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Severe Pulmonary Hypertension Caused by Smoldering Plasma Cell Myeloma: An Autopsy Case of POEMS Syndrome
Katsuya Chinen,Yasunori Fujioka
Case Reports in Medicine , 2012, DOI: 10.1155/2012/836893
Abstract: The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is a rare variant of plasma cell disorders with multiple systemic manifestations. Recently, pulmonary hypertension (PH) has become established as a complication, but pathological studies of this condition are scarce and the detailed pathogenesis remains to be elucidated. We present herein a case of a 49-year-old woman who was diagnosed as having idiopathic PH and was treated in accordance. However, she eventually died of respiratory failure and an autopsy revealed the presence of smoldering plasma cell myeloma and multiple organomegaly in addition to severe PH. The latter was attributed to stenosis and occlusion of the arterioles of the lungs due to marked plasma cell proliferation, quite different from the histology of idiopathic PH. From these findings, together with the clinical details, we concluded that the patient’s PH was a complication of the POEMS syndrome. This case showed a unique pulmonary vascular pathology featuring plasma cell proliferation and it provides clues towards understanding the pathogenesis with this background.
Severe Pulmonary Hypertension Caused by Smoldering Plasma Cell Myeloma: An Autopsy Case of POEMS Syndrome  [PDF]
Katsuya Chinen,Yasunori Fujioka
Case Reports in Medicine , 2012, DOI: 10.1155/2012/836893
Abstract: The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is a rare variant of plasma cell disorders with multiple systemic manifestations. Recently, pulmonary hypertension (PH) has become established as a complication, but pathological studies of this condition are scarce and the detailed pathogenesis remains to be elucidated. We present herein a case of a 49-year-old woman who was diagnosed as having idiopathic PH and was treated in accordance. However, she eventually died of respiratory failure and an autopsy revealed the presence of smoldering plasma cell myeloma and multiple organomegaly in addition to severe PH. The latter was attributed to stenosis and occlusion of the arterioles of the lungs due to marked plasma cell proliferation, quite different from the histology of idiopathic PH. From these findings, together with the clinical details, we concluded that the patient’s PH was a complication of the POEMS syndrome. This case showed a unique pulmonary vascular pathology featuring plasma cell proliferation and it provides clues towards understanding the pathogenesis with this background. 1. Introduction The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is well known to be associated with a variety of clinical presentations, including pulmonary manifestations, renal impairment, hematological disorders, and extravascular volume overload [1–6]. The pathogenesis of POEMS syndrome is not well understood, but overproduction of proinflammatory cytokines, such as interleukin (IL)-1β, IL-6, tumor necrosis factor (TNF)-α, and vascular endothelial growth factor (VEGF), has been reported and could play very important etiological roles [2, 7, 8]. Monoclonal plasma cell proliferative disorders, such as plasma cell neoplasms and Castleman’s disease, are known to be an underlying condition responsible for the syndrome [2–6]. Recently, pulmonary hypertension (PH) is attracting increased attention as a clinical manifestation of the POEMS syndrome and its incidence is reported to be 5–40% [2, 3, 8]. Previous studies indicated that abnormal release of vasoactive cytokines, such as VEGF, is involved in the pathogenesis of this condition [8–12]. However, pathological studies of PH in the POEMS syndrome have been lacking. Herein, we report an autopsy case of a 49-year-old woman who was diagnosed with idiopathic PH and treated accordingly. The autopsy revealed the presence of smoldering plasma cell myeloma and multiple organomegaly in addition to severe
The place of thalidomide in the treatment of multiple myeloma
Alessandro Corso,Silvia Mangiacavalli
Oncology Reviews , 2011, DOI: 10.4081/157
Abstract: Thalidomide represents the first attempt in multiple myeloma (MM) patients to overcome resistance to chemotherapy through a biological agent. The exciting results reported in the first study by Singhal et al. in 1999 [1] led to several other studies which aimed to evaluate its efficacy in different settings and disease phases, to define its toxicity, and to establish the optimal dose. Some of these questions have already been answered while others, such as the best dosage or the best schedule to obtain the highest efficacy with the lowest toxicity, still remain. Thalidomide has been studied as a single agent or in association with other drugs (dexamethasone, chemotherapy and new drugs) showing a synergic activity. We review the results of the main studies on the efficacy and toxicity of thalidomide used as a single agent or in association with other drugs, reflect on its present role, and consider its future contribution to the treatment of MM.
