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Constitutional Symptoms (constitutional + symptom)
Selected AbstractsHigh-dose immunoglobulines and extracorporeal photochemotherapy in the treatment of febrile ulceronecrotic Mucha-Habermann diseaseDERMATOLOGIC THERAPY, Issue 4 2010Federica Marenco ABSTRACT Febrile ulcero-necrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta (only 41 cases described to date), characterized by an acute onset of ulcero-necrotic papules accompanied by high fever and severe constitutional symptoms. We report a case of a 23-year-old man with a steroid-resistant FUMHD treated by intravenous immunoglobulins (IVIG) combined with methotrexate. Only one case of FUMHD treated by IVIG has been reported to date in literature. Also in our case, IVIG proved to be effective in inducing a dramatic improvement of ulceration and in arresting the appearance of new lesions. Moreover, in our experience we decided to perform a maintenance treatment with extracorporeal photochemotherapy (ECP), to the best of our knowledge not previously used in the treatment of pityriasis lichenoides et varioliformis acuta. ECP, which involves extracorporeal exposure of peripheral blood mononuclear cells to photo-activated 8-methoxypsoralen, induces an immunological reaction against auto-reactive T cell clones, without immune-depression and thus could potentially be useful particularly in FUMHD avoiding the risk of an infective reactivation. [source] Treatment options for hydroxyurea-refractory disease complications in myeloproliferative neoplasms: JAK2 inhibitors, radiotherapy, splenectomy and transjugular intrahepatic portosystemic shuntEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2010Elena Mishchenko Abstract Clinical care of patients with polycythemia vera, essential thrombocythemia and myelofibrosis (MF) requires not only a broad understanding of general treatment principles but also familiarity with the management of hydroxyurea-refractory disease complications. The latter include progressive splenomegaly, symptomatic portal hypertension (e.g. ascites, variceal bleeding), pulmonary hypertension, bone pain, intractable pruritus, constitutional symptoms (e.g. fatigue, night sweats) and cachexia (i.e. loss of lean body mass, general ill health, poor appetite). Some of these symptoms are directly or indirectly related to extramedullary hematopoiesis (EMH) and others to proinflammatory cytokine excess. Results from recent clinical trials of JAK inhibitors suggest remarkable activity in MF-associated constitutional symptoms, cachexia, pruritus and hydroxyurea-refractory splenomegaly. Involved-field radiotherapy is best utilized in the setting of EMH-associated symptoms, including ascites, bone (extremity) pain and pulmonary hypertension. Splenectomy is indicated in the presence of drug-refractory splenomegaly and frequent red cell transfusion requirement. Transjugular intrahepatic portosystemic shunt is used to alleviate symptoms of portal hypertension. [source] Role of apoptosis and cytokines in influenza virus morbidityFEMS MICROBIOLOGY REVIEWS, Issue 4 2005Edward W.A. Brydon Abstract Influenza virus is a major human pathogen that causes epidemics and pandemics with increased morbidity and, especially in the elderly and those with pre-existing medical conditions, increased mortality. Influenza is characterised by respiratory symptoms and constitutional symptoms. Whilst knowledge of the mechanisms underlying host and tissue specificity has advanced considerably of late we still know relatively little about other aspects of influenza virus virulence. In this review, we will explore what is known about the role of apoptosis in respiratory epithelial cell damage and the role of cytokines in inflammation and constitutional symptoms with particular emphasis on the link between apoptosis, inflammation, fever and cytokine production. [source] Unilateral sacroiliitis associated with systemic isotretinoin treatmentINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 3 2010Mauro Barbareschi MD Background, Acne fulminans is the most severe form of inflammatory acne characterized by the acute onset of inflammatory nodules and plaques, most commonly on the chest and the back. The lesions undergo rapid suppuration, leaving ragged hemorrhagic ulcers. Typically, it affects adolescent males with a history of mild to moderate acne. The affected patients often have constitutional symptoms such as fever, malaise, arthralgias, and myalgias. Leukocytosis is commonly associated. Sacroiliitis is reported in 21% of acne fulminans patients in association with arthritis and in a few cases it is reported during isotretinoin treatment, suggesting the drug triggering. Conclusion, We report a case of a young male patient in whom the induction of acne fulminans by systemic isotretinoin was associated with unilateral sacroiliitis. [source] An approach to the management of necrotising fasciitis in neonatesINTERNATIONAL