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• W4328000830 abstract "To the Editor: Monoclonal gammopathies are frequently encountered in clinical practice, and most have so-called undetermined significance. Current diagnostic criteria for monoclonal gammopathy of undetermined significance (MGUS), previously known as benign monoclonal gammopathy, include presence of a monoclonal immunoglobulin in serum of 30 g/L or less, 10% or less of plasma cells in bone marrow, and absence of clinical manifestations related to the M protein, such as anemia, hypercalcemia, lytic bone lesions, and renal insufficiency.1Bladé J Monoclonal gammopathy of undetermined significance.N Engl J Med. 2006; 355: 2765-2770Crossref PubMed Scopus (90) Google Scholar, 2Kyle RA Rajkumar SV Monoclonal gammopathy of undetermined significance.Br J Haematol. 2006; 134: 573-589Crossref PubMed Scopus (182) Google Scholar MGUS is found in 3% of persons older than 50 years, in 5% of persons older than 70 years, and in up to 7% of patients seeking medical evaluation. The importance of MGUS lies in its well-known potential to progress to multiple myeloma or a related plasma cell disorder at an average rate of 1% per year. However, even small and nonprogressive B-cell clones can have profound consequences that may be less familiar to clinicians. We present illustrative cases of probable immune-mediated disorders strongly associated with MGUS. These nonmalignant disorders are rare, and with such disorders patients may present to a variety of medical specialists. Presence of the M protein may be a valuable clue to the diagnosis. This case series is intended to increase awareness of these disorders and their association with M proteins. Case 1: Acquired C1 Inhibitor Deficiency. A 60-year-old woman was referred for investigation of recurrent abdominal pain of 1 year's duration. Seven months before presentation, urgent laparoscopy performed elsewhere was unremarkable other than revealing the presence of ascites. Two weeks before referral, another laparoscopic procedure was performed during a bout of intense abdominal pain. Again, ascites was evident. Acute abdominal pain recurred, and the patient was transferred to our hospital. MGUS, IgG κ monoclonal protein, had been diagnosed 9 years previously; family history was noncontributory. The patient's abdomen was diffusely tender without rebound tenderness or guarding. Results of all routine laboratory tests, including C-reactive protein levels, were normal. Complement assays showed low levels of CH50, C1q, and C4, whereas C3 levels were normal. C1 inhibitor levels were low, both quantitatively (0.12 g/L; reference range, 0.21-0.39 g/L) and functionally (<26%; reference range, 70%-130%). Acquired C1 inhibitor deficiency was diagnosed. During a subsequent attack of abdominal pain, computed tomography confirmed bowel wall edema. At last follow-up, 14 years after the diagnosis, the patient was doing well. With administration of 200 mg of danazol, up to 4 times a day, the C1 inhibitor level and function normalized. Attacks became less frequent and less severe and could be managed with addition of tranexamic acid. The monoclonal gammopathy was not progressive. Discussion. C1 inhibitor is an inhibitor of the first component of the classic complement pathway and of the kinin-generating pathways. When the C1 inhibitor level is deficient, uncontrolled complement activation and release of inflammatory mediators cause angioedema, characterized by capillary hyperpermeability and extravasation of fluid and proteins.3Agostoni A Aygören-Pürsün E Binkley KE et al.Hereditary and acquired angioedema: problems and progress: Proceedings of the Third C1 Esterase Inhibitor Deficiency Workshop and Beyond.J Allergy Clin Immunol. 2004; 114: S51-S131Abstract Full Text Full Text PDF PubMed Scopus (565) Google Scholar, 4Rosen FS Davis III, AE Deficiencies of C1 inhibitor.Best Pract Res Clin Gastroenterol. 