Spectrum of Ankyrin Mutations in Hereditary Spherocytosis: A Case Report and Review of the Literature
Conclusion: A novelANK1 mutation (NM_000037.3, c.2960+2T#x3e;G, intron 26) that is potentially associated with HS was identified. To date, 80ANK1 mutations have been reported to be associated with HS in humans.Acta Haematol 2018;140:77 –86 (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

Microarray-Based Gene Expression Analysis Identifies Potential Diagnostic and Prognostic Biomarkers for Waldenstr öm Macroglobulinemia
This study assessed differentially expressed genes (DEGs) to identify potential WM biomarkers and uncover the underlying the molecular mechanisms of WM progression us ing gene expression profiles from the Gene Expression Omnibus database. DEGs were identified using the LIMMA package and their potential functions were then analyzed by using the gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analyses and the protein-protein intera ction (PPI) network analysis by using the Search Tool for the Retrieval of Interacting Genes/Proteins database. Data showed that among 1,756 DEGs, 926 were upregu...
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

Mediastinal Myeloid Sarcoma with TP53 Mutation Preceding Acute Myeloid Leukemia with a PICALM-MLLT10 Fusion Gene
Conclusion: MS with a mediastinal localization is rare and often misdiagnosed as malignant lymphoma. Acute leukemia harboring a PICALM-MLLT10 fusion gene is characterized by a mixed T cell and myeloid phenotype. The rearrangement is a rare recurrent translocation associated with specific clinical features, as illustrated in this case report.Acta Haematol 2018;140:97 –104 (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

Real-Life Management of Children and Adolescents with Chronic Myeloid Leukemia: The Italian Experience
Conclusions: Our experience shows that having specific guidelines is an important tool for an optimal management of childhood CP-CML, together with exchange of knowledge and proactive discussions within the network.Acta Haematol 2018;140:105 –111 (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

A New < b > < i > Trans < /i > < /b > -Acting Modulator of Fetal Hemoglobin?
Acta Haematol 2018;140:112 –113 (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

Geographic Hematology: Some Observations in Mexico
In 1963 Jean Bernard introduced the concept of “geographic hematology” and distinguished 2 branches, i.e., “ethnic hematology,” which deals with differences between populations, and “environmental hematology,” which considers factors such as food habits, infections, and others. Both of these branches have implications in the distribu tion of hematological diseases worldwide. In comparison with Caucasian populations, in Mexico a significantly higher prevalence of acute lymphoblastic, acute promyelocytic, and acute megakaryoblastic leukemias has been described. The rate of chronic myeloid ...
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

Efficacy of Recombinant Human Soluble Thrombomodulin in Treating Disseminated Intravascular Coagulation Complicating Allogeneic Hematopoietic Stem Cell Transplantation
The prognosis for patients who experience hemostatic complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT) is poor. However, no report has investigated disseminated intravascular coagulation (DIC) caused by the complications of allo-HSCT without infection. Recombinant human soluble thrombomodulin (rhTM) was used to treat 12 episodes of DIC (n = 10; group 1) caused by allo-HSCT complications such as acute graft-versus-host disease (aGVHD) or thrombotic microangiopathy (TMA), and the clinical outcomes were compared with those of historical controls (n = 9; group 2) treated for DIC without rhTM. I...
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

Disseminated Intravascular Coagulation Complicating Allogeneic Hematopoietic Stem Cell Transplantation
Acta Haematol 2018;140:128 –129 (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Source Type: research

A New Trans-Acting Modulator of Fetal Hemoglobin?
PMID: 30227393 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Steinberg MH Tags: Acta Haematol Source Type: research

Efficacy of Recombinant Human Soluble Thrombomodulin in Treating Disseminated Intravascular Coagulation Complicating Allogeneic Hematopoietic Stem Cell Transplantation.
Abstract The prognosis for patients who experience hemostatic complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT) is poor. However, no report has investigated disseminated intravascular coagulation (DIC) caused by the complications of allo-HSCT without infection. Recombinant human soluble thrombomodulin (rhTM) was used to treat 12 episodes of DIC (n = 10; group 1) caused by allo-HSCT complications such as acute graft-versus-host disease (aGVHD) or thrombotic microangiopathy (TMA), and the clinical outcomes were compared with those of historical controls (n = 9; group 2) treated for D...
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Inoue Y, Matsunawa M, Sano F, Miura I Tags: Acta Haematol Source Type: research

