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Animesh Pardanani
: Disease overview:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extra-cutaneous organs. DIAGNOSIS: The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V. Risk stratification: The 2008 World Health Organization (WHO) classification of SM has been shown to be prognostically relevant...
November 2016: American Journal of Hematology
Mohammad Adawi, Bishara Bisharat, Abdalla Bowirrat
BACKGROUND: Erdheim-Chester disease (ECD) is an uncommon aggressive, multisystem form of non-Langerhans' cell histocytosis, which was firstly reported by Jakob Erdheim and William chester in 1930. The disease pathological features encompass an aberrant multiplication, overproduction and accumulation of white blood cells called histiocytes within multiple tissues and organs. Herein, we present a case of ECD owing to the rarity of this disease (roughly 550 cases have been described in the literature to date)...
October 2016: Medicine (Baltimore)
Chantal Fridle, Michael Medinger, Matthias C Wilk, Katja Seipel, Jakob Passweg, Markus G Manz, Thomas Pabst
BACKGROUND: The prognosis for relapsing AML patients is disappointing and the preferred salvage chemotherapy is unclear. Among other regimens, cladribine, cytarabine, and idarubicin (CLA-Ida) is used. METHODS: We analyzed relapsing AML patients receiving CLA-Ida chemotherapy between July 2012 and April 2015 at three academic centers in Switzerland. RESULTS: Thirty-four patients underwent at least one cycle of CLA-Ida chemotherapy, with 6 patients having two cycles...
October 13, 2016: Leukemia & Lymphoma
Celalettin Ustun, Michel Arock, Hanneke C Kluin-Nelemans, Andreas Reiter, Wolfgang R Sperr, Tracy George, Hans-Peter Horny, Karin Hartmann, Karl Sotlar, Gandhi Damaj, Olivier Hermine, Srdan Verstovsek, Dean D Metcalfe, Jason Gotlib, Cem Akin, Peter Valent
Systemic mastocytosis is a heterogeneous disease characterized by the accumulation of neoplastic mast cells in the bone marrow and other organ organs/tissues. Mutations in KIT, most frequently KIT D816V, are detected in over 80% of all systemic mastocytosis patients. While most systemic mastocytosis patients suffer from an indolent disease variant, some present with more aggressive variants, collectively called "advanced systemic mastocytosis", which include aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic, clonal non mast cell-lineage disease, and mast cell leukemia...
October 2016: Haematologica
Michael Leung, Kaitlin Highsmith, Amber Rexwinkle
Myelofibrosis is a BCR-ABL-negative myeloproliferative neoplasm characterized by abnormal hematopoiesis. Alterations to the Janus kinase-signal transducer and activator of transcription pathway result in dysregulation of gene transcription and cell proliferation. Patients with symptomatic myelofibrosis present with a variety of signs and symptoms including, but not limited to myelosuppression, marked splenomegaly, abdominal discomfort, fatigue, and blood transfusion-dependence. Traditional myelosuppressive therapies including hydroxyurea, azacitidine, and cladribine aim to reduce constitutional symptoms and control the burden of disease...
September 26, 2016: Journal of Oncology Pharmacy Practice
A M Novakovic, E H J Krekels, A Munafo, S Ueckert, M O Karlsson
In this study, we report the development of the first item response theory (IRT) model within a pharmacometrics framework to characterize the disease progression in multiple sclerosis (MS), as measured by Expanded Disability Status Score (EDSS). Data were collected quarterly from a 96-week phase III clinical study by a blinder rater, involving 104,206 item-level observations from 1319 patients with relapsing-remitting MS (RRMS), treated with placebo or cladribine. Observed scores for each EDSS item were modeled describing the probability of a given score as a function of patients' (unobserved) disability using a logistic model...
September 15, 2016: AAPS Journal
Agnieszka Wierzbowska, Ewa Wawrzyniak, Monika Siemieniuk-Rys, Aleksandra Kotkowska, Agnieszka Pluta, Aleksandra Golos, Tadeusz Robak, Marta Szarawarska, Anna Jaskowiec, Ewa Duszenko, Justyna Rybka, Jadwiga Holojda, Sebastian Grosicki, Barbara Pienkowska-Grela, Renata Woroniecka, Anna Ejduk, Marzena Watek, Malgorzata Wach, Barbara Mucha, Katarzyna Skonieczka, Maria Czyzewska, Anna Jachalska, Agnieszka Klonowska, Mariola Iliszko, Wanda Knopinska-Posluszny, Malgorzata Jarmuz-Szymczak, Anna Przybylowicz-Chalecka, Lidia Gil, Agnieszka Kopacz, Jerzy Holowiecki, Olga Haus
Monosomal karyotype (MK) and complex karyotype (CK) are poor prognostic factors in acute myeloid leukemia (AML). A comprehensive analysis of cytogenetic and clinical factors influencing an outcome of AML-CK(+ ) was performed. The impact of cladribine containing induction on treatment results was also evaluated. We analyzed 125 patients with AML-CK(+ ) treated within PALG protocols. MK was found in 75 (60%) individuals. The overall complete remission (CR) rate of 66 intensively treated patients was 62% vs...
