was investigated by questionnaires. We sent out questionnaires to 72 institutions and received 51 replies (72.2%). RIST was performed in 16 institutions (31.4%). A total of 91 cases were included of whom approximately 70% were non-remission or advanced hematological malignancies. Various conditioning regimens were used for RIST, most often consisting of fludarabine, busulfan and antithymocte globulin. Acute GVHD developed in 57% of all patients. Relapse or disease progression developed in 42.7% of evaluable patients. The overall survival rate was 54.6%, and 80% of low risk patients were alive at the time of analysis.

Adolescent↗
PubMed2003

[A case of multiple superficial esophageal cancers with liver cirrhosis successfully treated by radiotherapy].

We report a case of multiple superficial esophageal cancers with liver cirrhosis successfully treated by radiotherapy. The patient was a 55-year-old man with liver cirrhosis who was under the treatment of internists at our hospital. Upper digestive tract endoscopic examination revealed multiple superficial esophageal cancers. We performed radiotherapy because pancytopenia was found in his peripheral blood data. The treatment was started on a outpatient basis, but the patient was hospitalized after sixteen treatments were finished because of hematemesis. Total irradiation was 66 Gy, and complete reduction was obtained. Eight months after the treatment, the patient shows no signs of recurrence. These results suggest that radiotherapy is an effective treatment for multiple superficial esophageal cancer.

Carcinoma, Squamous Cell↗
PubMed2003

[Pancytopenia and ARDS with high dose hepatic arterial infusion].

The patient was a 74-year-old woman with gastric cancer with multiple liver metastasis. She was treated with daily oral administration of TS-1 100 mg/day (day 1-21) and systemic administration of CDDP 90 mg (day 8) as neoadjuvant chemotherapy for 2 courses. As metastatic lesions became smaller, we performed distal gastrectomy. TS-1 was started for the residual cancer lesion. However, liver metastatic lesions increased in size, so we carried out intraarterial chemotherapy (IAC), Nausea appeared at 9 days, pancytopenia at 28 days and ARDS at 78 days after IAC. She died due to ARDS.

Aged↗
PubMed2002

Insufficient duration of action of antihypertensive drugs mediates high blood pressure in the morning in hypertensive population: the Ohasama study.

Blood pressure (BP) usually peaks in the morning. The circadian variation of the onset of cardiovascular disease mimics this circadian BP variation. To examine the determinants of the BP difference between the self-recorded BP in the morning (home BP) and daytime average ambulatory BP a cross sectional study was done in the general population of Ohasama, Japan. 1207 subjects > or = 20 years measured both home (more than 14 times) and ambulatory BPs (326 treated for hypertension and 881 untreated subjects), The prevalence of subjects with the systolic BP difference (home BP in the morning - daytime ambulatory BP) of > or = 10 mmHg (high morning BP) was 5.6% in untreated normotensives, 2.9% in untreated hypertensives, and 25.8% in treated hypertensives. This trend was also observed for diastolic pressure. Multiple regression analysis demonstrated that age, male sex, and use of antihypertensive drugs were positively associated and day-night difference of BP was negatively associated with the high morning BP, respectively. These results suggest an insufficient duration of antihypertensive action of widely used antihypertensive drugs in Japan from the 1980s to 1990s. The amplitude of the day-night difference of ambulatory BP in subjects with a high morning BP was lower (non-dipping) than that without high morning BP. The high morning BP is not necessarily accompanied by hypertension but might be mediated, at least in part, by an insufficient duration of action of antihypertensive drugs. The high morning BP accompanies so-called non-dipper pattern of circadian BP variation. An insufficient duration of action of drugs may partly mediate non-dipping in subjects with antihypertensive medication.

Adult↗
PubMed2002

Successful cochlear implantation in prelingual profound deafness resulting from the common 233delC mutation of the GJB2 gene in the Japanese.

OBJECTIVES: Recently, we identified three novel mutations of the GJB2 gene in Japanese families with autosomal-recessive non-syndromic deafness.1 Seven of 11 mutated chromosomes (63.6%) contained a 233delC allele, suggesting that the 233delC mutation is the most common mutation of the GJB2 gene in the Japanese population. After it was recognized that cochlear implantation (CI) is of benefit to children with prelingual deafness, we have had a number of prelingual pediatric CI patients. Because children carrying the homozygous 233delC mutation show bilateral prelingual profound deafness, they could be enrolled in the CI program at Osaka University Graduate School of Medicine. The purposes of this study were 1) to analyze the occurrence of the GJB2 mutations in our 15 prelingual pediatric CI patients in whom the cause of non-syndromic deafness was unknown, and 2) to evaluate the auditory function and postoperative speech perception with CI of those GJB2-related deaf subjects. STUDY DESIGN: Retrospective analysis. METHODS: Mutation analysis of the GJB2 gene by direct sequencing was performed with genomic DNA from 15 children born profoundly deaf as a result of unknown causes and implanted with CI. Intraoperative electrically evoked auditory brainstem response (EABR) and intra-/postoperative EAP were measured. The speech perception was evaluated with Infants and Toddlers Meaningful Auditory Integration Scale (IT-MAIS). RESULTS AND CONCLUSIONS: We identified 4 CI patients (26.7%) out of 15 children carrying the homozygous 233delC mutation. Intra- and postoperative evaluation of the auditory system revealed almost intact cochlear and retrocochlear auditory function in these 4 patients. Postoperative auditory testing indicates that their speech perception had become significantly higher in comparison with that of other prelingual CI patients. These results suggest that prelingual deaf children carrying the homozygous 233delC mutation of the GJB2 gene can benefit from CI.

Audiometry↗