Mediastinal Myeloid Sarcoma with TP53 Mutation Preceding Acute Myeloid Leukemia with a PICALM-MLLT10 Fusion Gene.
CONCLUSION: MS with a mediastinal localization is rare and often misdiagnosed as malignant lymphoma. Acute leukemia harboring a PICALM-MLLT10 fusion gene is characterized by a mixed T cell and myeloid phenotype. The rearrangement is a rare recurrent translocation associated with specific clinical features, as illustrated in this case report. PMID: 30227397 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Naesens L, Devos H, Nollet F, Michaux L, Selleslag D Tags: Acta Haematol Source Type: research

Real-Life Management of Children and Adolescents with Chronic Myeloid Leukemia: The Italian Experience.
CONCLUSIONS: Our experience shows that having specific guidelines is an important tool for an optimal management of childhood CP-CML, together with exchange of knowledge and proactive discussions within the network. PMID: 30227398 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Giona F, Santopietro M, Menna G, Putti MC, Micalizzi C, Santoro N, Ziino O, Mura R, Ladogana S, Iaria G, Sau A, Burnelli R, Vacca N, Bernasconi S, Consarino C, Petruzziello F, Moleti ML, Biondi A, Locatelli F, Foà R Tags: Acta Haematol Source Type: research

Disseminated Intravascular Coagulation Complicating Allogeneic Hematopoietic Stem Cell Transplantation.
PMID: 30227403 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Nomura S Tags: Acta Haematol Source Type: research

Microarray-Based Gene Expression Analysis Identifies Potential Diagnostic and Prognostic Biomarkers for Waldenstr öm Macroglobulinemia.
This study assessed differentially expressed genes (DEGs) to identify potential WM biomarkers and uncover the underlying the molecular mechanisms of WM progression using gene expression profiles from the Gene Expression Omnibus database. DEGs were identified using the LIMMA package and their potential functions were then analyzed by using the gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analyses and the protein-protein interaction (PPI) network analysis by using the Search Tool for the Retrieval of Interacting Genes/Proteins database. Data showed that among 1,756 DEGs, 926 were upregula...
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Xu H, Yao F Tags: Acta Haematol Source Type: research

Spectrum of Ankyrin Mutations in Hereditary Spherocytosis: A Case Report and Review of the Literature.
CONCLUSION: A novel ANK1 mutation (NM_000037.3, c.2960+2T>G, intron 26) that is potentially associated with HS was identified. To date, 80 ANK1 mutations have been reported to be associated with HS in humans. PMID: 30227413 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Luo Y, Li Z, Huang L, Tian J, Xiong M, Yang Z Tags: Acta Haematol Source Type: research

Geographic Hematology: Some Observations in Mexico.
GJ Abstract In 1963 Jean Bernard introduced the concept of "geographic hematology" and distinguished 2 branches, i.e., "ethnic hematology," which deals with differences between populations, and "environmental hematology," which considers factors such as food habits, infections, and others. Both of these branches have implications in the distribution of hematological diseases worldwide. In comparison with Caucasian populations, in Mexico a significantly higher prevalence of acute lymphoblastic, acute promyelocytic, and acute megakaryoblastic leukemias has been described. The rate of c...
Source: Acta Haematologica - September 18, 2018 Category: Hematology Authors: Colunga-Pedraza PR, Gomez-Cruz GB, Colunga-Pedraza JE, Ruiz-Argüelles GJ Tags: Acta Haematol Source Type: research

< b > < i > TMEM18 < /i > < /b > : A Novel Prognostic Marker in Acute Myeloid Leukemia
Conclusion: Because the current AML prognostic factors do not take mRNA expression into consideration unlike other cancers, the development of mRNA-based prognostic factors would be beneficial for accurate prediction of the survival of AML patients. Therefore,TMEM18 gene is a potential biomarker for AML.Acta Haematol 2018;140:71 –76 (Source: Acta Haematologica)
Source: Acta Haematologica - September 10, 2018 Category: Hematology Source Type: research

