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Kawano N.,Miyazaki Prefectural Miyazaki Hospital
Journal of clinical and experimental hematopathology : JCEH | Year: 2016

Although clinical trials of first- and second-generation tyrosine kinase inhibitors (TKIs) have been shown to improve the prognosis of chronic myeloid leukemia (CML), there is still uncertainty about the clinical features, treatment outcomes, adverse effects, and other possible problems of their use in the clinical setting. We retrospectively analyzed 51 CML patients treated with TKIs at a single institution between 2002 and 2014. The patients (median age: 53.8 years) were classified as having chronic (n = 48), accelerated (n = 2), or blastic phase (n = 1) CML. Our treatments included both 1st generation TKIs (60.8%) and 2nd generation TKIs (39.2%). We found that the overall response rates of complete cytogenetic response (CCyR), major molecular response (MMR), and MR4 (molecular response 4) were 90.2%, 78.4%, and 64.7%, respectively. Second line 2nd generation TKIs had response rates equivalent to those of 1st line 1st generation TKIs. Moreover, 1st line 2nd generation TKIs tended to achieve an early response rate. Overall survival (OS) at 5 years was 93.2%. Sudden blastic crisis (BC) occurred in 2 CML patients receiving TKI with CCyR status. Hematopoietic stem cell transplantation was performed for BC (n = 1) and sudden BC (n = 2). Side effects of all grades (1-3) and grade 3 alone were 64.7% and 11.8%, respectively. Dose reduction, replacement with another TKI, or low dose TKI treatment may be useful methods to control side effects. Further reasons of TKI discontinuation were economic problems (n = 3) and pregnancy (n = 1). Consequently, our treatment strategy for CML demonstrated good response rate and OS. Currently, treatment discontinuation due to intolerance, resistance, economic problems, pregnancy, and sudden BC remains a concern in clinical practice.


Kuriyama T.,Miyazaki Prefectural Miyazaki Hospital
Journal of clinical and experimental hematopathology : JCEH | Year: 2016

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a serious disorder in which monoclonal growth of T cells infected with EBV and macrophage activation cause pancytopenia, high fever and acute liver failure. Patients with chemotherapy- or immunosuppression-resistant EBV-HLH require allogeneic hematopoietic stem cell transplantation (allo-HSCT), but patients who have no sibling donors may not have time to wait for an unrelated donor. In pediatric patients, there are some reports on allogeneic cord blood transplantation (allo-CBT) for the treatment of EBV-HLH; however, in adult patients, reports of allo-CBT for EBV-HLH are quite limited. The present case of a 20-year-old woman with chemotherapy-resistant EBV-HLH and systemic lupus erythematosus (SLE) who underwent allo-CBT following reduced-intensity conditioning (RIC-CBT). She achieved and maintained a complete donor type and the EBV-DNA load, and the titers of anti-double stranded DNA and antinuclear antibodies became negative. It is therefore considered that RIC-CBT is an effective treatment option for adult onset HLH. However, because the effectiveness of allo-HSCT for SLE remains unclear and transplant-related mortality is high, it is recommended that allo-HSCT for SLE is restricted to patients concomitant with oncohematological disease as with our present case.


PubMed | Fukuoka University, Tokuyama City Hospital, Beppu Medical Center, Kurume University and 10 more.
Type: Journal Article | Journal: International journal of hematology | Year: 2016

In a previous study of childhood acute lymphoblastic leukemia (ALL) by the Kyushu-Yamaguchi Childrens Cancer Study Group, ALL-96, we achieved a 72.1 % 5-year event-free survival (EFS) and an 84.8 % 5-year overall survival (OS). In a subsequent study, ALL-02, we adopted a vincristine dexamethasone (VCR/DEX) pulse regimen as maintenance therapy in the context of the ALL-96 study using the same risk classification and treatment schedule. A total of 156 pediatric cases of ALL were treated with ALL-02. All of the patients were classified as standard-risk or high-risk. Risk stratification was based on white cell counts, immunophenotype, the presence of central nervous system (CNS) disease at diagnosis, organomegaly, and early treatment response (day 14 bone marrow status). The 7-year EFS and OS rates were 77.7 % (95 % CI 70.6-84.8 %) and 89.5 % (95 % CI 84.6-94.4 %), respectively. CNS 3 status [hazard ratio (HR) = 5.0, p = 0.009] and high white blood cell count at diagnosis (HR = 2.6, p = 0.047) were risk factors for poor EFS in multivariate analysis. Our strategies to categorize patients into two risk groups, and to treat with a VCR/DEX pulse were feasible and reasonably effective treatments for pediatric ALL.


