Publications by authors named "Hideo Harigae"

274 Publications

Multi-targeted therapy for refractory eosinophilic granulomatosis with polyangiitis characterized by intracerebral hemorrhage and cardiomyopathy: a case-based review.

Rheumatol Int 2021 Jul 21. Epub 2021 Jul 21.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic autoimmune disorder classified under anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, predominantly affecting small- to medium-sized vessels, characterized by asthma, eosinophilia, and necrotizing granulomatous inflammation. Most patients with EGPA experience peripheral neuropathy, whereas intracerebral hemorrhage is rare as EGPA-related presentation in central nervous system involvement, causing severe morbidity and mortality. Here, we present a 45-year-old man with refractory EGPA who developed intracerebral hemorrhage as the first manifestation, followed by cardiac involvement. This patient with a history of bronchial asthma developed a right putaminal hemorrhage caused by EGPA. Although intravenous cyclophosphamide (IVCY) and mepolizumab (MPZ) induced remission, relapse was frequently observed. Subsequently, he developed cardiomyopathy despite administration of rituximab (RTX) substituted from IVCY and MPZ. Combined immunosuppressive therapy, including IVCY, MPZ, and RTX was required to inhibit vascular inflammation, leading to sustained remission. We review previously published literature while focusing on the clinical features of patients with intracerebral hemorrhage caused by EGPA and describe clinical characteristics for detecting EGPA in patients with intracerebral hemorrhage, emphasizing rapid evaluation and recognition of EGPA and adequate intervention in the early vasculitic phase of this disease. We also refer to the immunological aspects of this case. It is important to consider "multi-targeted therapy" through interleukin-5 suppression and B cell depletion in the management of refractory EGPA.
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http://dx.doi.org/10.1007/s00296-021-04950-zDOI Listing
July 2021

Dental care using an oral appliance to support hematopoietic stem cell transplantation for NK/T cell lymphoma, nasal type, with palatal perforation.

J Prosthodont Res 2021 Jul 15. Epub 2021 Jul 15.

Division of Advanced Prosthetic Dentistry, Tohoku University Graduate School of Dentistry, Sendai.

Patient: A 33-year-old man diagnosed with extranodal natural killer cell/T-cell lymphoma, nasal type (ENKTCL-NT) inducing palatal perforation was referred to the perioperative oral care support center of Tohoku University Hospital for dental care to support cancer treatment including chemotherapy and hematopoietic stem cell transplantation (HSCT). Dental review during chemotherapy revealed mucositis suspected to be caused by mucosal trauma from altered jaw function (chewing and speech) due to palatal perforation. Although the patient was already in the cleanroom, an oral appliance as well as conservative care as recommended in oral management guidelines for HSCT were used to prevent worsening of oral mucositis at subsequent HSCT including High-dose chemotherapy and total body irradiation. After HSCT, a prosthodontist fitted a palatal obturator made by a dental technician and an oral surgeon reviewed the necrotic bone and removed the sequestra according to the changes in the palate. This approach involving a multidisciplinary team including a hematologist improved the impaired oral function and minimized oral complications .

Discussion: ENKTCL-NT and its treatment have a significant impact on patients' oral status. Hence, it is important to provide customized dental care based on previously endorsed guidelines according to the type of disease, treatment requirements, and oral and systemic status.

Conclusions: This report indicated the importance of dental care with a customized plan before, during, and after HSCT for ENKTCL-NT with multidisciplinary supportive care for cancer patients to improve the impaired oral function and to minimize oral complications.
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http://dx.doi.org/10.2186/jpr.JPR_D_20_00270DOI Listing
July 2021

Cyclophosphamide-associated enteritis presenting with severe protein-losing enteropathy in granulomatosis with polyangiitis: A case report.

World J Gastroenterol 2021 May;27(20):2657-2663

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai 9808574, Japan.

Background: Although cyclophosphamide (CPA) is the key drug for the treatment of autoimmune diseases including vasculitides, it has some well-known adverse effects, such as myelosuppression, hemorrhagic cystitis, infertility, and infection. However, CPA-associated severe enteritis is a rare adverse effect, and only one case with a lethal clinical course has been reported. Therefore, the appropriate management of patients with CPA-associated severe enteritis is unclear.

Case Summary: We present the case of a 61-year-old woman diagnosed with granulomatosis with polyangiitis based on the presence of symptoms in ear, lung, and, kidney with positive myeloperoxidase-antineutrophil cytoplasmic antibody. She received pulsed methylprednisolone followed by prednisolone 55 mg/d and intravenous CPA at a dose of 500 mg/mo. Ten days after the second course of intravenous CPA, she developed nausea, vomiting, and diarrhea, and was admitted to the hospital. Laboratory testing revealed hypoalbuminemia, suggesting protein-losing enteropathy. Computed tomography revealed wall thickening of the stomach, small intestine, and colon with contrast enhancement on the lumen side. Antibiotics and immunosuppressive therapy were not effective, and the patient's enteritis did not improve for > 4 mo. Because her condition became seriously exhausted, corticosteroids were tapered and supportive therapies including intravenous hyperalimentation, replenishment of albumin and gamma globulin, plasma exchange, and infection control were continued. These supportive therapies improved her condition, and her enteritis gradually regressed. She was finally discharged 7 mo later.