TMEM18: A Novel Prognostic Marker in Acute Myeloid Leukemia.
CONCLUSION: Because the current AML prognostic factors do not take mRNA expression into consideration unlike other cancers, the development of mRNA-based prognostic factors would be beneficial for accurate prediction of the survival of AML patients. Therefore, TMEM18 gene is a potential biomarker for AML. PMID: 30199869 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 10, 2018 Category: Hematology Authors: Ha M, Kim JY, Han ME, Kim GH, Park SY, Jeong DC, Oh SO, Kim YH Tags: Acta Haematol Source Type: research

Atypical Phenotype and Treatment Response Pattern in a Patient with FIP1L1-PDGFR α Mutation
We present the case of a 71-year-old male who presented with abdominal discomfort, fevers, and leukocyt osis with eosinophilia. The patient was diagnosed with a myeloproliferative neoplasm with eosinophilia and FIP1L1-PDGFRα rearrangement after a bone marrow evaluation revealed hypercellular marrow with eosinophilia and fluorescence in situ hybridization identified the FIP1L1-PDGFRα rearrangement. T he patient was successfully treated with imatinib. Within months he relapsed and converted into acute myeloid leukemia. The patient was then treated with ponatinib which induced and maintained clinical and hematolog...
Source: Acta Haematologica - September 7, 2018 Category: Hematology Source Type: research

Atypical Phenotype and Treatment Response Pattern in a Patient with FIP1L1-PDGFR α Mutation.
We present the case of a 71-year-old male who presented with abdominal discomfort, fevers, and leukocytosis with eosinophilia. The patient was diagnosed with a myeloproliferative neoplasm with eosinophilia and FIP1L1-PDGFRα rearrangement after a bone marrow evaluation revealed hypercellular marrow with eosinophilia and fluorescence in situ hybridization identified the FIP1L1-PDGFRα rearrangement. The patient was successfully treated with imatinib. Within months he relapsed and converted into acute myeloid leukemia. The patient was then treated with ponatinib which induced and maintained clinical and hematologic...
Source: Acta Haematologica - September 5, 2018 Category: Hematology Authors: Bikhchandani M, Johnson R, Tuan B, Tefferi A Tags: Acta Haematol Source Type: research

The Levels of T Lymphocyte Subsets in Immune Thrombocytopenia Associated with Anti-GPIIb/IIIa- and/or Anti-GPIb α-Mediated Responses Are Differentially Sensitive to Dexamethasone
Conclusions: There was insensitivity to dexamethasone treatment in ITP patients who were anti-GPIb α antibody positive. The detection of T lymphocyte subsets is useful in ITP patients for forecasting the outcome of dexamethasone treatment. There were some relationships between the different antibodies and the levels of T lymphocyte subsets.Acta Haematol 2018;140:60 –66 (Source: Acta Haematologica)
Source: Acta Haematologica - August 21, 2018 Category: Hematology Source Type: research

The Levels of T Lymphocyte Subsets in Immune Thrombocytopenia Associated with Anti-GPIIb/IIIa- and/or Anti-GPIb α-Mediated Responses Are Differentially Sensitive to Dexamethasone.
The Levels of T Lymphocyte Subsets in Immune Thrombocytopenia Associated with Anti-GPIIb/IIIa- and/or Anti-GPIbα-Mediated Responses Are Differentially Sensitive to Dexamethasone. Acta Haematol. 2018 Aug 21;140(1):60-66 Authors: Chen Y, Xie Y, Ruan M, Shi J Abstract OBJECTIVE: The aim of this work was to investigate the influence of T lymphocyte subsets and platelet-specific autoantibodies on immune thrombocytopenia (ITP) with dexamethasone therapy. METHODS: The samples were obtained from patients before therapy. T lymphocyte subsets were measured by flow cytometry, and platelet-specific aut...
Source: Acta Haematologica - August 21, 2018 Category: Hematology Authors: Chen Y, Xie Y, Ruan M, Shi J Tags: Acta Haematol Source Type: research

