The Reality of Pixantrone in Real Life
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - October 20, 2020 Category: Hematology Source Type: research

Effectiveness and Safety of Pixantrone for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma in Every-Day Clinical Practice: The Italian Cohort of the PIXA Registry
Conclusion: Our data documented drug efficacy that is satisfactory for this high-risk subset of patients with an acceptable toxicity profile. Results indicate that pixantrone could be a significant treatment option in patients with R/R aggressive DLBCL treated in everyday clinical practice.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - October 9, 2020 Category: Hematology Source Type: research

Effectiveness and Safety of Pixantrone for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma in Every-Day Clinical Practice: The Italian Cohort of the PIXA Registry.
CONCLUSION: Our data documented drug efficacy that is satisfactory for this high-risk subset of patients with an acceptable toxicity profile. Results indicate that pixantrone could be a significant treatment option in patients with R/R aggressive DLBCL treated in everyday clinical practice. PMID: 33040061 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 9, 2020 Category: Hematology Authors: Zinzani PL, Bregni M, Spione M, Mitterer M, Musuraca G, Bugli A, Piazza F, Pinto A Tags: Acta Haematol Source Type: research

A Haematologist ’s Guide to Coronavirus Disease 2019: Encyclopaedia or Doorstop?
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - October 8, 2020 Category: Hematology Source Type: research

A Haematologist's Guide to Coronavirus Disease 2019: Encyclopaedia or Doorstop?
PMID: 33032279 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 8, 2020 Category: Hematology Authors: Gale RP Tags: Acta Haematol Source Type: research

Leukemic Phase of Histiocytic Sarcoma of the Digestive System: A Rare Manifestation of a Rare Disease
We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss...
Source: Acta Haematologica - October 5, 2020 Category: Hematology Source Type: research

Leukemic Phase of Histiocytic Sarcoma of the Digestive System: A Rare Manifestation of a Rare Disease.
We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss...
Source: Acta Haematologica - October 4, 2020 Category: Hematology Authors: Hofstetter L, Aranovich D, Bernstine H, Hayman L, Shahal-Zimra Y, Rabizadeh E, Cohen A, Lahav M, Raanani P, Wolach O Tags: Acta Haematol Source Type: research

Perls Stain Grade in Bone Marrow Aspirate Correlates with Overall Survival in Low-Risk Myelodysplastic Patients
Low-risk patients with myelodysplastic syndromes (MDS) are inclined to long-term accumulation of iron in the organs due mostly to red blood cell transfusion and ineffective erythropoiesis. The effect of free toxic iron species in the liver and heart sites is well known, but recent knowledge assumes that oxidant-mediated tissue injury is also effective in the bone marrow. We aimed to investigate the predictive value of bone marrow iron accumulation as demonstrated by Perls staining on the overall survival (OS) of MDS patients. We retrospectively analyzed 114 low and intermediate-I IPSS risk MDS patients who were diagnosed a...
Source: Acta Haematologica - October 2, 2020 Category: Hematology Source Type: research

Perls Stain Grade in Bone Marrow Aspirate Correlates with Overall Survival in Low-Risk Myelodysplastic Patients.
Abstract Low-risk patients with myelodysplastic syndromes (MDS) are inclined to long-term accumulation of iron in the organs due mostly to red blood cell transfusion and ineffective erythropoiesis. The effect of free toxic iron species in the liver and heart sites is well known, but recent knowledge assumes that oxidant-mediated tissue injury is also effective in the bone marrow. We aimed to investigate the predictive value of bone marrow iron accumulation as demonstrated by Perls staining on the overall survival (OS) of MDS patients. We retrospectively analyzed 114 low and intermediate-I IPSS risk MDS patients wh...
Source: Acta Haematologica - October 1, 2020 Category: Hematology Authors: Pilo F, Caocci G, Mele G, La Nasa G Tags: Acta Haematol Source Type: research

