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Hematology Reports

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https://www.readbyqxmd.com/read/27781079/atypical-hemolytic-uremic-syndrome-secondary-to-lupus-nephritis-responsive-to-eculizumab
#1
Alexander G Raufi, Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, Minh-Ha Tran
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27757216/gbt440-inhibits-sickling-of-sickle-cell-trait-blood-under-in-vitro-conditions-mimicking-strenuous-exercise
#2
Kobina Dufu, Josh Lehrer-Graiwer, Eleanor Ramos, Donna Oksenberg
In sickle cell trait (SCT), hemoglobin A (HbA) and S (HbS) are co-expressed in each red blood cell (RBC). While homozygous expression of HbS (HbSS) leads to polymerization and sickling of RBCs resulting in sickle cell disease (SCD) characterized by hemolytic anemia, painful vaso-occlusive episodes and shortened life-span, SCT is considered a benign condition usually with minor or no complications related to sickling. However, physical activities that cause increased tissue oxygen demand, dehydration and/or metabolic acidosis leads to increased HbS polymerization and life-threatening complications including death...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27757215/brentuximab-vedotin-therapy-for-cutaneous-lesions-in-t-prolymphocytic-leukemia-a-case-report
#3
Jordan Senchak, Peter Pickens
We present an 88-year-old male with simultaneous T-cell prolymphocytic leukemia and stable smoldering myeloma with excellent initial response to three months of alemtuzumab. The patient relapsed at twelve months with severe cutaneous disease. Biopsy of a representative plaque demonstrated CD30 positivity in rare malignant lymphocytes. The patient demonstrated no response to reintroduction with a full course of alemtuzumab. He was therefore treated with brentuximab vedotin, resulting in partial remission of skin involvement that persisted for three months...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27757214/positive-impact-of-eculizumab-therapy-on-surgery-for-budd-chiari-syndrome-in-a-patient-with-paroxysmal-nocturnal-hemoglobinuria-and-a-long-term-history-of-thrombosis
#4
Silvia De-la-Iglesia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, Teresa Molero
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27499838/refractory-ascites-with-liver-fibrosis-developed-in-late-phase-allogeneic-hematopoietic-stem-cell-transplantation-report-of-three-patients
#5
Hiroki Hosoi, Kenji Warigaya, Shogo Murata, Toshiki Mushino, Kodai Kuriyama, Akinori Nishikawa, Shinobu Tamura, Kazuo Hatanaka, Nobuyoshi Hanaoka, Yasuteru Muragaki, Shinichi Murata, Hideki Nakakuma, Takashi Sonoki
We report cases of three patients of refractory ascites without other fluid retention that occurred around five months after allogeneic hematopoietic stem cell transplantation (allo-HSCT). All three patients expired and postmortem examinations revealed unexpected liver fibrosis lacking histological evidences of graft-versus-host-disease (GVHD). The three patients showed normal hepatic function and size before transplantation. During their clinical courses, serum biochemistry test showed no elevation of hepatic enzymes and bilirubin; however, imaging studies demonstrated hepatic atrophy at the onset of ascites...
May 10, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27499837/autoimmune-syndromes-presenting-as-a-paraneoplastic-manifestation-of-myelodysplastic-syndromes-clinical-features-course-treatment-and-outcome
#6
Bradley T Williamson, Lynda Foltz, Heather A Leitch
Autoimmune manifestations (AIM) are reported in up to 10-30% of myelodysplastic syndromes (MDS) patients; this association is not well defined. We present herein a retrospective chart review of single center MDS patients for AIM, a case discussion and a literature review. Of 252 MDS patients examined, 11 (4.4%) had AIM around MDS diagnosis. International Prognostic Scoring System scores were: low or intermediate (int)-1 (n=7); int-2 or high (n=4). AIM were: culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); sweats; pericarditis; polymyalgia rheumatica (n=2 each); mouth ulcers; pulmonary infiltrates; suspicion for Behcet's; polychondritis and undifferentiated (n=1 each)...
May 10, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27499836/cd30-primary-cutaneous-post-transplant-lymphoproliferative-disorder-with-signet-ring-cell-features
#7
Neeta Malviya, Heather Wickless
We report a case of primary cutaneous CD30+ post-transplant lymphoproliferative disorder with an uncommon finding of signet ring cell features in a heart transplant patient. The neoplastic cells were CD4 and CD30 positive, and negative for S-100, pancytokeratin, myeloperoxidase, and CD56. In situ hybridization for Epstein Barr Virus (EBV) was negative, even though the patient did have EBV viremia.
May 10, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27499835/recurrent-syncope-and-cardiac-arrest-in-a-patient-with-systemic-light-chain-amyloidosis-treated-with-bortezomib
#8
Navin Jaipaul, Alexander Pi, Zhiwei Zhang
About 10-15% of patients with multiple myeloma develop light chain (AL) amyloidosis. AL amyloidosis is a systemic disease that may involve multiple organs, often including the heart. It may present clinically with bradyarrhythmia and syncope. The proteasome inhibitor bortezomib has been used with clinical efficacy in treating patients with AL amyloidosis but also implicated as a possible cause of cardiomyocyte injury. We report a case of a 48-year-old man with AL amyloidosis and increased frequency of syncope and cardiac arrest after starting bortezomib...