August 26, 2016: Leukemia & Lymphoma
Arnaud Payerols, Eric Frouin, Aurélie Schiffmann, Nicolas Menjot de Champfleur, Danielle Canioni, Olivia Chandesris, Valérie Costes, Max Villain, Frédéric Mura
PURPOSE: Mastocytosis is characterized by clonal mast cell proliferation with accumulation within various organs and uncontrolled activation with excessive mast cell mediator release. Ocular manifestations have rarely been published. We describe a 63-year-old man with bilateral exophthalmos that led to the diagnosis of systemic mastocytosis. CASE REPORT: A patient presented with bilateral eyelid edema with exophthalmos associated with binocular diplopia. Ophthalmologic examination showed bilateral axial, symmetrical, and painless exophthalmos with eyelid edema, and limitation in elevation of the right eye...
August 24, 2016: Optometry and Vision Science: Official Publication of the American Academy of Optometry
Ramya Uppuluri, Sreejith Ramachandrakurup, Divya Subburaj, Atish Bakane, Revathi Raj
Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. We used a novel protocol including six cycles of pulse dexamethasone and lenalidomide in four children with LCH refractory to first-line agents and courses of cladribine and cytarabine or single-agent cladribine. All four children completed the protocol without any significant adverse effects and remain in complete and durable remission 15-18 months posttreatment. The novel protocol we propose for relapsed/refractory LCH is cost-effective and outpatient-based with durable remission and minimal toxicity...
August 24, 2016: Pediatric Blood & Cancer
Hyunkyung Park, Jeonghwan Youk, Inho Kim, Sung-Soo Yoon, Seonyang Park, Jeong-Ok Lee, Soo-Mee Bang, Youngil Koh
Induction regimens integrating cladribine or fludarabine have shown promising outcomes in relapsed or refractory (R/R) acute myeloid leukaemia (AML). We compared the outcome of a cladribine- versus a fludarabine-based regimen as induction chemotherapy for R/R-AML. We included patients with R/R-AML who were treated with a cladribine- or fludarabine-based chemotherapy between 2006 and 2015. We analysed 120 patients, 65 treated with cladribine and 55 treated with fludarabine. The CR rates were 62.7 and 61.4 % for the cladribine group and fludarabine group, respectively (p = 0...
October 2016: Annals of Hematology
M H Duan, Y Zhang, M Zhang, X Han, Y Zhang, C Yang, J Feng, L Zhang, W Zhang, J Li, L P Tian, Y Zhang, D B Zhou
OBJECTIVE: To analyze efficacy and safety of CLAG regimen in patients with refractory or relapsed acute myeloid leukemia (AML). METHODS: Efficacy and adverse events of patients with refractory or relapsed AML who were treated with one course of CLAG from April 1st, 2014 through December 9th, 2015 in our hospital were retrospectively reviewed. RESULTS: Thirty- three patients (16 males and 17 females) with refractory or relapsed AML were treated with one course of CLAG with a median age of 49 (14-68) years...
July 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Charles Jian, Cyrus C Hsia
A 65-year-old woman presented with easy bruising, left upper quadrant pain, decreased appetite, and weight loss. She had splenomegaly and lymphocytosis (lymphocyte count of 11.6 × 10(9)/l), with remarkably abnormal appearing morphology. Her hemoglobin and platelet counts were normal. Peripheral blood flow cytometry revealed a monoclonal B-cell population expressing CD11c, CD25, CD19, CD20, and CD103. An initial diagnosis of hairy cell leukemia (HCL) was made, and the patient was treated with a standard 5-day course of cladribine...
May 2016: Case Reports in Oncology
D Miguel, J Lukacs, T Illing, P Elsner
Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG...
