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Volume 54, Issue 3
Displaying 1-12 of 12 articles from this issue
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Picture in Clinical Hematology
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[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2013 Volume 54 Issue 3 Pages 261
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSDownload PDF (1504K)
Clinical Study
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Naoko MAEDA, Takashi HAMAJIMA, Yuko YAMBE, Masahiro SEKIMIZU, Keizo HO ...2013 Volume 54 Issue 3 Pages 263-268
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSWe performed a physical examination and ultrasonography of the thyroid gland in 24 patients who had received hematopoietic stem cell transplantation with a total-body irradiation (TBI)-containing regimen during childhood. When ultrasonography revealed thyroid nodules larger than 1 cm in diameter, fine-needle aspiration biopsies were performed. Of 5 patients with palpable masses and thyroid nodules larger than 1 cm, adenomatous goiter was diagnosed in 4 cases and thyroid cancer in 1. Of the remaining 19 patients in whom no palpable mass was detected in the physical examination, 5 had thyroid nodules (including 1 adenomatous goiter), 6 had cystic lesions, and 8 exhibited no abnormalities on ultrasonography. No significant differences in sex, age at transplantation, interval between transplantation and evaluation, primary disease, preconditioning regimen, status at transplantation, stem cell source, chronic graft-versus-host disease, hypogonadism, or hypothyroidism were observed between patients with and without nodules. Individuals who received hematopoietic stem cell transplantation with a TBI-containing regimen are at risk of secondary thyroid cancer due to radiotherapy and require regular clinical evaluations of the thyroid gland by palpation, and ultrasonography should be incorporated into these checkups.View full abstractDownload PDF (1141K)
Case Reports
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Minoru MIZUTANI, Yoshiki NAKAMORI, Haruna SAKAGUCHI, Yuki KAGEYAMA, Ei ...2013 Volume 54 Issue 3 Pages 269-272
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSA 61-year-old woman presented with a right mandibular tumor and was diagnosed with DLBCL clinical stage IIIA from the biopsy results of the tumor and CT examination. An initial rituximab was administrated a week after the first CHOP treatment. During the infusion of rituximab, she exhibited disorientation, seizure, and consciousness disturbance. Hyponatremia due to SIADH and hypertension were coincidentally observed. MRI revealed T2 and FLAIR hyperintense signals involving the bilateral occipital, parietal, frontal lobes and the cerebellum that were consistent with reversible posterior leukoencephalopathy syndrome (RPLS). Her consciousness level recovered in parallel with corrections in serum sodium levels and blood pressure. Although she presented with transient cortical blindness, all neurological abnormalities disappeared 40 hours after the occurrence of seizure. She received a further 7 cycles of CHOP followed by 7 cycles of rituximab treatment with no relapse of RPLS. After irradiation for a residual abdominal tumor, she has maintained complete remission for 2 years. Although RPLS is a rare complication of rituximab-CHOP chemotherapy, it should be considered in patients with DLBCL who present with acute neurological deterioration.View full abstractDownload PDF (822K) -
Daisuke SAWA, Takuro NISHIKAWA, Kentaro NAKASHIMA, Hiromi MORITA, Nobu ...2013 Volume 54 Issue 3 Pages 273-278
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSThis report describes two infants with recurrent pulmonary edema after umbilical cord blood transplantation (CBT). A 3-month-old boy and a 7-month-old boy with infant acute lymphoblastic leukemia underwent CBT from an unrelated donor in the first complete remission. The conditioning regimen consisted of busulfan, etoposide, and cyclophosphamide. Tacrolimus and short-term methotrexate were administered for the prophylaxis of acute graft-versus-host disease (GVHD). Neutrophil engraftment was achieved on days 17 and 19, respectively. Neither infant developed acute GVHD. They both exhibited tachypnea and weight gain on days 25 and 30, respectively, which were diagnosed as pulmonary edema by chest X rays. The respiratory condition of the patients improved within a few days with the close monitoring of weight changes after the administration of diuretics. However, they suddenly developed dyspnea and pulmonary edema again on days 37 and 59, respectively. Steroid therapy was initiated for both patients. Their respiratory condition again improved quickly after the initiation of steroid therapy. Their symptoms and clinical courses may be classified as a new entity of idiopathic pneumonia syndrome (IPS). Therefore, these cases may represent a new unclassifiable IPS associated with either CBT or infants.View full abstractDownload PDF (1349K) -
Tomoko NARITA, Satoshi KAYUKAWA, Takashi YOSHIDA, Shigeru KUSUMOTO, Ta ...2013 Volume 54 Issue 3 Pages 279-283
