Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 54, Issue 4
Displaying 1-13 of 13 articles from this issue
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Picture in Clinical Hematology
The 74th Annual Meeting of the Japanese Society of Hematology
Presidential Symposium
JSH-EHA Joint Symposium
Symposium 1
Clinical Study
  • Relationship between red blood cell transfusion volume and posttransfusional iron overload in hematological diseases
    Takashi ASHIDA, Ami KAWANO, Erika YAMADA, Daisuke IDE, Chiemi SUGANO, ...
    2013 Volume 54 Issue 4 Pages 365-369
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.365
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    We retrospectively investigated the status of transfusional iron overload at Kinki University Hospital. One hundred and sixty three patients received more than 10 red blood cell (RBC) units per year in 2009 and 2010. Myelodysplastic syndrome (37.4%) and aplastic anemia (11.0%) accounted for about 50% of the underlying diseases. At the time of receiving a total of 20 RBC units, 90.8% and 66.2% of the 65 patients evaluated had more than 500 and 1,000 ng/ml of serum ferritin, respectively. The frequency of organ dysfunction associated with iron overload was 56.9% of all the patients assessed, 37.8% of patients with serum ferritin levels of 500∼999 ng/ml, and 67.4% of patients with serum ferritin levels >1,000 ng/ml. Although the Japanese guidelines propose 40 units of RBC transfusion and/or a serum ferritin level of 1,000 ng/ml as a good point to start iron chelation therapy, our results suggest that iron overload and consequent organ dysfunction may occur earlier than this. Therefore, it may be necessary to start iron chelation therapy earlier than that suggested by the Japanese guidelines.
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  • The feasibility of Erwinia asparaginase for pediatric patients who developed an allergic reaction to E.coli asparaginase during treatment of acute lymphoblastic leukemia
    Hiroyoshi TAKAHASHI, Katsuyoshi KOH, Motohiro KATO, Kiyotaka ISOBE, Na ...
    2013 Volume 54 Issue 4 Pages 370-377
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.370
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    Asparaginase (ASNase) is one of the most important key drugs in the treatment of acute lymphoblastic leukemia (ALL). However, clinical hypersensitivity reactions often occur and lead to the discontinuation of ASNase treatment. Here, we report a retrospective study of 68 Erwinia ASNase (Erw-ASNase) administrations in 11 patients with childhood ALL who developed allergic reactions to E.coli-ASNase in our hospital between 2006 and 2012. The median age of the patients was 6 (range, 0 to 14). Erw-ASNase purchased overseas by the patients' guardians had already been administered when we obtained informed consent from the guardians. In all patients, fibrinogen and/or anti-thrombin III levels were decreased, but thrombosis did not develop. There was only one mild adverse event (grade 2 urticaria) in one patient, in whom Erw-ASNase could be continued after increasing the doses of premedication with antihistamine and prednisolone. Erw-ASNase could be safely administered to all patients.
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Case Reports
  • Transfusion-related acute lung injury during the treatment of EBV-associated hemophagocytic lymphohistiocytosis
    Eriko NAKASHIMA, Motoaki SHIRATSUCHI, Emi HONDA, Eriko FUJIOKA, Hirofu ...
    2013 Volume 54 Issue 4 Pages 378-382
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.378
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    Transfusion-related acute lung injury (TRALI) is a severe pulmonary complication following blood transfusions. We experienced a case of possible TRALI during the course of EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH). A 19-year-old woman was admitted to our hospital suffering from fever and abdominal pain. Her laboratory data revealed pancytopenia, liver damage, coagulopathy, and a high titer of EBV-DNA. Computed tomography showed hepatosplenomegaly and bone marrow aspiration revealed hemophagocytosis and the proliferation of atypical lymphocytes. A diagnosis of EBV-HLH was made and plasma exchange was performed. Severe hypoxia due to pulmonary edema developed two hours after starting the plasma transfusion. Methylprednisolone pulse therapy and non-invasive positive pressure ventilation ameliorated her respiratory condition. Anti-HLA class I and II antibodies were detected in donor sera and a cross-match test between patient lymphocytes and donor plasma was positive. To the best of our knowledge, this is the first case report of TRALI complicated with EBV-HLH. It is possible that hypercytokinemia accompanied by HLH was associated with the onset of TRALI.
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  • Refractory acute myeloid leukemia developed malignancy-associated hemophagocytic lymphohistiocytosis during treatment of invasive fungal infection
    Ai YAMADA, Hiroshi MORITAKE, Daisuke SAWA, Hidemi SHIMONODAN, Hitomi K ...