Relationship of Thrombospondin 1 to von Willebrand Factor and ADAMTS-13 in Sickle Cell Disease Patients of Arab Ethnicity
Conclusions: Our findings confirm the inhibitory effects of TSP-1 on ADAMTS-13 activity in adult SCD patients. The negative correlation reported between TSP-1 and ADAMTS-13/vWF antigen ratio in pediatric subjects suggests a possible protective mechanism in younger individuals, although this is not related to the presence of SCD. This work emphasizes the impact of age on interpreting results related to the regulation of vWF expression and interaction with TSP-1 and ADAMTS-13 in SCD.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 28, 2020 Category: Hematology Source Type: research

Eltrombopag for the Treatment of Severe Inherited Thrombocytopenia
Inherited thrombocytopenias correspond to a group of hereditary disorders characterized by a reduced platelet count, platelet dysfunction, and a family history of thrombocytopenia. It is commonly associated with mucocutaneous bleeding. Thrombocytopenia results from mutations in genes involved in megakaryocyte differentiation, platelet formation, and clearance. Here we report on a patient presenting with severe syndromic inherited thrombocytopenia manifesting as spontaneous mucocutaneous bleeds, requiring frequent platelet transfusions. Thrombocytopenia was explained by the presence of 4 mutations in 3 hematopoietic transcr...
Source: Acta Haematologica - September 28, 2020 Category: Hematology Source Type: research

Relationship of Thrombospondin 1 to von Willebrand Factor and ADAMTS-13 in Sickle Cell Disease Patients of Arab Ethnicity.
CONCLUSIONS: Our findings confirm the inhibitory effects of TSP-1 on ADAMTS-13 activity in adult SCD patients. The negative correlation reported between TSP-1 and ADAMTS-13/vWF antigen ratio in pediatric subjects suggests a possible protective mechanism in younger individuals, although this is not related to the presence of SCD. This work emphasizes the impact of age on interpreting results related to the regulation of vWF expression and interaction with TSP-1 and ADAMTS-13 in SCD. PMID: 32987383 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 27, 2020 Category: Hematology Authors: Al-Awadhi A, Adekile A, Marouf R Tags: Acta Haematol Source Type: research

Eltrombopag for the Treatment of Severe Inherited Thrombocytopenia.
Abstract Inherited thrombocytopenias correspond to a group of hereditary disorders characterized by a reduced platelet count, platelet dysfunction, and a family history of thrombocytopenia. It is commonly associated with mucocutaneous bleeding. Thrombocytopenia results from mutations in genes involved in megakaryocyte differentiation, platelet formation, and clearance. Here we report on a patient presenting with severe syndromic inherited thrombocytopenia manifesting as spontaneous mucocutaneous bleeds, requiring frequent platelet transfusions. Thrombocytopenia was explained by the presence of 4 mutations in 3 hem...
Source: Acta Haematologica - September 27, 2020 Category: Hematology Authors: Abdelmoumen K, Fabre M, Ducastelle-Lepretre S, Favier R, Ballerini P, Bordet JC, Dargaud Y Tags: Acta Haematol Source Type: research

Severe Protein C Deficiency due to Novel Biallelic Variants in < b > < i > PROC < /i > < /b > and Their Phenotype Correlation
Severe protein C deficiency due to biallelicPROC mutations is an extremely rare thrombophilia, most commonly presenting during the neonatal period as purpura fulminans. Despite treatment, severe morbidity and mortality are frequent. The current study reports 3 unrelated patients harboring novel homozygousPROC mutations and their clinical phenotypes. We discuss how the cytoprotective activity of protein C and its role in the stabilization of endothelial barriers may account for the unique symptoms of this thrombophilia.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 25, 2020 Category: Hematology Source Type: research

StALKing Histiocytosis: ALK-Positive Histiocytosis Identified through Peripheral Blood Smear
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 25, 2020 Category: Hematology Source Type: research

