Oral cyclophosphamide was effective for Coombs-negative autoimmune hemolytic anemia in CD16+CD56- chronic lymphoproliferative disorder of NK-cells.

Abstract

An 84-year-old woman was referred to our hospital presenting anemia. Her hemoglobin level was 5.8 g/dL, and white blood cell count was 9400/μL, consisting of 82% lymphocytes. Given the lymphocyte phenotype (CD2+, CD3-, CD16+, and CD56-) and negative whole blood EBV viral load, we made a diagnosis of chronic lymphoproliferative disorder of NK cells (CLPD-NK). We suspected hemolytic anemia because of the high levels of reticulocytes in the peripheral blood and the low haptoglobin value. Although the direct Coombs test was negative and there was no cold agglutination, we examined her red-blood-cell-bound IgG (RBC-IgG), which was elevated. She was diagnosed as having as Coombs-negative autoimmune hemolytic anemia (AIHA). We report the effectiveness of oral cyclophosphamide for Coombs-negative autoimmune hemolytic anemia in CLPD-NK.

• Authors: Nodoka Sekiguchi, Sayaka Nishina, Toru Kawakami, Hitoshi Sakai, Noriko Senoo, Yasushi Senoo, Toshiro Ito, Hiroshi Saito, Hideyuki Nakazawa, Tomonobu Koizumi, Fumihiro Ishida
• Journal: International journal of hematology (Int J Hematol) Vol. 105 Issue 6 Pg. 854-858 (Jun 2017) ISSN: 1865-3774 [Electronic] Japan
• PMID: 28028749 (Publication Type: Case Reports, Journal Article)

Chemical References

• Autoantibodies
• CD56 Antigen
• FCGR3B protein, human
• GPI-Linked Proteins
• Immunoglobulin G
• NCAM1 protein, human
• Receptors, IgG
• Cyclophosphamide

Topics

• Aged, 80 and over
• Anemia, Hemolytic, Autoimmune (blood, diagnosis, drug therapy)
• Autoantibodies (blood)
• CD56 Antigen (blood)
• Chronic Disease
• Cyclophosphamide (administration & dosage)
• Female
• GPI-Linked Proteins (blood)
• Humans
• Immunoglobulin G (blood)
• Killer Cells, Natural (metabolism)
• Lymphoproliferative Disorders (blood, diagnosis, drug therapy)
• Receptors, IgG (blood)
• Reticulocytes (metabolism)