Here, we present the case of a patient with a 32-year history of type III VWD that was ultimately found to be AVWS related to an IgG MGUS. [pubmed.ncbi.nlm.nih.gov]

Further, our first case suggests that a monoclonal gammopathy may not be present at the onset of disease but may present later in the course. [omicsonline.org]

Four of the patients presented hypocomplementaemia but the cryoglobulin determinations were negative for all of them. [revistanefrologia.com]

An M protein is typically an incidental finding when a patient is being assessed for any of a number of presenting symptoms or conditions. [mdedge.com]

Abstract A case of chronic non-neuronopathic Gaucher's disease is presented. The diagnosis was based on the finging of typical Gaucher cells in the bone marrow spleen. The patient also had a benign monoclonal gammapathy. [ncbi.nlm.nih.gov]

• Splenomegaly

  General radiographs revealed no evidence of bone lesions, but splenomegaly was detected. Primary macroglobulinemia was suspected based on these findings. [ci.nii.ac.jp]

  […] differential; however, this disease is usually characterized by a monoclonal IgM immunoglobulin produced by a clonal malignancy of well-differentiated lymphocytes that display plasmacytoid features. 3 Clinical features include anemia, lymphadenopathy, and splenomegaly [path.upmc.edu]

• Lymphadenopathy

  Lymphadenopathy (more than 1 cm) attributable to metastatic carcinoma of the prostate occurred in one patient with MGUS. No patient had macroglossia. [mayoclinicproceedings.org]

  Computed tomography (CT) revealed mediastinal lymphadenopathy and bilateral lung involvement. [ehoonline.biomedcentral.com]

• Hodgkin Lymphoma

  Nonetheless, the Hodgkin disease was apparently diagnosed after a follow-up duration of only 2 months, suggesting, again, that the monoclonal gammopathy was merely an indication of unsuspected underlying Hodgkin lymphoma. [academic.oup.com]

  lymphoma. 1 It is clear that our understanding of MGUS has improved tremendously throughout the years, but were it not for Dr. [hematology.org]

  Keywords Diffuse large B-cell lymphoma Monoclonal gammopathy Serum free light chain Lenalidomide Background Diffuse large B-cell lymphoma (DLBCL) is the most common B-cell non-Hodgkin lymphoma (NHL), accounting for about 30 % of all new diagnoses [ 1 [ehoonline.biomedcentral.com]

  Long-term follow-up of 213 patients with IgM MGUS showed the development of non-Hodgkin lymphoma (NHL) in 17 patients, WM in six patients, CLL in three patients, and light-chain (AL) amyloidosis in three patients. [f1000research.com]

• Weight Loss

  A female patient, 67 years old, was admitted with dyspnoea, malaise, weight loss, and disseminated radiological lesions in the lungs. [ncbi.nlm.nih.gov]

  When to Test for MGUS Symptomatic Patients In general, we test for the presence of monoclonal gammopathy in patients who have clinical symptoms and signs of the presence of MM, AL amyloidosis, or WM (i.e., bone pain, unexplained weight loss, hyperviscosity [ashclinicalnews.org]

  His weight loss and fatigue have resolved and his presenting symptoms were attributable primarily to his chronic alcohol abuse, anemia of chronic disease, and refractory hypertension. [path.upmc.edu]

  Your doctor is likely to watch for the development of signs and symptoms including: Pain Fatigue or weakness Unintentional weight loss Fever or night sweats Headache, dizziness, nerve pain, or changes in vision or hearing Bleeding Anemia or other blood [drugs.com]

  The disease can present with fatigue, weight loss, edema, malabsorption, neuropathy, macroglossia, skin fragility, or periorbital purpura, and more severe symptoms include nephrosis, cardiomyopathy, autonomic insufficiency, and pulmonary failure with [consultant360.com]

• Diarrhea

  Major clinical manifestations include heart failure, hepatosplenomegaly, nephrotic syndrome, macroglossia, carpal tunnel syndrome, diarrhea, sensorimotor neuropathy, and autonomic neuropathy. [haematologica.org]

