Clinical analysis of 52 patients with granular lymphocyte proliferative disorder (GLPD) showed frequent anemia in indolent T-cell GLPD in Japan.

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/microL 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.

[Gaucher disease type I diagnosed at 63 years old presenting with thrombocytopenia].

A 63-year-old man who was incidentally found to have thrombocytopenia at a periodic physical examination visited our hospital. The spleen was palpable 3 finger-breadths below the navel level, and the liver was palpable 1 finger-breadth below the right costal margin. Peripheral blood examination showed WBC 2,900/microl, Hb 13.4 g/dl, and platelets 54 X 10(3)/ microl. Gaucher cells were recognized in the bone marrow by aspiration, and serum levels of total acid phosphatase and angiotensin converting enzyme were increased. Glucocerebrosidase activity was lower than the control level in bone marrow stroma cells, and modification of glucocerebrosidase genotype N188S was shown, which had been identified in the past. Furthermore, neurological examination was normal. Based on these results, we diagnosed the patient with Gaucher disease type I, and started enzyme replacement therapy. Gaucher disease is rare in Japanese, approximately 100 cases having been reported; diagnosis at older age is also relatively rare and, as far as we know, the oldest age reported in Japanese was 57 years old. Gaucher disease should be considered a differential diagnosis when thrombocytopenia and splenomegaly are found in elderly patients, although it is relatively rare.