Journal Article

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

ITO Yoshikazu, SHIMAMOTO Takashi, KATAGIRI Tomoko, NAKAJIMA Akihiro, OHYASHIKI Kazuma

2003

Overview

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.

• Publisher: Springer,Springer Nature B.V
• Subject: Acute Disease / Adult / Antibiotics, Antineoplastic - therapeutic use / Anticoagulants - therapeutic use / Antimetabolites, Antineoplastic - therapeutic use / Biological and medical sciences / Cytarabine - therapeutic use / Hematologic and hematopoietic diseases / Heparin, Low-Molecular-Weight - therapeutic use / Homozygote / Homozygous protein C deficiency / Humans / Idarubicin - therapeutic use / Killer Cells, Natural - pathology / Leukemia, Myeloid - complications / Leukemia, Myeloid - drug therapy / Leukemia, Myeloid - pathology / Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis / Low molecular weight heparin / Male / Medical sciences / Myeloid Cells - pathology / Myeloid natural killer cell precursor acute leukemia / Neoplastic Stem Cells - pathology / Pedigree / Platelet diseases and coagulopathies / Protein C Deficiency - complications / Protein C Deficiency - drug therapy / Protein C Deficiency - genetics