A favorable prognosis and a low rate of leukemic transformation has been attributed to the 5q- syndrome, a myelodysplastic syndrome (MDS) characterized by macrocytic anemia, hypolobulated micromegakaryocytic hyperplasia, and an interstitial deletion of chromosome 5. We examined the characteristics and outcome of 43 consecutive patients in our institution strictly defined by morphologic criteria...

BACKGROUND In March 1997 an international workshop introduced a new International Prognostic Scoring System (IPSS) for MDS. The goal of the present study was to apply the IPSS to a large group of MDS patients from one centre and to compare it to the FAB-classification. PATIENTS One hundred eighty-four MDS patients were included on the basis of similar criteria as used by the workshop but some...

of viable CD41+ cells be harvested at day 14 of culture. Bcl-xL mRNA was detected at high levels in all samples of RAEB CD34+ cells at day 0, and it appeared progressively down-regulated at days 7 and 14 of culture (Figure 2A). Moreover, the Bcl-xL expression pattern was similar in control cultures and in cultures containing anti-TGF-β1 neutralizing antibody (Figure 2B). The deregulated express...

Granulocytic sarcoma (GS) is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. Most GS reported in large series were not associated with overt acute myelogenous leukemia. Gastric perforation occurred during prednisolone therapy in a 72-year-old Japanese male with a four-month history of a myelofibrosis-like state. Subtotal gastrectomy was performed for a su...

A cohort of MDS patients was examined for mutations affecting 4 splice genes (SF3B1, SRSF2, ZRSR2, and U2AF35) and evaluated in the context of clinical and molecular markers. Splice gene mutations were detected in 95 of 221 patients. These mutations were mutually exclusive and less likely to occur in patients with complex cytogenetics or TP53 mutations. SF3B1(mut) patients presented with lower ...

Acute myeloid leukemia (AML) is a myeloid malignancy that arises spontaneously or that may evolve from myelodysplastic syndrome (MDS). AML is characterized by somatic cytogenetic and molecular mutations associated with distinct clinical outcomes. In patients with normal cytogenetics, genetic techniques have been used to discover novel mutations. In order to identify new candidate mutations invo...

Haemosiderosis bulbi is a degenerative condition of the eye bulb caused by the toxic effects of an intracellular accumulation of haemosiderin. Haemosiderin is a product of the decomposition of haemoglobin. The most common and severe damage takes place in the epithelial cells of the eye tissues. Haemosiderosis bulbi is a complication of the long existing haemophthalmus, the intravitreal bleeding...

Azacitidine (AZA), as demonstrated in the phase III trial (AZA-001), is the first MDS treatment to significantly prolong overall survival (OS) in higher risk MDS pts ((2007) Blood 110 817). Approximately, one-third of the patients (pts) enrolled in AZA-001 were FAB RAEB-T (≥20-30% blasts) and now meet the WHO criteria for acute myeloid leukaemia (AML) ((1999) Blood 17 3835). Considering the poo...

Aplastic anemia (AA) may evolve into clonal diseases like myelodysplastic syndrome (MDS) and acute myeloblastic leuke¬mia (AML). Monosomy 7 is a poor prognostic chromosomal abnormality commonly associated with therapy related MDS and secondary AML. It has also been associated with leukomogenic transformation in AA. We present here two adult ma¬le patients with acquired severe AA. Both patients ...