  An anemia (hemoglobin concentration of less than 11 g/dl) was found in 17 patients with MGUS—due to metastatic carcinoma in 4, gastrointestinal bleeding in 3, myelodysplastic syndrome in 2, and agnogenic myeloid metaplasia, chronic diarrhea, renal insufficiency [mayoclinicproceedings.org]

• Hepatosplenomegaly

  In the course of his stay, the hepatosplenomegaly regressed, his serum globulins had fallen (total proteins-7.2 g/dL, albumin-3.7 g/dL, globulin-3.5 g/dL). [scielo.br]

  An increased frequency of hepatosplenomegaly and lymphadenopathy is expected in LP but not in MGUS. In the MGUS group, however, the presence of hepatosplenomegaly was explained by causes other than lymphoproliferation. [mayoclinicproceedings.org]

  Patients may also have hepatosplenomegaly or excessive bleeding/bruising. [cancertherapyadvisor.com]

  When symptoms are present, they can include fatigue and drowsiness from severe anemia, hepatosplenomegaly, enlarged lymph nodes, Raynaud’s disease, hyperviscosity, and neuropathy. Unlike multiple myeloma, there are no bone lesions. [consultant360.com]

• Macroglossia

  Some patients also show purpura and macroglossia. 4 The exact mechanism of disease is not fully realized, but it may involve certain translocations of the IgH gene, which is seen in a majority of patients primary amyloidosis. 4 Monoclonal Gammopathy of [path.upmc.edu]

  It can also cause renal insufficiency, but other clinical features include macroglossia and restrictive cardiomyopathy. [cancertherapyadvisor.com]

  The disease can present with fatigue, weight loss, edema, malabsorption, neuropathy, macroglossia, skin fragility, or periorbital purpura, and more severe symptoms include nephrosis, cardiomyopathy, autonomic insufficiency, and pulmonary failure with [consultant360.com]

  However, with the exception of diffuse lytic bone lesions, macroglossia, infiltrative cardiomyopathy, and engorgement of retinal veins, most presenting symptoms and signs of LPMs are nonspecific. [bloodjournal.org]

• Skin Rash

  Top ↑ Case Presentation A 76-year-old Japanese man presented to our hospital with persistent fever and skin rash on both forearms which emerged one week earlier. [elynsgroup.com]

  6p21.33, 7p15.3, 17p11.2 and 22q13.1 ( Blood 2014;123:2513 ) Clinical features Most patients are asymptomatic; usually diagnosed as an incidental finding on protein electrophoresis performed to evaluate peripheral neuropathy, vasculitis, hemolytic anemia, skin [pathologyoutlines.com]

  It is commonly found by primary care physicians while investigating a broad-spectrum of symptoms and disorders such as fatigue, vasculitis, hemolytic anemia, arthralgias, neuropathies, and skin rashes. [cancertherapyadvisor.com]

  Terpos E, Asli B, Christoulas D, et al. : Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome. [f1000research.com]

• Skin Lesion

  The more nodular skin lesions demonstrated changes consistent with erythema nodosum. [omicsonline.org]

  Extensive skin lesions with livedo reticularis entirely disappeared prior to initiation of the second cycle in association with the declined serum level of IgM. [intechopen.com]

• Night Sweats

  Your doctor is likely to watch for the development of signs and symptoms including: Pain Fatigue or weakness Unintentional weight loss Fever or night sweats Headache, dizziness, nerve pain, or changes in vision or hearing Bleeding Anemia or other blood [drugs.com]

  Patients with constitutional symptoms including fever, night sweats, and unintentional weight loss, in the presence of lymphadenopathy and/or hepatosplenomegaly suggest lymphoma. [cancertherapyadvisor.com]

• Fracture

  The risk was significantly higher for axial (skull, vertebral/pelvis, and sternum/costae) compared with distal (arm and leg) fractures ( P < 0.001). [emedicine.medscape.com]

