Elizabeth F Plocharczyk, Sarah H O'Brien, Jill L Crumbacher, Samir B Kahwash
{"title":"Platelet hyperreactivity: report of a case with catastrophic arterial thrombosis in a child.","authors":"Elizabeth F Plocharczyk, Sarah H O'Brien, Jill L Crumbacher, Samir B Kahwash","doi":"10.1532/LH96.12006","DOIUrl":"https://doi.org/10.1532/LH96.12006","url":null,"abstract":"<p><p>Platelet hyperreactivity is an important but under-recognized cause of idiopathic arterial thrombosis. We describe a case of arterial thrombosis in a previously healthy 12-year-old girl after minor trauma to the knee, resulting in lower extremity amputation. Family history was positive for arterial and venous thrombosis, but an extensive work-up for the usual inherited thrombophilia risk factors was negative. The patient was eventually diagnosed with platelet hyperreactivity and remains on life-long antiplatelet therapy.Consideration of platelet hyperreactivity in the evaluation of unusual arterial thrombotic events allows for targeted therapy, potential avoidance of future events, and timely screening of family members.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 4","pages":"22-6"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31134633","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maria Coutinho, Emília Costa, Tânia Monteiro, Gisela Silva, Helena Costa, Ana Guedes, Inês Freitas, José Barbot
{"title":"Infantile pyknocytosis: an under-recognized form of neonatal hemolytic anemia?","authors":"Maria Coutinho, Emília Costa, Tânia Monteiro, Gisela Silva, Helena Costa, Ana Guedes, Inês Freitas, José Barbot","doi":"10.1532/LH96.12005","DOIUrl":"https://doi.org/10.1532/LH96.12005","url":null,"abstract":"<p><p>Infantile pyknocytosis (IP) is an under-recognized hematological entity of newborns that can cause a severe neonatal hemolytic anemia. A careful, prompt, and accurate peripheral blood smear examination is essential to establish the diagnosis. Here we describe the clinical features and histological parameters of 1 case of IP. Spontaneous resolution usually occurs by 4 to 6 months, but red blood cell transfusion may be needed if the anemia is severe.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 4","pages":"27-9"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31134634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Saeid Abroun, Najmaldin Saki, Rahim Fakher, Farahnaz Asghari
{"title":"Biology and bioinformatics of myeloma cell.","authors":"Saeid Abroun, Najmaldin Saki, Rahim Fakher, Farahnaz Asghari","doi":"10.1532/LH96.11003","DOIUrl":"https://doi.org/10.1532/LH96.11003","url":null,"abstract":"<p><p>Multiple myeloma (MM) is a plasma cell disorder that occurs in about 10% of all hematologic cancers. The majority of patients (99%) are over 50 years of age when diagnosed. In the bone marrow (BM), stromal and hematopoietic stem cells (HSCs) are responsible for the production of blood cells. Therefore any destruction or/and changes within the BM undesirably impacts a wide range of hematopoiesis, causing diseases and influencing patient survival. In order to establish an effective therapeutic strategy, recognition of the biology and evaluation of bioinformatics models for myeloma cells are necessary to assist in determining suitable methods to cure or prevent disease complications in patients. This review presents the evaluation of molecular and cellular aspects of MM such as genetic translocation, genetic analysis, cell surface marker, transcription factors, and chemokine signaling pathways. It also briefly reviews some of the mechanisms involved in MM in order to develop a better understanding for use in future studies.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 4","pages":"30-41"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31134635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Derek L Vanhille, Roberto H Nussenzveig, Christopher Glezos, Sherrie Perkins, Archana M Agarwal
{"title":"Best practices for use of the HEMOX analyzer in the clinical laboratory: quality control determination and choice of anticoagulant.","authors":"Derek L Vanhille, Roberto H Nussenzveig, Christopher Glezos, Sherrie Perkins, Archana M Agarwal","doi":"10.1532/LH96.12001","DOIUrl":"https://doi.org/10.1532/LH96.12001","url":null,"abstract":"The HEMOX Analyzer (TCS Scientific) has been used to measure the full oxygen-dissociation curve (ODC) and to calculate P(50) and the Hill coefficient. The effects of different anticoagulants on sample stability and P(50) values have not been evaluated extensively for this instrument. We characterized an artificial hemoglobin (Equil QC463) for quality control (QC) and compared P(50) values for blood samples drawn into 3 different anticoagulants (acid citrate dextrose [ACD], heparin, and EDTA). P(50) values were not stable in ACD but were stable in heparin and EDTA anticoagulants for up to 4 days. Tests with Equil QC463 showed that P(50) values were quite sensitive to small variations in buffer pH. Use