Management of COVID-19 Coagulopathy in a Patient with Severe Haemophilia A
A 54-year-old man with a long history of severe haemophilia A treated prophylactically with efmoroctocog alpha (3,000 IU twice weekly) was diagnosed with COVID-19 infection. He had multiple risk factors for COVID-19 severity including obesity, diabetes mellitus and hypertension. He required prolonged intensive care unit (ICU) stay due to the severity of respiratory failure until his death on day 24. During his ICU stay, he received a continuous infusion of efmoroctocog alpha in order to maintain factor VIII activity between 80 and 100%, together with therapeutic doses of low-molecular-weight heparin targeting anti-Xa activ...
Source: Acta Haematologica - September 25, 2020 Category: Hematology Source Type: research

Management of COVID-19 Coagulopathy in a Patient with Severe Haemophilia A.
Abstract A 54-year-old man with a long history of severe haemophilia A treated prophylactically with efmoroctocog alpha (3,000 IU twice weekly) was diagnosed with COVID-19 infection. He had multiple risk factors for COVID-19 severity including obesity, diabetes mellitus and hypertension. He required prolonged intensive care unit (ICU) stay due to the severity of respiratory failure until his death on day 24. During his ICU stay, he received a continuous infusion of efmoroctocog alpha in order to maintain factor VIII activity between 80 and 100%, together with therapeutic doses of low-molecular-weight heparin targe...
Source: Acta Haematologica - September 24, 2020 Category: Hematology Authors: Pinto Pereira J, Hantson P, Gerard L, Wittebole X, Laterre PF, Lambert C, Hermans C Tags: Acta Haematol Source Type: research

Severe Protein C Deficiency due to Novel Biallelic Variants in PROC and Their Phenotype Correlation.
Abstract Severe protein C deficiency due to biallelic PROC mutations is an extremely rare thrombophilia, most commonly presenting during the neonatal period as purpura fulminans. Despite treatment, severe morbidity and mortality are frequent. The current study reports 3 unrelated patients harboring novel homozygous PROC mutations and their clinical phenotypes. We discuss how the cytoprotective activity of protein C and its role in the stabilization of endothelial barriers may account for the unique symptoms of this thrombophilia. PMID: 32980846 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 24, 2020 Category: Hematology Authors: Barg AA, Dardik R, Levin C, Koren A, Levy-Mendelovich S, Pode-Shakked B, Kenet G Tags: Acta Haematol Source Type: research

StALKing Histiocytosis: ALK-Positive Histiocytosis Identified through Peripheral Blood Smear.
PMID: 32980847 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 24, 2020 Category: Hematology Authors: Andolina JR Tags: Acta Haematol Source Type: research

Pretreatment Immature Platelet Fraction as a Surrogate of Reticulated Platelets Predicts the Response to Corticosteroids in Adults with Immune Thrombocytopenia
Conclusions: Our findings thus suggest that measuring the IPF% as a surrogate of reticulated platelets is useful to identify patients likely to respond to corticosteroids.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 17, 2020 Category: Hematology Source Type: research

Pretreatment Immature Platelet Fraction as a Surrogate of Reticulated Platelets Predicts the Response to Corticosteroids in Adults with Immune Thrombocytopenia.
Abstract OBJECTIVES: Reticulated platelets circulating in the blood reflect megakaryopoietic activity and platelet turnover and can be automatically and low-invasively measured as the immature platelet fraction (IPF) using a Sysmex XN hematocytometer. The present study retrospectively investigated whether or not the IPF can predict the treatment response to corticosteroids in adult patients with primary immune thrombocytopenia (ITP). METHODS: Forty-six patients who had been newly diagnosed with primary treatment-naïve ITP and started treatment with corticosteroids were analyzed. RESULTS: Among the 4...
Source: Acta Haematologica - September 16, 2020 Category: Hematology Authors: Takami A, Mizuno S, Nakamura A, Kanasugi J, Yamamoto H, Vu Quang L, Nakagami Y, Nakano Y, Yamada S, Matsumura S, Takasugi S, Uchino K, Horio T, Murakami S, Oohigashi Y, Nakayama T, Tani H, Enomoto M, Hanamura I Tags: Acta Haematol Source Type: research