  Osteolytic foci in the skull are usually diagnostic for MM, but vertebral fracture may also occur in osteopenia. It is evident that a period of at least three years of close observation without treatment should be the routine. [ncbi.nlm.nih.gov]

  People with this disorder also are more likely to have bone loss and fractures. The M-protein levels in people with MGUS often remain stable for years—25 years in some people—and do not require treatment. [merckmanuals.com]

  […] year about 1% of people with MGUS go on to develop certain types of blood cancers or other serious diseases such as: Multiple myeloma Light chain amyloidosis Waldenstrom macroglobulinemia Lymphoma Other complications associated with MGUS include bone fractures [mayoclinic.org]

• Bone Pain

  When to Test for MGUS Symptomatic Patients In general, we test for the presence of monoclonal gammopathy in patients who have clinical symptoms and signs of the presence of MM, AL amyloidosis, or WM (i.e., bone pain, unexplained weight loss, hyperviscosity [ashclinicalnews.org]

  Patients with MGUS should watch for any symptoms, such as bone pain and fatigue, as they might indicate progression to myeloma. [cancer.uams.edu]

  It is diagnosed by the combination of SPEP findings and bone marrow biopsy showing greater than 10% of plasma cells (expressing CD38 and CD138), along with predominant clinical symptoms of bone pain/fracture (from lytic bone lesions), fatigue (from anemia [path.upmc.edu]

  Presence of symptoms, such as bone pain or recurrent infections, blood test abnormalities such as anaemia, raised calcium level, raised bone marrow plasma cell numbers or kidney failure, or x-ray abnormalities within the bones, means the diagnosis is [melbournehaematology.com.au]

  If you are experiencing bone pain, your doctor might recommend an MRI or CT-positron emission tomography (PET) scan. The images can help your doctor find bone abnormalities related to MGUS. Bone marrow test. [drugs.com]

• Arthralgia

  However, his fever, arthralgias and fatigue recurred when the dose of prednisone was below 20 mg/day. A thorough malignancy work-up was completed and was unremarkable. [omicsonline.org]

  It is commonly found by primary care physicians while investigating a broad-spectrum of symptoms and disorders such as fatigue, vasculitis, hemolytic anemia, arthralgias, neuropathies, and skin rashes. [cancertherapyadvisor.com]

  IgM (8% ref -15% ref -17.2% ref -24% ref ) Schnitzler's syndrome (SS), initially described in 1974 (around 70 cases reported to date ref ), is a rare clinical condition characterized by chronic urticaria, intermittent fever, elevated ESR, bone pain, arthralgia [ufrgs.br]

  […] complement activation. 84, 85 In Schnitzler syndrome there is deposition of para-protein (IgM-κ is most common) at the dermoepidermal junction and in capillary walls which probably contributes to development of chronic urticaria, intermittent fever, arthralgia [haematologica.org]

• Renal Injury

  First, the electrophoretic pattern is helpful in distinguishing the type of renal injury in patients with M gammopathy or MM. [degruyter.com]

• Peripheral Neuropathy

  […] doi: 10.1097/WCO.0b013e32832fd563 Nerve, neuro-muscular junction and motor neuron diseases: Edited by Gerard Said Abstract Author Information Authors Article Metrics Metrics The management of peripheral neuropathy associated with monoclonal gammopathies [journals.lww.com]

  […] syndromes with autoantibody activity against peripheral nerve glycoproteins, such as myelin-associated glycoprotein (MAG), are generally IgM forms. • Monoclonal gammopathy-associated peripheral neuropathy is potentially treatable; however, the preferred [medlink.com]

  MGUS usually is asymptomatic, but peripheral neuropathy can occur. Common symptoms reported by people with monoclonal gammopathy of undetermined significance Reports may be affected by other conditions and/or medication side effects. [patientslikeme.com]

  Moderately elevated levels of this protein in the blood rarely result in symptoms, but some people with MGUS may experience peripheral neuropathy — pain or burning in the hands and feet. MGUS has been linked to exposure to certain chemicals. [mskcc.org]