Agranulocytosis Associated with Waldenstr öm Macroglobulinemia
Currently, there are only 2 case reports of Waldenstr öm macroglobulinemia (WM) associated with severe neutropenia. This is a case report of a woman with a past medical history of WM who presented with neutropenic fever. The patient’s febrile neutropenia resolved after RCD chemotherapy (cyclophosphamide 750 mg/m2, dexamethasone 20 mg, and rituximab 375 mg/m2). Fourteen days after administration, the neutrophil level had started to rise and normalized after 6 days. To the best of our knowledge, this is the 3rd reported case of agranulocytosis due to WM.Acta Haematol 2018;140:42 –45 (Source: Acta Haematologica)
Source: Acta Haematologica - August 16, 2018 Category: Hematology Source Type: research

Factor X Deficiency Caused by Nonsecretory Myeloma Successfully Corrected with Bortezomib: A Case Report and Review of the Literature
A 62-year-old female presenting with anemia and extensive hemorrhages is reported. Coagulation tests showed significantly prolonged prothrombin time (PT) and activated partial thromboplastin time. Decreased levels of clotting factor X activity were determined (5.4%). Bone marrow biopsy revealed neoplastic plasma cells. Serum and urine protein electrophoresis were both negative for monoclonal gammopathy, and both bone marrow and abdominal fat biopsies were negative for amyloid deposition. The patient was diagnosed as suffering from nonsecretory multiple myeloma (MM) complicated by acquired factor X deficiency. A complete re...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Source Type: research

Why Is the Immunoglobulin Heavy Chain Gene Mutation Status a Prognostic Indicator in Chronic Lymphocytic Leukemia?
The immunoglobulin heavy chain gene (IgHV) mutation status correlates with the clinical outcome of patients with chronic lymphocytic leukemia (CLL) treated with chemoimmunotherapy. Why the survival rate of patients with unmutatedIgHV is worse than that of patients with mutatedIgHV is unknown. CLL cells with unmutated IgHV were thought to originate from na ïve B lymphocytes, whereas CLL cells with mutatedIgHV were thought to arise from B cells that have undergone somatic hypermutation (SHM). Cell surface protein expression profile and gene expression studies showing that all CLL cells, regardless of theirIgHV mutation ...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Source Type: research

< b > < i > ANTXR1 < /i > < /b > Intronic Variants Are Associated with Fetal Hemoglobin in the Arab-Indian Haplotype of Sickle Cell Disease
Disease severity of sickle cell anemia is highly variable, and it is commonly accepted that fetal hemoglobin (HbF) levels play a major role as an ameliorating factor. Investigation of genetic variants have identified several genes to be the principal influencers of HbF regulation. Here, we further elucidated the association of rs4527238 and rs35685045 ofANTXR1 genes in the context of HbF level variance in sickle cell anemia patients of the Arab-Indian haplotype. Samples from 630 sickle cell anemia patients were analyzed for the mutations at 2 specific locations of theANTXR1 gene by TaqMan ®-based real-time PCR. The CC ...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Source Type: research

Factor X Deficiency Caused by Nonsecretory Myeloma Successfully Corrected with Bortezomib: A Case Report and Review of the Literature.
Abstract A 62-year-old female presenting with anemia and extensive hemorrhages is reported. Coagulation tests showed significantly prolonged prothrombin time (PT) and activated partial thromboplastin time. Decreased levels of clotting factor X activity were determined (5.4%). Bone marrow biopsy revealed neoplastic plasma cells. Serum and urine protein electrophoresis were both negative for monoclonal gammopathy, and both bone marrow and abdominal fat biopsies were negative for amyloid deposition. The patient was diagnosed as suffering from nonsecretory multiple myeloma (MM) complicated by acquired factor X deficie...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Authors: Jia J, Wang H, Wu M, Zhang F, Liu X, Chen W, Liu A Tags: Acta Haematol Source Type: research

Agranulocytosis Associated with Waldenstr öm Macroglobulinemia.
Agranulocytosis Associated with Waldenström Macroglobulinemia. Acta Haematol. 2018 Aug 16;140(1):42-45 Authors: Vaxman I, Shepshelovich D, Hayman L, Raanani P, Lahav M Abstract Currently, there are only 2 case reports of Waldenström macroglobulinemia (WM) associated with severe neutropenia. This is a case report of a woman with a past medical history of WM who presented with neutropenic fever. The patient's febrile neutropenia resolved after RCD chemotherapy (cyclophosphamide 750 mg/m2, dexamethasone 20 mg, and rituximab 375 mg/m2). Fourteen days after administration, the neutrophil level ha...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Authors: Vaxman I, Shepshelovich D, Hayman L, Raanani P, Lahav M Tags: Acta Haematol Source Type: research