COVID-19 in Patients with Hematologic Disorders Undergoing Therapy: Perspective of a Large Referral Hematology Center in Rome
Conclusion: In our experience, the prevalence of COVID-19 in hematologic patients, mainly affected by malignancies, was not significantly higher compared to that of the general population. Definition of adapted strategies for healthcare services, while continuing to administer the standard hematologic treatments, represents the crucial challenge for the management of hematologic diseases in the COVID-19 era.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 15, 2020 Category: Hematology Source Type: research

COVID-19 in Patients with Hematologic Disorders Undergoing Therapy: Perspective of a Large Referral Hematology Center in Rome.
CONCLUSION: In our experience, the prevalence of COVID-19 in hematologic patients, mainly affected by malignancies, was not significantly higher compared to that of the general population. Definition of adapted strategies for healthcare services, while continuing to administer the standard hematologic treatments, represents the crucial challenge for the management of hematologic diseases in the COVID-19 era. PMID: 32932252 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - September 14, 2020 Category: Hematology Authors: Girmenia C, Gentile G, Micozzi A, Petrucci L, Malaspina F, Di Prima A, Baldacci E, Bianchi S, Pugliese P, Turriziani O, Antonelli G, Tombolini V, Foà R, Martelli M Tags: Acta Haematol Source Type: research

Concomitant Primary Hyperparathyroidism in Patients with Multiple Myeloma: A Possible Link?
Hypercalcemia is a significant feature of patients with active multiple myeloma (MM) with extensive bone disease. Among the causes of non-neoplastic hypercalcemia, primary hyperparathyroidism (PHPT) is one of the most common, leading to osteoporosis and bone fractures. Interestingly, some preclinical data indicate that high secretion of parathyroid hormone (PTH) may have a negative impact on bone disease and MM progression. However, concomitant diagnosis of MM and PHPT has rarely been described. Here, we present 4 cases of patients with active MM and hypercalcemia with high or inappropriately normal PTH levels. Interesting...
Source: Acta Haematologica - September 9, 2020 Category: Hematology Source Type: research

Presence of Parvovirus B19 but Not Herpesvirus Genome in Acute Skin Rash after Allogeneic Stem Cell Transplantation Correlates with Outcome
Conclusions: Detection of ParvoB19 DNA in exanthema after ASCT might be associated with lower NRM.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 9, 2020 Category: Hematology Source Type: research

Presence of Parvovirus B19 but Not Herpesvirus Genome in Acute Skin Rash after Allogeneic Stem Cell Transplantation Correlates with Outcome.
LP Abstract INTRODUCTION: Skin rash is a first symptom of acute graft-versus-host disease (GvHD) after allogeneic stem cell transplantation (ASCT) but can also be caused by viruses. The relevance of virus DNA analyses in skin rash for diagnosis and clinical outcome is unknown. OBJECTIVES: To record the frequencies of detection of herpes and parvovirus B19 (ParvoB19) DNA in skin rash within 100 days after ASCT and to analyze their relevance for diagnosis, clinical course, and non-relapse mortality (NRM). METHODS: We retrospectively identified 55 patients with virus DNA analysis for CMV, EBV, HHV6, HHV8, ...
Source: Acta Haematologica - September 8, 2020 Category: Hematology Authors: Weber T, Schmidberger A, Ligeti K, Bauer M, Rosenwald A, Müller LP Tags: Acta Haematol Source Type: research

Concomitant Primary Hyperparathyroidism in Patients with Multiple Myeloma: A Possible Link?
Abstract Hypercalcemia is a significant feature of patients with active multiple myeloma (MM) with extensive bone disease. Among the causes of non-neoplastic hypercalcemia, primary hyperparathyroidism (PHPT) is one of the most common, leading to osteoporosis and bone fractures. Interestingly, some preclinical data indicate that high secretion of parathyroid hormone (PTH) may have a negative impact on bone disease and MM progression. However, concomitant diagnosis of MM and PHPT has rarely been described. Here, we present 4 cases of patients with active MM and hypercalcemia with high or inappropriately normal PTH l...
Source: Acta Haematologica - September 8, 2020 Category: Hematology Authors: Notarfranchi L, Marchica V, Dalla Palma B, Pelagatti L, Burroughs-Garcia J, Pedrazzoni M, Ruffini L, Cetani F, Marcocci C, Giuliani N Tags: Acta Haematol Source Type: research