  The peripheral neuropathy associated with IgM MGUS usually presents as a distal, symmetric, and slowly progressive sensorimotor neuropathy with predominantly demyelinating features in the nerve conduction studies. [f1000research.com]

• Polyneuropathy

  […] antibody associated polyneuropathy syndrome. [medlink.com]

  Echocardiography revealed severe pulmonary hypertension, and electromyography revealed sensoromotoric polyneuropathy with axon and myelin damage. [ncbi.nlm.nih.gov]

  There is a clear association between peripheral neuropathy and IgM MGUS with characteristic clinical, electrophysiology and pathologic features that make the disorder distinct from chronic inflammatory demyelinating polyneuropathy. [journals.lww.com]

  We identified 36 consecutive patients who had no evidence of a haematological malignancy (multiple myeloma, osteosclerotic myeloma, Waldenstrom’s macroglobulinaemia, amyloidosis, or lymphoma) or other diseases that might have explained the polyneuropathy [jnnp.bmj.com]

  POEMS is an acronym for the key features of the disease -Polyneuropathy (such as chronic inflammatory demyelinating polyneuropathy), organomegaly (hepatosplenomegaly or less often lymphadenopathy), endocrinopathies (such as hypothyroidism or hypogonadism [cancertherapyadvisor.com]

Complete hematologic workup with serum and urine protein electrophoresis, immunofixation, and serum-free light-chain assay is required. [theisn.org]

A kappa-lambda ratio below 0.37 or above 3 is rarely seen in chronic kidney disease and should prompt workup for MGUS. 16 Tests in combination. [mdedge.com]

Consensus recommendations for standard investigative workup: report of the International Myeloma Workshop Consensus Panel 3. Blood 2011;117:4701–5. Google Scholar 7. [degruyter.com]

Thus, renal biopsy along with detailed hematologic workup is essential to link the presence of the paraprotein to the associated renal disease. [cjasn.asnjournals.org]

BM examination should be part of the diagnostic workup for all IgA and IgM M-proteins. [haematologica.org]

[…] service, product or treatment in your circumstances. [leukaemia.org.au]

It is evident that a period of at least three years of close observation without treatment should be the routine. "Prophylactic" treatment of BMG with cytostatic drugs should never be attempted. [ncbi.nlm.nih.gov]

Those who failed to improve with the initial treatment received an alternative second or third treatment at the discretion of the treating physician. [jnnp.bmj.com]

Conclusions : Exudative maculopathy unresponsive to conventional treatment may suggest an underlying hematologic condition. [iovs.arvojournals.org]

Characterization of primary systemic amyloidosis, clinical manifestations and disease prognosis. [cima.unav.edu]

In addition, serum FLC measurements have shown utility in determining the prognosis of and in detecting and monitoring light chain amyloidosis. [aacc.org]

A long-term study of prognosis in monoclonal gammopathy of undetermined significance. N Engl J Med 2002;346:564–9. Google Scholar 5. Dispenzieri A, Katzmann JA, Kyle RA, Larson DR, Melton LJ 3rd, Colby CL, et al. [degruyter.com]

Monoclonal gammopathy is identified in 10% of patients with idiopathic peripheral neuropathy, and the presence of a monoclonal protein may have significant implications regarding the etiology, progression, and prognosis of the neuropathy. [medlink.com]

Prognosis MGUS is generally considered benign and has no ill effects on overall health or survival. However, regular check-ups are essential, since myeloma typically develops in 1% to 2% of MGUS patients per year. [cancer.uams.edu]

Monoclonal gammopathy is identified in 10% of patients with idiopathic peripheral neuropathy, and the presence of a monoclonal protein may have significant implications regarding the etiology, progression, and prognosis of the neuropathy. [medlink.com]

Only a few case reports in the past have implicated visceral leishmaniasis as an etiology of monoclonal gammopathy. 2-4 In one case, the patient had a monoclonal triple band. 2 In few cases BMA did not reveal LD bodies, and antileishmanial serology was [scielo.br]