Why Is the Immunoglobulin Heavy Chain Gene Mutation Status a Prognostic Indicator in Chronic Lymphocytic Leukemia?
Abstract The immunoglobulin heavy chain gene (IgHV) mutation status correlates with the clinical outcome of patients with chronic lymphocytic leukemia (CLL) treated with chemoimmunotherapy. Why the survival rate of patients with unmutated IgHV is worse than that of patients with mutated IgHV is unknown. CLL cells with unmutated IgHV were thought to originate from naïve B lymphocytes, whereas CLL cells with mutated IgHV were thought to arise from B cells that have undergone somatic hypermutation (SHM). Cell surface protein expression profile and gene expression studies showing that all CLL cells, regardless of...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Authors: Rozovski U, Keating MJ, Estrov Z Tags: Acta Haematol Source Type: research

ANTXR1 Intronic Variants Are Associated with Fetal Hemoglobin in the Arab-Indian Haplotype of Sickle Cell Disease.
Abstract Disease severity of sickle cell anemia is highly variable, and it is commonly accepted that fetal hemoglobin (HbF) levels play a major role as an ameliorating factor. Investigation of genetic variants have identified several genes to be the principal influencers of HbF regulation. Here, we further elucidated the association of rs4527238 and rs35685045 of ANTXR1 genes in the context of HbF level variance in sickle cell anemia patients of the Arab-Indian haplotype. Samples from 630 sickle cell anemia patients were analyzed for the mutations at 2 specific locations of the ANTXR1 gene by TaqMan®-based rea...
Source: Acta Haematologica - August 16, 2018 Category: Hematology Authors: Al-Ali ZA, Fallatah RK, Aljaffer EA, Albukhari ER, Sadek Al-Ali N, Al-Ghannam ZT, Sayeb Al-Atrash R, Alsuliman A, Vatte C Tags: Acta Haematol Source Type: research

CD56 for Multiple Myeloma: Lack of CD56 May Be Associated with Worse Prognosis
Acta Haematol 2018;140:40 –41 (Source: Acta Haematologica)
Source: Acta Haematologica - August 15, 2018 Category: Hematology Source Type: research

Contents Vol. 139, 2018
Acta Haematol 2018;139:I –IV (Source: Acta Haematologica)
Source: Acta Haematologica - August 15, 2018 Category: Hematology Source Type: research

CD56 for Multiple Myeloma: Lack of CD56 May Be Associated with Worse Prognosis.
PMID: 30110676 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 15, 2018 Category: Hematology Authors: Miyazaki K, Suzuki K Tags: Acta Haematol Source Type: research

Transplant Outcomes in Beta-Thalassemia Major Patients Receiving Combined Granulocyte Colony-Stimulating Factor-Primed Bone Marrow and Cord Blood Graft Compared to Granulocyte Colony-Stimulating Factor-Primed Bone Marrow Alone
Conclusions: A combined CB and G-BM graft from an HLA-identical sibling donor is an effective treatment option for TM in children, with less acute and chronic GVHD.Acta Haematol 2018;140:20 –29 (Source: Acta Haematologica)
Source: Acta Haematologica - August 2, 2018 Category: Hematology Source Type: research

A Pilot Phase II Study of Erlotinib for the Treatment of Patients with Relapsed/Refractory Acute Myeloid Leukemia
Erlotinib, an epidermal growth factor receptor (EGFR) inhibitor, may have off-target activity inducing acute myeloid leukemia (AML) differentiation, possibly through SYK inhibition. We investigated erlotinib in a pilot phase II study for efficacy in relapsed/refractory AML patients at a dose of 150 mg once daily in 28-day cycles. Twenty-nine patients were treated for a median of 29 days (range 12 –142 days). Seven patients (24%) received #x3e; 1 cycle of therapy and 12 (41%) discontinued treatment before day 28 due to disease progression. One patient (3%) achieved complete remission and 2 (7%) a #x3e; 50% reduction i...
Source: Acta Haematologica - August 2, 2018 Category: Hematology Source Type: research