Conclusion: Immediate discontinuation of CPA and intensive supportive therapy are crucial for the survival of patients with CPA-associated severe enteritis.
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http://dx.doi.org/10.3748/wjg.v27.i20.2657DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8160622PMC
May 2021

Retrospective study revealed that Zn relate to improvement of swallowing function in the older adults.

BMC Geriatr 2021 04 26;21(1):279. Epub 2021 Apr 26.

Department of Education and Support for Regional Medicine, Tohoku University Hospital, 1-1, Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Background: Zinc is an essential micronutrient for maintaining biological activity. The level of zinc in the blood is known to decrease with age, especially in those over 75 years of age. In older adults patients with impaired functional status, aspiration pneumonia based on dysphagia often becomes problematic. However, the relationship between zinc deficiency and swallowing function has not been studied before.

Methods: A total of 52 older adults subjects (15 males and 37 females) living in a nursing home were enrolled for this study. At the time of enrollment, data of gender, age, body weight, serum zinc levels, serum albumin levels, and the time in a simple 2-step swallowing provocation test (S-SPT) were collected. In patients with serum zinc levels < 60 μg/dL, we initiated 2 months of oral zinc supplementation therapy with a 34 mg/day zinc load. Those who underwent zinc supplementation were re-evaluated after the treatment period and serum zinc levels and S-SPT time were measured.

Results: At the time of enrollment, serum zinc level was significantly correlated with serum albumin levels (Pearson's R = 0.58, p < 0.0001) and time in the S-SPT (Spearman's rho = - 0.32, p = 0.0219). Twenty-five of the 52 patients had zinc deficiency with a serum zinc level < 60 μg/dL. After 2 months of oral zinc supplementation, both serum zinc levels (p < 0.0001) and time in the S-SPT (p = 0.04) significantly improved. Meanwhile, serum albumin level (p = 0.48) or body weight (p = 0.07) did not significantly change following zinc supplementation. Zinc supplementation significantly improved swallowing function, especially in the older adults who had comorbid dysphagia and zinc deficiency.

Conclusions: Zinc deficiency is associated with compromised swallowing function in older adults patients with impaired general functions. Oral zinc supplementation can alleviate dysphagia in older adults patients with zinc deficiency even though this is a retrospective study. Further study will be needed to confirm this positive effect.
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http://dx.doi.org/10.1186/s12877-021-02224-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8075009PMC
April 2021

Disseminated gonococcal infection in a patient with paroxysmal nocturnal haemoglobinuria receiving eculizumab.

Lancet Infect Dis 2021 05;21(5):741

Department of Infectious Diseases, Internal Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan; Department of Intelligent Network for Infection Control, Tohoku University Graduate School of Medicine, Sendai, Japan; Division of Infection Control, Tohoku University Hospital, Sendai, Japan.

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http://dx.doi.org/10.1016/S1473-3099(20)30930-0DOI Listing
May 2021

Association of various myositis-specific autoantibodies with dermatomyositis and polymyositis triggered by pregnancy.

Rheumatol Int 2021 Apr 10. Epub 2021 Apr 10.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Although pregnancy is an important risk factor for autoimmune rheumatic diseases, little is known regarding the association between pregnancy and dermatomyositis (DM) or polymyositis (PM). Herein, we present two patients with DM that developed during the perinatal period. The first patient was positive for anti-aminoacyl synthetase (ARS) antibody and developed DM in the 14th week of pregnancy. Despite treatment, her foetus died of intrauterine growth restriction in the 27th week. The second patient was positive for anti-melanoma differentiation-associated gene 5 (MDA-5) antibody and developed DM 1 week after miscarriage at 9 weeks of gestation. The patient developed severe interstitial pneumonia, and intensive therapy including tofacitinib and rituximab administration was required. Our cases and a literature review revealed that various myositis-specific autoantibodies, including anti-ARS, anti-Mi-2, anti-TIF-1γ, and anti-MDA-5, are associated with DM and PM triggered by pregnancy. We also found that delay in commencing treatment in case of active disease including myositis and interstitial pneumonia, and poor response to corticosteroids were related to poor foetal outcomes in DM and PM. Although rare in pregnant women, it is critical to consider the possibility of DM and PM in patients presenting with rash, fever, weakness, and cough, and testing for myositis-specific autoantibodies is recommended.
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http://dx.doi.org/10.1007/s00296-021-04851-1DOI Listing
April 2021

Targeting stanniocalcin-1-expressing tumor cells elicits efficient antitumor effects in a mouse model of human lung cancer.

