Copyright (c) 2013 S. Karger AG, Basel
t(8;22)(p11;q11) is a rare but recurrent chromosome translocation selleck chemical Ponatinib that has been reported in 11 cases of myeloproliferative neoplasm or B-acute lymphoblastic leukemia. This translocation results in an in-frame fusion of FGFR1 on 8p11 and BCR on 22q11, and causes constitutive activation of the tyrosine kinase of the BCR/FGFR1 chimera protein. Here, we report the twelfth case of hematological tumor bearing t(8;22)(p11;q11). The bone marrow showed hypoplastic and tri-lineage dysplasia with 24.4% abnormal cells. The abnormal cells were not defined as myeloid or lymphoid morphologically, lacking a myeloperoxidase reaction. Flow cytometric analysis of the bone marrow cells revealed that the abnormal cells expressed CD13, CD33, CD34, and CD19, and that a fraction of the abnormal cells was positive for CD10.

Southern blot analysis of the bone marrow cells showed rearrangement of the immunoglobulin heavy chain gene, a genetic hallmark of B-cell differentiation. Previously reported cases with t(8;22)(p11;q11) suggested an association between myeloid and B-lymphoid tumors, whereas other chromosome translocations involving FGFR1 on 8p11 showed a link between myeloid and T-lymphoid tumors. Our observation supports that t(8;22)(p11;q11) might define a dual myeloid and B-lymphoid disorder. Copyright (c) 2013 S. Karger AG, Basel
Background: The International Prognostic Staging System (IPSS) for myelodysplastic syndromes (MDS) was developed predominately in patients of European ancestry and is not validated in Asians.

In a recently revised IPSS (IPSS-R), several new prognostic variables are included, i.e. age, Eastern Cooperative Oncology Group performance score (ECOG PS), serum ferritin and lactate dehydrogenase. Chinese with MDS offer a unique opportunity to distinguish the prognostic impacts of haemoglobin (HGB) concentration, red blood cell (RBC) transfusions and serum ferritin because in China, patients rarely receive RBC transfusions unless the HGB concentration is < 6.0 g/dl. Methods: We studied prognostic variables in 191 untreated Chinese primary patients with MDS intermediate-1 (INT-1) in the IPSS. Results: Serum ferritin level >= 500 AV-951 mu g/l at diagnosis was a strong independent predictor of survival. Although baseline serum ferritin level was inversely correlated with baseline HGB, it was the serum ferritin, not the baseline HGB level, that was significantly correlated with survival in Chinese patients.

A new prognostic scoring system including the ECOG PS, absolute neutrophil level, serum ferritin, percentage of bone marrow blasts and poor karyotype was developed for Chinese with IPSS INT-1 MDS. Conclusions: This revised scoring system identified a subgroup of Chinese with MDS and INT-1 IPPS who have a poor prognosis our site and may benefit from more intensive therapy.