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Hematology Reports

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https://www.readbyqxmd.com/read/28286634/the-efficacy-of-colchicine-and-dapsone-combination-therapy-in-relapsed-immune-thrombocytopenia
#1
Thanawat Rattanathammethee, Wasan Theerajangkhaphichai, Ekarat Rattarittamrong, Sasinee Hantrakool, Chatree Chai-Adisaksopha, Lalita Norasetthada, Adisak Tantiworawit
The aim of the present paper is to evaluate the efficacy and safety of colchicine and dapsone combination therapy in cases of steroid-dependent, relapsed and refractory immune thrombocytopenia (ITP). This is a retrospective study of ITP patients who attended the Hematology Clinic at Chiang Mai University Hospital (Thailand) from 1 January 2008 to 30 September 2014. Medical records and clinical data were reviewed for efficacy and adverse effects. Sixty-four ITP patients received the combination therapy. The median age was 46 years and 70...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286633/successful-intrathecal-chemotherapy-combined-with-radiotherapy-followed-by-pomalidomide-and-low-dose-dexamethasone-maintenance-therapy-for-a-primary-plasma-cell-leukemia-patient
#2
Yusuke Yamashita, Shinobu Tamura, Takehiro Oiwa, Hiroshi Kobata, Kodai Kuriyama, Toshiki Mushino, Shogo Murata, Hiroki Hosoi, Akinori Nishikawa, Nobuyoshi Hanaoka, Takashi Sonoki
Primary plasma cell leukemia (PPCL) is a rare aggressive variant of plasma cell disorder and frequently presents with extramedullary disease. Central nervous system (CNS) involvement with PPCL has an extremely poor prognosis. We describe a 46-year-old man with PPCL treated with a combination of lenalidomide, bortezomib, and dexamethasone as induction therapy following upfront allogeneic stem cell transplantation (allo-SCT). Despite achieving a very good partial response, the patient suffered from an isolated CNS relapse 12 months after allo-SCT...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286632/can-ratio-of-neutrophil-to-lymphocyte-count-and-erythrocyte-sedimentation-rate-in-diabetic-foot-infecti-on-predict-osteomyelitis-and-or-amputation
#3
Oktay Yapıcı, Hande Berk, Nefise Öztoprak, Derya Seyman, Alper Tahmaz, Alparslan Merdin
The aim of this study was to search for any relations between the neutrophil-to-lymphocyte ratio (NLR) and the development of osteomyelitis and the need for amputation in patients with diabetic foot infection (DFI). All data included DFI patients who were hospitalized in our Infectious Diseases Clinic between 2012 and 2015 and who were classified according to International Classification Disease Code System. 75 patients were analyzed in the study. The DFI patients were stratified into 3 groups of whom had amputation procedure, whom had only debridement/drainage procedure and whom had any surgery procedure...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286631/hemoglobin-willamette-%C3%AE-51pro-%C3%A2-arg-case-report-and-literature-review
#4
Orivaldo Alves Barbosa, Matheus Martins de Sousa Dias, Saymon Medeiros Távora, Gentil Claudino de Galiza Neto, Jacqueline Holanda de Souza, Herivaldo Ferreira da Silva
We report a case of hemoglobin (Hb) Willamette (β51 Pro → Arg) in the Hematology Department of a tertiary hospital in Fortaleza, Northeast of Brazil. A literature review of the cases described in health sciences databases using as a descriptor Hb Willamette was performed, revealing 12 reported cases, of which only one presented with anemia. Herein, we describe a case of a female 29 years old, with hemoglobinopathy Willamette presenting clinically with anemia, having the lowest hemoglobin rate of the published cases...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286630/a-fatal-case-of-immune-hyperhemolysis-with-bone-marrow-necrosis-in-a-patient-with-sickle-cell-disease
#5
Matthew S Karafin, Arun Singavi, Susan T Johnson, Joshua J Field
In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286629/impact-of-crab-symptoms-in-survival-of-patients-with-symptomatic-myeloma-in-novel-agent-era
#6
Aya Nakaya, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yoshiko Azuma, Yukie Tsubokura, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito, Shosaku Nomura
The acronym CRAB summarizes the most typical clinical manifestations of multiple myeloma, these being hypercalcemia, renal failure, anemia, and bone disease. CRAB can be used to distinguish between active, symptomatic multiple myeloma and monoclonal gammopathy of undermined significance or smoldering myeloma. The distinction is relevant not only for classification and diagnosis but also for therapy. CRAB factors influence the prognosis of multiple myeloma. However, it is unclear whether the presence of CRAB factors has an influence on the prognosis of myeloma treated with novel agents...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286628/hemophagocytic-lymphohistiocytosis-an-unclear-nosologic-entity-case-report-of-an-adult-man-with-rising-of-amylase-and-lipase-and-spinal-cord-infiltration
#7
Moris Sangineto, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso, Carlo Sabbà
Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28090281/successful-treatment-of-immune-reconstitution-inflammatory-syndrome-related-hemophagocytic-syndrome-in-an-hiv-patient-with-primary-effusion-lymphoma