Cancer Med 2021 05 7;10(9):3085-3100. Epub 2021 Apr 7.

Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.

Lung cancer is the most common cause of cancer-related death in developed countries; therefore, the generation of effective targeted therapeutic regimens is essential. Recently, gene therapy approaches toward malignant cells have emerged as attractive molecular therapeutics. Previous studies have indicated that stanniocalcin-1 (STC-1), a hormone involved in calcium and phosphate homeostasis, positively regulates proliferation, apoptosis resistance, and glucose metabolism in lung cancer cell lines. In this study, we investigated if targeting STC-1 in tumor cells could be a promising strategy for lung cancer gene therapy. We confirmed that STC-1 levels in peripheral blood were higher in lung cancer patients than in healthy donors and that STC-1 expression was observed in five out of eight lung cancer cell lines. A vector expressing a suicide gene, uracil phosphoribosyltransferase (UPRT), under the control of the STC-1 promoter, was constructed (pP -UPRT) and transfected into three STC-1-positive cell lines, PC-9, A549, and H1299. When stably transfected, we observed significant cell growth inhibition using 5-fluorouracil (5-FU) treatment. Furthermore, growth of the STC-1-negative lung cancer cell line, LK-2 was significantly arrested when combined with STC-1-positive cells transfected with pP -UPRT. We believe that conferring cytotoxicity in STC-1-positive lung cancer cells using a suicide gene may be a useful therapeutic strategy for lung cancer.
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http://dx.doi.org/10.1002/cam4.3852DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8085941PMC
May 2021

Salvage Cord Blood Transplantation for Sustained Remission of Acute Megakaryoblastic Leukemia That Relapsed Early after Myeloablative Transplantation.

Intern Med 2021 Apr 5. Epub 2021 Apr 5.

Department of Hematology, Tohoku University Hospital, Japan.

Acute megakaryoblastic leukemia (AMKL) is a rare subtype of acute myeloid leukemia accompanied by an aggressive clinical course and dismal prognosis. We herein report a case of AMKL preceded by mediastinal germ cell tumor that relapsed early after allogeneic hematopoietic stem cell transplantation with myeloablative conditioning but was successfully treated using salvage cord blood transplantation (CBT) with reduced-intensity conditioning. Although several serious complications developed, sustained remission with a favorable general condition was ultimately achieved. Although an optimal therapeutic strategy remains to be established, the graft-versus-leukemia effect of CBT may be promising, even for the treatment of refractory AMKL.
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http://dx.doi.org/10.2169/internalmedicine.6796-20DOI Listing
April 2021

Hepatitis B Virus Reactivation with Discontinuation of Nucleoside Analogue in Patients Who Received Allogeneic Hematopoietic Stem Cell Transplantation.

Case Rep Gastroenterol 2021 Jan-Apr;15(1):178-187. Epub 2021 Feb 12.

Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan.

Reactivation of hepatitis B virus (HBV) is known to occur frequently after hematopoietic stem cell transplantation (HSCT). The reactivation can be prevented by nucleos(t)ide analogue (NA), but it is unclear how long NA should be continued. Here, we report 3 cases of HBV reactivation with discontinuation of NA following the discontinuation of immunosuppressive therapies after HSCT. Three male patients aged 34, 59, and 54 years received allogeneic HSCT (allo-HSCT) for chronic myeloid leukemia, mixed phenotype acute leukemia, and myelodysplastic syndrome, respectively. Before HSCT, 2 patients were positive for hepatitis B surface antigen (HBsAg) and 1 patient was negative for HBsAg and positive for antibodies to hepatitis B core antigen. NA (lamivudine or entecavir) was started at the same time as HSCT and stopped after the discontinuation of immunosuppressive therapies. In all patients, the serum HBV DNA levels were increased after the discontinuation of NAs. Two of the three patients developed severe hepatitis with high levels of HBV DNA (7.5 and 7.4 log IU/mL, respectively). A patient without hepatitis was re-administered NA soon after the HBV DNA started to increase (3.3 log IU/mL). Interestingly, the 2 patients who developed hepatitis cleared HBsAg promptly after the recovery from hepatitis and they could stop NAs without the reversion of HBsAg. It was speculated that transplanted immune cells, which were naïve for HBV, react strongly with HBV antigens that were increased after the NA discontinuation. The discontinuation of NA after allo-HSCT is not recommended generally because strong hepatitis might be induced even after several years.
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http://dx.doi.org/10.1159/000512397DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7923699PMC
February 2021

The Potential of Computed Tomography Volumetry for the Surgical Treatment in Bilateral Macronodular Adrenal Hyperplasia: A Case Report.

Tohoku J Exp Med 2021 02;253(2):143-150

Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Hospital.

