Premium
Cyclophosphamide therapy for pure red cell aplasia associated with granular lymphocyte‐proliferative disorders
Author(s) -
YAMADA OSAMU,
MIZOGUCHI HIDEAKI,
OSHIMI KAZUO
Publication year - 1997
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1997.282672.x
Subject(s) - cyclophosphamide , pure red cell aplasia , lymphocyte , aplasia , clone (java method) , medicine , immunology , biology , chemotherapy , bone marrow , genetics , dna
Granular lymphocytes have been characterized as cells with azurophilic granules in the cytoplasm. Patients with increased numbers of granular lymphocytes are designated as granular lymphocyte‐proliferative disorders (GLPDs). A variety of haematological abnormalities are associated with T‐cell‐lineage GLPD. Among these, pure red cell aplasia is frequent, and adequate therapy is required. Seven patients with pure red cell aplasia, or a related condition complicating T‐cell‐lineage GLPD, were entered into this study. Cyclophosphamide was initiated at a daily oral dose of 100 mg. After 2 weeks the dose was reduced to 50 mg/d, and maintained at that dose. Cyclophosphamide was administered until the lymphocyte count was <1 ×10 9 l and T‐cell receptor‐β gene analysis was used to monitor the response to treatment. All the patients were successfully treated, irrespective of their former treatment. Clinical remission was associated with the disappearance of the abnormal granular lymphocyte clone, as detected by Southern blot hybridization analysis. Therapeutic responses began after 8 weeks, and clinical complete remissions were obtained after 6 months. Oral cyclophosphamide monotherapy can successfully treat the pure red cell aplasia associated with T‐cell‐lineage GLPD.