2005; 19: 251-261Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar, 5Markovic SN Inwards DJ Frigas EA Phyliky RP Acquired C1 esterase inhibitor deficiency.Ann Intern Med. 2000; 132: 144-150Crossref PubMed Scopus (113) Google Scholar, 6Zuraw BL Hereditary angioedema.N Engl J Med. 2008; 359: 1027-1036Crossref PubMed Scopus (595) Google Scholar Typically, episodes of angioedema recur without urticaria or pruritus. This nonallergic angioedema most often affects the skin, upper airway, and bowel. Symptoms range from minor inconvenience to life-threatening laryngeal edema. Abdominal attacks can cause severe pain, nausea, and vomiting, and unnecessary abdominal surgery may be performed. A shift of fluids into the interstitium or peritoneal cavity during abdominal attacks can cause hemoconcentration and clinically important hypotension. Despite the impressive clinical picture, inflammatory signs are typically absent (eg, normal C-reactive protein level). Most episodes are self-limiting, lasting 1 to 7 days and recurring every 1 to 9 months, often in a uniform pattern. Hereditary angioedema usually presents during the first or second decade of life.6Zuraw BL Hereditary angioedema.N Engl J Med. 2008; 359: 1027-1036Crossref PubMed Scopus (595) Google Scholar Family history usually points to autosomal dominant inheritance. Hereditary angioedema is due to either a lack of synthesis of the C1 inhibitor (85% of cases) or the synthesis of a nonfunctional protein (15% of cases). With the hereditary form, no associated disease is found, and C1q levels are normal. Acquired C1 inhibitor deficiency is less frequent: 145 cases have been reported since the first description in 1972.7Castelli R Deliliers DL Zingale LC Pogliani EM Cicardi M Lymphoproliferative disease and acquired C1 inhibitor deficiency [letter].Haematologica. 2007; 92: 716-718Crossref PubMed Scopus (63) Google Scholar Acquired C1 inhibitor deficiency affects adult or elderly patients with no family history of this deficiency and is often associated with low-grade lymphoproliferative disorders, including MGUS in up to 40% of cases.3Agostoni A Aygören-Pürsün E Binkley KE et al.Hereditary and acquired angioedema: problems and progress: Proceedings of the Third C1 Esterase Inhibitor Deficiency Workshop and Beyond.J Allergy Clin Immunol. 2004; 114: S51-S131Abstract Full Text Full Text PDF PubMed Scopus (565) Google Scholar, 4Rosen FS Davis III, AE Deficiencies of C1 inhibitor.Best Pract Res Clin Gastroenterol. 2005; 19: 251-261Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar, 5Markovic SN Inwards DJ Frigas EA Phyliky RP Acquired C1 esterase inhibitor deficiency.Ann Intern Med. 2000; 132: 144-150Crossref PubMed Scopus (113) Google Scholar, 7Castelli R Deliliers DL Zingale LC Pogliani EM Cicardi M Lymphoproliferative disease and acquired C1 inhibitor deficiency [letter].Haematologica. 2007; 92: 716-718Crossref PubMed Scopus (63) Google Scholar The M protein can be IgG, IgM, or IgA. The immunoglobulin is hypothesized to mediate consumption and/or inactivation of the C1 inhibitor. In the acquired form of C1 inhibitor deficiency, C1q serum levels are decreased, which is attributed to consumption due to immune complex formation. C4 levels are low in both the inherited and the acquired form, whereas C3 levels are normal. Management of acquired C1 inhibitor deficiency consists of treating the underlying disorder, if possible, and preventing and treating angioedema. Acute angioedema is treated with antifibrinolytic agents, such as tranexamic acid. These agents have been advocated especially for prophylactic use.8Cicardi M Zingale LC Pappalardo E Folcioni A Agostoni A Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies.Medicine (Baltimore). 2003; 82: 274-281PubMed Google Scholar 17-α-Alkylated androgens (including danazol) have been used prophylactically because androgens increase liver protein synthesis, including C1 inhibitor levels and function.5Markovic SN Inwards DJ Frigas EA Phyliky RP Acquired C1 esterase inhibitor deficiency.Ann Intern Med. 2000; 132: 144-150Crossref PubMed Scopus (113) Google Scholar Response to C1 inhibitor substitution or fresh frozen plasma in patients with the acquired form of C1 inhibitor deficiency varies because of hypercatabolism. Case 2: Systemic Capillary Leak Syndrome. A 61-year-old man was referred to our hospital because of hypo volemic shock. Hypotension progressed to immeasurable blood pressure levels, and associated edema developed. Within 24 hours, his hemoglobin concentration increased from 15.3 g/dL to 22.9 g/dL (reference ranges shown parenthetically) (12.0-16.0 g/dL), along with an increase in blood urea nitrogen levels; his serum albumin level decreased from 40 g/L to 35 g/L (40.2-47.6 g/L). Intense fluid resuscitation was initiated. After a few hours, hemodynamic stabilization was obtained, and a polyuric phase followed. During the 18 months before the index admission, the patient was admitted to a regional hospital 5 times because of shock. Episodes were variably preceded by malaise, abdominal discomfort, fatigue, rhinitis, palpitations, dyspnea, thirst, oliguria, orthostatic