Although adrenal resection is a major option to control hypercortisolemia in patients with bilateral macronodular adrenal hyperplasia, a predictive method for postoperative cortisol production has not been established. A 53-year-old man with ulcerative colitis was referred to our hospital for bilateral multiple adrenal nodules and hypertension. Physical and endocrinological examination revealed inappropriate cortisol production and suppressed secretion of adrenocorticotropic hormone with no typical signs of Cushing's syndrome. Imaging analysis revealed bilateral adrenal nodular enlargement, the nodules of which had the radiological features of adrenocortical adenomas without inter-nodular heterogeneity. In addition, computed tomography volumetry demonstrated that the left adrenal gland (70 mL) accounts for three quarters of the total adrenal volume (93 mL). The patient was diagnosed as subclinical Cushing's syndrome due to bilateral macronodular adrenal hyperplasia, and subsequently underwent a left laparoscopic adrenalectomy with the estimation of 75% decrease in the cortisol level based on the adrenal volume. The surgical treatment ultimately resulted in control of the cortisol level within the normal range, which was compatible to our preoperative prediction. However, regardless of the sufficient cortisol level, ulcerative colitis was exacerbated after the surgery, which needed a systemic therapy for remission. This case indicates successful surgical control of hypercortisolemia based on computed tomography volumetry in bilateral macronodular adrenal hyperplasia, as well as the perioperative exacerbation risk for inflammatory diseases in Cushing's syndrome. We report the potential utility of computed tomography volumetry as a quantitative method with retrospective evaluation of our historical cases.
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http://dx.doi.org/10.1620/tjem.253.143DOI Listing
February 2021

Interferon Enhances B Cell Activation Associated With FOXM1 Induction: Potential Novel Therapeutic Strategy for Targeting the Plasmablasts of Systemic Lupus Erythematosus.

Front Immunol 2020 3;11:498703. Epub 2021 Feb 3.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

Systemic lupus erythematosus (SLE) is an autoimmune disease. It is characterized by the production of various pathogenic autoantibodies and is suggested to be triggered by increased type I interferon (IFN) signature. Previous studies have identified increased plasmablasts in the peripheral blood of SLE patients. The biological characteristics of SLE plasmablasts remain unknown, and few treatments that target SLE plasmablasts have been applied despite the unique cellular properties of plasmablasts compared with other B cell subsets and plasma cells. We conducted microarray analysis of naïve and memory B cells and plasmablasts (CD38CD43 B cells) that were freshly isolated from healthy controls and active SLE (n = 4, each) to clarify the unique biological properties of SLE plasmablasts. The results revealed that all B cell subsets of SLE expressed more type I IFN-stimulated genes. In addition, SLE plasmablasts upregulated the expression of cell cycle-related genes associated with higher FOXM1 and FOXM1-regulated gene expression levels than that in healthy controls. This suggests that a causative relationship exists between type I IFN priming and enhanced proliferative capacity through FOXM1. The effects of pretreatment of IFN on B cell activation and FOXM1 inhibitor FDI-6 on B cell proliferation and survival were investigated. Pretreatment with IFN promoted B cell activation after stimulation with anti-IgG/IgM antibody. Flow cytometry revealed that pretreatment with IFN preferentially enhanced the Atk and p38 pathways after triggering B cell receptors. FDI-6 inhibited cell division and induced apoptosis in activated B cells. These effects were pronounced in activated B cells pretreated with interferon . This study can provide better understanding of the pathogenic mechanism of interferon-stimulated genes on SLE B cells and an insight into the development of novel therapeutic strategies.
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http://dx.doi.org/10.3389/fimmu.2020.498703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7902015PMC
April 2021

Second direct-acting antiviral therapy for hepatitis C virus infection after umbilical cord blood transplantation: A case report.

J Infect Chemother 2021 Aug 12;27(8):1230-1233. Epub 2021 Feb 12.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

Hepatitis C virus (HCV) infection has an adverse impact on outcomes after allogeneic hematopoietic stem cell transplantation (HSCT). It is recommended that HSCT candidates infected with HCV receive the treatment prior to transplantation. Although the recent approval of direct-acting antivirals (DAAs) has led to great advances in the treatment of HCV infection, little information is available on the efficacy and safety of DAA therapy in patients receiving allogeneic HSCT. Herein, we report the clinical course of an umbilical cord blood (UCB) recipient treated with DAAs for HCV infection. The patient achieved HCV RNA negativity with glecaprevir and pibrentasvir after consolidation therapy for acute myeloid leukemia (AML), and underwent transplantation before confirming sustained virological response (SVR) at 12 weeks. The HCV viral load became detectable on day +28 after transplantation and second HCV treatment with sofosbuvir, velpatasvir, and ribavirin was required. It is important to confirm SVR prior to transplantation, but it is often difficult. If early transplantation is required, close monitoring of HCV RNA after transplantation is needed. Further investigation is required to clarify the optimal management of HCV infection for allogeneic HSCT recipients in the DAA era.
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http://dx.doi.org/10.1016/j.jiac.2021.02.002DOI Listing
August 2021

Nicotinamide Attenuates the Progression of Renal Failure in a Mouse Model of Adenine-Induced Chronic Kidney Disease.

