Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 49, Issue 5
Displaying 1-11 of 11 articles from this issue
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Picture in Clinical Hematology No.27
The 69th Annual Meeting of the Japanese Society of Hematology/
The 49th Annual Meeting of the Japanese Society of Clinical Hematology
Symposium
Review
Case Reports
  • ATIC-ALK-positive anaplastic large cell lymphoma: a case report and review of the literature
    Kousaku MATSUBARA, Takayuki TANAKA, Tomohiko TAKI, Atsuko NAKAGAWA, Hi ...
    2008 Volume 49 Issue 5 Pages 325-330
    Published: 2008
    Released on J-STAGE: September 05, 2008
    DOIhttps://doi.org/10.11406/rinketsu.49.325
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    We report a 10-year-old girl with ATIC-anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL). She presented with inguinal, axillary, and paraaortic lymph node swellings that showed spontaneous regression over a 3-month period, followed by recurrence after an interval of 8 months. Radiological and clinical findings indicated Ann Arbor stage IIIA. Pathological findings showed that staining of ALK was restricted to the cytoplasm of ALCL cells. ATIC-ALK chimeric transcripts were detected by reverse transcriptase polymerase chain reaction. The patient was assigned to the standard risk group proposed by the international multicenter study for pediatric ALCL, ALCL99. The patient responded well to the treatment and remained in complete remission for more than 26 months. To date, 7 genes have been identified as a fusion partner of ALK, with the highest frequency in nucleophosmin (NPM). Little is known about the clinical implications of subtypes of ALCL harboring each of the 7 fusion genes, especially those of variant fusion genes other than NPM-ALK. In this paper, we review 9 patients with ATIC-ALK-positive ALCL in the literature in addition to discussing our patient. In eight of these 10 cases, disease occurred within the first three decades. Five of 6 cases that were followed continuously remained in complete remission.
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  • Enhanced post-herpetic neuralgia by Bortezomib
    Kazutaka KIRIZUME, Osamu IMATAKI, Takamichi SHINTANI, Shintaro FUJIHAR ...
    2008 Volume 49 Issue 5 Pages 331-334
    Published: 2008
    Released on J-STAGE: September 05, 2008
    DOIhttps://doi.org/10.11406/rinketsu.49.331
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    Bortezomib, a proteasome inhibitor, has been used for patients with refractory multiple myeloma. We present a 58-year old man who had IgG-λ-type multiple myeloma, refractory for MP (melphalan-predonisolone) and VAD (vincrisitine-doxorubicin-dexamethasone) therapy. He was complicated with reactivation of varicella-zoster virus (VZV) 4 weeks before bortezomib administration. Two weeks of consolidation treatment with standard dose valaciclovir caused VZV infection to settle down and, after a further 2 weeks, VZV remission was confirmed. Bortezomib was started at a dose of 1.3 mg/m2 with prophylactic use of valaciclovir for VZV reactivation, post-herpetic neuralgia exacerbated the following day and grade 3 neuralgia developed the following week without recurrence of skin eruption. Neuralgia improved after the cessation of bortezomib with various supportive treatments and interventions. Although the reactivation of VZV was suspicious, no apparent skin lesions were observed. Although the mechanisms of post-herpetic neuralgia and chemotherapy-induced neuropathy are different, bortezomib might enhance post-herpetic neuralgia independent of the manner of viral reactivation.
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  • Gaucher disease type I diagnosed at 63 years old presenting with thrombocytopenia
    Shimpei KAWAHARA, Atushi KATO, Kazuo OSHIMI, Hiroyuki IDA
    2008 Volume 49 Issue 5 Pages 335-339
    Published: 2008
    Released on J-STAGE: September 05, 2008
    DOIhttps://doi.org/10.11406/rinketsu.49.335
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    A 63-year-old man who was incidentally found to have thrombocytopenia at a periodic physical examination visited our hospital. The spleen was palpable 3 finger-breadths below the navel level, and the liver was palpable 1 finger-breadth below the right costal margin. Peripheral blood examination showed WBC 2,900/μl, Hb 13.4 g/dl, and platelets 54×103/μl. Gaucher cells were recognized in the bone marrow by aspiration, and serum levels of total acid phosphatase and angiotensin converting enzyme were increased. Glucocerebrosidase activity was lower than the control level in bone marrow stroma cells, and modification of glucocerebrosidase genotype N188S was shown, which had been identified in the past. Furthermore, neurological examination was normal. Based on these results, we diagnosed the patient with Gaucher disease type I, and started enzyme replacement therapy.
    Gaucher disease is rare in Japanese, approximately 100 cases having been reported; diagnosis at older age is also relatively rare and, as far as we know, the oldest age reported in Japanese was 57 years old. Gaucher disease should be considered a differential diagnosis when thrombocytopenia and splenomegaly are found in elderly patients, although it is relatively rare.
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  • Acute myeloid leukemia invasion of the central nervous system, detected only along the Virchow Robin space
    Kei SUZUKI, Kazuko INO, Yumiko SUGAWARA, Minoru MIZUTANI, Takao SEKINE ...
    2008 Volume 49 Issue 5 Pages 340-343
    Published: 2008
    Released on J-STAGE: September 05, 2008
    DOIhttps://doi.org/10.11406/rinketsu.49.340
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    A 66-year-old man with sustained fever was diagnosed as having acute myeloid leukemia with multilineage dysplasia. Induction therapy with etoposide and AraC was initiated, but was ineffective. Although fever had persisted for more than a few days, there was no evidence of any infection on radiological examination or culture studies. The patient was disorientated and demonstrated personality change. After a severe convulsive seizure, the patient died. Autopsy findings showed that the leukemic cells had permeated the Virchow Robin space, but without a mass lesion in the cerebral parenchyma. He was diagnosed as having had central nervous system leukemia (CNSL) that provoked sustained fever, consciousness disturbance and convulsive seizure. These findings suggested that the Virchow Robin space plays a particular role in the development of CNSL. Even with repeated cerebrospinal fluid examinations and radiological tests, we were unable to correctly diagnose CNSL before death, which may indicate the intractability of diagnosing CNSL spread along the Virchow Robin space. This case provides useful information about the pathophysiology and diagnosis of CNSL.
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A Short Report
  • Hemophagocytic lymphohistiocytosis as an initial manifestation in an HIV-infected patient
    Takao SUDO, Satoru KOSUGI, Hiroshi SATA, Soichi NAKATA, Hironori TAKE, ...
    2008 Volume 49 Issue 5 Pages 344-346
    Published: 2008
    Released on J-STAGE: September 05, 2008
    DOIhttps://doi.org/10.11406/rinketsu.49.344
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    A 48-year old man was admitted with idiopathic fever, and subsequently diagnosed as having hemophagocytic lymphohistiocytosis (HLH). Though an extensive series of laboratory examinations failed to demonstrate an apparent etiology, empirical use of steroids achieved remission. About two years later, the patient developed Pneumocystis Jiroveci pneumonia and was diagnosed as HIV-positive. Based on this case, HIV-screening tests would be performed whenever we encounter HLH in Japan, where the number of HIV-positive patients is increasing.
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