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The full spectrum of Castleman disease: 273 patients studied over 20 years.

The full spectrum of Castleman disease: 273 patients studied over 20 years.

Br J Haematol. 2017 Nov 16;:

Authors: Oksenhendler E, Boutboul D, Fajgenbaum D, Mirouse A, Fieschi C, Malphettes M, Vercellino L, Meignin V, Gérard L, Galicier L

Abstract
The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Twenty of these patients with Castleman-like histopathology were removed from analyses, because they were diagnosed with an exclusionary disorder (18 with haematological malignancy). Among the 253 remaining patients, 57 were considered unicentric CD (UCD), 169 were multicentric CD associated with Human Herpesvirus 8 (HHV-8+MCD), including 140 patients with human immunodeficiency virus (HIV) infection and 29 patients without HIV infection, and 27 were HHV-8 negative/idiopathic multicentric CD (iMCD). 2-((18) F)fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography was useful in 62 patients for staging/classification of the disease and for excluding associated lymphoma. UCD was mainly associated with hyaline-vascular histopathological features, and most patients were asymptomatic. Of the 27 patients that we had originally diagnosed with iMCD, 26 met the newly established diagnostic criteria. Patients with iMCD and HHV-8+ MCD demonstrated similar characteristics, including fever, splenomegaly, cytopenia and inflammatory symptoms. However, the disease was more aggressive in HHV-8+ MCD, particularly in HIV-infected patients.

PMID: 29143319 [PubMed - as supplied by publisher]




Castleman disease: delineating the spectrum.

Castleman disease: delineating the spectrum.

Br J Haematol. 2017 Nov 16;:

Authors: Alzahrani M, Leitch HA

PMID: 29143317 [PubMed - as supplied by publisher]




Fetal haemoglobin induction in sickle cell disease.

Fetal haemoglobin induction in sickle cell disease.

Br J Haematol. 2017 Nov 16;:

Authors: Paikari A, Sheehan VA

Abstract
Fetal haemoglobin (HbF, α2γ2) induction has long been an area of investigation, as it is known to ameliorate the clinical complications of sickle cell disease (SCD). Progress in identifying novel HbF-inducing strategies has been stymied by limited understanding of gamma (γ)-globin regulation. Genome-wide association studies (GWAS) have identified variants in BCL11A and HBS1L-MYB that are associated with HbF levels. Functional studies have established the roles of BCL11A, MYB, and KLF1 in γ-globin regulation, but this information has not yielded new pharmacological agents. Several drugs are under investigation in clinical trials as HbF-inducing agents, but hydroxycarbamide remains the only widely used pharmacologic therapy for SCD. Autologous transplant of edited haematopoietic stem cells holds promise as a cure for SCD, either through HbF induction or correction of the causative mutation, but several technical and safety hurdles must be overcome before this therapy can be offered widely, and pharmacological therapies are still needed.

PMID: 29143315 [PubMed - as supplied by publisher]




Erythrophagocytosis by T-cell lymphoma cells in a patient with hereditary spherocytosis post-splenectomy.

Erythrophagocytosis by T-cell lymphoma cells in a patient with hereditary spherocytosis post-splenectomy.

Br J Haematol. 2017 Nov 16;:

Authors: Frame IJ, Coberly J, Fuda FS, Chen M

PMID: 29143312 [PubMed - as supplied by publisher]




Interleukin-12 and its procoagulant effect on erythrocytes, platelets and fibrin(ogen): the lesser known side of inflammation.

Interleukin-12 and its procoagulant effect on erythrocytes, platelets and fibrin(ogen): the lesser known side of inflammation.

