ISRN Hematology最新文献

Primary dural lymphoma is a rare disease with more indolent clinical behavior compared to primary central nervous system lymphoma. The majority of the reported cases were indolent marginal zone lymphoma subtype with more predilections to the spine. Herein, we are presenting a case of intracranial, diffuse large B-cell lymphoma of the dura that was diagnosed and treated at our institution. We are presenting the challenges in the treatment based on a review of the literature.

We report some observations from our laboratory practice that might be important for the treatment of sickle cell disease (SCD). We describe data from two cases indicating that iron depletion might have a beneficial effect diminishing the formation of HbS in favor of HbF, possibly reducing the severity of the disease. We believe that it would be worthwhile to monitor the course of the disease comparing cases with identical genotypes with and without iron depletion, and we advise to consider chelation therapy to reduce iron overload in patients with SCD.

Anthracycline-based chemotherapy remains standard treatment for peripheral T-cell lymphoma (PTCL) although its benefits have been questioned. We performed systematic literature review and meta-analyses examining the complete response (CR) and overall survival (OS) rates for patients with PTCL. The CR rate for PTCL patients ranged from 35.9% (95% CI 23.4-50.7%) for enteropathy-type T-cell lymphoma (ETTL) to 65.8% (95% CI 54.0-75.9%) for anaplastic large cell lymphoma (ALCL). The 5-year OS was 38.5% (95% CI 35.5-41.6%) for all PTCL patients and ranged from 20.3% (95% CI 12.5-31.2%) for ETTL to 56.5% (95% CI 42.8-69.2%) for ALCL. These data suggest that there is marked heterogeneity across PTCL subtypes in the benefits of anthracycline-based chemotherapy. While anthracyclines produce CR in half of PTCL patients, this yields reasonable 5-year OS for patients with ALCL but not for those with PTCL-NOS or ETTL. Novel agents and regimens are needed to improve outcomes for these patients.

Background. Most orthopaedic units do not have a policy for reversal of anticoagulation in patients with hip fractures. The aim of this study was to examine the current practice in a district general hospital and determine difference in the time to surgery, if any, with cessation of warfarin versus cessation and treatment with vitamin K. Methods. A retrospective review of the case notes between January 2005 and December 2008 identified 1797 patients with fracture neck of femur. Fifty seven (3.2%) patients were on warfarin at the time of admission. Patients were divided into 2 groups (A and B). Group A patients (16/57; 28%) were treated with cessation of warfarin only and group B patients (41; 72%) received pharmacological therapy in addition to stopping warfarin. Time to surgery between the two groups was compared. Results. The mean INR on admission was 2.9 (range 1.7-6.5) and prior to surgery 1.4 (range 1.0-2.1). Thirty eight patients received vitamin K only and 3 patients received fresh frozen plasma and vitamin K. The average time to surgery was 4.4 days in group A and 2.4 days in group B. The difference was statistically significant (P < .01). Conclusion. Reversal of high INR is important to avoid significant delay in surgery. There is a need for a national policy for reversing warfarin anticoagulation in patients with hip fractures requiring surgery. Vitamin K is safe and effective for anticoagulation reversal in hip fracture patients.

Cervical radiotherapy may leads to elevated caries risk in Hodgkin-lymphoma (HL) patients. Our aim was to estimate the late effect of cervical irradiation on periodontal status in HL patients. Patients filled out query-form, their clinical data were collected, periodontal status was examined, decayed-missing-filled-teeth and periodontal-indexes were calculated. We examined 68 patients who received, 64 patients who did not received cervical radiotherapy and 51 control person. 23.5% of cervical irradiated, 18.15% of not irradiated patients and 17.64% of controls had subjective xerostomia, but it was not objective by sialometry. Mean decayed-missing-filled-teeth-index was 22.53 among irradiated, 21.54 among not irradiated patients while it was 17.23 in control group. Periodontal index was 2.47, 2.42, and 2.14 in different groups. Difference between decayed-missing-filled-teeth indexes of irradiated patients and controls was significant. We have to emphasize the importance of prevention and closer dental observation of HL patients.

POEMS Syndrome is a rare cause of demyelinating and axonal mixed neuropathy. Plasmacytomas are usually seen in POEMS syndrome and can be osseous or extramedullary. Plasmacytomas presenting as an abscess has not been noted earlier. Our patient presented with localized hyperpigmented patch on the back and later developed progressive weakness in upper and lower limbs. Initially serum and urine protein electrophoresis were normal. The patient was thought to have Chronic Inflammatory Demyelinating Polyneuropathy and was treated accordingly without any improvement. Repeat serum protein electrophoresis showed monoclonal gammopathy. MRI of the back revealed an abscess in the paravertebral soft tissues reaching up to the skin. Needle biopsy was consistent with plasmacytoma. Later, he developed a purulent fungating lesion in the lower midback. Antibiotics were started and local resection was done followed by radiation. Pathology of the resected mass showed plasmacytoma extensively involving subcutaneous soft tissue and bone. The patient improved with the treatment. Cystic plasmacytomas and abscess within the plasmacytoma has not been reported earlier. Whether abscess formation is part of the disease spectrum due to infiltration of overlying tissue or is secondary to localized immunosuppression is unknown. Local treatment of a single plasmacytoma is useful in ameliorating systemic symptoms.

Introduction. Coexistence of myeloproliferative neoplasms with lymphoproliferative syndromes has been described in the past, whereas plasma cell dyscrasias seem to be the most common cases. Case Presentation. We present a case of a 59-year-old Caucasian female of Greek origin who presented with thrombocytosis. Clinical and laboratory investigation disclosed the presence of a smoldering myeloma with coexisting histological and molecular characteristics of primary myelofibrosis. The patient had the acquired point mutation V617F in the JAK2 gene but not the bcr-abl rearrangement and was treated for myelofibrosis with subsequent improvement of all haematological parameters without evidence of myelomatic evolution. Conclusion. We present the first case in the literature of a smoldering myeloma coexisting with primary myelofibrosis. The underlying pathogenetic mechanism could be either related to the presence of a pluripotent neoplastic stem cell capable to differentiate into both lymphoid and myeloid cells or be related to two separate nosologic entities.

B cell non-Hodgkin lymphoma is a typical extrahepatic manifestation frequently associated with hepatitis C virus (HCV) infection. The mechanism by which HCV infection leads to lymphoproliferative disorder remains unclear. Our group established HCV transgenic mice that expressed the full HCV genome in B cells (RzCD19Cre mice). We observed a 25.0% incidence of diffuse large B cell non-Hodgkin lymphomas (22.2% in male and 29.6% in female mice) within 600 days of birth. Interestingly, RzCD19Cre mice with substantially elevated serum-soluble interleukin-2 receptor α-subunit (sIL-2Rα) levels (>1000 pg/mL) developed B cell lymphomas. Another mouse model of lymphoproliferative disorder was established by persistent expression of HCV structural proteins through disruption of interferon regulatory factor-1 (irf-1(_/_)/CN2 mice). Irf-1(_/_)/CN2 mice showed extremely high incidences of lymphomas and lymphoproliferative disorders. Moreover, these mice showed increased levels of interleukin (IL)-2, IL-10, and Bcl-2 as well as increased Bcl-2 expression, which promoted oncogenic transformation of lymphocytes.