It has been recognized that some patients with myelodysplastic syndromes (MDS) develop immunologic abnormalities, but little is known of its correlations to MDS-specific disease features. In a retrospective study of 284 MDS patients, we identified 32 patients (11.3%) with clinical or serologic immunological abnormalities (group A) and compared them to the remaining 252 cases (group B). Group A consisted of 20 patients with clinical signs of autoimmune disease and 12 asymptomatic patients with serologic immunological abnormalities only. Apart from significant female predominance in group A (M/F = 2.5 vs M/F = 0.7, p = 0.001), the other clinical and biological features such as median age, distribution of MDS subtypes, incidence of karyotyopic abnormalities, "abnormal" in vitro growth of GM-progenitors and survival times were similar in the two groups. Autoimmune manifestations partially responded to immunosuppressive therapy, with moderate improvement of peripheral cytopenia. In addition, CD3(+), CD4(+), CD8(+), CD19(+), and CD56(+) cells were quantified in peripheral blood of 38 patients. Matched with similarly aged healthy control group, most MDS patients showed significant lymphocytopenia, mainly due to the reduction of T-helper series (in both absolute numbers and percentage). B-cells were reduced in absolute numbers, but their percentage still overlapped with the control. No major abnormalities of natural killer cells (CD56(+)) were seen. We conclude that autoimmune diseases and asymptomatic immunologic abnormalities are common in patients with MDS, but except for female predominance, no correlation between these abnormalities and MDS-specific disease features were found.
B-Chronic lymphocytic leukaemia (B-CLL) is a monoclonal malignancy characterized by an accumulation of terminally differentiated small and anergic B lymphocytes in the blood, bone marrow and other tissues. CD23 antigen, a trans-membrane glycoprotein, promotes the activation and proliferation of normal B lymphocytes and has an important role in the process of malignant transformation in B-CLL. This retrospective cohort study of 77 consecutive newly diagnosed B-CLL patients, 43 males, 34 females, median age of 62 years, examined CD23 expression and correlations with clinical parameters. CD23+ was negatively correlated with pro-lymphocyte infiltration of the bone marrow (P<0.01) and peripheral blood lymphocyte counts (P<0.001). Lower CD23 expression was correlated with lower serum immunoglobulin levels (P<0.05), especially IgG; while greater CD23 expression was positively correlated with higher CD5 levels. B-CLL patients with a percentage of CD23+ lymphocytes >40% had longer survival (92.8 months) than those expressing <40% (35.3 months) (P=0.001). CD23 is not uniformly expressed by lymphocytes in B-CLL patients, and the differences in expression are dependent on a number of clinical parameters, including the peripheral blood lymphocyte count and the degree of pro-lymphocyte infiltration of the bone marrow. CD23 expression is significantly decreased in patients with extremely high lymphocyte counts (PBL counts of >100 x 10(9)/l) and in the advanced stages of disease.
A retrospective survey of 210 consecutive patients aged ≥ 65 years (median age 69 years, range 65-88 years) with acute myeloid leukemia (AML) diagnosed at a single center over a 6-year period (January 2001 to December 2006) is presented. De novo AML was diagnosed in 179 (85.2 %) patients and 31 (14.7 %) patients had a secondary AML. Twenty-three patients had M0 (11 %), 36 M1 (17.15 %), 57 M2 (27.1 %), eight M3 (3.8 %), 45 M4 (21.4 %), 31 M5 (14.8 %), one M6 (0.5 %), one M7 (0.5 %), and eight patients had unclassified myeloid leukemia (3.8 %) according to French-American-British (FAB) Study Group Classification. Eight patients with M3 (acute promyelocytic leukemia) were excluded from the study. Cytogenetic analysis was performed in 172/202 (85 %) patients. The normal karyotype was found in 81/172 (47 %), high risk aberrations in 32/172 (18.6 %), and favorable karyotype in 13/172 (7.5 %) patients. Supportive and palliative therapies were applied in 115 (56.9 %) patients, a no induction chemotherapy (NIC) group, and 87 (43.1 %) patients received induction chemotherapy (IC group). Complete remission (CR) was achieved in 45/87 (51.7 %) in the IC group and in 5/115 (4.3 %) in the NIC group of patients. After a median follow up of 4 years, 194 (96 %) patients died. The variables significantly associated with a longer overall survival (OS) by univariate analysis were an age of <75 years, a better ECOG performance status (PS) (p = 0.000, CI 95.0 %, 1.358-2.049), a serum LDH activity <600 U/l (p = 0.000, CI 95.0 %, 1.465-2.946), lower white blood cell (WBC) count at diagnosis (p = 0.011, CI 95.0 %, 1.102-2.100), lower comorbidity HCT-CI index (p = 0.000, CI 95 % 2.209-3.458), absence of splenomegaly (p = 0.015, CI 95.0 %, 1.082-2.102) and hepatomegaly (p = 0.008, CI 95.0 %, 1.125-2.171), and no preceding nonhematological malignancy. Multivariate analysis showed that significant factors affecting OS in the IC group were achievement of CR (p = 0.000), the ECOG PS (p = 0.045) and the ECOG PS (p = 0.000), and HCT-CI (p = 0.000) in the NIC group of elderly patients. The present study suggests that a subgroup of elderly patients with both ECOG PS and HCT-CI ≤ 2 at presentation may be eligible for intensive induction chemotherapy.
Myeloid sarcoma (MS) is
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