symptoms, fever, and palpebral edema. Duration of attacks ranged from 1 day to several days, and they were characterized by extreme circulatory collapse and high fluid requirements. Between crises, the patient was free of symptoms. A monoclonal IgG κ gammopathy was found and systemic capillary leak syndrome diagnosed. A prophylactic regimen that consisted of terbutaline and theophyllines was initiated. The patient has been followed up for 9 years. Although the attacks are less severe since the diagnosis, except for 2 occasions that required admission to the intensive care unit, symptoms recur irregularly and are accompanied by weight gain, lowering of blood pressure, and decreased urinary output. At onset of a prodromal phase, methylprednisolone is added for 1 or 2 days to the usual prophylactic regimen. The IgG monoclonal gammopathy remains stable at a low level. Discussion. The systemic capillary leak syndrome is a rare but life-threatening disorder, characterized by recurrent attacks of hypovolemic shock and generalized edema.9Clarkson B Thompson D Horwith M Luckey EH Cyclical edema and shock due to increased capillary permeability.Am J Med. 1960 Aug; 29: 193-216Abstract Full Text PDF PubMed Scopus (278) Google Scholar About 100 cases have been reported.10Dhir V Arya V Malav IC Suryanarayanan BS Gupta R Dey AB Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years.Intern Med. 2007; 46 (Epub 2007 Jun 15.): 899-904Crossref PubMed Scopus (90) Google Scholar The syndrome is attributed to transient capillary hyperpermeability with extravasation of albumin-rich plasma, up to 70% of total plasma volume. The acute phase is characterized by a typical triad of hypotension, increased hematocrit, and hypoalbuminemia. Monoclonal gammopathy, usually IgG, is typically present. The pathophysiologic role of the M protein is unclear. Attacks of the systemic capillary leak syndrome vary in frequency, severity, and duration. The systemic capillary leak syndrome affects otherwise healthy middle-aged individuals with no positive family history of this condition. The classic clinical evolution is triphasic. First, a prodromal phase of a few hours to days is characterized by polydipsia and oliguria and by nonspecific symptoms such as those described in the case report. Second, the capillary leak phase, lasting 1 to 4 days, is heralded by generalized edema accompanied by hemoconcentration, hypotension that can lead to hypovolemic shock, and weight gain. Intestinal edema, ascites, pleural and pericardial effusions, muscular edema (with the compartment syndrome), and oliguric nonproteinuric prerenal failure may occur. Third, during the recruitment phase, fluid and proteins are reabsorbed, and polyuria replaces oliguria. In this latter phase, overagressive fluid correction may lead to pulmonary edema. Supportive treatment consists of judicious volume therapy: fluid substitution in the capillary leak phase and promotion of diuresis of accumulated fluid in the recruitment phase. β2-Agonists (eg, terbutaline) and theophylline have been recommended as prophylactic therapy, without definite proof of efficacy.11Amoura Z Papo T Ninet J et al.Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment.Am J Med. 1997; 103: 514-519Abstract Full Text Full Text PDF PubMed Scopus (131) Google Scholar, 12Droder RM Kyle RA Greipp PR Control of systemic capillary leak syndrome with aminophylline and terbutaline.Am J Med. 1992; 92: 523-526Abstract Full Text PDF PubMed Scopus (92) Google Scholar, 13Tahirkheli NK Greipp PR Treatment of the systemic capillary leak syndrome with terbutaline and theophylline: a case series.Ann Intern Med. 1999; 130: 905-909Crossref PubMed Scopus (113) Google Scholar Recently, 3 patients were treated successfully with high-dose intravenous immunoglobulins.14Lambert M Launay D Hachulla E et al.High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome.Crit Care Med. 2008; 36: 2184-2187Crossref PubMed Scopus (91) Google Scholar Case 3: Acquired von Willebrand Syndrome. A 38-year-old woman with an enlarged left ovary was referred for preoperative evaluation. She noted prolonged bleeding after minor cutaneous injury and menorrhagia of 1 year's duration. No other bleeding symptoms were evident. Previous dental surgery (4 years previously) and knee surgery (25 years previously) were uncomplicated. The patient took no regular medication and had no family history of bleeding. Platelet count, prothrombin time, and D-dimer level were normal. The activated partial