WOUND JOURNAL, Issue 2 2005Soraya Zuloaga-Salcedo MD Abstract Necrotising fasciitis is a severe, life-threatening soft tissue infection. It produces an extensive cellulitis with severe involvement of subcutaneous tissue, fascia, muscle or both, resulting in necrosis of the tissue. All age groups, including neonates, can be affected. Patients with necrotising fasciitis present with more severe constitutional symptoms and have a poor outcome, unless aggressive antibiotic therapy and surgical debridement are instituted promptly. The debridement of necrotic tissue is imperative to control the infection, but results in deep wounds that require further treatment. In this study, the neonate was treated with alginate dressings and negative pressure therapy after resolution of cellulitis, with excellent results and no untoward events. [source] Atypical lymphocytic lobular panniculitisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 4 2004Cynthia M. Magro Background:, Although subcutaneous T-cell lymphoma (SCTCL) is considered an aggressive form of lymphoma, some patients manifest a long waxing and waning phase unaccompanied by constitutional symptoms. Methods:, Twelve patients were prospectively encountered, presenting with a lymphocytic panniculitis accompanied by lymphoid atypia, although not fulfilling criteria for SCTCL. Clinical, histologic, phenotypic, and genotypic analyses were conducted. Results:, There were five men, one boy, and six women; none had symptoms compatible with lupus erythematosus or aggressive SCTCL. All but two had a waxing and waning course of years. Four patients had periodic cytopenias accompanied by fevers. While responding somewhat to prednisone, the lesions relapsed. In one patient, treatment with alemtuzumab (CAMPATH-1) led to complete lesional resolution with no recurrence. Light microscopy showed expansion of the interstices of the fat lobule by mildly atypical lymphocytes of the CD4 subset in 10 biopsies from eight patients; in the other four patients, there was an increase in CD8 lymphocytes. There was diminished expression of CD5 and/or CD7 in the majority of biopsies. Ten of 13 biopsies showed clonal T-cell receptor-, rearrangements. Conclusions:, We apply the term atypical lymphocytic lobular panniculitis to this distinctive form of lymphocytic panniculitis manifesting this light microscopic, phenotypic, and genotypic profile. [source] Waldenstrom's macroglobulinemia presenting with spinal cord compression: A case reportAMERICAN JOURNAL OF HEMATOLOGY, Issue 12 2006Hani Al-Halabi Abstract Waldenstrom's macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma characterized by a wide range of clinical presentations related to direct tumor infiltration and the production of IgM. Most commonly it presents with cytopenia, hepatosplenomegaly, lymphadenopathy, constitutional symptoms, and hyperviscosity syndrome. We report a case of WM in an 81-year-old man who initially presented with severe back pain. The patient had no peripheral lymphadenopathy or hepatosplenomegaly and his peripheral blood smear was normal. MRI of the spine revealed an epidural mass causing spinal cord compression at T9. Surgical decompression was performed and pathological analysis of the mass revealed a lymphoproliferative B-cell process. The diagnosis of WM was established after cytomorphologic and immunohistochemical analysis of the patient's bone marrow revealed the presence of a lymphoid/lymphoplasmacytoid-like bone marrow infiltrate along with an elevated serum IgM level. The patient responded both clinically and serologically to local radiotherapy. This case is unusual because the patient lacked all common clinical features of WM. This is the first reported case of epidural spinal cord compression as the initial manifestation of WM, adding to the spectrum of clinical presentations seen in this disease. Am. J. Hematol., 2006. © 2006 Wiley-Liss, Inc. [source] HLA,DRB4 as a genetic risk factor for Churg-Strauss syndromeARTHRITIS & RHEUMATISM, Issue 9 2007Augusto Vaglio Objective To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease. Methods Low-resolution genotyping of HLA,A, HLA,B, and HLA,DR loci and genotyping of TNFA ,238A/G and TNFA ,308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls. Results The frequency of the HLA,DRB1*07 allele was higher in the CSS patients than in controls (27.1% versus 13.3%; ,2 = 12.64, P = 0.0003, corrected P [Pcorr] = 0.0042, odds ratio [OR] 2.42, 95% confidence interval [95% CI] 1.47,3.99). The HLA,DRB4 gene, present in subjects carrying either HLA,DRB1*04, HLA,DRB1*07, or HLA,DRB1*09 alleles, was also far more frequent in patients than in controls (38.5% versus 20.1%; ,2 = 16.46, P = 0.000058, Pcorr = 0.000232, OR 2.49, 95% CI 1.58,3.09). Conversely, the frequency of the HLA,DRB3 gene was lower in patients than in controls (35.4% versus 50.4%; ,2 = 7.62, P = 0.0057, Pcorr = 0.0228, OR 0.54, 95% CI 0.35,0.84). CSS has 2 major clinical