Thalidomide and its analogues in the treatment of Multiple Myeloma
Tahir Latif, Nabeel Chauhan, Rashid Khan, Andrea Moran, Saad Z Usmani
Experimental Hematology & Oncology , 2012, DOI: 10.1186/2162-3619-1-27
Abstract: Multiple myeloma is a mature B-cell neoplasm characterized by a monoclonal expansion of plasma cells in the bone marrow often accompanied by hemocytopenias, immunodeficiency, osteolytic lesions, hypercalcemia and renal failure. Myeloma accounts for about 10% of all hematological malignancies and 1% of all cancers [1]. In United States alone an estimated 20,520 new cases (11,400 men and 9120 women) will be diagnosed in 2011 and 10,610 people will die of myeloma [2]. Ever since the first reported case of ‘mollities ossium’ described in 1844 [3], the disease has remained incurable, despite a better understanding of its pathogenesis and recent advancements in therapeutics. Prior to the advent of alkylating agents, the median survival for multiple myeloma was 1–1.5 years [4]. Following the introduction of L-phenylalanine mustard or melphalan in 1958 [5] and prednisone in 1962 [6], the combination of these two drugs remained the cornerstone of therapy for more than two decades, even though the complete remission rate remained less than 5% and the median survival with this treatment did not exceed 3 years [7]. Since the late 1990s, a number of new classes of drugs have been incorporated in the treatment of multiple myeloma and additional agents are under investigation (Figure 1). The present paper will review the clinical data for the use of thalidomide and its analogs, collectively referred as immunomodulatory drugs or IMiDs in multiple myeloma.Thalidomide was first introduced as an oral sedative and anti-emetic in 1957, but was quickly abandoned due to its profound teratogenic effects. Almost four decades passed before studies demonstrated thalidomide to have anti-cancer properties, specifically showing significant in vitro anti-myeloma activity. It found its use as a novel anti-myeloma drug for relapsed and refractory disease in 1999 [8], later yielding impressive overall response rates of up to 50% when used in combination with dexamethasone and up to 65% when combined
An unexpected case of venous and pulmonary thrombo-embolism in a patient treated with thalidomide for refractory erythema nodosum leprosum: a case report
Riyaaz Ahamed, Wijesiriwardena Bandula, Ratnayake Chamara
Thrombosis Journal , 2011, DOI: 10.1186/1477-9560-9-2
Abstract: The benefit of the immunomodulatory effects of thalidomide was discovered accidentally in 1965 when it was given to patients with leprosy as a sedative to relieve their suffering. Subsequently, researchers noticed an unexpected clinical improvement in the signs and symptoms of ENL. Since then, the off-label use of thalidomide has become increasingly popular due to its effectiveness in the treatment of a variety of malignant and non-malignant conditions refractory to other treatments. Although common adverse effects of thalidomide therapy are well-documented in literature since its introduction, some of the uncommon risks associated with its use are still being discovered. In the recent past, there has been an increased incidence of adverse events being reported in patients treated with thalidomide including thromboembolic complications such as deep vein thrombosis (DVT) and pulmonary embolism (PE). Many studies have reported thomboembolic events with thalidomide when used in combination with multi-drug chemotherapy regimens for the treatment of multiple myeloma [1]. These thrombotic events have also been reported in non malignant conditions treated with thalidomide. A literature search shows few cases of DVT in patients treated with thalidomide for ENL and so far only two cases have been reported with concurrent DVT and pulmonary embolism. [2,3]A 60-year old man from Sri Lanka presented with a history of sudden onset breathlessness and right sided pleuritic chest pain. 5 days before he had developed a painful swelling of his right leg while he was being treated in the hospital for recurrent episodes of ENL. 3 years ago he was diagnosed to have lepromatous leprosy following a positive slit skin smear for mycobacterium leprae. He was commenced on World Health Organization (WHO) multi-drug therapy (MDT) regimen for multi-bacilary (MB) leprosy, which was complicated by type II lepra reaction (ENL) with right sided panuveitis and treated with oral prednisolone 30 mg/day
The role of thalidomide as maintenance after autologous stem cell transplantation in multiple myeloma
Maiolino, Angelo;
Revista Brasileira de Hematologia e Hemoterapia , 2009, DOI: 10.1590/S1516-84842009000800009
Abstract: major progress was obtained over the last ten years in the treatment of multiple myeloma (mm). high dose chemotherapy with autologous stem cell transplantation (asct) and new drugs such as thalidomide, lenalidomide and bortezomib have completely changed the scenario of mm treatment. asct has become the mainstay of mm treatment for patients up to 65 years old. this strategy has been tested in randomized clinical trials which proved that asct can improve overall survival of mm patients. unfortunately, the great majority of these patients will relapse in a few years after asct. strategies of maintenance have been developed trying to improve the results of asct. thalidomide has been tested in this setting in at least 4 randomized clinical trials with a significant impact on response rate, and event free and overall survival.l.
Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
M. Humbert
European Respiratory Review , 2010,
Abstract: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis), and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy.
Renal safety of zoledronic acid with thalidomide in patients with myeloma: a pharmacokinetic and safety sub-study
Andrew Spencer, Andrew Roberts, Nola Kennedy, Christina Ravera, Serge Cremers, Sanela Bilic, Terry Neeman, Michael Copeman, Horst Schran, Kevin Lynch
BMC Pharmacology and Toxicology , 2008, DOI: 10.1186/1472-6904-8-2
Abstract: Twenty-four patients who were enrolled in a large randomized trial of thalidomide vs no thalidomide maintenance therapy for myeloma, in which all patients also received zoledronic acid, were recruited to a pharmacokinetic and renal safety sub-study, and followed for up to 16 months.No significant differences by Wilcoxon rank-sum statistic were found in zoledronic acid pharmacokinetics or renal safety for up to 16 months in patients randomized to thalidomide or not.In myeloma patients receiving maintenance therapy, the combination of zoledronic acid and thalidomide appears to confer no additional renal safety risks over zoledronic acid alone.Modern therapy of multiple myeloma (MM) combines treatments to induce disease response (e.g. thalidomide, corticosteroids and cytotoxic agents) with supportive care to prevent bone and infective complications (e.g. bisphosphonates and antibiotics). However, combining agents might cause interactions that increase toxicity or lessen efficacy. Case reports suggested that MM patients receiving zoledronic acid combined with thalidomide had higher risks of renal impairment [1]. Hence, the United States' Food and Drug Administration requested that Novartis (manufacturer of zoledronic acid) conduct a study to look for pharmacokinetic interaction between zoledronic acid and thalidomide, and to monitor renal safety of MM patients receiving both drugs.The Australasian Leukaemia and Lymphoma Group (ALLG) was conducting a large (>200 patients), randomized clinical trial (ALLG MM6) in MM patients, who had received high-dose therapy and autologous stem cell transplantation (ASCT), to ascertain the efficacy and safety of thalidomide and prednisolone maintenance therapy compared to prednisolone maintenance therapy post-transplantation. All patients were scheduled to receive adjunctive zoledronic acid intravenously on a 4th-weekly basis. The ALLG agreed to incorporate a safety sub-study into the ALLG MM6 study to assess the pharmacokinetics of zol
Pulmonary hypertension  [cached]
Andrew Peacock
European Respiratory Review , 2013,
Abstract: 2011 to 2012 has seen an explosion in published research in the field of pulmonary vascular disease, especially pulmonary hypertension. In conjunction with this research has been an explosion in clinical interest in treating pulmonary hypertension. This is possible because we now have three different generic classes of drug therapy: endothelin receptor antagonists, phosphodiesterase-5 inhibitors and prostacyclins. Clearly, however, we need to be careful that we are treating the correct disease with the correct drug and measuring the correct response. Herein, I will review the papers published over the last year that offer the most insight into the pathobiology, but also those that give us the clinical information we need in epidemiology, treatment and end-points so that we can treat this devastating group of disease.
Atrial septostomy in pulmonary hypertension  [cached]
Nihal ?zdemir
Anadolu Kardiyoloji Dergisi , 2010,
Abstract: Despite the fact that remarkable improvements have been achieved in quality of life and survival through increasing number of specific medical treatment alternatives in severe pulmonary hypertension, many patients have resistance to medical treatment. Atrial septostomy is considered as a palliative procedure in severe pulmonary hypertension to relieve symptoms of severe pulmonary hypertension by reducing right ventricular preload and increasing systemic flow. Gradual balloon dilatation and blade balloon atrial septostomy are the most frequently used methods. If atrial septostomy is performed in experienced medical centers, it provides clinical improvement and even has tendency to extend life expectancy in patients with pulmonary hypertension resistant to medical treatment.
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