Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 55, Issue 4
Displaying 1-14 of 14 articles from this issue
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Picture in Clinical Hematology
The 75th Annual Meeting of the Japanese Society of Hematology
Symposium 2
Symposium 3
Clinical Study
  • Impacts of new agents for multiple myeloma on development of secondary myelodysplastic syndrome and acute myeloid leukemia
    Airi HAMANO, Sumito SHINGAKI, Yu ABE, Kanji MIYAZAKI, Rieko SEKINE, Ya ...
    2014 Volume 55 Issue 4 Pages 428-435
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.428
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    The use of new agents (NAs) such as bortezomib, thalidomide, and lenalidomide has extended the survival of patients with multiple myeloma (MM). However, whether long-term treatment using NAs may increase the risk of second primary malignancies is a concern. Three hundred and thirty-three patients with MM were treated at our hospital from 1998 to 2013. Additional chromosomal abnormalities (CAs), associated with secondary myelodysplastic syndrome/acute myeloid leukemia, were observed in 13 of 152 users of NAs, but in 38 of 181 non-users of NAs. The cumulative CA incidence was higher in non-users of NAs. The CAs frequently observed were 13q-, 20q-, +8 in users of NAs, while -5/5q- and -7/7q- were detected in non-users of NAs. The total dose and treatment period of NAs did not differ between CAs-positive and -negative patients. However, a higher dose of melphalan was observed to have been used in patients who had CAs. Longer follow-up periods are necessary for an accurate risk assessment.
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  • Pagophagia in iron deficiency anemia
    Tatsumi UCHIDA, Yasunori KAWATI
    2014 Volume 55 Issue 4 Pages 436-439
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.436
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    The relationship between pagophagia (ice pica) and iron deficiency anemia was studied. All 81 patients with iron deficiency anemia defined as hemoglobin <12.0 g/dl and ferritin level <12 ng/ml were interviewed about their habits of eating ice or other non-food substances. Pagophagia was defined as compulsive and repeated ingestion of at least one tray of ice or ice eating which was relieved after iron administration. Pagophagia was present in 13 patients (16.0%). All patients who received oral iron were periodically assessed employing a questionnaire on pagophagia and laboratory data. Iron therapy can cure the pagophagia earlier than hemoglobin recovery and repair of tissue iron deficiency. Although the pathogenesis of pagophagia is unclear, a biochemical approach involving the central nervous system might elucidate the mechanism underlying these abnormal behaviors.
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Case Reports
  • Successful Treatment of an essential thrombocythemia patient complicated by Sweet's syndrome with combination of chemotherapy and lenalidomide
    Michiko YAMADA, Hiroyuki KURODA, Masahiro YOSHIDA, Wataru JOMEN, Tomoy ...
    2014 Volume 55 Issue 4 Pages 440-444
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.440
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    A 79-year-old man had been followed up since July 2003 based on a diagnosis of essential thrombocythemia (ET). The patient visited our hospital after developing a high fever and rash in August 2010, and Sweet's syndrome was diagnosed based on skin biopsy results. The bone marrow aspirate showed features like those of myelodysplastic/myeloproliferative neoplasm (MDS/MPN, unclassifiable). Administration of metenolone and azacitidine was initiated in March and May 2011, respectively, but the rash associated with Sweet's syndrome showed exacerbation. Ranimustine was therefore administered starting in July 2011 to control the blood cell count, but the rash associated with Sweet's syndrome persisted. Combination therapy with lenalidomide was initiated in September 2012, and resulted in control of the blood cell count and marked improvement of Sweet's syndrome.
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  • Development of acute pure red cell aplasia after deferasirox administration in two cases of myelodysplastic syndrome
    Fumihiko HAYAKAWA, Akihiro TOMITA, Tomoki NAOE
    2014 Volume 55 Issue 4 Pages 445-449
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.445
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    Cases 1 and 2 were 55- and 68-year-old males, respectively. Both were administered deferasirox (DFX) because they received red blood cell transfusions regularly as treatment for myelodysplastic syndrome refractory anemia. DFX administrations were stopped on the 22nd day in case 1 and on the 78th day in case 2 because significantly reduced hemoglobin values and reticulocyte counts were observed. Bone marrow examinations showed pure red cell aplasia in both cases. In case 1, the reticulocyte ratio recovered to the value before drug administration 21 days after drug withdrawal. In case 2, it started increasing on the 14th day, and had recovered to the value before drug administration by the 42nd day after drug withdrawal. Human parvovirus B19 infections were negative in both cases. Both cases were thought to have drug-induced pure red cell aplasia, probably due to DFX. This drug should be used carefully with regular follow-ups of the reticulocyte count.
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  • Long-term survival of a patient with multiple myeloma-associated severe cardiac AL amyloidosis after implantation of a cardioverter-defibrillator
    Minako MORI, Tomoya KITAGAWA, Yuya SASAKI, Takashi ONAKA, Akihito YONE ...