May 10, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27103981/presentation-and-management-of-paroxysmal-nocturnal-hemoglobinuria-a-single-center-experience
#9
Mehmet Sinan Dal, Abdullah Karakuş, Mehmet Önder Ekmen, Orhan Ayyildiz
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder characterized by intravascular hemolysis. Real-world experience of PNH management is largely unreported. A retrospective analysis was undertaken based on medical records from six patients with PNH [two with aplastic anemia (AA)] treated at our center, Dicle University, Turkey. Diagnosis was based on granulocyte PNH clones, ranging from 93% to 66%. All patients had symptoms consistent with PNH. One patient was managed adequately with supportive measures only...
March 17, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27103980/effect-of-intravenous-iron-supplementation-on-iron-stores-in-non-anemic-iron-deficient-patients-with-hereditary-hemorrhagic-telangiectasia
#10
Torbjörn Karlsson, Honar Cherif
In hereditary hemorrhagic telangiectasia (HHT), frequent episodes of nasal and gastrointestinal bleeding commonly lead to iron-deficiency with or without anemia. In the retrospective study presented here we assessed the iron stores, as determined by analysis of plasma ferritin, during oral and intravenous iron supplementation, respectively, in a population of iron-deficient non-anemic HHT patients who were inadequately iron-repleted by oral supplementation. A switch from oral to intravenous iron supplementation was associated with a significant increase in ferritin in this patient population...
March 17, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27103979/the-high-effect-of-chemomobilization-with-high-dose-etopside-granulocyte-colony-stimulating-factor-in-autologous-hematopoietic-peripheral-blood-stem-cell-transplantation-a-single-center-experience
#11
Şebnem Izmir Güner, Mustafa Teoman Yanmaz, Ahmet Selvi, Cigdem Usul
Autologous hematopoietic stem cell transplantation (auto-HSCT) provides hematopoietic support after high-dose chemotherapy and is the standard of care for patients with multiple myeloma (MM), chemo sensitive relapsed high or intermediate grade non-Hodgkin's lymphoma (NHL) and Hodgkin's lymphoma (HL). However, yields of hematopoietic stem cells vary greatly between patients, and the optimal strategy to mobilize hematopoietic stem cells into peripheral blood for collection has not been defined yet. We investigated the efficacy and safety of chemo mobilization with an intermediate dose etoposide (VP-16; 200 mg/m(2) on days 1-3) and granulocyte-colony stimulating factor (G-CSF)(5 µg/kg twice daily from day 4 through the final day of collection)...
March 17, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27103978/immunoglobulin-d-multiple-myeloma-plasma-cell-leukemia-and-chronic-myelogenous-leukemia-in-a-single-patient-treated-simultaneously-with-lenalidomide-bortezomib-dexamethasone-and-imatinib
#12
Naveed Ali, Peter V Pickens, Herbert E Auerbach
Multiple myeloma (MM) is a neoplastic lymphoproliferative disorder characterized by uncontrolled monoclonal plasma cell proliferation. Among different isotypes of MM, immunoglobulin D (IgD) MM is very rare, representing only 1 to 2% of all isotypes. Chronic myelogenous leukemia (CML) is a neoplastic myeloproliferative disorder of pluripotent hematopoietic stem cell, which is characterized by the uncontrolled proliferation of myeloid cells. An 88-year-old male was diagnosed simultaneously with IgD kappa MM and CML...
March 17, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27103977/coexistence-of-essential-thrombocythemia-iron-refractory-iron-deficiency-anemia-and-renal-cell-carcinoma
#13
Sinem Namdaroğlu, Emre Tekgündüz, Fevzi Altuntaş
Essential thrombocythemia (ET) is a Philadelphia chromosome (Ph)-negative myeloproliferative neoplasm. It is characterized by thrombocytosis and megakaryocytic hyperplasia of the bone marrow with JAK2V617F mutation. Iron-refractory iron deficiency anemia (IRIDA) is an autosomal recessive disorder, which is mainly characterized by iron deficiency anemia not responding to oral iron intake, but partially responding to parenteral iron therapy. Recently, it has been shown that IRIDA has stemmed from mutations in the gene TMPRSS6, which encodes a transmembrane serine protease (matriptase-2) expressed by the liver...
March 17, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27103976/serum-endocan-levels-in-children-with-febrile-neutropenia
#14
Eylem Kiral, Ener Cagri Dinleyici, Ayse Bozkurt-Turhan, Ozcan Bor, Yurdanur Akgun, Necat Akdeniz Akgun
Endocan is an endotelial cell specific molecule; previous studies have shown that serum endocan levels increased in cancer and sepsis and are also related to the severity of sepsis. There are no clinical study about serum endocan levels in children with febrile neutropenia. The aim of this study was to evaluate serum endocan levels in pediatric leukemia patients with febrile neutropenia (n=33) and compare them with children with leukemia without fever (n=33) and also with healthy children (n=24). The median serum endocan level in the first group (children with febrile neutropenia) was statistically significantly higher compared to the leukemic children without febrile neutropenia and also control group (P<0...