Br J Haematol. 2017 Nov 16;:

Authors: Page MJ, Bester J, Pretorius E

Abstract
Inflammation, with its associated inflammatory molecules, is integral to most chronic diseases, including the various cardiovascular diseases. Interleukin 12 (IL12) is one of the inflammatory cytokines that is upregulated during inflammation; however, we know very little about its exact effect on red blood cells (RBCs), platelets and fibrin(ogen). IL12 is an important pleiotropic cytokine in early inflammatory responses and has potent immunomodulatory, antitumour and anti-infection activity. Here we investigate how low levels of circulating IL12, comparable to levels found during chronic inflammation, affect coagulation parameters, platelets and RBCs. We used thromboelastography, scanning electron microscopy, refractometery and wide-field microscopy. Our results show that IL12 caused hypercoagulation, platelet activation and spreading, as well as RBC agglutination. This phenomenon has far-reaching implications for treatment of the plethora of conditions where IL12 is upregulated, since it suggests aberrant haemorheology as agglutination affects blood flow. This information might be used in future to target the lowering of IL12 in inflammatory conditions, as well as address RBC agglutination.

PMID: 29143311 [PubMed - as supplied by publisher]




Clinical features and outcomes of secondary intraocular lymphoma.

Clinical features and outcomes of secondary intraocular lymphoma.

Br J Haematol. 2017 Nov 16;:

Authors: Karakawa A, Taoka K, Kaburaki T, Tanaka R, Shinozaki-Ushiku A, Hayashi H, Miyagi-Maeshima A, Nishimura Y, Uekusa T, Kojima Y, Fukayama M, Kurokawa M, Aihara M

PMID: 29143310 [PubMed - as supplied by publisher]




Bone marrow Dikkopf-1 levels are a new independent risk factor for progression in patients with smouldering myeloma.

Bone marrow Dikkopf-1 levels are a new independent risk factor for progression in patients with smouldering myeloma.

Br J Haematol. 2017 Nov 16;:

Authors: Dalla Palma B, Marchica V, Pedrazzoni M, Accardi F, Notarfranchi L, Goldoni M, De Luca F, Costa F, Storti P, Toscani D, Sammarelli G, Bonomini S, Aversa F, Giuliani N

PMID: 29143308 [PubMed - as supplied by publisher]




Inter-individual variability in phospholipid-dependent interference of C-reactive protein on activated partial thromboplastin time.

Inter-individual variability in phospholipid-dependent interference of C-reactive protein on activated partial thromboplastin time.

Br J Haematol. 2017 Nov 16;:

Authors: Erdem-Eraslan L, Hens JJH, van Rossum AP, Frasa MAM, Keuren JFW

PMID: 29143304 [PubMed - as supplied by publisher]




Is food intolerance a factor underlying chronic immune thrombocytopenia (ITP)?

Is food intolerance a factor underlying chronic immune thrombocytopenia (ITP)?

Br J Haematol. 2017 Nov 16;:

Authors: Batty CA, Hunter JO, Woolner J, Baglin T, Turner C

PMID: 29143303 [PubMed - as supplied by publisher]




Potentially inappropriate medication use in elderly non-Hodgkin lymphoma patients is associated with reduced survival and increased toxicities.

Potentially inappropriate medication use in elderly non-Hodgkin lymphoma patients is associated with reduced survival and increased toxicities.

Br J Haematol. 2017 Nov 16;:

Authors: Lin RJ, Ma H, Guo R, Troxel AB, Diefenbach CS

Abstract
Survival outcomes for elderly lymphoma patients are disproportionally inferior to those of younger patients. We examined medication usage at diagnosis for 171 elderly patients (median age 70 years) with aggressive non-Hodgkin lymphoma treated between 2009 and 2014. At least one potentially inappropriate medication was used in 47% of patients according to the Beers Criteria, 59% experienced treatment delays and/or dose reduction and 65% experienced ≥ grade 3 treatment-related toxicities. We report here for the first time that potentially inappropriate medication use was associated with reduced progression-free survival and overall survival, and increased ≥ grade 3 treatment-related toxicities in multivariate analysis.

PMID: 29143301 [PubMed - as supplied by publisher]