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International Journal of Hematology

, Volume 78, Issue 2, pp 149–153 | Cite as

Myeloid/Natural Killer Cell Precursor Acute Leukemia Accompanied by Homozygous Protein C Deficiency

  • Takashi Shimamoto
  • Akihiro Nakajima
  • Tomoko Katagiri
  • Yoshikazu Ito
  • Kazuma Ohyashiki
Case Report

Abstract

A patient with myeloid/natural killer (NK) cell precursor acute leukemia who was also homozygous for protein C deficiency was treated and showed a complete remission while he simultaneously received low molecular weight heparin. He presented with fever spikes, lymphadenopathy, and a bulky tumor of the anterior mediastinum.A bone marrow aspirate showed the infiltration of immature lymphoblastoid cells. The patient’s diagnosis was determined to be myeloid/NK cell precursor acute leukemia by morphologic and immunophenotypic analysis (CD7+CD33+CD34+CD56+). The patient developed a thrombosis in his jugular vein on cannulation of the internal jugular vein. An examination of the serum levels and the activities of proteins C and S demonstrated a slight decrease in the protein C level but an undetectable protein C activity.The patient received the diagnosis of homozygous protein C deficiency, because both parents were found to have heterozygous protein C activity. Treatment of the patient’s leukemia included induction chemotherapy (Ara-C and idarubicin) with concomitant administration of low molecular weight heparin for his homozygous protein C deficiency. He achieved a complete remission without expressing any thrombosis during the course of chemotherapy.To our knowledge, this is the first case ever described in which acute myeloid leukemia was complicated with homozygous protein C deficiency. Int J Hematol. 2003;78:149-153.

Key words

Myeloid/natural killer cell precursor acute leukemia Homozygous protein C deficiency Low molecular weight heparin 

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Copyright information

© The Japanese Society of Hematology 2003

Authors and Affiliations

  • Takashi Shimamoto
    • 1
  • Akihiro Nakajima
    • 1
  • Tomoko Katagiri
    • 1
  • Yoshikazu Ito
    • 1
  • Kazuma Ohyashiki
    • 1
  1. 1.First Department of Internal MedicineTokyo Medical UniversityTokyoJapan

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