A Pilot Phase II Study of Erlotinib for the Treatment of Patients with Relapsed/Refractory Acute Myeloid Leukemia.
Abstract Erlotinib, an epidermal growth factor receptor (EGFR) inhibitor, may have off-target activity inducing acute myeloid leukemia (AML) differentiation, possibly through SYK inhibition. We investigated erlotinib in a pilot phase II study for efficacy in relapsed/refractory AML patients at a dose of 150 mg once daily in 28-day cycles. Twenty-nine patients were treated for a median of 29 days (range 12-142 days). Seven patients (24%) received> 1 cycle of therapy and 12 (41%) discontinued treatment before day 28 due to disease progression. One patient (3%) achieved complete remission and 2 (7%) a> 50% redu...
Source: Acta Haematologica - August 2, 2018 Category: Hematology Authors: Abou Dalle I, Cortes JE, Pinnamaneni P, Lamothe B, Diaz Duque A, Randhawa J, Pemmaraju N, Jabbour E, Ferrajoli A, Wierda WG, Estrov Z, Konopleva M, Ravandi F, Alvarado Y, Borthakur G, Gandhi V, Kantarjian HM Tags: Acta Haematol Source Type: research

Transplant Outcomes in Beta-Thalassemia Major Patients Receiving Combined Granulocyte Colony-Stimulating Factor-Primed Bone Marrow and Cord Blood Graft Compared to Granulocyte Colony-Stimulating Factor-Primed Bone Marrow Alone.
CONCLUSIONS: A combined CB and G-BM graft from an HLA-identical sibling donor is an effective treatment option for TM in children, with less acute and chronic GVHD. PMID: 30071526 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 2, 2018 Category: Hematology Authors: Wen J, Haque Q, Pei F, Chen L, Ruan Y, Liu X, He Y, Feng X, Li C, Wu X Tags: Acta Haematol Source Type: research

Direct Antiglobulin Test-Negative Autoimmune Hemolytic Anemia
Acta Haematol 2018;140:18 –19 (Source: Acta Haematologica)
Source: Acta Haematologica - July 13, 2018 Category: Hematology Source Type: research

Psychometric Properties of a Modified KINDL-R Questionnaire for Adolescents and Adults, and Construction of a Brief Version, the KINDL-A(dult)B(rief) Questionnaire, KINDL-AB
Conclusions: The new KINDL-AB suggests sufficient to good psychometric properties in adult patients with hereditary and acquired bleeding disorders.Acta Haematol 2018;140:1 –9 (Source: Acta Haematologica)
Source: Acta Haematologica - July 13, 2018 Category: Hematology Source Type: research

A Comprehensive Diagnostic Algorithm for Direct Antiglobulin Test-Negative Autoimmune Hemolytic Anemia Reveals the Relative Ratio of Three Mechanisms in a Single Laboratory
Conclusion: Relative ratios of patients with DAT-negative AIHA resulting from RBC-bound IgG, RBC-bound IgA, RBC-bound IgM, and low-affinity IgG were estimated as 80, 4, 1 and 15%, respectively. A new classification and diagnostic algorithm for DAT-negative AIHA were proposed.Acta Haematol 2018;140:10 –17 (Source: Acta Haematologica)
Source: Acta Haematologica - July 13, 2018 Category: Hematology Source Type: research

Psychometric Properties of a Modified KINDL-R Questionnaire for Adolescents and Adults, and Construction of a Brief Version, the KINDL-A(dult)B(rief) Questionnaire, KINDL-AB.
l U Abstract BACKGROUND/AIMS: The generic quality of life KINDL-R -questionnaire is validated for use in children/adolescents ≤16 years. The aim of this cross-sectional investigation was to modify the KINDL-R questionnaire for use in adults and to validate its psychometric properties. METHODS: Five items of the KINDL-R questionnaire were adapted and the newly developed KINDL-A(dult) questionnaire administered to 255 patients with hereditary and acquired bleeding disorders (mean age 53 years). Its internal consistency and convergent and divergent construct validity were investigated and confirmatory factor ...
Source: Acta Haematologica - July 13, 2018 Category: Hematology Authors: Neuner B, Krampe H, McCarthy WJ, Reinke S, Kowalski D, Clausnizer H, Shneyder M, Rocke A, Nowak-Göttl U Tags: Acta Haematol Source Type: research