PubMed | Miyazaki Prefectural Miyazaki Hospital, Japan Community Health care Organization Kyushu Hospital, Kagoshima University and Kyushu University
Type: Journal Article | Journal: Pediatrics international : official journal of the Japan Pediatric Society | Year: 2016

Determining the cause of bowel obstruction without a history of laparotomy (BO without HL) is difficult and can result in delay of treatment and development of a potentially life-threatening situation. We herein investigated the clinical characteristics of pediatric patients who underwent laparotomy due to BO without HL.All surgical pediatric patients aged <16 age who were diagnosed with BO without HL between January 2004 and September 2014 were included. Etiology of BO, intraoperative findings and postoperative outcome were investigated retrospectively.Seventy patients were diagnosed with BO without HL in this period. In these 70 patients, malrotation (n = 34), intussusception (n = 19), internal hernia (n = 6) and Meckels diverticulum (n = 5) were predominantly identified. Regarding preoperative definitive diagnosis, prevalence of internal hernia, Meckels diverticulum or idiopathic volvulus was significantly lower than that of malrotation or intussusception (P < 0.05). Intraoperatively, the rates of strangulation and bowel resection were 55.7% and 30.0%, respectively. The optimal time for emergency operation in order to avoid strangulated bowel resection was <19 h from onset of symptoms.Malrotation and intussusception are major causes of BO without HL in children, but internal hernia, Meckels diverticulum and idiopathic volvulus should always be taken into account, particularly because of the preoperative diagnostic difficulty and resulting high rate of intestinal resection. In order to avoid resection of the bowel, surgery should be done within 19 h before bowel ischemic change occurs.


PubMed | University of Miyazaki, Miyazaki Prefectural Miyazaki Hospital, Fukuoka University, Nagasaki University and University of Tokyo
Type: Journal Article | Journal: Nephrology (Carlton, Vic.) | Year: 2016

Mutations of LMX1B cause nail-patella syndrome, a rare autosomal dominant disorder. Recently, LMX1B R246Q heterozygous mutations were recognised in nephropathy without extrarenal manifestation. The aim of this study was to clarify characteristics of nephropathy caused by R246Q mutation.Whole exome sequencing was performed on a large family with nonsyndromic autosomal dominant nephropathy without extrarenal manifestation. Clinical and histological findings of patients with LMX1B mutation were investigated.LMX1B R246Q heterozygous mutation was identified in five patients over three generations. Proteinuria or haematoproteinuria was recognized by urinary screening from all patients in childhood. Proteinuria gradually increased to nephrotic levels and renal function decreased in adolescence. Two patients progressed to end-stage renal disease in adulthood. Renal histology demonstrated minimal change in childhood and focal segmental glomerulosclerosis in adulthood. Using electron microscopy, focal collagen deposition could be detected in glomeruli even when a moth-eaten appearance was not apparent in the glomerular basement membrane. In addition, podocin expression in glomerular podocytes was significantly decreased, even in the early stages of disease progression.Comprehensive genetic analyses and collagen or tannic acid staining may be useful for diagnosis of LMX1B-associated nephropathy. While renal prognosis of R246Q may be worse than that of typical NPS nephropathy, signs of podocytopathy can be detected during the infantile period; thus, childhood urinary screening may facilitate early detection.


Kawano N.,Miyazaki Prefectural Miyazaki Hospital
Journal of clinical and experimental hematopathology : JCEH | Year: 2013

Crystal-storing histiocytosis is a rare clinical entity characterized by an increase in the number of abnormal histiocytes accompanied by accumulation of crystallized immunoglobulins. We describe the case of an 80-year-old man who presented with crystal-storing histiocytosis of the lung 13 years after receiving a diagnosis of gastric non-Hodgkin lymphoma (NHL ; clinical stage, Lugano IA). After wedge resection of the left upper lobe, the histological findings showed crystal-storing histiocytosis with CD68(+), some small to medium lymphoid cells with CD79a(+) with κ(+(weekly)) and λ(-), and some plasma cells with CD138(+), and rearrangement of the immunoglobulin heavy chain. Based on the nonrecurrent gastric NHL, small B-cell population, and failure to detect the same clone by polymerase chain reaction analysis, our case was classified as pulmonary localized crystal-storing histiocytosis without underlying lymphoproliferative or plasma cell disorder. The findings of minor B-cell populations harboring a heavy chain rearrangement with slight light-chain restriction (κ > λ) may be related to the pathogenesis of crystallogenesis and crystal-storing histiocytosis. Moreover, surgical treatment may be an effective therapeutic option for solitary crystal-storing histiocytosis.


Chosa N.,Miyazaki Prefectural Miyazaki Hospital
Nishinihon Journal of Dermatology | Year: 2012