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSA 45-year-old man with chronic myelogenous leukemia (CML) in the chronic phase was treated with imatinib mesylate (IM). Although partial cytogenetic response (CyR) was obtained in 3 months, the patient exhibited back pain after treatment with IM for 5 months. He was diagnosed with myeloblastic crisis of CML with 30% blasts in the bone marrow. An extramedullary tumor with a diameter of 5-cm was found adjacent to the pancreatic head. Mutation analysis of the bcr/abl chimeric gene was negative. After the treatment with dasatinib (140 mg/day) for 40 days, complete CyR was obtained by bone marrow examination and the extramedullary tumor shrunk resulting in partial response on computed tomography. Allogeneic peripheral blood stem cell transplantation (allo-PBSCT) was performed from his HLA-DR one locus-mismatched sister. He has been in molecular remission for 24 months after allo-PBSCT and maintenance therapy with dasatinib has been administered. Dasatinib was tolerable without severe adverse events before and after allo-PBSCT in this case.View full abstractDownload PDF (1431K) -
Sachie WADA, Koichi KITAZUME, Takayuki SUZUKI, Akira FUJITA, Seiichiro ...2013 Volume 54 Issue 3 Pages 284-289
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSA 65-year-old man was diagnosed with leukocytosis in a routine medical examination. Further laboratory examinations showed increased LDH and sIL-2R levels in the serum. There was no evidence of infiltrative lesions or organomegaly. Bone marrow aspiration revealed many atypical small-sized lymphocytes without apparent nucleoli. Flow cytometric analysis of atypical lymphocytes was positive for T-cell markers, and chromosome analysis showed a normal karyotype. He was diagnosed with the small cell variant of T-PLL. Approximately 34 months later, having received no treatment, his cervical lymph nodes increased in size and number, and his white blood cell count, LDH and sIL-2R levels also rapidly increased. He was then admitted to our hospital. Bone marrow aspiration and cervical lymph node biopsy revealed complex chromosome abnormalities including inv(14)(q11;q32). Computed tomography showed swollen lymph nodes all over his body and hepatosplenomegaly. On the fourth hospital day, spontaneous splenic rupture occurred. Transcatheter arterial embolization was unsuccessful and the patient died. We report this case with rare autopsy findings.View full abstractDownload PDF (3140K) -
Ichiro KAWASHIMA, Yuki SHOBU, Takeo YAMAMOTO, Satoshi HAMANAKA, Yumi N ...2013 Volume 54 Issue 3 Pages 290-294
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSRearrangements of the mixed lineage leukemia MLL gene at chromosome 11q23 are common chromosomal abnormalities in human leukemia. MLL fused with numerous partner genes causes different leukemia phenotypes that depend on the function of partner genes. MLLT3-MLL is generated by translocation t(9;11), which primarily induces acute myeloid leukemia in humans, whereas MLLT3-MLL induces ALL or biphenotypic leukemia in mice. The microenvironment that surrounds leukemia cells plays a central role in this process. We report a patient with mixed phenotype acute leukemia with MLLT3-MLL. This patient, a 44-year-old woman, initially exhibited extramedullary leukemia with multiple tumors and subsequently developed bone marrow disease. The leukemia cells exhibited myeloid (CD13 and MPO) and B cell (CD19 and CD79a) phenotypes. Chromosomal analysis and RT-PCR assay revealed tumor cells with the MLLT3-MLL fusion gene. We treated this patient with a drug regimen for AML (Ara-C plus anthracycline), and complete remission was obtained. This report describes the fourth case of mixed phenotypic leukemia with extramedullary disease. The extramedullary circumstance may underlie the biphenotypic features of these patients.View full abstractDownload PDF (1971K) -
Keita KIRITO, Norio KOMATSU2013 Volume 54 Issue 3 Pages 295-299
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSRomiplostim is a thrombopoietin (TPO) receptor agonist that has attracted attention as a novel drug for the treatment of refractory immune thrombocytopenia (ITP). However, bone marrow reticulin and collagen fibrosis during the long-term use of romiplostim has recently become a concern. Here, we present a patient with ITP who exhibited bone marrow fibrosis after the completion of a Japanese phase III clinical trial and long-term extension study of romiplostim. The patient was a 64-year-old woman. She was diagnosed with refractory ITP and participated in a clinical trial of romiplostim. Myeloblasts were found in her peripheral blood in Week 116 of treatment with romiplostim in the long-term extension study, and romiplostim was discontinued. In Week 118, teardrop cells appeared and she underwent a bone marrow biopsy. Results showed reticulin and collagen fiber hyperplasia and her platelet count decreased markedly to 0.4×104/μl with macroscopic hematuria. Thereafter, a reduced dose of romiplostim was resumed. Approximately one year after the resumption of romiplostim, a bone marrow biopsy revealed a decrease in reticulin and collagen fibrosis. Although few patients exhibited bone marrow fibrosis with TPO and the event may be reversible, our observations indicate that careful monitoring is required for general clinical use.View full abstractDownload PDF (2291K) -