Rituximab Monotherapy or in Combination with Bendamustine Is Not Inferior to Rituximab-CHOP Regimen in the Treatment of Patients with Splenic Marginal Zone Lymphoma in the Real Life
Splenic marginal zone lymphoma (SMZL) is a rare lymphoma belonging to the marginal zone lymphoproliferative disorders. Usually, SMZL occurs with indolent presentation and, when required, the standard of care is represented by rituximab-based regimens. No direct comparison of different rituximab-based combinations and polychemotherapy regimens has been conducted to date. In a monocentric cohort of 68 SMLZ patients, we showed that rituximab in monotherapy or in combination with bendamustine, compared with rituximab associated with the polychemotherapy cycle cyclophosphamide, hydroxydaunorubicin, vincristine and prednisolone ...
Source: Acta Haematologica - September 4, 2020 Category: Hematology Source Type: research

Rituximab Monotherapy or in Combination with Bendamustine Is Not Inferior to Rituximab-CHOP Regimen in the Treatment of Patients with Splenic Marginal Zone Lymphoma in the Real Life.
asa G Abstract Splenic marginal zone lymphoma (SMZL) is a rare lymphoma belonging to the marginal zone lymphoproliferative disorders. Usually, SMZL occurs with indolent presentation and, when required, the standard of care is represented by rituximab-based regimens. No direct comparison of different rituximab-based combinations and polychemotherapy regimens has been conducted to date. In a monocentric cohort of 68 SMLZ patients, we showed that rituximab in monotherapy or in combination with bendamustine, compared with rituximab associated with the polychemotherapy cycle cyclophosphamide, hydroxydaunorubicin, vincr...
Source: Acta Haematologica - September 3, 2020 Category: Hematology Authors: Mulas O, Caocci G, Dessì D, Mantovani D, Moi G, Cabras MG, La Nasa G Tags: Acta Haematol Source Type: research

PNH Clones for Aplastic Anemia with Immunosuppressive Therapy: A Systematic Review and Meta-Analysis
Conclusions: The meta-analysis suggested an evidence-based role for PNH clones in predicting a better response in AA patients with immunosuppression.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - September 2, 2020 Category: Hematology Source Type: research

PNH Clones for Aplastic Anemia with Immunosuppressive Therapy: A Systematic Review and Meta-Analysis.
Abstract OBJECTIVES: PNH clones, also aptly called "escape clones," are evidence of acquired immune-mediated bone marrow failure and have a high prevalence in patients with aplastic anemia (AA). Several studies have reported contradictory results regarding the impact of PNH clones on AA patients with immunosuppression treatment, and PNH clones have not been confirmed as positive predictors of response in the AA guidelines of the British Society for Standards in Haematology. METHODS: We performed a meta-analysis to address this issue by searching for articles in PubMed, EMBASE, The Coch-rane Library,...
Source: Acta Haematologica - September 1, 2020 Category: Hematology Authors: Tu J, Pan H, Li R, Wang Z, Lian Y, Li W, Shi J, Fang L Tags: Acta Haematol Source Type: research

Red Blood Cell Distribution Is a Significant Predictor of Severe Illness in Coronavirus Disease 2019
Conclusion: These results, along with data published in other studies, support the use of RDW for assessing the risk of unfavorable COVID-19 progression.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - August 25, 2020 Category: Hematology Source Type: research

Red Blood Cell Distribution Is a Significant Predictor of Severe Illness in Coronavirus Disease 2019.
Abstract INTRODUCTION: As red blood cell distribution width (RDW) significantly predicts clinical outcomes in patients with respiratory tract infections and in those with critical illnesses, we performed a critical analysis of the literature to explore the potential prognostic role of this laboratory parameter in coronavirus disease 2019 (COVID-19). METHODS: An electronic search was conducted in Medline, Scopus and Web of Science, using the keywords "coronavirus disease 2019" OR "COVID-19" AND "red blood cell distribution width" OR "RDW" in all fields, up to the present...
Source: Acta Haematologica - August 24, 2020 Category: Hematology Authors: Lippi G, Henry BM, Sanchis-Gomar F Tags: Acta Haematol Source Type: research