thromboplastin time was prolonged. Factor VIII coagulant, von Willebrand antigen, and ristocetin cofactor were profoundly decreased, and platelet aggregation was severely depressed. A monoclonal gammopathy IgG κ was found, without evidence of multiple myeloma. Acquired von Willebrand syndrome was diagnosed. Correction of the hemostatic defect after desmopressin administration was brief. Likewise, the effect of von Willebrand factor concentrate substitution was limited and temporary. Intravenous IgG was infused with total correction for several days, facilitating laparoscopic resection of a benign ovary tumor without complications. During the following years, hip arthroplasty and dental extractions were performed safely with prior administration of intravenous immunoglobulin. Discussion. The acquired von Willebrand syndrome is an uncommon hemostatic disorder, usually arising in elderly patients with no prior personal or family history of bleeding. Since 1968, more than 300 cases have been reported.15Kumar S Pruthi RK Nichols WL Acquired von Willebrand disease.Mayo Clin Proc. 2002; 77: 181-187PubMed Scopus (85) Google Scholar, 16Franchini M Lippi G Acquired von Willebrand syndrome: an update.Am J Hematol. 2007; 82: 368-375Crossref PubMed Scopus (88) Google Scholar Of the various diseases associated with acquired von Willebrand syndrome, hematoproliferative disorders, including MGUS, are most frequent, occurring in 50% to 60% of patients.15Kumar S Pruthi RK Nichols WL Acquired von Willebrand disease.Mayo Clin Proc. 2002; 77: 181-187PubMed Scopus (85) Google Scholar, 16Franchini M Lippi G Acquired von Willebrand syndrome: an update.Am J Hematol. 2007; 82: 368-375Crossref PubMed Scopus (88) Google Scholar, 17Frederici AB Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders.Semin Hematol. 2006; 43: S48-S58Abstract Full Text Full Text PDF PubMed Scopus (66) Google Scholar Other disorders associated with the acquired von Willebrand syndrome include hypothyroidism, solid tumors, and autoimmune disorders. Laboratory findings and clinical presentation are the same as in patients with the congenital form of von Willebrand disease. The pathogenesis is unclear; however, autoantibody-mediated inhibition of von Willebrand factor function or enhanced clearance of the factor VIII von Willebrand factor complex is thought to play a role. Management consists of treating the underlying disorder, if possible, and correcting and preventing bleeding episodes. The effects of desmopressin infusion, which induces release of von Willebrand factor from endothelial cells, are transient and less pronounced than in congenital von Willebrand disease, which has been attributed to an accelerated plasma clearance of the von Willebrand factor. Similarly, large doses of von Willebrand factor-containing concentrates may be required to overwhelm an inhibitory autoantibody. Administration of high-dose intravenous IgG can produce more sustained responses in patients with acquired von Willebrand syndrome, especially when associated with MGUS or lymphoproliferative disorders.15Kumar S Pruthi RK Nichols WL Acquired von Willebrand disease.Mayo Clin Proc. 2002; 77: 181-187PubMed Scopus (85) Google Scholar, 16Franchini M Lippi G Acquired von Willebrand syndrome: an update.Am J Hematol. 2007; 82: 368-375Crossref PubMed Scopus (88) Google Scholar, 17Frederici AB Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders.Semin Hematol. 2006; 43: S48-S58Abstract Full Text Full Text PDF PubMed Scopus (66) Google Scholar Case 4: Schnitzler Syndrome. A 37-year-old man presented with progressive aching of the joints, especially of his wrists and ankles, and morning stiffness. During the past 2.5 years, he had repeatedly been seen at the emergency department because of bouts of fever. Laboratory tests revealed chronic inflammation, also occurring between febrile episodes, with C-reactive protein levels varying between 30 and 94 mg/L (<5.0 mg/L) and erythrocyte sedimentation rates higher than 55 mm/h (1-15 mm/h). Chronic urticaria waxed and waned, with minor itching. Evidence of monoclonal gammopathy IgM κ, without evidence of Waldenström disease, led to the diagnosis of Schnitzler syndrome. Bone scintigraphy showed periosteal reaction at both knees. Treatment withanakinra, 100 mg/d subcutaneously, induced prompt resolution of articular and cutaneous symptoms and normalization of the laboratory inflammatory markers but resulted in moderate neutropenia. Temporary interruption of therapy provoked recurrence of symptoms and signs. Discussion. Schnitzler syndrome, described in 1972, is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy, primarily of the IgM type.18de Koning HD Bodar EJ van der Meer JW Simon A Schnitzler Syndrome Study Group Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment.Semin Arthritis Rheum. 