ABSTRACT
<p><b>OBJECTIVE</b>To analyse the clinical feature and natural course of essential thrombocythemia (ET).</p><p><b>METHODS</b>A retrospective analysis was conducted in ET patients treated in our hospital during May 1980 to December 2006.</p><p><b>RESULTS</b>Four hundred and thirty eight patients (201 males and 237 females with a median age of 48 years) were diagnosed. Hemorrhage occurred in 101 cases (23.1%), thrombosis in 86 cases (19.6%), and both hemorrhage and thrombosis in 13 cases (3.0%). Splenomegaly occurred in 150 cases and hepatomegaly occurred in 60 cases. One hundred and forty-nine cases (34%) had no symptoms at diagnosis and 145 cases (33.1%) confirmed by routine blood tests due to other diseases. The median platelet count at diagnosis was 1000 x 10(9)/L [(533 -3740) x 10(9)/L]. Bone marrow biopsy was performed in 255 cases which showed mainly increase of enlarged mature megakaryocytes with hyper-lobulated nuclei and local proliferation of reticular fiber was revealed in 51 cases. JAK2V617F mutation was detected in 90(78.9%) of 114 patients studied. Karyotype analysis was performed in 180 cases and 6 (3.3%) had clonal chromosomal aberrations. Two hundred and sixty-one patients were followed up over 12 months with a median of 60 months (range from 12 to 300 months). Seventeen cases (6.5%) evolved into marrow fibrosis (MF) and one case into polycythemia vera (PV). One case evolved into PV 6 years and then MF 20 years after diagnosis of ET. Three cases developed acute monocyte leukemia (M5), myelodysplastic syndrome (MDS) and multiple myeloma (MM), respectively.</p><p><b>CONCLUSIONS</b>ET is a chronic myeloproliferative disorder characterized predominantly by thrombocytosis and hemorrhage. The percentage of asymptomatic cases is high. The prognoses for most cases were good with a few cases may evolve into MF.</p>
Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Follow-Up Studies , Prognosis , Retrospective Studies , Thrombocythemia, Essential , Genetics , PathologyABSTRACT
<p><b>BACKGROUND</b>Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with chronic ITP. This study aimed at defining the treatment outcome and prognostic factors for chronic ITP based on a large cohort of Chinese patients followed up for over 25 years at a single center.</p><p><b>METHODS</b>The medical records of 1791 patients aged 14 years or older who were diagnosed as having chronic ITP at our hospital from 1974 to 1999 were retrospectively analyzed.</p><p><b>RESULTS</b>The female-to-male ratio was 2:1, with a median age of 34 years (ranging from 14 to 80 years), median platelet count of 38 x 10(9)/L [range (1-99) x 10(9)/L], and median follow-up of 36 months (range 1-220 months). Steroids were used in 689 patients, among them 209 (30.3%) achieved complete remission (CR). A splenectomy was performed in 124 patients, and response to steroid pre-splenectomy was not available in 14 patients. The CR rate after a splenectomy was lower in steroid nonresponders (29 of 90, 32.2%) than in those who relapsed after successful steroid treatment (12 of 20, 60.0%) (P < 0.05). In comparison with patients negative for antinuclear antibody (ANA), those who were ANA positive had similar responses to steroids, but a significantly shorter remission period after a splenectomy (P < 0.01).</p><p><b>CONCLUSIONS</b>Adult Chinese chronic ITP patients can have long-term remission after steroid therapy and splenectomies. Primary steroid refractoriness is a prognostic factor predicting poor subsequent response to a splenectomy.</p>