Ruxolitinib in COVID-19 Hyperinflammation and Haematologic Malignancies
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - August 20, 2020 Category: Hematology Source Type: research

Ruxolitinib in COVID-19 Hyperinflammation and Haematologic Malignancies.
ée P PMID: 32818929 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 19, 2020 Category: Hematology Authors: La Rosée F, La Rosée P Tags: Acta Haematol Source Type: research

A Novel Combination for Graft-versus-Host-Disease Prophylaxis: Lessons Learned from the Birth of Sushi Burrito!
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - August 19, 2020 Category: Hematology Source Type: research

Extramedullary Acute Myeloid Leukemia of the Renal Pelvis: Insights into a Visceral Niche
Acute myeloid leukemia (AML) is a stem cell malignancy that originates in the bone marrow and involves the peripheral blood. Extramedullary AML is rarer, but it is most commonly associated with the former French-American-British (FAB) subtypes M4 or M5 of AML. AML cells may also home to the central nervous system and other solid organs such as cortical bone and skin. Such target sites of metastasis depend on microenvironmental niche interactions, which have not been fully elucidated to date. Visceral organs usually do not represent a favorable niche for AML stem cell occupancy. Herein, we describe the case of an 80-year-ol...
Source: Acta Haematologica - August 19, 2020 Category: Hematology Source Type: research

A Novel Combination for Graft-versus-Host-Disease Prophylaxis: Lessons Learned from the Birth of Sushi Burrito!
PMID: 32814312 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 18, 2020 Category: Hematology Authors: Hashmi SK Tags: Acta Haematol Source Type: research

Extramedullary Acute Myeloid Leukemia of the Renal Pelvis: Insights into a Visceral Niche.
Abstract Acute myeloid leukemia (AML) is a stem cell malignancy that originates in the bone marrow and involves the peripheral blood. Extramedullary AML is rarer, but it is most commonly associated with the former French-American-British (FAB) subtypes M4 or M5 of AML. AML cells may also home to the central nervous system and other solid organs such as cortical bone and skin. Such target sites of metastasis depend on microenvironmental niche interactions, which have not been fully elucidated to date. Visceral organs usually do not represent a favorable niche for AML stem cell occupancy. Herein, we describe the cas...
Source: Acta Haematologica - August 18, 2020 Category: Hematology Authors: Patel SA, Bledsoe JR, Gordon J, Cerny J Tags: Acta Haematol Source Type: research

Current and Emerging Treatments for Waldenstr öm Macroglobulinemia
Waldenstr öm macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma. The primary goal of therapy is to reduce symptoms related to direct infiltration of the bone marrow and decrease monoclonal IgM-associated complications. Active agents in the management of WM can be broadly classified as rituximab-alky lator combination therapy, proteasome inhibitor-based therapy, and Bruton’s tyrosine kinase inhibitor-based therapy.MYD88L265P andCXCR4 genetic status are pivotal for tailoring treatment options. Ibrutinib is a suitable treatment option for both treatment-na ïve and relapsing WM patients. Recent advance...
Source: Acta Haematologica - August 18, 2020 Category: Hematology Source Type: research

Current and Emerging Treatments for Waldenstr öm Macroglobulinemia.
Current and Emerging Treatments for Waldenström Macroglobulinemia. Acta Haematol. 2020 Aug 18;:1-12 Authors: Grimont CN, Castillo Almeida NE, Gertz MA Abstract Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma. The primary goal of therapy is to reduce symptoms related to direct infiltration of the bone marrow and decrease monoclonal IgM-associated complications. Active agents in the management of WM can be broadly classified as rituximab-alkylator combination therapy, proteasome inhibitor-based therapy, and Bruton's tyrosine kinase inhibitor-based therapy. MYD88L265P an...
Source: Acta Haematologica - August 17, 2020 Category: Hematology Authors: Grimont CN, Castillo Almeida NE, Gertz MA Tags: Acta Haematol Source Type: research