March 17, 2016: Hematology Reports
https://www.readbyqxmd.com/read/26734129/eculizumab-treatment-in-a-patient-with-hematopoietic-stem-cell-transplantation-associated-thrombotic-microangiopathy-and-steroid-refractory-acute-graft-versus-host-disease
#15
Cristina Fernández, Ana Lario, Rafael Forés, Rafael Cabrera
A 30-year-old man with acquired aplastic anemia underwent an HLA-identical bone marrow transplant. He developed a grade III acute graft versus host disease (GVHD) refractory to various lines of treatment. On post-transplant day 196, he was diagnosed with stem cell transplantation-associated thrombotic micro-angiopathy (HSCT-TMA) and he received treatment with eculizumab 900 mg iv weekly for 4 doses followed by a single dose of 1200 mg 2 weeks later. After the first dose of eculizumab, the patient ceased to require transfusions and a progressive improvement in analytical parameters for microangiopathy was observed until their complete normalization...
November 23, 2015: Hematology Reports
https://www.readbyqxmd.com/read/26734128/docosahexaenoic-acid-induces-apoptosis-in-primary-chronic-lymphocytic-leukemia-cells
#16
Romain Guièze, Emmanuel Gyan, Olivier Tournilhac, Christelle Halty, Richard Veyrat-Masson, Saïda Akil, Marc Berger, Olivier Hérault, Mary Callanan, Jacques-Olivier Bay
Chronic lymphocytic leukemia is an indolent disorder with an increased infectious risk remaining one of the main causes of death. Development of therapies with higher safety profile is thus a challenging issue. Docosahexaenoic acid (DHA, 22:6) is an omega-3 fatty acid, a natural compound of normal cells, and has been shown to display antitumor potency in cancer. We evaluated the potential in vitro effect of DHA in primary CLL cells. DHA induces high level of in vitro apoptosis compared to oleic acid in a dose-dependent and time-dependent manner...
November 23, 2015: Hematology Reports
https://www.readbyqxmd.com/read/26734127/the-role-of-pten-in-myeloid-malignancies
#17
REVIEW
Alessandro Morotti, Cristina Panuzzo, Sabrina Crivellaro, Giovanna Carrà, Davide Torti, Angelo Guerrasio, Giuseppe Saglio
PTEN deletion in the mouse and in the zebrafish highlights the essential role of this tumor suppressor in the development of myeloid malignancies, in particular acute myeloid leukemia and myeloproliferative disorders. In humans, extensive genetic sequences of myeloid malignancies did not reveal recurrent PTEN mutations and deletions. However, PTEN was shown to be functionally inactivated in several acute myeloid leukemia and chronic myeloid leukemia samples, through both post-trasductional modifications, changes in protein levels and cellular compartmentalization...
November 23, 2015: Hematology Reports
https://www.readbyqxmd.com/read/26733269/the-anti-factor-xa-range-for-low-molecular-weight-heparin-thromboprophylaxis
#18
REVIEW
Matthew Y Wei, Salena M Ward
Low molecular weight heparins (LMWHs) are now the mainstay option in the prevention and treatment of venous thromboembolism. In some patients receiving therapeutic doses of LMWH, activity can be measured by quantifying the presence of Anti-factor Xa (AFXa) for dose adjustment. However, currently there are no guidelines for LMWH monitoring in patients on thromboprophylactic, doses, despite certain patient populations may be at risk of suboptimal dosing. This review found that while the AFXa ranges for therapeutic levels of LMWHs are relatively well defined in the literature, prophylactic ranges are much less clear, thus making it difficult to interpret current research data...
November 23, 2015: Hematology Reports
https://www.readbyqxmd.com/read/26487936/idiopathic-hemophagocytic-lymphohistiocytosis-during-pregnancy-treated-with-steroids
#19
Bachar Samra, Mohamad Yasmin, Sami Arnaout, Jacques Azzi
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome characterized by a dysregulated hyperinflammatory immune response. The diagnosis of HLH during pregnancy is especially challenging due to the rarity of this condition. The highly variable clinical presentation, laboratory findings, and associated diagnoses accompanying this syndrome further complicate the problem. A pronounced hyperferritinemia in the setting of systemic signs and symptoms along with a negative infectious and rheumatological workup should raise suspicions for HLH...
September 23, 2015: Hematology Reports
https://www.readbyqxmd.com/read/26487935/acute-liver-failure-in-a-pediatric-patient-with-congenital-dysery-thropoietic-anemia-type-i-treated-with-deferasirox
#20
Galina Ling, Vered Pinsk, Inbal Golan-Tripto, Eduard Ling
Congenital dyserythropoietic anemias (CDA) represent a heterogeneous group of disorders characterized by morphological abnormalities of erythroid precursor cells and various degrees of hemolysis. Iron overload is a result of continuous hemolysis and recurrent transfusions. It is treated with iron chelators, including deferasirox. We present here a case of acute liver failure in a 12 years old girl with CDA type I treated with deferasirox and discuss the approach to treatment.
September 23, 2015: Hematology Reports
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