Toxins (Basel) 2021 01 11;13(1). Epub 2021 Jan 11.

Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.

Nicotinamide adenine dinucleotide (NAD) supplies energy for deoxidation and anti-inflammatory reactions fostering the production of adenosine triphosphate (ATP). The kidney is an essential regulator of body fluids through the excretion of numerous metabolites. Chronic kidney disease (CKD) leads to the accumulation of uremic toxins, which induces chronic inflammation. In this study, the role of NAD in kidney disease was investigated through the supplementation of nicotinamide (Nam), a precursor of NAD, to an adenine-induced CKD mouse model. Nam supplementation reduced kidney inflammation and fibrosis and, therefore, prevented the progression of kidney disease. Notably, Nam supplementation also attenuated the accumulation of glycolysis and Krebs cycle metabolites that occurs in renal failure. These effects were due to increased NAD supply, which accelerated NAD-consuming metabolic pathways. Our study suggests that Nam administration may be a novel therapeutic approach for CKD prevention.
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http://dx.doi.org/10.3390/toxins13010050DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7827863PMC
January 2021

Acquisition of monosomy 7 and a RUNX1 mutation in Pearson syndrome.

Pediatr Blood Cancer 2021 02 16;68(2):e28799. Epub 2020 Nov 16.

Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan.

Pearson syndrome (PS) is a very rare and often fatal multisystem disease caused by deletions in mitochondrial DNA that result in sideroblastic anemia, vacuolization of marrow precursors, and pancreatic dysfunction. Spontaneous recovery from anemia is often observed within several years of diagnosis. We present the case of a 4-month-old male diagnosed with PS who experienced prolonged severe pancytopenia preceding the emergence of monosomy 7. Whole-exome sequencing identified two somatic mutations, including RUNX1 p.S100F that was previously reported as associated with myeloid malignancies. The molecular defects associated with PS may have the potential to progress to advanced myelodysplastic syndrome .
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http://dx.doi.org/10.1002/pbc.28799DOI Listing
February 2021

JAK2 mutation-positive polycythaemia vera associated with IgA vasculitis and nephrotic syndrome: a case report.

Mod Rheumatol Case Rep 2020 07 13;4(2):289-295. Epub 2020 Feb 13.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

We report a case of polycythaemia vera (PV) associated with IgA vasculitis. A 45-year-old man was admitted for evaluation of abdominal pain and palpable purpura. IgA vasculitis was diagnosed, and oral prednisolone therapy (30 mg/day) was initiated. On day 6, the patient developed left hemiparesis, and magnetic resonance imaging revealed acute cerebral infarction. Bone marrow biopsy results and the identification of a Janus kinase 2 () mutation led to the diagnosis of PV. Despite steroid therapy, urine protein levels increased to 15 g/g・Cre. Renal biopsy demonstrated mild mesangial proliferation with IgA deposits, but immunosuppressive therapy was partially effective. This case suggests that PV can be a complication of IgA vasculitis and that preventive measures for thrombosis should be taken in such cases.
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http://dx.doi.org/10.1080/24725625.2020.1728061DOI Listing
July 2020

Primary adrenal extranodal NK/T-cell lymphoma: A case report and literature review.

Leuk Res Rep 2020 28;14:100223. Epub 2020 Sep 28.

Department of Hematology, Tohoku University Hospital, Sendai, Japan.

A 37-year-old man was admitted to our department following the detection of bulky tumors in his bilateral adrenal glands. A biopsy resulted in the diagnosis of extranodal NK/T cell lymphoma, nasal type (ENKL). After debulking by chemotherapy, allogeneic hematopoietic stem cell transplantation (alloHCT) was performed. Relapses in the liver and adrenal glands were identified 2 months post alloHCT, for which temporary administration of l-asparaginase resulted in complete metabolic response. However, multiple relapses in the central nervous system and lethal lymphomatous meningitis successively developed. Primary adrenal ENKL could tend to present as bulky lesion and follow an aggressive clinical course.
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http://dx.doi.org/10.1016/j.lrr.2020.100223DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7527573PMC
September 2020

One after another retinal involvement in lupus.

Eur J Rheumatol 2020 Sep 3. Epub 2020 Sep 3.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

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http://dx.doi.org/10.5152/eurjrheum.2020.20022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8133891PMC
September 2020

Low-dose lenalidomide and dexamethasone therapy after melphalan-prednisolone induction in elderly patients with newly diagnosed multiple myeloma.

Ann Hematol 2020 Oct 31;99(10):2351-2356. Epub 2020 Aug 31.

Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-857, Japan.

