Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 51, Issue 4
Displaying 1-11 of 11 articles from this issue
Picture in Clinical Hematology No.43
Clinical Study
  • Chihiro SHIMAZAKI, Hirokazu MURAKAMI, Morio SAWAMURA, Masayuki MATSUDA ...
    2010 Volume 51 Issue 4 Pages 245-252
    Published: 2010
    Released on J-STAGE: May 11, 2010
    JOURNAL RESTRICTED ACCESS
    Recently, a highly sensitive assay (FREELITE) capable of measuring serum immunoglobulin-free light chains (FLC) was developed. An abnormal κ/λ ratio supports the presence of clonal plasma cell expansion. Using this assay, we measured serum and urine samples of 178 healthy volunteers, 184 patients with polyclonal γ-globulinemia and 150 patients with monoclonal γ-globulinemia. The diagnostic sensitivity of the FLC assays for monoclonal gammopathies was 88.0% and the specificity for healthy volunteers and polyclonal gammopathies was 96.1%. The minimal detection sensitivity of this assay for serum FLC was 0.3 mg/l and was greater than 100-fold more sensitive than serum protein electrophoresis (SPE). The combination of FLC with SPE and immunoelectrophoresis identified 99% of patients with monoclonal gammopathies. Effective treatment often leads to a more rapid reduction of the involved FLC level relative to the intact immunoglobulin or total light chain concentration because the half-life of FLC is <6 hours. These observations suggest that FREELITE is useful for diagnosis, disease monitoring and assessment of response to treatment in patients with monoclonal gammopathies such as multiple myeloma and AL amyloidosis.
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Case Reports
  • Dai CHIHARA, Takashi SAKAMOTO, Gaku MURAKAMI, Tomoharu TAKEOKA, Tatsuh ...
    2010 Volume 51 Issue 4 Pages 253-257
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    Patients receiving splenectomy are at risk of a fatal fulminant infection called overwhelming post-splenectomy infection (OPSI). Here we report a rare case of toxic shock syndrome (TSS) evoked by group B streptococcus (GBS) in an asplenic young woman, which we considered a case of OPSI. A 34-year old woman consulted our hospital complaining of vomiting, diarrhea and fever that developed early in the morning. As the physical examination and routine laboratory tests did not disclose any serious abnormalities, she returned home after symptomatic treatment under a provisional diagnosis of acute enterocolitis. However, the next morning, she was transferred to the hospital complicated by acute renal failure, severe liver damage, respiratory insufficiency, disseminated intravascular coagulation and hypotension. She was admitted to ICU and treated with intravenous antibiotics, frequent transfusions of platelet concentrates, hemodialysis, and non-invasive positive pressure ventilation. Blood cultures grew gram-positive cocci, which later proved to be Streptococcus agalactiae (GBS). We diagnosed the patient with TSS due to GBS. Organ damage and symptoms improved gradually with intensive treatment, she was discharged from the hospital 26 days after admission. Although cases of TSS due to GBS are very rare, we must be aware of the potential risk of OPSI in a splenectomized patient.
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  • Kazuko INO, Masahiro MASUYA, Yoshiki NAKAMORI, Kei SUZUKI, Minoru MIZU ...
    2010 Volume 51 Issue 4 Pages 258-263
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 46-year-old Japanese man was admitted to our hospital because of prolonged fever. Laboratory examination demonstrated leukopenia, thrombocytopenia, marked liver dysfunction, and elevation of serum ferritin. A bone marrow examination showed several hemophagocytic macrophages, and a diagnosis of hemophagocytic syndrome was made. He was treated using HLH-94 protocol, and his clinical symptoms and laboratory data were rapidly improved. After 5 weeks, fever and liver dysfunction reappeared. A repeat bone marrow examination demonstrated that 28.4% of marrow nucleated cells were atypical lymphocytes, which were positive for CD2, CD7, CD16, CD56, and HLA-DR. Clonality of these proliferating NK cells was confirmed by an analysis of EB virus terminal repeat sequence and cytogenetic analysis, and final diagnosis of aggressive NK-cell leukemia was made. After induction chemotherapy consisting of dexamethasone, etoposide, ifosfamide, and L-asparaginase, the patient achieved partial remission. He received allogeneic peripheral blood stem cell transplantation from his one locus mismatched son, and is alive with no evidence of disease 20 months after transplantation.
