Myelodysplastic Syndrome in a Patient with Familial Pelger-Huet Anomaly

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Other Title
  • 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 1.8×104l, 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

  • Rinsho Ketsueki

    Rinsho Ketsueki 32 (11), 1453-1457, 1991

    The Japanese Society of Hematology

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