急性単球性白血病を発症したピルビン酸キナーゼ欠乏症の1例

Abstract

A case of pyruvate kinase deficiency anemia associated with acute monocytic leukemia is reported with a review of the literature. A 57-year-old man who was treated for diabetes mellitus at a nearby hospital was admitted to our hospital for precise examination of the progression of anemia. Blood examination showed RBC 207×10⁴/μl, Hb 5.9 g/dl, Ht 18%, reticulocytes 134‰, WBC 5,200/μl, platelets 9.3×10⁴/μl, indirect bilirubin 0.6 mg/dl, serum iron 112 μg/dl, haptoglobin under 10 μg/dl. After admission to our hospital, serum indirect bilirubin level was increased to 2.3 mg/dl, and pancytopenia was noted in the peripheral blood. the half life span of erythrocytes had shortened to 16 days. A bone marrow examination revealed prominent erythroid hyperplasia. More detailed examination of this hemolysis revealed pyruvate kinase deficiency of erythrocytes. Three months after admission, blast cells were noted in the peripheral blood and they increased progressively. The bone marrow contained 43.6% of blast cells. Subsequently, a diagnosis of acute monocytic leukemia was made. Although intensive chemotherapy was performed, the patient achieved no remission and died 6 months after admission to our hospital.