Etiologic Factors for MGUS and Prospects for Primary Prevention Recent observations offer important clues, indicating that genetic predisposition and potentially preventable environmental factors may play a key role in the development of MGUS. [clincancerres.aacrjournals.org]

Repeat CT of his chest revealed a new right lower lobe consolidation abutting the diaphragm with surrounding ground-glass opacity most consistent with pneumonia and two new lung nodules that were likely infectious in etiology. [omicsonline.org]

[…] include IgH translocation, hyperdiploidy and cyclin D dysregulation Secondary genetic events include: NRAS, KRAS and BRAF mutations NFkB pathway mutations TP53, PTEN and RB inactivation Most non-IgM MGUS cases are IgG, followed by IgA, biclonal and IgD Etiology [pathologyoutlines.com]

“Monoclonal gammopathy of undetermined significance and smoldering myeloma: new insights into pathophysiology and epidemiology”. Hematology Am Soc Hematol Educ Program. vol. 2010. 2010. pp. 295-302. [cancertherapyadvisor.com]

History of the Rochester Epidemiology Project. Mayo Clin Proc 1996 ;71: 266 - 274 16. Kyle RA, Katzmann JA, Lust JA, Dispenzieri A. Immunochemical characterization of immunoglobulins. In: Rose NR, Hamilton RG, Detrik B, eds. [nejm.org]

Detailed classification of MGUS: A roadmap for monitoring patients Extensive epidemiologic and clinical studies have refined the classification of MGUS 3-5 and related disorders ( TABLES 2-4 ), 3 providing physicians with guidance on how to monitor patients [mdedge.com]

J Clin Pathol 35:63–68 PubMed CrossRef Google Scholar Salmon SE, Kyle RA (1994) Silicone gels, induction of plasma cell tumors, and genetic susceptibility in mice: a call for epidemiologic investigation of women with silicone breast implants. [link.springer.com]

"Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma: a review of the current understanding of epidemiology, biology, risk stratification, and management of myeloma precursor disease". [en.wikipedia.org]

PMID: 31262628 Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Michiels JJ, Budde U, van der Planken M, van Vliet HH, Schroyens W, Berneman Z. Michiels JJ, et al. [pubmed.ncbi.nlm.nih.gov]

“Monoclonal gammopathy of undetermined significance and smoldering myeloma: new insights into pathophysiology and epidemiology”. Hematology Am Soc Hematol Educ Program. vol. 2010. 2010. pp. 295-302. [cancertherapyadvisor.com]

More prevalent in African Americans than Caucasians (2 - 3 fold) ( Leukemia 2014;28:1537 ) More common in men than women (1.5:1) Non-IgM MGUS represents up to 85% of MGUS cases Pathophysiology Origin is clonal mature plasma cells residing in the bone [pathologyoutlines.com]

[…] numbness or tingling. [1] Severe renal disease has also been found in a subset of those with monoclonal gammopathy. [1] MGUS is usually detected by chance when the patient has a blood test for another condition or as part of standard screening. [1] Pathophysiology [en.wikipedia.org]

Pathophysiology The reason for the monoclonal expansion of a single Ig-secreting plasma cell population in what appears to be a nonmalignant manner is unknown in most cases. Most cases involve IgG or IgA monoclonal cell populations. [emedicine.medscape.com]

Etiologic Factors for MGUS and Prospects for Primary Prevention Recent observations offer important clues, indicating that genetic predisposition and potentially preventable environmental factors may play a key role in the development of MGUS. [clincancerres.aacrjournals.org]

This progression cannot be prevented. [merckmanuals.com]

In some cases, treatment aimed at preventing progression to active myeloma is indicated. Prognosis MGUS is generally considered benign and has no ill effects on overall health or survival. [cancer.uams.edu]

Blood disorders affect one or more parts of the blood and prevent your blood from doing its job. They can be acute or chronic. Many blood disorders are inherited. [icdlist.com]

Asymptomatic Patients Practice guidelines do not recommend routine screening for MGUS in the general population because of the lack of proven benefit and the absence of curative or preventive therapy. [ashclinicalnews.org]