Mariko YAMAMOTO, Hisaya NAKADATE, Umefumi IGUCHI, Hiroshi MASUDA, Hiro ...2013 Volume 54 Issue 3 Pages 300-304
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSThis report describes the successful management of neurosurgical procedures with continuous infusion of recombinant factor IX (rFIX). A 1-year-old boy with severe hemophilia B was administered prophylactic therapy with rFIX after intracranial bleeding. We found the enlargement of an arachnoid cyst in a follow-up CT scan. He underwent marsupialization of the cyst under the continuous infusion of rFIX. FIX levels were examined in our hospital and the rFIX infusion rate was adjusted in an attempt to keep FIX levels above 90% intraoperatively, and 70% until his 7th post-operative day. We studied the pharmacokinetic profile of rFIX and found a half-time of 25 hours and mean in vivo recovery of 0.69 IU/dl/IU/kg. Reconstituted rFIX also retained at least 95% activity after 72 hours at room temperature. This is the first report of the perioperative management of a child undergoing a neurosurgical procedure under the continuous infusion of rFIX in Japan. Further studies are required before the routine use of this product for continuous infusion.View full abstractDownload PDF (893K) -
Yuichi KODAMA, Miho NISHIMURA, Kentaro NAKASHIMA, Nobuhiro ITO, Reiji ...2013 Volume 54 Issue 3 Pages 305-310
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSA 3-year-old girl with acute myeloid leukemia underwent unrelated cord blood stem cell transplantation (UCBT) due to primary induction failure. Fourteen days after UCBT, she developed central venous catheter (CVC)-related bloodstream infection due to Chryseobacterium indologenes. Despite ciprofloxacin and minocycline being administered according to the results of susceptibility, a high grade fever recurred. Therefore, the CVC was removed 21 days after UCBT and symptoms related to CVC infection improved. Although C. indologenes is widely distributed in nature, it is a rare pathogen in humans. Most cases of C. indologenes bacteremia have been found in immunocompromised patients with malignancies and diabetes mellitus. C. indologenes exhibits specific characteristics, including the progression of resistance to antibiotics and the formation of a biofilm. Therefore, removal of the CVC appears to be the most reasonable treatment for CVC infection due to C. indologenes in patients undergoing hematopoietic stem cell transplantation if clinical symptoms do not improve after appropriate antibiotic therapy.View full abstractDownload PDF (1046K) -
Satoko KOTEDA, Kei NOMURA, Michitoshi HASHIGUCHI, Kuniki KAWAGUCHI, Ei ...2013 Volume 54 Issue 3 Pages 311-315
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSA 62-year-old woman with acute lymphoblastic leukemia in first complete remission was treated with unrelated cord blood transplantation, but exhibited primary graft failure. She then underwent HLA-haploidentical peripheral blood stem cell transplantation from her daughter. The conditioning regimen consisted of fludarabine 30 mg/m2/day for 6 days, intravenous busulfan 3.2 mg/kg/day for 2 days, and thymoglobulin 1 mg/kg/day for 2 days. Voriconazole was administered to prevent fungal infections. The patient achieved prompt hematopoietic recovery. Fever was observed 21 days after the second transplant, followed by sigmoid colon perforation and a liver space occupying lesion (SOL). A filamentous fungus was detected in a percutaneous biopsy of the liver SOL. In spite of changing the antifungal drug from voriconazole to liposomal amphotericin B, the patient died on day 41. The fungus was identified as Mucor indicus, a type of zygomycete. Although Mucor indicus inhabits soil, an infectious disease is extremely rare, and breakthrough infection after voriconazole prophylaxis had not been reported until now. It is mandatory to consider preventive antifungal treatment for drug-resistant fungal infectious diseases in patients after neutrophilic recovery with a strongly immunocompromised state after a HLA-haploidentical transplant.View full abstractDownload PDF (2047K)
A Short Report
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Chitose OGAWA, Atsushi MANABE, Akira OHARA, Akira ISHIGURO2013 Volume 54 Issue 3 Pages 316-318
Published: 2013
Released on J-STAGE: April 03, 2013
JOURNAL RESTRICTED ACCESSWe investigated supportive therapy against coagulopathy associated with L-asparaginase treatment in patients with acute lymphoblastic leukemia who were enrolled in the Japanese Pediatric Leukemia/Lymphoma Study Group (JPLSG), Japan Adult Leukemia Study Group (JALSG), and foreign institutes. Fresh frozen plasma (FFP) was administered as a supplement in 46% patients in the JPLSG and 86% in the JALSG. The threshold level of FFP infusion was less than 100 mg/dl plasma fibrinogen in 70% of the JALSG and 20% of the JPLSG, while in another 20% of the JPLSG, FFP was administered when the fibrinogen level was less than 50 mg/dl. The preventive use of antithrombin products (AT) was prescribed in 93% of the JPLSG and 63% of the JALSG: The threshold level of AT supplementation was less than 70% of plasma antithrombin activity, which was similar in both groups. Most foreign institutes do not routinely use FFP or AT.View full abstractDownload PDF (591K)
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