A Comprehensive Diagnostic Algorithm for Direct Antiglobulin Test-Negative Autoimmune Hemolytic Anemia Reveals the Relative Ratio of Three Mechanisms in a Single Laboratory.
CONCLUSION: Relative ratios of patients with DAT-negative AIHA resulting from RBC-bound IgG, RBC-bound IgA, RBC-bound IgM, and low-affinity IgG were estimated as 80, 4, 1 and 15%, respectively. A new classification and diagnostic algorithm for DAT-negative AIHA were proposed. PMID: 30007985 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 13, 2018 Category: Hematology Authors: Kamesaki T, Kajii E Tags: Acta Haematol Source Type: research

Direct Antiglobulin Test-Negative Autoimmune Hemolytic Anemia.
PMID: 30007988 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 13, 2018 Category: Hematology Authors: Takahashi T Tags: Acta Haematol Source Type: research

Leptomeningeal Myelomatosis: A Rare but Devastating Manifestation of Multiple Myeloma Diagnosed Using Cytology, Flow Cytometry, and Fluorescent in situ Hybridization
Conclusion: By combining several technical procedures, it is possible to identify most patients with LMM. Management of affected patients is challenging and the survival short after a diagnosis of LMM.Acta Haematol 2018;139:247 –254 (Source: Acta Haematologica)
Source: Acta Haematologica - July 11, 2018 Category: Hematology Source Type: research

Pharmacogenetic Warfarin Dosing Algorithms: Validity in Egyptian Patients
Conclusions: The Gage ­genetic warfarin dosing algorithm is the best model that could be implemented in Egyptian patients starting warfarin therapy.Acta Haematol 2018;139:255 –262 (Source: Acta Haematologica)
Source: Acta Haematologica - July 11, 2018 Category: Hematology Source Type: research

Leptomeningeal Myelomatosis: A Rare but Devastating Manifestation of Multiple Myeloma Diagnosed Using Cytology, Flow Cytometry, and Fluorescent in situ Hybridization.
CONCLUSION: By combining several technical procedures, it is possible to identify most patients with LMM. Management of affected patients is challenging and the survival short after a diagnosis of LMM. PMID: 29996124 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 11, 2018 Category: Hematology Authors: Bommer M, Kull M, Teleanu V, Schwarzwälder P, Feuring-Buske M, Kroenke J, Bunjes D, Langer C Tags: Acta Haematol Source Type: research

Pharmacogenetic Warfarin Dosing Algorithms: Validity in Egyptian Patients.
CONCLUSIONS: The Gage -genetic warfarin dosing algorithm is the best model that could be implemented in Egyptian patients starting warfarin therapy. PMID: 29996126 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 11, 2018 Category: Hematology Authors: Selim TE, Azzam HA, Ghoneim HR, Mohamed AA, El Wakeel H, Abu Bakr HM Tags: Acta Haematol Source Type: research

Gaucher Disease and Myelofibrosis: A Combined Disease or a Misdiagnosis?
Conclusion: In order to avoid a misdiagnosis, a diagnostic algorithm for patients with hepatosplenomegaly combined with cytopenia is suggested.Acta Haematol 2018;139:240 –242 (Source: Acta Haematologica)
Source: Acta Haematologica - June 26, 2018 Category: Hematology Source Type: research

Management of Abdominoperineal Excision of the Rectum in a Patient with Glanzmann Thrombasthenia
Acta Haematol 2018;139:243 –246 (Source: Acta Haematologica)
Source: Acta Haematologica - June 26, 2018 Category: Hematology Source Type: research

Genetic Predisposition to Myeloproliferative Neoplasms: Another Piece of the Puzzle
Acta Haematol 2018;139:238 –239 (Source: Acta Haematologica)
Source: Acta Haematologica - June 26, 2018 Category: Hematology Source Type: research