Lenalidomide (Len) and dexamethasone (dex) therapy is a standard therapy in patients with multiple myeloma. Elderly or unfit patients may reduce Len or dex doses to prevent toxicities that lead to treatment discontinuation. However, there have been few studies evaluating the efficacy and safety of lower doses of Len and dex. We conducted a phase II study of 1.5-year low-dose Len and dex therapy following melphalan and prednisolone (MP), the number of which cycles was determined by a response within 9 cycles. The Len dose was 10 mg daily and the dex dose was 20 mg weekly, which were continued for 1.5 years. Twenty-one patients were enrolled. The median number of cycles of MP was 3 (range, 2-9). The overall response rate was 81% and a very good partial response or better was achieved in 33.3% of patients. The median follow-up time for survivors was 70.5 months (range, 42-83 months), the median progression-free survival (PFS) was 27 months (95% CI, 21-33 months), and the median overall survival was not reached. Grade 3 or 4 adverse events were observed in 28.6% of patients. In conclusion, the low-dose Len and dex therapy safely achieved comparable efficacies to the standard-dose regimen in elderly patients with newly diagnosed multiple myeloma. UMIN000007889.
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http://dx.doi.org/10.1007/s00277-020-04240-xDOI Listing
October 2020

Treatment of Refractory Hypertension with Timely Angioplasty in Total Renal Artery Occlusion with Atrophic Kidney.

Intern Med 2021 Jan 22;60(2):287-292. Epub 2020 Aug 22.

Division of Nephrology, Endocrinology, and Vascular Medicine, Tohoku University Graduate School of Medicine, Japan.

Angioplasty for cases of chronic total occlusion of renal artery with/without atrophic kidney is generally not recommended. We herein report a 57-year-old man who presented with renin-mediated refractory hypertension caused by occlusion of a unilateral renal artery leading to kidney atrophy (length: 69 mm). Angioplasty favorably achieved blood pressure control with normalized renin secretion and enlargement of the atrophic kidney to 85 mm. Timely angioplasty can be beneficial in select patients, even with an atrophic kidney and total occlusion, especially in cases with deterioration of hypertension within six months and the presence of collateral perfusion to the affected kidney.
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http://dx.doi.org/10.2169/internalmedicine.5290-20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7872794PMC
January 2021

Concurrent analogous organ damage in the brain, eyes, and kidneys in malignant hypertension: reversible encephalopathy, serous retinal detachment, and proteinuria.

Hypertens Res 2021 Jan 27;44(1):88-97. Epub 2020 Jul 27.

Division of Nephrology, Endocrinology, and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan.

Malignant hypertension, a form of hypertensive emergency, causes acute damage in vital organs such as the brain, eyes, and kidneys. We aimed to examine the concurrency of acute hypertensive damage across the target organs to elucidate the underlying analogous pathophysiology. This single-center retrospective study evaluated the characteristics of organ damage, short-term clinical course, and interorgan relationships in patients with malignant hypertension treated between 2008 and 2019. Baseline characteristics of 20 patients who met our inclusion criteria were mean age 48 ± 13 years and blood pressure 222 ± 18/142 ± 16 mmHg; the median estimated glomerular filtration rate and urinary protein level were 49 mL/min/1.73 m (interquartile range [IQR] 27-79) and 1.9 g/g creatinine (IQR 0.2-4.0), respectively. Posterior reversible encephalopathy syndrome (PRES) was found in 60% of patients with major involvement and a wide variety of distribution patterns in the brainstem. In the fundus, serous retinal detachment was found in 60% of patients. Patients with PRES and serous retinal detachment showed higher levels of urinary protein than those without symptoms (P = 0.007 and 0.02, respectively), and proteinuria >1 g/g creatinine highly complicated both PRES and serous retinal detachment (91%). Matrix analysis also showed that the three symptoms were highly associated with each other. These results demonstrate the close relationship and concurrency of hypertensive acute organ damage in the brain, eyes, and kidneys. A common analogous mechanism, such as hyperperfusion-induced capillary leakage in each organ, implies an underlying pathophysiology of PRES, serous retinal detachment, and proteinuria.
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http://dx.doi.org/10.1038/s41440-020-0521-2DOI Listing
January 2021

High Relapse Rate in Patients with Polymyalgia Rheumatica despite the Combination of Immunosuppressants and Prednisolone: A Single Center Experience of 89 patients.

Tohoku J Exp Med 2020 06;251(2):125-133

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine.

