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Acta Haematol [journal]
- Development of JAK2V617F-Positive Polycythemia Vera after Chemotherapy-Induced Remission of Primary Central Nervous System Diffuse Large B Cell Non-Hodgkin's Lymphoma: A Case Report and Review of the Literature. [JOURNAL ARTICLE]
- Acta Haematol 2013 May 7; 130(3):142-145.
The coexistence or the development of Philadelphia chromosome-negative myeloproliferative neoplasms after a lymphoproliferative disease in the same patient is an extremely rare event. We report the case of a 72-year-old man who developed JAK2V617F polycythemia vera 3 years after the diagnosis and treatment of primary diffuse large B cell non-Hodgkin's lymphoma of the central nervous system. We also review the literature regarding the pathogenesis underlying the association of myeloproliferative and lymphoproliferative chronic disorders.
- Inhalation as a Source of Iron in Secondary Iron Overload. [JOURNAL ARTICLE]
- Acta Haematol 2013 May 7; 130(3):138-141.
- Absence of Merkel Cell Polyomavirus in Monocytic Leukemias. [JOURNAL ARTICLE]
- Acta Haematol 2013 Apr 19; 130(3):135-137.
- B-Lymphoblastic Leukemia/Lymphoma Associated with t(8;13)(p11;q12)/ ZMYM2 (ZNF198)-FGFR1 : Rare Case and Review of the Literature. [JOURNAL ARTICLE]
- Acta Haematol 2013 Apr 16; 130(3):127-134.
Myeloid and lymphoid neoplasms with fibroblastic growth factor receptor-1 (FGFR1) abnormalities originate from mutated pluripotent stem cells and have a heterogeneous clinical presentation. There are 12 identified partner genes commonly involved in FGFR1 translocation at an 8p11 breakpoint. In FGFR1-related neoplasms, T-lymphoblastic lymphoma with eosinophilia is the most common clinical scenario, whereas acute B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is rare. To date, only 7 cases of B-ALL/LBL with FGFR1 abnormalities have been reported. Here, we report an additional case of a 64-year-old gentleman with leukocytosis, eosinophilia and diffuse mediastinal and general lymphadenopathy. Bone marrow examination showed patchy infiltrates of immature precursors/blasts, along with myeloid/eosinophilic hyperplasia. Immunophenotyping confirmed increased B lymphoblasts (30-40%). Karyotyping revealed cytogenetic abnormalities, including t(8;13)(p11;q12)/ZMYM2 (ZNF198)-FGFR1 and trisomy 21. The patient did not respond to hyper-CVAD chemotherapy and within 4 months developed acute myelomonocytic leukemia and expired 11 months after the initial diagnosis. Similar cases from the literature are reviewed.
- Characterization of a Novel Mutation in the NADH-Cytochrome b5 Reductase Gene Responsible for Rare Hereditary Methaemoglobinaemia Type I. [JOURNAL ARTICLE]
- Acta Haematol 2013 Apr 13; 130(2):122-125.
- Quantitative Comparison of CDKN2B Methylation in Pediatric and Adult Myelodysplastic Syndromes. [JOURNAL ARTICLE]
- Acta Haematol 2013 Apr 3; 130(2):115-121.
Background/Aims:Transcriptional repression of tumor suppressor genes is determined by the quantity of promoter hypermethylation. We analyzed the methylation quantity of CDKN2B in pediatric myelodysplastic syndromes (MDS).
Methods:Quantitative measurement of CDKN2B methylation was performed in 25 pediatric MDS patients and 12 controls using pyrosequencing, and the result was compared with those from 74 adult MDS cases and 31 adult controls. The association between CDKN2B methylation quantity and factors related to prognosis including bone marrow blast percentage and karyotype was analyzed.
Results:Pediatric MDS patients showed a higher methylation level (MtL) of CDKN2B than pediatric controls (2.94 vs. 1.62; p = 0.031) but a lower level than adult MDS patients (8.76; p < 0.001). MtL was higher in pediatric MDS cases with >5% blasts than in pediatric controls (3.78 vs. 1.62; p = 0.052). Pediatric MDS cases with abnormal karyotype showed a higher MtL than pediatric controls (5.95 vs. 1.62; p = 0.045).
Conclusions:We confirmed that methylation of CDKN2B is associated with the pathogenesis and prognosis in pediatric MDS. The difference in MtLs between pediatric and adult MDS might be related to the physiological hypermethylation of tumor suppressor genes in aging.
- Dasatinib Maintenance Therapy after Allogeneic Hematopoietic Stem Cell Transplantation for an Isolated Central Nervous System Blast Crisis in Chronic Myelogenous Leukemia. [JOURNAL ARTICLE]
- Acta Haematol 2013 Mar 29; 130(2):111-114.
A 22-year-old male with Ph-positive chronic myelogenous leukemia (CML) was started on treatment with imatinib. After 12 months of therapy, he achieved a complete cytogenetic response (CCyR). Although the CCyR persisted in his bone marrow, he developed an isolated CML blast crisis in his central nervous system (CNS) after 29 months of therapy. He underwent allogeneic hematopoietic stem cell transplantation (HSCT) following combination therapy with dasatinib, intrathecal chemotherapy and cranial irradiation. Subsequently, 168 days after allogeneic HSCT, he was started on dasatinib maintenance therapy to prevent a CNS relapse. Thirty-eight months after allogeneic HSCT, he has sustained a complete molecular response in both bone marrow and CNS. We believe dasatinib has the potential to prevent CNS relapse if used for maintenance therapy after allogeneic HSCT.
- Taming of the Shrew - Overcoming Extramedullary Blast Crisis in the Era of the New Tyrosine Kinase Inhibitors. [EDITORIAL]
- Acta Haematol 2013 Mar 29; 130(2):108-110.
- Specific Binding of Paraprotein to Platelet Receptors as a Cause of Platelet Dysfunction in Monoclonal Gammopathies. [JOURNAL ARTICLE]
- Acta Haematol 2013 Mar 28; 130(2):101-107.
The study included 48 untreated patients with monoclonal gammopathies (MG). Paraprotein was isolated from the serum of 10 patients with decreased platelet aggregation. Platelet aggregation was measured before and after the addition of the isolated paraprotein to platelet-rich plasma (PRP) from 10 healthy donors, in vitro. Expression of platelet von Willebrand factor (vWF) receptor glycoprotein (GP)Ib and platelet collagen receptor GPVI was determined by flow cytometry in the PRP of healthy donors before and after the addition of isolated paraprotein using the monoclonal antibodies, CD42b (for GPIb) and CD36 (for GPVI). Flowcytometry showed that expression of CD42b and CD36 positive cells was reduced after the addition of isolated paraprotein to PRP from healthy donors (p < 0.001). These investigations demonstrated that paraprotein causes platelet dysfunction in patients with MG due to specific binding to the platelet vWF receptor GPIb and platelet collagen receptor GPVI.
- Plasma Cell Neoplasms Showing Multilobulated Nuclei. [JOURNAL ARTICLE]
- Acta Haematol 2013 Mar 27; 130(2):98-100.