#8
Markela-P Zorzou, Maria Chini, Athina Lioni, Georgios Tsekes, Thomas Nitsotolis, Ioannis Tierris, Nicolaos Panagiotou, Dimitra Rontogianni, Nicolaos Harhalakis, Marios Lazanas
Although the connection of [secondary hemophagocytic syndrome (sHS)] with HIV has been well documented, optimal treatment regimen is not well established. This is due not only to the rarity of the syndrome, but also to the heterogeneity of the involved population. Most cases are related to opportunistic infections or malignancies in advanced stage, but many cases are also related to seroconversion, in the primary infection setting. Moreover, in the [antiretroviral treatment (ART)] era, rare cases of ART-related sHS have been reported...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/28053696/invasive-thymoma-with-pure-red-cell-aplasia-and-amegakaryocytic-thrombocytopenia
#9
Takuya Onuki, Yusuke Kiyoki, Sho Ueda, Masatoshi Yamaoka, Seiich Shimizu, Masaharu Inagaki
We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 10(4)/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal tumor was detected, and bone marrow biopsy revealed few megakaryocytes and severely reduced numbers of erythroid cells. The diagnosis was thymoma with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT)...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/28053695/study-on-hydroxyurea-response-in-hemoglobinopathies-patients-using-genetic-markers-and-liquid-erythroid-cultures
#10
Serena Sclafani, Alice Pecoraro, Veronica Agrigento, Antonio Troia, Rosario Di Maggio, Massimiliano Sacco, Aurelio Maggio, Elena D'Alcamo, Rosalba Di Marzo
Increased expression of fetal hemoglobin (HbF) may ameliorate the clinical course of hemoglobinopathies. Hydroxyurea (HU) is the only inducer approved for the treatment of these diseases able to stimulate HbF production but patients' response is highly variable indicating the utility of the identification of pharmacogenomic biomarkers in order to predict pharmacological treatment efficacy. To date few studies to evaluate the role of genetic determinants in HU response have been conducted showing contradictory results...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27994839/romidepsin-controls-chronic-lymphocytic-leukemia-in-a-patient-with-mycosis-fungoides
#11
David M Lemchak, Oleg E Akilov
Romidepsin belongs to a class of medications called histone deacetylase inhibitors and is currently approved for treatment of cutaneous and peripheral T-cell lymphomas. Romidepsin was previously investigated for the treatment of chronic lymphocytic leukemia (CLL), and demonstrated potential benefit, but interest in its use declined following phase I clinical trials that showed poor tolerance of a significant side effect profile. We presented a patient with a history of stage II CLL, referred to dermatology for treatment of new-onset of mycosis fungoides (MF), who was treated with romidepsin over seven months...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27994838/reversible-cerebral-vasoconstriction-syndrome-promptly-diagnosed-with-magnetic-resonance-imaging-including-magnetic-resonance-angiography-during-immunosuppressive-therapy-in-a-16-year-old-girl-with-refractory-cytopenia-of-childhood
#12
Hideaki Ueki, Yasushi Sanayama, Akiyo Miyajima, Taichiro Tsuchimochi, Shunji Igarashi, Shosuke Sunami
Reversible cerebral vasoconstriction syndrome (RCVS) is a syndrome characterized by severe headache with segmental vasoconstriction of the cerebral arteries that resolves within 12 weeks. A 16-year-old girl with refractory cytopenia of childhood, who was receiving the immunosuppressant cyclosporine, developed severe headache and was diagnosed with RCVS using magnetic resonance imaging, including magnetic resonance angiography (MRA). MRA is a non-invasive and very effective technique for diagnosing RCVS. MRA should be performed at the onset of severe headache during immunosuppressant administration for children with hematological disorders and may prevent sequelae such as posterior reversible encephalopathy syndrome or ischemic attack...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27994837/treatment-of-refractory-anemia-with-ring-sideroblasts-associated-with-marked-thrombocytosis-with-lenalidomide-in-a-patient-testing-negative-for-5q-deletion-and-jak2-v617f-and-mpl-w515k-l-mutations
#13
Ryan Keen, Jeremy Pantin, Natasha Savage, Paul M Dainer
Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a hematologic malignancy that often results in transfusion dependency and a hypercoagulable state. This rare disease currently lacks formal guidelines for treatment; however, various case reports have demonstrated efficacy in the use of lenalidomide. This immunomodulatory drug has shown promise in patients with 5q deletions, with reports of achieving transfusion independence and normalization of platelet counts. Herein we present the case of a 68-year-old African American woman with RARS-T who tested negative for 5q deletion and JAK2 V617F and MPL W515K/L mutations...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27781079/atypical-hemolytic-uremic-syndrome-secondary-to-lupus-nephritis-responsive-to-eculizumab
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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