Panagiotis Tsaftaridis

CD138 Expression Helps Distinguishing Waldenström's Macroglobulinemia (WM) From Splenic Marginal Zone Lymphoma (SMZL)

The distinction between WM and SMZL may be difficult since both entities share overlapping clinical, immunophenotypic, and histopathologic characteristics. In this context we evaluated whether CD138 expression could be added in the armamentarium of tools used to differentiate these entities. Sixty-nine patients were studied, 47 WM and 22 SMZL. Paraffin-embedded sections of bone marrow biopsies were quantitatively and qualitatively evaluated for CD138 expression. Sixty percent of WM cases expressed CD138 in contrast to 18% of SMZL patients. Intermediate/high intensity of CD138 expression was observed in 47% of WM while it was low in all SMZL patients. Differences between WM and SMZL regarding the intensity and the percentage of CD138 positive cells were both significant (P=.0008 and, .00021 respectively). Moreover, CD138 expression was related to serum IgM levels in WM patients (P=.0006). In conclusion, CD138 expression may constitute an additional aid in the distinction between WM and SMZL.

Carrier Screening and Prenatal Diagnosis of Hemoglobinopathies. A Study of Indigenous and Immigrant Couples in Northern Greece, Over the Last 5 Years

Hemoglobinopathies constitute the most frequent monogenic disorders worldwide and thalassemias are the most frequent genetic disorders in Greece. Over a 5-year period (2002–2006), 1,375 couples were screened for hemoglobinopathies and counseled at our Thalassaemia Prevention Unit, Hippokration Hospital, Thessaloniki, Greece. In 148 cases (10.7%), both partners carried an abnormal hemoglobin (Hb) gene and genetic counseling was offered. One hundred out of 116 pregnancies were at-risk of giving birth to an offspring carrying either the homozygous or double heterozygous forms of the mutations under discussion. The remaining 16 pregnancies involved couples who were heterozygous for mutations that did not cause severe clinical disease, and were exempted from prenatal diagnosis. Twenty-six fetuses were found to be homozygotes or double heterozygotes for clinically significant mutations. These couples were informed of the danger of having an affected child but the termination or continuation of the pregnancy was left to the couples to decide. Nevertheless, all the couples preferred to terminate the pregnancies. The National Thalassaemia Prevention Programme has effectively decreased the incidence of thalassemia major and sickle cell syndromes in Greece.

Macrophage Inflammatory Protein‐1 alpha (MIP‐1α) is over‐expressed in a cohort of patients with myelodysplastic syndromes

To the Editor: Macrophage inflammatory protein-1 alpha (MIP1a) is a low molecular weight chemokine that belongs to the RANTES family and is primarily associated with cell adhesion and migration, being chemotactic for monocytes, T lymphocytes, natural killer cells, immature dendritic cells and granulocytes (1). Due to its activating effect on premature osteoclasts MIP-1a correlated with lytic bone disease in multiple myeloma (2). The MIP-1a has also been shown to inhibit the proliferation of primitive progenitors and has been implicated in the pathogenesis of acute and chronic myeloid leukaemia as well as adult T-cell leukaemia (3–6). Maciejewski et al. have reported very significant increases in the levels of MIP-1a RNA transcripts in the bone marrow nucleated cells from patients with aplastic anaemia and myelodysplastic syndromes (MDS) compared to normal individuals, suggesting that the expression of MIP-1a in bone marrow may reflect dysregulated cytokine production and activation of the immune system that may possibly contribute to disease progression (7). However, there is no information for the serum levels of MIP-1a in patients with MDS. The aim of this study was the evaluation of MIP-1a serum levels in MDS patients and the correlation with clinical and laboratory data. Therefore, MIP-1a was measured in the serum of 62 patients with MDS and 20 normal, ageand gender-matched individuals (control group) using an ELISA method (Quantikine ELISA, R & D Systems, Minneapolis, MN, USA). After veinpuncture, serum was separated within 4 h and stored at )70 C until the day of measurement. The MDS patients had a median age of 76.5 yr (range: 45– 91 yr). Thirty-three patients were males and 29 were females. The type of MDS according to recent WHO classification was as follows: 25 patients had refractory anaemia (RA), 20 patients had refractory cytopenia with multilineage dysplasia (RCMD), five patients had refractory anaemia with ringed sideroblasts (RARS), one patient had RCMD-RS, seven patients had refractory anaemia with excess of blasts type-I (RAEB-I), and four patients had RAEB-II. No patient had any kind of infection at the time of measurement and their medical records were reviewed to exclude any chronic disease or osteoporosis. Differences between patients and controls were evaluated using the Wilcoxon rank sum test. Associations between serum levels of MIP-1a and clinical or laboratory data were examined by the Kruskal–Wallis test, while the Spearman Rank correlation test was employed to examine relationships between various parameters. Differences between patients and controls were evaluated using the Wilcoxon rank sum test. All P values were two sided and confidence intervals refer to 95% boundaries. The MIP-1a serum levels presented no significant difference between patients and controls (median values ± SD: 29.21 ± 9.48 pg/mL vs. 25.10 ± 4.14 pg/mL, for patients and controls, respectively, P > 0.1). However, four MDS patients (6.4%), two with RA and two with RAEB-II, had at least a twofold higher concentration of MIP-1a in their serum compared to the mean value of control group. We found no correlation between MIP-1a levels and clinical or laboratory data of the patients, such as haemoglobin, neutrophil and platelet count, cytogenetic abnormalities, bone marrow blast count, lactate dehydrogenase serum levels, age, gender, number of transfusion requirements of red blood cells units, presence of organomegaly, IPSS or disease progression. Therapeutic decisions in patients with MDS are very complex due to the heterogeneity of this group of haematological disorders. The dilemma that confronts the management of MDS is illustrated by the presence of only one agent, which has been recently approved by the USA Food and Drug Administration with an indication for high-risk MDS, 5-azacytidine. Supportive care, low-intensity Eur J Haematol 2005: 75: 85–86 All rights reserved Copyright Blackwell Munksgaard 2005