An 83-year-old woman had left hemiplegia caused by a thalamic hemorrhage in 2001, and from 2002 an edema developed gradually in the left lower extremity, which was diagnosed as chronic lymphedema of the left lower extremity in 2006. In March 2011, she developed a skin ulcer on the left leg. Because the lesion worsened, she was referred to our institution in May 2011. We found a lymphedema of the left lower extremity, a pigmented leg, and infectious, necrotic, bleeding skin ulcers on the leg that were between 5 cm and 13 cm in diameter. Computed tomography angiography revealed an obstruction and varication of the left femoral vein on the level of the inguinal ligament, and an obstruction in the left external iliac vein. Early perfusion of the veins of the left lower extremity suggested strongly that there was an arteriovenous fistula. Although the lesions were treated with topical conventional wound therapy, the lesions were not improved. Therefore, we performed a custom-made negative pressure wound therapy (NPWT) on the lesions, using the hospital's central vacuum system, and a good granulating bed was obtained. Because the granulating bed was large, we conducted a split-thickness skin graft (a patch graft ; the grafted skin was removed by the wheal shaving method) for the wound at the bedside, and NPWT was applied to the wound postoperatively. The patch graft with concurrent NPWT was performed every 2 weeks, for a total of 3 times, and the lesions were healed. The healing of skin ulcers from chronic lymphedema are markedly delayed. NPWT will be a significant therapeutic option for skin ulcers from chronic lymphedema. And then, when the obtained granulating bed is large, the patch graft (using the wheal shaving method) is simple and easy to use together with NPWT, which will contribute to the earlier healing of the wound bed.


Kawano N.,Miyazaki Prefectural Miyazaki Hospital
Journal of clinical and experimental hematopathology : JCEH | Year: 2011

Disseminated intravascular coagulation (DIC) is a clinical entity with high mortality and is characterized by multiple organ failure caused by activation of systemic intravascular coagulation. Although a standard treatment for DIC has not been established owing to the absence of randomized controlled trials, recent reports have indicated that recombinant human soluble thrombomodulin (rTM) is effective against DIC. To elucidate the clinical characteristics and outcomes of DIC, we retrospectively analyzed 35 DIC patients treated with rTM at our institution over a 2-year period (infectious disease: 21 cases; hematological disease: 14 cases). Diagnosis of DIC was based on the diagnostic criteria for DIC of the Japanese Ministry of Health and Welfare. In addition to the treatment of underlying diseases, we administered rTM for 6 consecutive days. Twenty-one (60.0%) of the DIC patients attained resolution of DIC at 7 days after administration (infectious disease: 61.9%; hematological disease: 57.1%). Furthermore, 7 of the remaining 14 DIC patients (who did not attain resolution at 7 days) attained resolution at an average of 12.1 days. Consequently, 28 (80.0%) of the 35 patients were alive with resolution of DIC after a 28-day observation period (infectious disease: 76.2%; hematological disease: 85.7%). Among them, for 7 (70%) of the 10 DIC patients with severe life-threatening bleeding symptoms without hemorrhagic shock, treatment with heparin was contraindicated; these patients were successfully treated with rTM without the progression of hemorrhage. In the majority of DIC patients, rTM administration may be an effective, safe, and feasible therapeutic modality producing a good outcome.


PubMed | Miyazaki Prefectural Miyazaki Hospital
Type: Journal Article | Journal: Journal of clinical and experimental hematopathology : JCEH | Year: 2016

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a serious disorder in which monoclonal growth of T cells infected with EBV and macrophage activation cause pancytopenia, high fever and acute liver failure. Patients with chemotherapy- or immunosuppression-resistant EBV-HLH require allogeneic hematopoietic stem cell transplantation (allo-HSCT), but patients who have no sibling donors may not have time to wait for an unrelated donor. In pediatric patients, there are some reports on allogeneic cord blood transplantation (allo-CBT) for the treatment of EBV-HLH; however, in adult patients, reports of allo-CBT for EBV-HLH are quite limited. The present case of a 20-year-old woman with chemotherapy-resistant EBV-HLH and systemic lupus erythematosus (SLE) who underwent allo-CBT following reduced-intensity conditioning (RIC-CBT). She achieved and maintained a complete donor type and the EBV-DNA load, and the titers of anti-double stranded DNA and antinuclear antibodies became negative. It is therefore considered that RIC-CBT is an effective treatment option for adult onset HLH. However, because the effectiveness of allo-HSCT for SLE remains unclear and transplant-related mortality is high, it is recommended that allo-HSCT for SLE is restricted to patients concomitant with oncohematological disease as with our present case.


PubMed | Miyazaki Prefectural Miyazaki Hospital
Type: Journal Article | Journal: International journal of hematology | Year: 2016

Primary bone lymphomas comprise <1% of all malignant lymphomas, and there have been only a limited number of reports on primary adult T-cell leukemia/lymphoma (ATLL) of bone. Here, we report two cases of primary ATLL of bone. The first case was a 41-year-old woman with multiple bone tumors. She was diagnosed with ATLL of the skull through biopsy and treated with chemotherapy. Although the bone lesions showed transient improvement, the subsequent central nervous system (CNS) invasion of ATLL occurred and she died 7months after diagnosis. The second case was a 65-year-old man with right coxodynia. He was diagnosed with ATLL through right femoral biopsy, and the lesion improved with chemotherapy. However, CNS invasion of ATLL developed during chemotherapy, and he died 10months after diagnosis. Both the patients with primary ATLL of the bone reported here experienced CNS invasion. Thus, ATLL treatment should aim to prevent CNS invasion at an early stage.

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