2007 Dec; 37 (Epub 2007 Jun 21.): 137-148Abstract Full Text Full Text PDF PubMed Scopus (203) Google Scholar Minor criteria include intermittent fever, arthralgia or arthritis, bone pain, lymphadenopathy, hepatomegaly and/or splenomegaly, elevated erythrocyte sedimentation rate and/or leukocytosis, and bone abnormalities.18de Koning HD Bodar EJ van der Meer JW Simon A Schnitzler Syndrome Study Group Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment.Semin Arthritis Rheum. 2007 Dec; 37 (Epub 2007 Jun 21.): 137-148Abstract Full Text Full Text PDF PubMed Scopus (203) Google Scholar, 19Lipsker D Veran Y Grunenberger F Cribier B Heid E Grosshans E The Schnitzler syndrome: four new cases and review of the literature.Medicine (Baltimore). 2001; 80: 37-44Crossref PubMed Scopus (223) Google Scholar Schnitzler syndrome can be diagnosed when both major criteria and 2 or more minor criteria are fulfilled, and other causes are excluded. Ninety-four patients have been reported as having this syndrome.18de Koning HD Bodar EJ van der Meer JW Simon A Schnitzler Syndrome Study Group Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment.Semin Arthritis Rheum. 2007 Dec; 37 (Epub 2007 Jun 21.): 137-148Abstract Full Text Full Text PDF PubMed Scopus (203) Google Scholar Chronic, often nonitching, urticaria is the hallmark and usually the first symptom. Of the various therapeutic options that have been proposed, anakinra, an interleukin 1 receptor antagonist, shows the most promise; it has induced complete remission (with continuous administration) in 7 of 7 previously described patients.18de Koning HD Bodar EJ van der Meer JW Simon A Schnitzler Syndrome Study Group Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment.Semin Arthritis Rheum. 2007 Dec; 37 (Epub 2007 Jun 21.): 137-148Abstract Full Text Full Text PDF PubMed Scopus (203) Google Scholar Case 5: Scleromyxedema. A 39-year-old man experienced 4 episodes of confusion during a 2-year period, each predated by a flulike illness and culminating in status epilepticus with prolonged postictal coma. Between episodes, cognitive dysfunction persisted, affecting memory, attention, executive function, and word finding. During the first episode, an M protein IgG κ was found. Neuroimaging and cerebrospinal fluid analysis were unrevealing. The appearance over a few weeks of erythematous plaques on the face and skin creases over the glabella suggested the diagnosis of scleromyxedema, which was confirmed pathologically. The M protein value increased from 7 g/L to 26 g/L in 1 month's time, anemia developed, and bony plasmocytoma was found; a diagnosis of multiple myeloma was established. Hematologic treatment consisted of plasmapheresis, dexamethasone, intravenous immunoglobulin, autologous stem cell transplant, and thalidomide. Six months after the diagnosis of scleromyxedema, the patient experienced an acute myocardial infarction. During the following year, the patient's condition improved, including substantial recovery of cognitive functions, and his antiepileptic medication was tapered. Discussion. Scleromyxedema is a rare disease of unknown etiology that is characterized by hyaluronic acid deposition into the superficial dermis and other organs and production of a monoclonal protein, primarily IgG λ.20Gabriel SE Perry HO Oleson GB Bowles CA Scleromyxedema: a scleroderma-like disorder with systemic manifestations.Medicine (Baltimore). 1988; 67: 58-65PubMed Google Scholar, 21Pomann JJ Rudner EJ Scleromyxedema revisited.Int J Dermatol. 2003; 42: 31-35Crossref PubMed Scopus (78) Google Scholar Papular mucinosis and generalized lichen myxedematosus are related terms. Usually, the disease appears equally in either sex and in persons between ages 30 and 50 years. The cutaneous eruption consists of waxy papules, varying from 2 to 5 mm, with marked sclerosis and induration of the skin, especially of the head, neck, and extremities. Involvement of the gastrointestinal tract, musculoskeletal system, renal system, and cardiopulmonary system is common.21Pomann JJ Rudner EJ Scleromyxedema revisited.Int J Dermatol. 