G-CSF and GM-CSF Are Different. Which One Is Better for COVID-19?
Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - August 13, 2020 Category: Hematology Source Type: research

G-CSF and GM-CSF Are Different. Which One Is Better for COVID-19?
PMID: 32791509 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 12, 2020 Category: Hematology Authors: Lazarus HM, Gale RP Tags: Acta Haematol Source Type: research

Plasma Exchange as an Initial Treatment for Severe Bleeding Induced by Acquired Factor V Deficiency: A Case Report and Mini Literature Review
Acquired factor V deficiency (AFVD) is a rare autoimmune bleeding disorder. Unlike acquired hemophilia, bypass therapies with recombinant activated factor VII and activated prothrombin complex concentrates are ineffective for severe bleeding due to AFVD. Although several treatment strategies have been attempted, a standard of care for severe hemorrhage induced by AFVD is lacking. Herein, we report a case of AFVD with severe bleeding that responded to plasma exchange (PE) combined with immunosuppression. We also reviewed previously reported AFVD cases with severe hemorrhage and suggest that PE may be an effective initial tr...
Source: Acta Haematologica - August 12, 2020 Category: Hematology Source Type: research

Plasma Exchange as an Initial Treatment for Severe Bleeding Induced by Acquired Factor V Deficiency: A Case Report and Mini Literature Review.
Abstract Acquired factor V deficiency (AFVD) is a rare autoimmune bleeding disorder. Unlike acquired hemophilia, bypass therapies with recombinant activated factor VII and activated prothrombin complex concentrates are ineffective for severe bleeding due to AFVD. Although several treatment strategies have been attempted, a standard of care for severe hemorrhage induced by AFVD is lacking. Herein, we report a case of AFVD with severe bleeding that responded to plasma exchange (PE) combined with immunosuppression. We also reviewed previously reported AFVD cases with severe hemorrhage and suggest that PE may be an ef...
Source: Acta Haematologica - August 11, 2020 Category: Hematology Authors: Yanagiya R, Kanouchi K, Toubai T, Yamada A, Aizawa K, Shiono Y, Ito S, Ishizawa K Tags: Acta Haematol Source Type: research

Subject Index
Acta Haematol 2020;143:402 (Source: Acta Haematologica)
Source: Acta Haematologica - July 30, 2020 Category: Hematology Source Type: research

Author Index
Acta Haematol 2020;143:401 (Source: Acta Haematologica)
Source: Acta Haematologica - July 30, 2020 Category: Hematology Source Type: research

Prelims
Acta Haematol 2020;143:297 –298 (Source: Acta Haematologica)
Source: Acta Haematologica - July 30, 2020 Category: Hematology Source Type: research

JAK/STAT Pathway Inhibition May Be a Promising Therapy for COVID-19-Related Hyperinflammation in Hematologic Patients
COVID-19 has rapidly become a major concern for the health systems worldwide. Its high contagiousness and associated mortality demand the discovery of helpful interventions with promising safety profile. Here, we report 3 severe COVID-19 cases, which achieved rapid and sustained improvement in outcome with the use of ruxolitinib, a JAK/STAT pathway inhibitor.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - July 29, 2020 Category: Hematology Source Type: research

JAK/STAT Pathway Inhibition May Be a Promising Therapy for COVID-19-Related Hyperinflammation in Hematologic Patients.
Abstract COVID-19 has rapidly become a major concern for the health systems worldwide. Its high contagiousness and associated mortality demand the discovery of helpful interventions with promising safety profile. Here, we report 3 severe COVID-19 cases, which achieved rapid and sustained improvement in outcome with the use of ruxolitinib, a JAK/STAT pathway inhibitor. PMID: 32726783 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 28, 2020 Category: Hematology Authors: Rojas P, Sarmiento M Tags: Acta Haematol Source Type: research