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  • Yukinari SANADA, Tomonori NAKAZATO, Kazuhito SUZUKI, Ai MIHARA, Yoshin ...
    2010 Volume 51 Issue 4 Pages 264-269
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 59-year-old man was diagnosed with IgA-κ multiple myeloma in October 2005. He was treated with 4 courses of VAD and autologous peripheral blood stem cell transplantation (auto-PBSCT) after 200 mg/m2 melphalan in September 2006, followed by a second auto-PBSCT after 200 mg/m2 melphalan in February 2007. However, he did not achieve a very good partial response (VGPR). Laboratory examinations showed increased serum IgA level and renal dysfunction gradually progressed. Bortezomib was then started at a dose of 1.3 mg/m2 in November 2008. After three cycles of bortezomib, the patient developed numbness, pain and weakness of his upper and lower extremities. The sensation of position and vibration was diminished in the fingers and toes. He developed left foot drop and gait disturbance due to left peroneal nerve palsy. Autonomic dysfunction such as orthostatic hypotension and urinary retention also occurred. Nerve conduction studies showed severe sensorimotor polyneuropathy particularly in the lower extremities. He developed grade 4 motor neuropathy and severe painful neuralgia. Six months after the cessation of bortezomib, these symptoms gradually improved and he was able to walk with support and discharged. Close monitoring of neurological symptoms and prompt dose-reduction or cessation of bortezomib are important to prevent the progression of irreversible peripheral neuropathy.
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  • Riko HONMA, Sachiko FUKASE, Masayuki SUZUKI, Eijiro OMOTO
    2010 Volume 51 Issue 4 Pages 270-274
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    Quantitative assay for serum free light chains (sFLC) is reported as a useful test for diagnosis and monitoring of patients with nonsecretory multiple myeloma (NSM). We performed serial sFLC assays in a patient with NSM with light chain cast nephropathy (LCCN) and light chain deposition disease (LCDD). After 3 cycles of VAD induction therapy, while plasma cells in the marrow decreased from 93.0% to 0.2%, sFLCκ/λ ratio remained abnormal. Flow cytometry assay also showed that these plasma cells were CD19 negative. After the subsequent high dose melphalan therapy followed by autologous peripheral blood stem cell transplantation (PBSCT), the sFLCκ/λ ratio returned to normal and the patient achieved a stringent complete response (sCR). One year after PBSCT, the patient remained in sCR with improved renal function. The quantitative FLC assay was useful for the diagnosis and monitoring of NSM and LCDD in this patient.
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  • Naoki KARASAWA, Yasuhiro TANIGUCHI, Tomonori HIDAKA, Keiko KATAYOSE, T ...
    2010 Volume 51 Issue 4 Pages 275-280
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 67-year-old woman was admitted to the hospital for lethargy, fever, hemolytic anemia, thrombocytopenia, and consciousness disturbance. Direct Coombs test was positive, and anti-cardiolipin β2-glycoprotein I antibody was detected. She was diagnosed with antiphospholipid syndrome complicated with autoimmune hemolytic anemia (AIHA). She demonstrated variable consciousness disturbance, inability to distinguish right from left, dysgraphia and dyscalculia. Multiple cerebral infarctions, especially dominant cerebral hemisphere infarctions, were observed on magnetic resonance imaging. A ventilation-perfusion scan demonstrated the presence of a ventilation-perfusion mismatch in both lung fields, and multiple veinous embolisms in the right femoral, bilateral the great saphenous and popliteal veins. Therefore, pulmonary embolism and thrombophlebitis were diagnosed. Based on these findings, it was necessary to distinguish this diagnosis from thrombotic thrombocytopenic purpura (TTP). As ADAMTS-13 activity was within the normal range, TTP was denied. Thereafter, the patient was treated with 1 mg/kg of prednisolone for AIHA, 3 mg of warfarin, and 3500 units of low-molecular-weight heparin for thrombosis, and her condition improved.