Polymyalgia rheumatica (PMR) is an inflammatory disorder in the elderly and is characterized by pain in the shoulders and lower back. Previous studies from western countries have shown that relapse is frequent; however, there are only a few reports on the relapse rate in Japan. Here we examined the relapse rate, and sought to identify factors that predict recurrence in patients with PMR. Of 110 patients who fulfilled the Bird's criteria for PMR between May 2011 and June 2019, 21 patients were excluded, and the remaining 89 patients were followed up until July 2019. Relapse was defined when clinical symptoms were exacerbated and serum C-reactive protein level increased. The relapse-free survival curves were plotted using the Kaplan-Meier method, and log-rank test was used for statistical analysis. The mean age of the 89 patients (50 males and 39 females) was 71.8 years. The mean dose of initial prednisolone (PSL) was 11.8 mg/day. The 1-, 3-, and 5-year relapse-free survival rates were 81.6%, 58.0%, and 52.3% (N = 59, 21, and 7), respectively. In patients who experienced recurrence, the 1- and 3-year second relapse-free survival rates were 58.3% and 27.3% (N = 18 and 3), respectively. Immunosuppressants, such as methotrexate and tacrolimus, were added to PSL in 19 of 30 patients who experienced relapse at the discretion of the attending physicians; however, none of the immunosuppressants worked for preventing second relapses and had steroid-sparing effects. These results indicate that effective immunosuppressants are required to suppress relapse in the treatment of PMR.
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http://dx.doi.org/10.1620/tjem.251.125DOI Listing
June 2020

Sustained remission of giant pancreatic plasmacytoma with daratumumab.

Ann Hematol 2020 Jun 18. Epub 2020 Jun 18.

Department of Hematology, Tohoku University Hospital, 1-1 Seiryo-cho, Sendai, 980-8574, Japan.

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http://dx.doi.org/10.1007/s00277-020-04145-9DOI Listing
June 2020

Rheumatoid Arthritis After Cord Blood Cell Transplantation.

J Clin Rheumatol 2020 Jun 12. Epub 2020 Jun 12.

From the Departments of Hematology and Rheumatology.

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http://dx.doi.org/10.1097/RHU.0000000000001430DOI Listing
June 2020

Extranasal extranodal NK/T-cell lymphoma associated with systemic lupus erythematosus.

Int J Hematol 2020 Oct 8;112(4):592-596. Epub 2020 Jun 8.

Department of Hematology, Tohoku University Hospital, 1-1 Seiryo-cho, Sendai, 980-8574, Japan.

Increased incidence of lymphoproliferative disorders is reported in patients with autoimmune diseases, majority of which have a B-cell phenotype and are pathogenetically associated with the reactivation of Epstein-Barr virus (EBV). However, EBV-associated T/NK-cell lymphoma has hardly been reported. We present the case of a 68-year-old-woman, who had been diagnosed with systemic lupus erythematosus (SLE) 28 years back and was treated with various immunosuppressive agents including steroids, cyclophosphamide, and tacrolimus. She presented with a progressively worsening swelling of the right thigh for the last few months. Radiological examination revealed an intramuscular bulky tumor without any other lesions and the biopsy results led to a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKL). Concurrent chemoradiotherapy resulted in a complete response, which has been sustained for more than 2 years without requiring additional therapy. After the initiation of chemotherapy, SLE did not worsen with the administration of low-dose corticosteroids. To the best of our knowledge, this is the first case report of a localized extranasal ENKL developing in a patient with SLE.
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http://dx.doi.org/10.1007/s12185-020-02914-wDOI Listing
October 2020

Questionnaire survey on the prescription of renal replacement therapy for acute phase patients on maintenance dialysis who developed cerebrovascular disease.

Clin Exp Nephrol 2020 Sep 25;24(9):821-828. Epub 2020 May 25.

Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan.

Background: There is limited information about acute phase renal replacement therapy (RRT) for maintenance hemodialysis patients after the onset of cerebrovascular disease. This study aimed to investigate which modality of renal replacement therapy is currently selected in practice.

Methods: We conducted a mail-based survey in 317 dialysis facilities that were certified by three academic societies that focus on dialysis, neurology, and neurosurgery in Japan.

Results: We received responses from 103 facilities (32.5%). In cases of cerebral infarction (CI) and intracerebral hemorrhage (ICH), more than 80% of the facilities selected only intermittent RRT, and 22.3% (CI)/8.7% (ICH) of the facilities selected intermittent HD which is the same setting in normal conditions. Although continuous hemodiafiltration and peritoneal dialysis are recommended in the Japanese guidelines, these were selected in only a few facilities: 16.5% and 0% in CI, 16.5% and 1% in ICH, respectively. RRT on the day of onset tended to be avoided, irrespective of the duration following the last HD session. Furthermore, physicians preferred to modify anticoagulants and reduce dialysis performance in the acute phase.

Conclusion: This questionnaire survey uncovered a gap between guidelines and actual practice, even in hospitals accredited as educational facility, which is a novel and important finding. Further studies with larger sample sizes are needed to determine the optimal modality of RRT for the acute phase of cerebrovascular disease.
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http://dx.doi.org/10.1007/s10157-020-01905-9DOI Listing
September 2020

Kidney enlargement effect of angioplasty for nonatherosclerotic renovascular disease: reversibility of ischemic kidney.