Rituximab monotherapy in splenic marginal zone lymphoma: prolonged responses and potential benefit from maintenance

TO THE EDITOR: Treatment of splenic marginal zone lymphoma (SMZL) is not standardized due to the lack of prospective randomized trials.[1][1][⇓][2][⇓][3][⇓][4][⇓][5][⇓][6][⇓][7][⇓][8][⇓][9][⇓][10][⇓][11][⇓][12]-[13][13] After our initial 2007 paper, we now present updated data

Prognostic significance of deletion of the long arm of chromosome 20 in patients with myelodysplastic syndrome (MDS): a study of the Greek MDS Study Group

To the Editor: Deletion of the long arm of the chromosome 20 (del 20q) was observed in 10% of patients with polycythemia vera as well as in other myeloproliferative disorders (MPD) (1), in 4% of patients with myelodysplastic syndromes (MDS) (2) and in 1–2% of patients with acute myeloid leukemia (AML) (3). Del 20q is believed to arise in a pluripotent stem cell precursor of both myeloid and lymphoid cells (4) and to contribute to malignancy due to the loss of tumor suppressor genes. More than one gene on 20q is probably involved in leukemogenesis, which could account for the different clinical characteristics of myeloid disorders with del (20q) (5, 6). The 20q deletions are usually interstitial, involving bands q11.2– q13.1 and molecular studies have now identified two overlapping regions of deletion, one observed in patients with MDS/AML and one in patients with MPD (5). Translocations involving 20q are rare in myeloid malignancies with only occasional reports of unbalanced translocations involving chromosomes 1, 11, and 21 (7–9). According to the International Prognostic Scoring System (IPSS), MDS patients with del (20q) have good prognosis and a low transformation rate to acute leukemia (10, 11). The prognostic significance of del (20q) is not clear, because it has not been confirmed by other authors (12). Del (20q) as a sole aberration is associated with a favorable outcome while with additional aberrations in chromosomes 5 or 7, or both, predicts a relatively poor prognosis connected with shorter survival (13). MDS with hypereosinophilic syndrome and del (20q) has been reported with an indolent course (14). Duplication of del (20q) in a few MDS patients is of unknown prognostic significance while a t(17;20) in MDS and AML, involving genes on 17p and 20q seems to play a role in myeloid leukemogenesis (15). The aim of the study was to evaluate the clinical characteristics, risk of progression to acute leukemia, and survival in Greek MDS patients with del 20q. From 708 MDS patients, 28 patients with primary MDS and del 20q (23 patients with del 20q, as a sole and five patients, as a complex cytogenetic aberration), were identified. The study included 22 men and six women with a median age of 73.5 years (range: 37–87 years). Thirteen patients were diagnosed with refractrory anemia (RA), three with RA with ringed sideroblasts (RARS), six with RA with excess of blasts (RAEB-1), one with RAEB in transformation (RAEB-2), three with chronic myelomonocytic leukemia (CMML) and two patients were unclassified according to the last WHO classification. Nine patients were classified as low, 13 as intermediate-1, and six as intermediate-2 risk group according to IPSS. Fifty-six percent of this group presented with anemia, Hb £ 10 g/dL, and 88% with thrombocytopenia, platelet count 6 100 · 10/L. Peripheral blasts of 8% and 12% were identified in two patients with RAEB and RAEB-T, respectively. Levels of serum LDH were increased in two CMML and one RAEB-T patient. Six patients were red cell transfusion dependent (two packs of red cells per month). Three patients were platelet transfusion dependent every 2 weeks, and two patients were admitted to the hospital for neutropenic fever. No difference in the risk of leukemic evolution was observed in 28 patients with del 20q (25%) and the cohort of 680 patients without del 20q (30%). No significant difference in median or overall survival was observed between the two groups. The median survival, as shown in Figure 1, for 28 and 680