of the correct anticoagulant and strict control of buffer pH are 2 parameters that need to be accounted for in best-practices use of this hemoximeter and before determining P(50).","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 3","pages":"17-9"},"PeriodicalIF":0.0,"publicationDate":"2012-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30925274","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinically silent massive fetomaternal hemorrhage: important lessons from an illustrative case.","authors":"Ginell R Post, John Lazarchick, Zeba N Singh","doi":"10.1532/LH96.12002","DOIUrl":"https://doi.org/10.1532/LH96.12002","url":null,"abstract":"<p><p>Massive fetomaternal hemorrhage (FMH) >150 mL is rare and may occur in the absence of high-risk obstetrical events. The significance of FMH in Rh D-negative women is alloimmunization with an increased risk of hemolytic disease of the newborn in subsequent Rh D-positive pregnancies and adverse outcomes for the fetus/neonate. The Kleihauer-Betke (KB) acid elution test is used to quantify fetal erythrocytes in the circulation of Rh D-negative women postpartum and to calculate the dose of Rh immune globulin (RhIG) needed for prophylaxis against alloimmunization. In this case, the KB stain unexpectedly revealed 4.5% fetal cells, a finding consistent with a massive FMH of 225 mL, in the absence of a predisposing cause and clinical signs in the infant. This case underscores the importance of FMH quantification in all Rh D-negative women with Rh D-positive fetuses, uncomplicated pregnancies, and healthy newborns. We discuss factors that can affect KB test performance and caveats in interpretation.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 2","pages":"11-3"},"PeriodicalIF":0.0,"publicationDate":"2012-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30701379","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S Anoun, Y Traoue, A Ouled Lahcen, K Gamraoui, S Faez, B Oukkache
{"title":"Reactive hemophagocytic syndrome caused by Rickettsial infection: report of a case.","authors":"S Anoun, Y Traoue, A Ouled Lahcen, K Gamraoui, S Faez, B Oukkache","doi":"10.1532/LH96.11008","DOIUrl":"https://doi.org/10.1532/LH96.11008","url":null,"abstract":"","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 2","pages":"14-6"},"PeriodicalIF":0.0,"publicationDate":"2012-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30701380","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Azza Mostafa Ibrahim, Iman Maher Mansour, Manal Michel Wilson, Doha Abdel-Hamid Mokhtar, Amani Mohamed Helal, Hanan Mohamed Al Wakeel
{"title":"Study of survivin and X-linked inhibitor of apoptosis protein (XIAP) genes in acute myeloid leukemia (AML).","authors":"Azza Mostafa Ibrahim, Iman Maher Mansour, Manal Michel Wilson, Doha Abdel-Hamid Mokhtar, Amani Mohamed Helal, Hanan Mohamed Al Wakeel","doi":"10.1532/LH96.11005","DOIUrl":"https://doi.org/10.1532/LH96.11005","url":null,"abstract":"<p><p>Apoptosis deregulation is important for cancer development, chemotherapy response, and prognosis. Survivin and X-linked inhibitor of apoptosis protein (XIAP) are 2 members of the inhibitor of apoptosis proteins family (IAP). We used semi-quantitative reverse transcriptase polymerase chain reaction (RT-PCR) to determine the levels of expression of survivin and XIAP in 30 patients with de novo acute myeloid leukemia (AML) and 20 age- and sex-matched healthy volunteers. Survivin and XIAP overexpression were detected in 36.7% and 43.3% of cases, respectively. Patients with overexpression of either survivin or XIAP showed unfavorable response to chemotherapy in 81.2% and 91.7%, respectively. Also, these cases showed shorter median survival time (30 days) compared to patients with normal expression of either survivin or XIAP (150 days and 180 days). Patients with overexpression of both survivin and XIAP showed unfavorable response to induction therapy in 100% of the patients and the shortest median survival (30 days). These findings suggest that survivin and XIAP may have a role in leukemogenesis and provide prognostic information.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"18 1","pages":"1-10"},"PeriodicalIF":0.0,"publicationDate":"2012-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30538973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Burkitt's leukemia with an atypical immunophenotype: report of a case and review of literature.","authors":"Neil J Rawlinson, Peter Baker, Samir B Kahwash","doi":"10.1532/LH96.11004","DOIUrl":"https://doi.org/10.1532/LH96.11004","url":null,"abstract":"<p><p>Burkitt's leukemia (BL) constitutes a small but important fraction of acute leukemias in children. It is an aggressive type of leukemia that is responsive to high-intensity, short-duration chemotherapy with complete remission possible in 75% to 90% of cases. The recognition and proper designation of BL is important because treatment differs from that of precursor B-cell acute lymphoblastic leukemia (pre-B ALL). Burkitt's leukemia is separated by its typical morphologic features (blasts with typical French-American-British [FAB] L-3 morphology compared to FAB L-1/L-2 morphology in pre-B ALL) and a classic immunophenotype (blast positivity for CD45 [bright], CD20 [bright], CD10, CD19, surface immunoglobulin [SIg], Ig light chain restriction, and negative terminal deoxynucleotidyl transferase [TdT]) compared to pre-B ALL blasts (which are positive for CD45 [dim], CD10, CD19, and TdT and negative for CD20 and SIg). The diagnosis of Burkitt's leukemia is confirmed by the characteristic cytogenetic findings.The combination of Burkitt's morphology with precursor B-cell immunophenotype may present a diagnostic pitfall, resulting in delay of proper management.We describe such an atypical case in a 12-year-old girl and emphasize that correct classification and treatment starts with proper morphologic/immunophenotypic correlation, and the awareness of the overlapping features in some cases.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"17 4","pages":"27-31"},"PeriodicalIF":0.0,"publicationDate":"2011-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30341855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Detection of morphologic changes in peripheral mononuclear cells in hepatitis B virus infection using the beckman coulter LH 750.","authors":"Y Zhu, X Cao, D Xu","doi":"10.1532/LH96.09013","DOIUrl":"https://doi.org/10.1532/LH96.09013","url":null,"abstract":"<p><p>The Beckman Coulter LH 750 with VCS technology can determine intrinsic biophysical properties of peripheral leukocytes in their \"near native state.\" We investigated the possibility of the LH 750 to detect morphologic changes in lymphocytes/monocytes in hepatitis B virus (HBV) infection. The VCS data from 37 active HBV patients, 140 HBV carriers, and 655 controls were analyzed. We observed significant increases in lymphocyte volume (LV) and the lymphocyte volume SD (LV-SD), and a significant decrease in lymphocyte conductivity (LC) in HBV infection, compared with controls. Similar changes in monocyte volume and conductivity were also observed. A simplified parameter, the Lymph-Index (calculated as [LV LV-SD]/LC]) is proposed. The Lymph-Index was significantly increased in HBV carriers compared with controls (13.86 ± 2.73 versus 11.83 ± 1.36; P < .001). The increase was more pronounced in patients with active HBV infection (18.84 ± 3.88 versus 11.83 ± 1.36; P < .0001). With a cutoff value of 18.2 for the Lymph-Index, a 91.7% sensitivity and a 95.7% specificity were achieved in diagnosing active HBV infection. This overall sensitivity and specificity were superior to those of the individual lymphocyte/monocyte VCS parameters. In conclusion, VCS parameters can reflect the morphologic changes of lymphocytes/monocytes in HBV infection and may be useful clinically to aid in identifying HBV infection in a population with a high HBV prevalence.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"17 3","pages":"22-6"},"PeriodicalIF":0.0,"publicationDate":"2011-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"30156207","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A Girolami, G Berti de Marinis, E Bonamigo, S Vettore
{"title":"Similarities and discrepancies in homozygous factor VII defects due to mutations in the region of residues Met298 to Cys310 (exon 8) in the catalytic domain of factor VII.","authors":"A Girolami, G Berti de Marinis, E Bonamigo, S Vettore","doi":"10.1532/LH96.11-001","DOIUrl":"https://doi.org/10.1532/LH96.11-001","url":null,"abstract":"<p><p>Patients with the Arg304Gln mutation in factor VII Padua (FVII Padua) show discrepant activity levels that depend on the thromboplastin used in the assay system. This report investigates the possibility that residues close to Arg304 (exon 8) show the same discrepant behavior. All available homozygous patients with a mutation in a 13-residue region (preceding and following Arg304) have been evaluated. Only the Arg304Trp mutation showed a discrepancy similar to that shown by the Arg304Gln mutation. Other homozygotes failed to show differences, despite their all being positive for cross-reacting material. Another FVII amino acid residue involved in tissue factor binding and activation is Arg79 (exon 4). No comparison could be carried out because no homozygotes for deficiency in this region have ever been described. The relationship between these 2 residues involved in tissue factor binding and activation has not yet been completely clarified; however, Arg residues 79 and 304 are the only 2 residues definitely shown thus far to be involved in this important function.</p>","PeriodicalId":85078,"journal":{"name":"Laboratory hematology : official publication of the International Society for Laboratory Hematology","volume":"17 2","pages":"17-21"},"PeriodicalIF":0.0,"publicationDate":"2011-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"29963111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}