    2013 Volume 54 Issue 4 Pages 383-387
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.383
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    We here report a 2-year-old female with relapsed acute myeloid leukemia (AML) with MLL gene rearrangement in the bone marrow and central nervous system. The 3'-RACE (Rapid Amplification of cDNA Ends) method identified the MLLT10 gene as a fusion partner of the MLL gene. The patient was complicated with hemophagocytic lymphohistiocytosis (HLH) and invasive aspergillosis (IPA) after re-induction treatment with FLAG-IDA following etoposide, cytarabine, and mitoxantrone. Although treatment with systemic anti-fungal drugs was effective for IPA, HLH did not improve. We considered tumor-associated HLH to be initiated from leukemic stem cells (LSCs) in the bone marrow niche because reverse transcription-polymerase chain reaction (RT-PCR) analysis of a bone marrow biopsy sample was positive for MLL-MLLT10. Gemtuzumab ozogamicin and sorafenib had no major effect on acquiring complete remission, and the patient died of progressive AML with an exacerbation of HLH and aspergillosis. LSCs are known to be resistant to conventional chemotherapy due to their quiescence in the cell cycle. Novel therapeutic concepts are important to eradicate LSCs in order to cure AML patients.
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  • The appearance of t(9;22)(q34;q11.2) in BJP-λ type multiple myeloma during maintenance therapy including lenalidomide
    Jun-nosuke UCHIHARA, Hitoshi ARAKAKI
    2013 Volume 54 Issue 4 Pages 388-391
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.388
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    A 61-year-old man, who was diagnosed with Bence-Jones protein (BJP)-λ type multiple myeloma, was treated with bortezomib. Although maintenance therapy including lenalidomide was continued, t(9;22)(q34;q11.2) was detected in the marrow cells by a cytogenetic study. The increased incidence of a secondary malignancy after treatment with lenalidomide for multiple myeloma has been highlighted in previous clinical trials; however, reports on the presence of t(9;22)(q34;q11.2) or the onset of chronic myelogenous leukemia have yet to be found. Although the cause of chronic myelogenous leukemia with the lenalidomide treatment is not yet clear, it is an interesting case.
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  • Primary diffuse large B-cell lymphoma of the uterus complicated with hydronephrosis
    Mai ISOSAKA, Toshiaki HAYASHI, Kei MITSUHASHI, Michihiro TANAKA, Takey ...
    2013 Volume 54 Issue 4 Pages 392-396
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.392
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    Malignant lymphoma sometimes originates from extranodal sites; however, the uterus has rarely been reported as the site of the primary lesion. We present a patient with malignant lymphoma of the uterus complicating bilateral hydronephrosis. A 67-year-old previously healthy woman was seen at a clinic because of massive genital bleeding. She was referred to our hospital for further examination of a uterine tumor. Computed tomography scans revealed a pelvic tumor invading to the retroperitoneal region, which caused bilateral obstruction of the ureters and hydronephrosis. No lymph node swelling was detected. Magnetic resonance imaging showed a bulky uterine tumor that was homogenously low on T1-weighted imaging and isointense on T2-weighted imaging, while the endometrium was intact. A pathological examination of the biopsy specimen from the uterine cervix revealed diffuse infiltration of CD20-positive atypical large lymphoid cells, which was compatible with diffuse large B-cell lymphoma (DLBCL). Since the tumor expanded from the uterus and no other abnormal lesion was observed in imaging studies including gallium scintigraphy, a diagnosis of DLBCL of the uterus, clinical stage IE was made. The patient received six cycles of rituximab plus CHOP chemotherapy followed by involved field irradiation. She achieved complete remission and has been alive for more than two years without relapse.
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  • Acute myeloid leukemia with monosomy 7 and inv(3)(q21q26.2) complicated with central diabetes insipidus
    Satoru NANNO, Kiyoyuki HAGIHARA, Manami SAKABE, Hiroshi OKAMURA, Akiko ...
    2013 Volume 54 Issue 4 Pages 397-402
    Published: 2013
    Released on J-STAGE: May 11, 2013
    DOIhttps://doi.org/10.11406/rinketsu.54.397
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    A 20-year-old female presented with thirst, polyposia, and polyuria and was referred to our hospital because of leukocytosis and anemia. Bone marrow aspiration revealed 66.8% myeloperoxidase-positive blasts and trilineage myelodysplasia. The karyotype was 45, XX, inv(3)(q21q26.2), -7[19]. Therefore, a diagnosis of AML with inv(3)(q21q26.2) complicated by -7 was made. Moreover, hyposthenuria and a low anti-diuretic hormone (ADH) level were observed. Although cerebrospinal fluid analysis was normal, magnetic resonance imaging (MRI) revealed the absence of hyperintensity in the neurohypophysis in T1-weighted images. Therefore, she was also diagnosed with diabetes insipidus. After she was administered a desmopressin nasal spray, the volume of urine produced decreased. Following treatment with second induction therapy containing high-dose cytarabine for AML, she achieved complete remission in the bone marrow. Moreover, when the abnormality on MRI and the volume of urine were normalized, she discontinued desmopressin. Although diabetes insipidus is a rare complication of AML, the majority of AML patients who have diabetes insipidus have the abnormal karyotypes with inv(3)(q21q26.2)/t(3;3)(q21;q26.2) and monosomy 7. Further study is required to clarify the pathogenesis and develop a strategy for the treatment of this category of AML.
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