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- HIRAGA Hiroyuki
- Department of Immunohematology, Kobe City General Hospital
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- YABE Hiroki
- Department of Immunohematology, Kobe City General Hospital
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- NAGAI Kenichi
- Department of Immunohematology, Kobe City General Hospital
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- NAKAYAMA Shiro
- Department of Immunohematology, Kobe City General Hospital
Bibliographic Information
- Other Title
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- 遺伝性Pelger-Huet核異常症患者に発症したMyelodysplastic Syndromeの1例
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Description
This paper reports on a patient with congenital Pelger-Huet anomaly who developed myelodysplastic syndrome (MDS). A 45-year-old female was referred for investigation of pancytopenia of 6 months' duration. Hereditary Pelger-Huet anomaly was diagnosed by family study 7 years prior to admission. On admission, Hb was 6.5 g/dl, Ht 19.9%, Platelets 1.8×104/μl, and WBC 1,200/μl with 2% myelocytes, 9% metamyelocytes, 14% bands, 2% segmented neutrophils, 58% lymphocytes and 5% monocytes. Most of the granulocytes were Pelger-Huet type with strikingly clumped nuclear chromatin. Bone marrow aspirate demonstrated 3.6% blasts and dysplastic changes including megaloblastoid features in erythroid series and micro-megakaryocytes compatible with refractory anemia, a subtype of MDS. The association of hereditary Pelger-Huet anomaly and MDS is discussed.
Journal
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- Rinsho Ketsueki
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Rinsho Ketsueki 32 (11), 1453-1457, 1991
The Japanese Society of Hematology