July 19, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Vijaya L Damaraju, Dwayne Weber, Michelle Kuzma, Carol E Cass, Michael B Sawyer
Human nucleoside transporters (hNTs) mediate cellular influx of anticancer nucleoside drugs, including cytarabine, cladribine, and fludarabine. BCR-ABL tyrosine kinase inhibitors (TKIs) imatinib and dasatinib inhibit fludarabine and cytarabine uptake. We assessed interactions of bosutinib, dasatinib, imatinib, nilotinib, and ponatinib with recombinant hNTs (hENT1, 2; hCNT1, -2, and -3) produced individually in yeast Saccharomyces cerevisiae Nilotinib inhibited hENT1-mediated uridine transport most potently (IC50 value, 0...
September 2, 2016: Journal of Biological Chemistry
Jan-Paul Bohn, Guenther Gastl, Michael Steurer
Classic hairy cell leukemia (HCL) is a rare indolent B‑cell-lymphoproliferative disorder, first described as a distinct disease entity in 1958. After more than two decades without effective chemotherapeutic options and a dismal prognosis of less than 5 years, only the introduction of interferon‑α (IFN‑α) allowed for response rates between 80-90 % and survival improvement. Nowadays, however, patients are rarely treated with IFN-α as purine analogues were found to be highly effective in HCL facilitating a near normal life span in most cases...
2016: Memo
A Santos-Lozano, A Morales-Gonzalez, F Sanchis-Gomar, C Cristi-Montero, C Fiuza-Luces, H Pareja-Galeano, J Martínez-López, N Garatachea, A Lucia
Waldenström macroglobulinemia (WM) is a malignant lymphoproliferative disorder characterized by the presence of a high level of serum monoclonal IgM and a lymphoplasmacytic infiltrate in the bone marrow. This meta-analysis sought to assess the effectiveness of the different treatments for WM tested in published trials using the response rate (RR) as the main outcome measure. Forty-six articles (1409 patients) identified were entered in a variable effects model meta-analysis of proportions (rates and sample sizes)...
September 2016: Critical Reviews in Oncology/hematology
Sriram Ravichandran, Rohith G Chitrapur, Saurabh Bhave, Anupam Chakrapani, Reena Nair, Mammen Chandy
Systemic mastocytosis is a rare and recalcitrant disorder with nonspecific clinical features. Hence, a high index of suspicion is required. Here, we report the case of a 64 years old male presenting with chronic diarrhoea that was evaluated at different centres and treated with multiple lines of therapy. The diagnosis of aggressive systemic mastocytosis was finally clinched following a holistic work up that included a Jejunal biopsy and a laparoscopic lymph node biopsy. Treatment of this disorder is difficult, responses are transient and most patients will eventually relapse, as illustrated by this case...
June 2016: Indian Journal of Hematology & Blood Transfusion
Luis Miguel Juárez Salcedo, Jose Sandoval-Sus, Ling Zhang, Donald Klippenstein, Julio C Chavez
No abstract text is available yet for this article.
September 2016: British Journal of Haematology
Dai Chihara, Hagop Kantarjian, Susan O'Brien, Jeffrey Jorgensen, Sherry Pierce, Stefan Faderl, Alessandra Ferrajoli, Rebecca Poku, Preetesh Jain, Phillip Thompson, Mark Brandt, Rajyalakshmi Luthra, Jan Burger, Michael Keating, Farhad Ravandi
Nucleoside analogues are highly active in patients with hairy cell leukaemia (HCL); however, patients continue to relapse. This phase II study evaluated the efficacy and safety of cladribine followed by rituximab in patients with untreated HCL (N = 59), relapsed HCL (N = 14) and HCL variant (HCLv, N = 7). Cladribine 5·6 mg/m(2) was given intravenously (IV) daily for 5 d and was followed approximately 1 month later with rituximab 375 mg/m(2) IV weekly for 8 weeks. Complete response rate in patients with untreated HCL, relapsed HCL and HCLv was 100%, 100% and 86%, respectively...
September 2016: British Journal of Haematology
Charlotte Rigaud, Mohamed A Barkaoui, Caroline Thomas, Yves Bertrand, Anne Lambilliotte, Jean Miron, Nathalie Aladjidi, Geneviève Plat, Eric Jeziorski, Claire Galambrun, Ludovic Mansuy, Patrick Lutz, Anne Deville, Corinne Armari-Alla, Yves Reguerre, Sylvie Fraitag, Aurore Coulomb, Virginie Gandemer, Nicolas Leboulanger, Despina Moshous, Khe Hoang-Xuan, Abdellatif Tazi, Sébastien Heritier, Jean-François Emile, Jean Donadieu
The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15-year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine...
September 2016: British Journal of Haematology
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