TEMPI Syndrome: Erythrocytosis in Plasma Cell Dyscrasia.
TEMPI Syndrome: Erythrocytosis in Plasma Cell Dyscrasia. Clin Lymphoma Myeloma Leuk. 2018 Aug 09;: Authors: Zhang X, Fang M Abstract TEMPI (telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, intrapulmonary shunting) syndrome is a newly described clinical entity that is generally considered a plasma cell dyscrasia with multiple system involvement. The etiology and pathophysiology of this condition remains elusive. Nevertheless, clonal plasma cells and monoclonal protein appear to be major contributors. The early diagnosis of TEMPI syndrome is essential because therapies targeting the underlying plasma cells can lead to a dramatic response. Bortezomib-based chemotherapy, daratumumab monotherapy, and autologous hematopoietic stem cell transplantation can result in reversal of most manifestations. Nevertheless, the diagnosis of TEMPI syndrome remains a substantial challenge owing to its rarity and the complexity of clinical presentations. TEMPI syndrome is often misdiagnosed as other causes of erythrocytosis, resulting in a delayed diagnosis and further clinical deterioration. The aim of the present review was to present the clinical and biologic features of TEMPI syndrome, highlighting the differential diagnosis and outlining the present understanding of its pathophysiology and treatment. PMID: 30100329 [PubMed - as supplied by publisher]
In this study, around 20% of all patients with HBV reactivation developed HBV reactivation after 2 years from the initiation of therapy, unlike malignant lymphoma. This might be due to the fact that almost all of the patients received chemotherapy for a long duration. Therefore, a new strategy for the prevention of HBV reactivation in MM patients is required.
Publication date: Available online 7 November 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Kaoru Morita, Masahiro Ashizawa, Yumiko Toda, Takashi Ikeda, Shin-ichiro Kawaguchi, Shoko Ito, Shin-ichi Ochi, Takashi Nagayama, Kiyomi Mashima, Kento Umino, Daisuke Minakata, Hirofumi Nakano, Ryoko Yamasaki, Chihiro Yamamoto, Kaoru Hatano, Shin-ichiro Fujiwara, Kazuya Sato, Iekuni Oh, Ken Ohmine, Kazuo MuroiAbstractAbout 40% of patients with diffuse large B cell lymphoma (DLBCL) relapse after or are refractory to standard chemotherapy with rituximab plus cyclophosphamide, hydroxydaunorubicin, vincristine, and prednis...
CONCLUSION: All tested biosimilars demonstrated similar effectiveness and safety profiles in patients with hematological tumors undergoing PBSC mobilization; therefore, they can be used interchangeably. PMID: 31663634 [PubMed - as supplied by publisher]
Publication date: Available online 9 October 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Joseph MikhaelAbstractThe advent of new, more effective, and less toxic therapies has revolutionized the management of multiple myeloma in the past decade. Despite the availability of new treatments, the majority of patients with multiple myeloma will become refractory to the therapies that currently comprise the hematologic standard of care for the malignancy: proteasome inhibitors, immunomodulatory agents, and monoclonal antibodies. Moreover, in recent years, a new subset of patients refractory to all 3 of these agen...
This study emphasizes the feasibility of autologous stem cell transplant in an outpatient model of care and outlines the resources necessary to run such a unit.
This study emphasizes the feasibility of ASCT in an outpatient model of care and outlines the resources necessary to run such a unit.
Publication date: Available online 2 October 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Avyakta Kallam, Julie M. VoseAbstractNon-Hodgkin lymphomas that are refractory to or relapse after frontline chemoimmunotherapy have a poor prognosis. Although high dose chemotherapy, followed by autologous stem cell transplantation remains the standard of care at relapse, this treatment modality leads to a cure in less than 50% of the patients. Adoptive cellular immunotherapy with anti CD 19 chimeric antigen receptor (CAR) T cell has changed the treatment landscape in B cell lymphomas. They have emerged as effective t...
A phase I/II trial was conducted to explore the safety and activity of addition of bortezomib on days -6, -3, and +1 relative to the day of autologous stem cell transplantation to a conditioning regimen with busulfan and melphalan (BuMel; 3.2 mg/kg/day busulfan on days -5 to -3 and 140 mg/m2/day melphalan on day -2) in patients with multiple myeloma (MM) following bortezomib-based induction chemotherapy. In phase I, doses of bortezomib (0.7, 1.0, and 1.3 mg/m2) with BuMel were administered to groups of three patients each.
Patients with Multiple Myeloma (MM) are enjoying significant improvement in overall survival as the result of advent of novel anti-myeloma agent that replace traditional chemotherapy, in addition to the increased use of transplant. The prices of novel agents, especially oral ones, have been rapidly escalating and there are well-described issues with affordability (Shih et al. JCO 2017). We therefore hypothesized that insurance status influences MM patients (pts) survival. National Cancer Database (NCDB), covering 70% of MM patient nationwide was utilized.
Bortezomib, doxorubicin and dexamethasone (PAd) and bortezomib, liposome doxorubicin and dexamethasone (PDd) are widely used as induction chemotherapy regimens in China in the new-diagnosed multiple myeloma patients before autologous stem cell transplantation. There was no data on the comparison in safety and efficiency between those two regimens, so we designed this open-label, randomized, multicenter clinical trial in 10 centers in China （NCT02577783）.