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  • Shinya RAI, Mitsuhiro MATSUDA, Nozomi YAMAIRI, Takao KAZUTA, Takayuki ...
    2010 Volume 51 Issue 4 Pages 281-285
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 51-year-old woman developed very severe aplastic anemia complicated by chronic renal failure. She underwent ATG therapy, resulting in a transient effect. Thereafter, renal insufficiency progressed, followed by the induction of CAPD therapy. Although rabbit ATG (rATG) was administered as the next immunotherapy for aplastic anemia, pancytopenia persisted and she experienced repeated episodes of severe infection including MRSA and fungal infections. She was transplanted with peripheral blood stem cells from her HLA-haploidentical son after a reduced-intensity conditioning regimen that included cyclophosphamide (three days of 30 mg/kg), fludarabine (two days of 20 mg/m2) and rATG (three days of 2.5 mg/kg). FK506 (0.03 mg/kg/24H) was administered for the prophylaxis of GVHD. Prompt trilineage engraftment occurred, resulting in the improvement of infections. Three months after grafting, she succumbed to cerebrovascular disease, although there was no apparent GVHD and she had remained well with stable hematopoiesis.
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  • Kazuhiro YAGO, Maki AONO, Hideto SHIMADA
    2010 Volume 51 Issue 4 Pages 286-290
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 79-year-old female with acute promyelocytic leukemia (APL) presented with second hematological relapse. She had been treated previously with modified AIDA protocol as the front-line therapy and had achieved complete remission. During ATRA maintenance therapy, the first hematological relapse occurred and she was treated with arsenic trioxide (ATO), achieving the second complete remission. After four courses of consolidation therapy of ATO, the second hematological relapse occurred. At this time, except for a transient effect of tamibarotene, neither arsenic trioxide nor combination chemotherapy was effective. The patient was then treated with two courses of gemtuzumab ozogamicin (GO) and achieved the third complete remission. At present, she is maintaining molecular remission more than one year after GO treatment. GO is considered to be a promising agent for elderly patients with relapsed acute promyelocytic leukemia resistant to arsenic trioxide.
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  • Toru MITSUMORI, Kei NAKAJIMA, Yumi NOZAKI, Satoshi HAMANAKA, Takahiro ...
    2010 Volume 51 Issue 4 Pages 291-296
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 67-year-old woman with refractory multiple myeloma was admitted to our hospital for salvage therapy. She developed fever several days after chemotherapy was initiated and complained of chest pain. Since abnormal electrocardiogram was demonstrated. Emergency coronary angiography was performed, but the coronary artery did not demonstrate stenosis. Thereafter, the patient was diagnosed as having takotsubo cardiomyopathy. Hydragogue and nitric acid preparation transiently improved chest symptoms, but high fever persisted despite antibiotic and antifungal agents. She died on the 9th day after the initiation of chemotherapy. Physicians need to be aware that cardiomyopathy may develop as a severe side effect of chemotherapy.
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A Short Report
  • Masashi OHE, Satoshi HASHINO
    2010 Volume 51 Issue 4 Pages 297-299
    Published: 2010
    Released on J-STAGE: May 11, 2010
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    A 79-year-old woman was admitted with submandibular lymphadenopathy and was diagnosed as having bacterial and fungal lymphadenitis by lymph nodal biopsy. Laboratory examination demonstrated leukopenia with 7% blasts. The bone marrow was infiltrated with about 90% blasts and surface antigen analysis of these blasts demonstrated expression of cyCD3, TdT and MPO. The diagnosis was Mixed phenotype acute leukemia, T/myeloid, NOS. The patient was treated with prednisone (10 mg/day) for fever and subsequently CAM (800 mg/day) for pneumonia. Three months later, leukemic cells had disappeared on both bone marrow aspiration and peripheral blood smear. This clinical course suggests CAM is effective for this leukemia.
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