    2014 Volume 55 Issue 4 Pages 450-455
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.450
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    Cardiac involvement is by far the most relevant factor impacting poor outcomes of patients with systemic light-chain (AL) amyloidosis. Median survival of patients with symptomatic cardiac AL amyloidosis is less than 6 months. Approximately two-thirds of these patients die suddenly due to ventricular arrhythmias and electromechanical dissociation. We report a 56-year-old female with very severe cardiac AL amyloidosis (NT-proBNP 13,355 ng/l, troponin T 0.16 μg/l, and systolic blood pressure 100 mmHg), who was successfully treated with diuretics and an implantable cardioverter-defibrillator (ICD) and has survived for more than 4 years, to date. During the 4-year period after receiving the ICD, she experienced several episodes of sustained ventricular tachycardia and ventricular fibrillation, all successfully terminated by anti-tachycardia pacing or electrical shock. The benefit of ICD for cardiac AL amyloidosis is unclear since there have been only a few reports of successful use of this therapy for patients with cardiac AL amyloidosis. Recently, new treatment options for AL amyloidosis, such as bortezomib and lenalidomide, have shown high response rates and improved outcomes. It is important to identify those cardiac amyloidosis patients who might be more likely to benefit from ICD implantation.
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  • Cerebral toxoplasmosis after umbilical cord blood transplantation diagnosed by the detection of anti-toxoplasma specific IgM antibody in cerebrospinal fluid
    Akiko INABA, Hideo KOH, Yasuhiro NAKASHIMA, Mitsutaka NISHIMOTO, Yoshi ...
    2014 Volume 55 Issue 4 Pages 456-460
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.456
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    Cerebral toxoplasmosis is a rare, potentially fatal, complication of hematopoietic cell transplantation. Early definitive diagnosis is very difficult and it may be associated with a poor prognosis. Herein, we describe a 60-year-old woman who developed cerebral toxoplasmosis after cord blood transplantation for myelodysplastic syndrome. During treatment with tacrolimus and methylprednisolone for relapsed grade 2 acute gut GVHD, fever and disturbance of consciousness occurred on day 210. Brain MRI showed multiple ring-enhancing nodular lesions in the thalamus, basal ganglia, brainstem, and subcortical white matter. Cerebrospinal fluid (CSF) assessment revealed elevations of both anti-to-xoplasma IgM and IgG, which were also elevated in serum, but no evidence of other infections or malignancies. Notably, the IgM level was higher in the CSF than in serum. Thus, cerebral toxoplasmosis was diagnosed. Soon after administration of oral sulfamethoxazole/trimethoprim and intravenous clindamycin in combination with short-term dexamethasone for the cerebral edema, her symptoms and signs began to improve. On day 229, both IgM and IgG titers in CSF had clearly decreased but remained essentially constant in serum. She was discharged without clinically significant neurological disorders. This case suggests that CSF specific anti-toxoplasma IgM titers might be useful for early diagnosis of cerebral toxoplasmosis after transplantation.
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  • Complete remission achieved by a combination regimen with bortezomib, cyclophosphamide, and dexamethasone in a multiple myeloma patient with elevated serum KL-6 level
    Yutaka OKUNO, Nao NISHIMURA, Kisato NOSAKA, Hiroyuki HATA, Hiroaki MIT ...
    2014 Volume 55 Issue 4 Pages 461-465
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.461
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    A 79-year-old woman suffering from double vision after a 4-year history of MGUS was referred to our hospital. MRI revealed that she had three intracranial plasmacytoma masses and one spinal plasmacytoma mass. Bone marrow aspirates showed 52.4% plasma cell infiltration and immunoelectrophoresis identified serum IgG-M protein, leading to a diagnosis of IgG-type multiple myeloma. IgG was elevated to 3,355 mg/dl and urine type Bence-Jones protein was positive. KL-6, a membrane-bound glycoprotein encoded by Mucin 1 and a marker of interstitial pneumonia, was also elevated to 1,409 mg/dl, but computed tomography of the lungs revealed no obvious pulmonary lesions. Previously reported studies showing that myeloma patients with elevated KL-6 might have a poor prognosis prompted us to treat this patient with a three-drug (bortezomib, cyclophosphamide, and dexamethasone: VCD) combination regimen. When 6 cycles of the regimen had been completed, no M-protein was detectable in her serum. Furthermore, κ free light chain had significantly decreased from 12,700 to 24.8 mg/l. In addition, 18F-FDG PET/CT revealed reduced mass sizes and no 18F-FDG uptakes by plasmacytomas. Thus, she was defined as having achieved a stringent complete remission (sCR). We therefore concluded that the VCD combination regimen was highly effective in this multiple myeloma patient with KL-6 elevation.
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Short Report
  • Hairy cell leukemia accompanied by Evans syndrome
    Shigeyuki EBARA, Mizuho KAGOSIMA, Mikio MARUMO, Yasusi ITO, Eiji TATUM ...
    2014 Volume 55 Issue 4 Pages 466-468
    Published: 2014
    Released on J-STAGE: May 20, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.466
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    We report a case of Western type hairy cell leukemia (HCL), a very rare leukemia in Japan. In this malignancy, leukemic cells in a peripheral blood film may be missed due in part to accompanying pancytopenia and in part to loss of typical cytoplasmic projections if prepared in a conventional Japanese way using forced air-drying. Our present patient also had a variety of autoantibodies and the clinical picture was primarily that of Evans syndrome (ES), suggesting disturbed immune responses associated with the HCL. Although HCL accompanied by either AIHA or ITP has been reported, the occurrence of ES in HCL is extremely rare.
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