Israel Medical Association Journal

Background: With advances in myelodysplastic syndromes (MDS), patient cohorts from different time periods might be different.

Objectives: To compare presentation and outcomes between two cohorts.

Methods: Data were collected from George Washington University Medical Center, Washington, DC, USA 1986–1987 (DC), and Tel Aviv Medical Center, Israel 1999–2009 (TA).

Results: The study comprised 227 patients (139 TA, 88 DC). TA patients were older (75.4 ± 9.8 vs. 63.8 ± 14.3 years, P < 0.001) and had more cardiovascular diseases (56.8% vs. 14.8%, P < 0.001), fewer cytopenias (1.67 ± 0.82 vs. 2.0 ± 0.93, P = 0.003), and lower mean corpuscular volume (94.3 ± 9.9 fl vs. 100.5 ± 15.3 fl, P < 0.001). Hemoglobin, leukocyte, neutrophil, and platelet counts were similar. More TA patients had dysplasias. Bone marrow cellularity and cytogenetics were similar, but more TA patients had blasts < 5% (73.4% vs. 50.6%, P = 0.003). More TA patients had early French-American-British (FAB) disease (66.9% vs. 40.9%, P < 0.001) and lower risk disease per International Prognostic Scoring System (81% vs. 50%, P < 0.001). The 5 year survival (5YS) of TA patients was not significantly greater (62% vs. 55%). 5YS by FAB was also slightly greater for TA patients (77% vs. 65% for early FAB; 43% vs. 37% for advanced FAB, P > 0.05).

Conclusions: Although patients diagnosed with MDS at a later period were older and had more cardiovascular co-morbidities, they had fewer cytopenias, tended to have earlier disease, and had minimally greater, but not significant, 5YS.

Background: Approximately 80% of patients with myelodysplastic syndromes (MDS) receive multiple red blood cells (RBC), often multiple transfusions, and are therefore prone to develop alloantibodies against RBC. Because of increasing evidence for the role of immune dysregulation in the pathobiology of MDS, we hypothesized that in patients with MDS there is an increase in alloantibody formation beyond that expected by multiple transfusions.

Objectives: To determine the prevalence rates of alloantibodies in patients with MDS who are transfusion dependent and compare them to those of non-MDS patients matched for number of RBC units they received. 

Methods: The blood bank database was screened to identify non-MDS patients matched for age and number of units transfused. Logistic regression analysis was applied to determine factors affecting alloantibody formation. 

Results: Of 60 patients with MDS, 18 (30%) developed alloantibodies against RBC. Transfusion-dependent MDS and non-MDS patients (N=56 each), matched for number of RBC units and age, were compared. Fifteen MDS patients (27%) but only 12 non-MDS patients (12%) developed alloantibodies (P = 0.057). The relative risk for developing antibodies in MDS patients was 2.14, and MDS was the strongest predictor for formation of alloantibodies during transfusion therapy (odds ratio 3.66, confidence interval 1.4–9.3). 

Conclusions: Patients with MDS are at increased risk to develop RBC alloantibodies, partly because these patients receive multiple RBC transfusions. Whether matching for RH and KEL would lead to lower rates of RBC alloantibodies remains to be determined.

 

Background: Janus kinase-2 (JAK2) is mutated in a high proportion of patients with polycythemia vera and in a smaller number with essential thrombocythemia and primary myelofibrosis. Mutated JAK2 is an important diagnostic marker for myeloproliferative neoplasm (MPN) and may also play a major role in the pathogenesis of MPN.

Objectives: To evaluate the prevalence of mutated JAK2 (JAK2-V617F) among patients with major intraabdominal vein thrombosis who had normal blood counts at diagnosis of the initial event.

Methods: The medical records of patients who presented with a major intraabdominal venous thrombosis and normal peripheral blood counts were obtained. JAK2-V617F mutation status was determined by real-time polymerase chain reaction.

Results: Twenty-two patients were available for this analysis and 9 (41%) were found to have JAK2-V617F. Patients with positive JAK2-V617F were younger and had more frequent clinical splenomegaly than those with wild-type JAK2.

Conclusions: A high proportion of patients presenting with “idiopathic” major intraabdominal vein thrombosis and normal blood counts carry JAK2-V617F. We recommend searching for the mutation in this clinical setting to detect patients with occult MPN.