2003; 42: 31-35Crossref PubMed Scopus (78) Google Scholar Reports of cardiac pathologic findings have included myocardial infarction. Neurologic manifestations include encephalopathy, stroke, seizures, coma, psychosis, and peripheral neuropathies.22Berger JR Dobbs MR Terhune MH Maragos WF The neurologic complications of scleromyxedema.Medicine (Baltimore). 2001; 80: 313-319Crossref PubMed Scopus (37) Google Scholar As in the current case, patients have been described in whom neurologic abnormalities preceded the classical skin lesions. The term dermato-neuro syndrome has been coined to describe a flulike prodrome, followed by fever (high temperatures), seizures, and coma in the setting of scleromyxedema.21Pomann JJ Rudner EJ Scleromyxedema revisited.Int J Dermatol. 2003; 42: 31-35Crossref PubMed Scopus (78) Google Scholar Treatment of scleromyxedema has included a number of modalities, including corticosteroids and immunosuppressive agents, antineoplastic agents, plasmapheresis, thalidomide, and stem cell transplant. Recently, treatment with intravenous immunoglobulin has been associated with high response rates but requires maintenance infusions to preserve disease control.23Blum M Wigley FM Hummers LK Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG).Medicine (Baltimore). 2008; 87: 10-20Crossref PubMed Scopus (90) Google Scholar This case series highlights the potentially profound clinical importance of a monoclonal protein, besides its capability of malignant transformation. Most of these M protein-related disorders are rare, probably in part due to underrecognition, and some are life-threatening. The mechanisms of these diseases are not fully understood, and the pathogenetic role of the monoclonal protein is uncertain. Immune-mediated mechanisms are commonly implicated. This contrasts with other monoclonal component-related diseases that are caused by M protein aggregation, such as light chain cast nephropathy, AL amyloidosis, light chain deposition disease, crystal-storing histiocytosis, and cryoglobulinemia type I.24Merlini G Stone MJ Dangerous small B-cell clones.Blood. 2006 Oct 15; 108 (Epub 2006 Jun 22.): 2520-2530Crossref PubMed Scopus (334) Google Scholar, 25Fain O Monoclonal gammopathies [in French].Rev Prat. 2006; 56: 40-50PubMed Google Scholar Examples of probable immune-mediated disorders strongly associated with MGUS are listed in the Table. The disorders with primary dermatological and neurologic manifestations have been reviewed.26Daoud MZ Lust JA Kyle RA Pittelkow MR Monoclonal gammopathies and associated skin disorders.J Am Acad Dermatol. 1999; 40: 507-535Abstract Full Text Full Text PDF PubMed Scopus (177) Google Scholar, 27Ropper AH Gorson KC Neuropathies associated with paraproteinemia.N Engl J Med. 1998; 338: 1601-1607Crossref PubMed Scopus (246) Google Scholar A monoclonal protein is almost a sine qua non in certain disorders (such as the systemic capillary leak syndrome, scleromyxedema, and Schnitzler syndrome), whereas the underlying diseases can be more variable in other disorders (such as acquired von Willebrand syndrome and acquired C1 inhibitor deficiency).TABLEExamples of Probable Immune-Mediated Disorders Strongly Associated With Monoclonal Gammopathy of Undetermined Signi.cance (MGUS) Primarily dermatological26Daoud MZ Lust JA Kyle RA Pittelkow MR Monoclonal gammopathies and associated skin disorders.J Am Acad Dermatol. 1999; 40: 507-535Abstract Full Text Full Text PDF PubMed Scopus (177) Google Scholar Schnitzler syndromeaPresence of monoclonal protein virtually obligatory.,bDiscussed in current series.ScleromyxedemaaPresence of monoclonal protein virtually obligatory.,bDiscussed in current series.ScleredemaNecrobiotic xanthogranulomaPlane xanthomaPrimarily neurologic27Ropper AH Gorson KC Neuropathies associated with paraproteinemia.N Engl J Med. 1998; 338: 1601-1607Crossref PubMed Scopus (246) Google Scholar MGUS neuropathyaPresence of monoclonal protein virtually obligatory.Miscellaneous Type II mixed cryoglobulinemiaaPresence of monoclonal protein virtually obligatory.Chronic cold agglutinin diseaseaPresence of monoclonal protein virtually obligatory.Acquired C1 inhibitor deficiencybDiscussed in current series.Systemic capillary leak syndromeaPresence of monoclonal protein virtually obligatory.,bAcquired von Willebrand syndromebDiscussed in current series.a Presence of monoclonal protein virtually obligatory.b Discussed in current series. 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• W4328000830 title "Monoclonal Gammopathy of Undetermined Significance: Significant Beyond Hematology" @default.
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