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Clinical analysis of 52 patients with granular lymphocyte proliferative disorder (GLPD) showed frequent anemia in indolent T‐cell GLPD in Japan
Author(s) -
Kawahara Shimpei,
Sasaki Makoto,
Isobe Yasushi,
Ando Jun,
Noguchi Masaaki,
Koike Michiaki,
Hirano Takao,
Oshimi Kazuo,
Sugimoto Koichi
Publication year - 2009
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2009.01213.x
Subject(s) - lymphocytosis , medicine , cd8 , anemia , lymphocyte , hepatosplenomegaly , immunology , gastroenterology , pathology , immune system , disease
We present here clinical and hematological findings of 52 cases of granular lymphocyte‐proliferative disorder (GLPD), which contained 35 indolent T‐cell lineage granular lymphocyte‐proliferative disorder (T‐GLPD), two atypical T‐GLPD, 12 chronic NK‐cell lymphocytosis (CNKL), and three aggressive NK‐cell leukemia (ANKL). The median period of follow up was 24 months. Hemoglobin level <8.0 g/dL was recognized in 21 cases of indolent T‐GLPD (60%), among which 15 patients met the criteria of pure red cell aplasia. Neutrophil counts <500/μL occurred only in two cases of T‐GLPD (6%). Although the median age and male‐to‐female distribution were similar, very frequent anemia and rare neutrocytopenia in indolent T‐GLPD in the present study keenly contrasted with previous reports. CD56 was positive in three of 29 indolent T‐GLPD cases with CD4−CD8+ phenotype, in three of four CD4+CD8−, and in none of two CD4−CD8− cases. Therefore, although two atypical T‐GLPD cases were CD56‐positive, CD56 should not be a specific marker for aggressive T‐GLPD. All CNKL patients had a chronic course with a stable granular lymphocyte count. All three ANKL patients presented high fever and hepatosplenomegaly, barely responded to chemotherapies and died within 6 months. The present analysis of 52 cases of GLPD in Japan showed that Japanese and Western cases of indolent T‐GLPD clearly differ in their hematological complications.