subsets, antineutrophil cytoplasmic antibody (ANCA),positive, with features of small-vessel vasculitis, and ANCA-negative, in which organ damage is mainly mediated by tissue eosinophilic infiltration; analysis of HLA,DRB4 in patients categorized by different numbers of vasculitic manifestations (purpura, alveolar hemorrhage, mononeuritis multiplex, rapidly progressive glomerulonephritis, and constitutional symptoms) showed that its frequency strongly correlated with the number of vasculitis symptoms (P for trend = 0.001). Conclusion These findings indicate that HLA,DRB4 is a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease. [source] Chronic pelvic pain in women: still a challengeBJU INTERNATIONAL, Issue 9 2008Orietta Dalpiaz Chronic pelvic pain (CPP), a common condition particularly in reproductive-aged women, causes disability and distress, and significantly compromises quality of life and affects healthcare costs. The pathogenesis of CPP is still poorly understood and consequently poorly managed. Furthermore, the lack of a consensus on the definition of CPP greatly hinders epidemiological studies. Patients present with various associated problems, including bladder or bowel dysfunction, gynaecological pathologies or sexual dysfunction, and other systemic or constitutional symptoms. Other conditions, e.g. depression, anxiety and drug addiction, can also coexist. Effective management presupposes an integrated knowledge of all pelvic organs and other systems, including musculoskeletal, neurological and psychiatric systems. The key to treating CPP is to treat it as the complex disease it is. Treatment options range from conservative medical therapy to surgical intervention, and are primarily directed towards symptom relief. Unsatisfactory results of treatment render this condition a frustrating problem for both patients and physicians. [source] Systemic pyoderma gangrenosum responding to infliximab and adalimumabBRITISH JOURNAL OF DERMATOLOGY, Issue 5 2005V.G. Hubbard Summary Pyoderma gangrenosum (PG) is frequently associated with constitutional symptoms as part of a nonspecific inflammatory response. However, extracutaneous discrete aseptic neutrophilic infiltrates are extremely rare. We report a patient with idiopathic PG with splenic and psoas muscle involvement. His disease was extremely aggressive and was unresponsive to conventional immunosuppressive therapy. His cutaneous and extracutaneous PG cleared with infliximab and adalimumab. [source] Phase II study of the histone deacetylase inhibitor MGCD0103 in patients with previously treated chronic lymphocytic leukaemiaBRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2009Kristie A. Blum Summary MGCD0103, an orally available class I histone deacetylase (HDAC) inhibitor, was examined for pre-clinical activity in chronic lymphocytic leukaemia (CLL). A phase II clinical trial was performed, starting at a dose of 85 mg/d, three times per week. Dose escalation to 110 mg or the addition of rituximab was permitted in patients without a response after two or more cycles. MGCD0103 demonstrated pre-clinical activity against CLL cells with a LC50 (concentration lethal to 50%) of 0·23 ,mol/l and increased acetylation of the HDAC class I specific target histone H3. Twenty-one patients received a median of two cycles of MGCD0103 (range, 0,12). All patients had previously received fludarabine, 33% were fludarabine refractory, and 71% had del(11q22·3) or del(17p13·1). No responses according to the National Cancer Institutes 1996 criteria were observed. Three patients received 110 mg and four patients received concomitant rituximab, with no improvement in response. Grade 3,4 toxicity consisted of infections, thrombocytopenia, anaemia, diarrhoea, and fatigue. HDAC inhibition was observed in six out of nine patients on day 8. Limited activity was observed with single agent MGCD0103 in high risk patients with CLL. Future investigations in CLL should focus on broad HDAC inhibition, combination strategies, and approaches to diminish constitutional symptoms associated with this class of drugs. [source] Giant cell arteritis with spontaneous remissionCLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 1 2007Valerie Purvin MD Abstract Background:, Clinical manifestations of giant cell arteritis (GCA) are variable. Whether signs and symptoms present in an explosive fashion or insidiously, once manifest the course is usually progressive unless treatment is initiated. Methods:, A retrospective review of patients with GCA seen in an outpatient neuro-ophthalmology clinic. Results:, We report four patients with biopsy-proven GCA who experienced spontaneous remission. Clinical manifestations consisted of headache and diplopia in two patients, constitutional symptoms in one patient and facial pain in another. Conclusions:, Clinicians should be aware of this aspect of the disease in order to avoid a delay in diagnosis and treatment. [source] | ||||||||||||||||||||||