Hypertens Res 2020 11 22;43(11):1214-1221. Epub 2020 May 22.

Division of Nephrology, Endocrinology, and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan.

Renal artery stenosis causes kidney ischemia, reducing the size of the affected kidney, which eventually results in atrophy. Although renal atrophy is considered irreversible, resolution of the ischemia occasionally restores kidney size when the cause is renal artery stenosis. Angioplasty is effective in patients with nonatherosclerotic renovascular diseases (non-ARVDs). Nevertheless, renal enlargement after angioplasty has not been fully examined. We conducted a retrospective study to examine this phenomenon in non-ARVD patients. Ten patients with a <100-mm pole-to-pole length of the poststenotic kidney were treated with angioplasty. Data were collected up to 12 months after angioplasty. The mean age was 28 years; the estimated glomerular filtration rate was 92 ± 7 mL/min/1.73 m (mean ± SEM); blood pressure was 150/99 mmHg; 80% were women; and fibromuscular dysplasia was present in 90% of the patients. All patients had hypertension. The lengths of the poststenotic and contralateral kidney before angioplasty were 91 ± 1 and 111 ± 3 mm, respectively. After angioplasty, the length of the poststenotic kidney gradually increased during the 3 months after treatment (+5.4 mm) and that of the contralateral kidney decreased over the same time course (-3.7 mm). Enlargement was also found in the moderate atrophy subgroup (length < 92 mm), and it was greater in the <30 years old group. In a noteworthy case, renal size in the poststenotic kidney recovered from 87 to 102 mm after angioplasty. Our findings demonstrated that reduced renal size can be reversed after optimal angioplasty in non-ARVD patients, especially young patients, suggesting reversibility of the ischemic kidney.
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http://dx.doi.org/10.1038/s41440-020-0473-6DOI Listing
November 2020

Migratory Aortitis Associated with Granulocyte-colony-stimulating Factor.

Intern Med 2020 Jun 19;59(12):1559-1563. Epub 2020 Mar 19.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Japan.

We herein report a case of migratory aortitis after the administration of granulocyte-colony-stimulating factor (G-CSF) to a 65-year-old woman with a history of pancreatic cancer. She was being administered pegfilgrastim and developed aortitis around the aortic arch. Although it resolved within two weeks, she again developed aortitis around the descending aorta, presenting as migratory aortitis, after pegfilgrastim was resumed. We further experienced three additional cases of G-CSF-induced aortitis that also showed spontaneous resolution, suggesting no or short-term use of immunosuppression. Aortitis due to G-CSF can present as migratory aortitis, since aortitis can quickly resolve and inflammation can recur at a different location.
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http://dx.doi.org/10.2169/internalmedicine.4331-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7364257PMC
June 2020

Kidney function, blood pressure and proteinuria were associated with pregnancy outcomes of pregnant women with chronic kidney disease: a single-center, retrospective study in the Asian population.

Clin Exp Nephrol 2020 Jun 11;24(6):547-556. Epub 2020 Mar 11.

Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, 2-1 Seiryo-Machi, Aoba-ku, Sendai, Miyagi, 980-8575, Japan.

Background: Studies among pregnant Asian women with chronic kidney disease (CKD) have not been widely performed; therefore, clinical criteria for these patients have not been well established.

Methods: We conducted a retrospective study among pregnant women with CKD who received prenatal care at our institution for 8 consecutive years. Primary outcome was the development of severe adverse events (SAEs). We analyzed correlations between primary outcome and CKD parameters [age, body mass index (BMI), estimated glomerular filtration rate (eGFR), urinary protein-creatinine ratio (UP), systolic blood pressure (SBP), diastolic blood pressure (DBP), and not normal blood pressure (non-NBP)] at the time of referral. Secondary outcomes were low birth weight (LBW), preterm delivery (PreD), and small for gestational age (SGA). We divided into two categories, CKD stage G1, and G2 or higher according to eGFR, and proteinuria negative and proteinuria positive according to UP, respectively.

Results: We observed 89 pregnancies. SAE was observed in 28 pregnancies. In live birth cases, there were 28 PreD, 28 LBW and 13 SGA. Major SAEs included preeclampsia, superimposed preeclampsia, unscheduled cesarean section, neonatal intensive care unit admission, and fetal death. Stepwise logistic regression analysis selected eGFR (OR = 0.847, p = 0.026), SBP (OR = 1.897, p = 0.006) and proteinuria positive (OR = 2.96, p = 0.046) as the significant predictors of SAEs. There were no significant differences among the baseline characteristics stratified by SGA.

Conclusions: This is the first study to report pregnancy outcomes among Japanese non-disease-oriented patients with CKD. In Asians, especially in the Japanese population, kidney function, blood pressure and proteinuria might affect pregnancy outcomes.
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http://dx.doi.org/10.1007/s10157-020-01865-0DOI Listing
June 2020
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