Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate. The episodes each lasted for over one week. Neutrophilic and eosinophilic inflammation was found on skin biopsy. However, serum and urine electrophoresis showed no evidence of monoclonal gammopathy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, and methotrexate, which were administered over a span of 3 years immediately preceding successful treatment. A dramatic response, however, was observed after a daily administration of anakinra. This observation suggests that the correct diagnosis of this case is Schnitzler's syndrome without monoclonal gammopathy. For an adult patient with refractory chronic urticaria and systemic inflammation, Schnitzler's syndrome could be considered as a possible differential diagnosis. Although the typical form of Schnitzler's syndrome exhibits the presence of monoclonal gammopathy as a diagnostic criterion, monoclonal gammopathy may be absent in an atypical form. In such a situation, an IL-1 antagonist should be effective for the management of chronic urticaria.
Aged ; Blood Sedimentation ; C-Reactive Protein/metabolism ; Chronic Disease ; Humans ; Interleukin 1 Receptor Antagonist Protein/therapeutic use ; Leukocytes/metabolism ; Male ; Paraproteinemias/complications ; Schnitzler Syndrome/blood ; Schnitzler Syndrome/drug therapy ; Urticaria/complications

No abstract available.
Aged ; Humans ; Leukemia, Plasma Cell/complications/*diagnosis/pathology ; Leukocytosis ; Lymph Nodes/pathology ; Lymphoma, T-Cell/complications/*diagnosis/pathology ; Male ; Paraproteinemias/complications ; Polymerase Chain Reaction ; Receptors, Antigen, T-Cell, gamma-delta/genetics/metabolism ; Tomography, X-Ray Computed

No abstract available.
Blood Cell Count ; Bone Marrow Cells/metabolism/pathology ; Humans ; Leukemia, Promyelocytic, Acute/complications/*diagnosis/pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multiple Myeloma/complications/*diagnosis/pathology ; Paraproteinemias/diagnosis ; Syndecan-1/metabolism

We hereby report a case of diffuse pelvic peritoneal involvement by immunoglobulin G4-related disease (IgG4-RD). Numerous pelvic masses and nodules showing delayed enhancement on enhanced abdominal CT were found to congregate in the pelvic organs of a 57-year-old female presenting with intestinal subocclusion. The differentiation between peritoneal IgG4-RD and pelvic peritoneal carcinomatosis was only made by histopathology and immunohistochemistry performed after surgical resection. Autoimmune pancreatitis represents the historical prototype of IgG4-RD, but the spectrum of manifestations involving various organs has expanded during the last decade. In this report, we shortly review this clinical entity.
Carcinoma/diagnosis ; Female ; Humans ; *Immunoglobulin G ; Immunohistochemistry ; Intestinal Obstruction/*etiology ; *Intestine, Small ; Middle Aged ; Paraproteinemias/*complications/diagnosis/pathology ; Peritoneal Diseases/*complications/diagnosis/pathology ; Peritoneal Neoplasms/diagnosis

Monoclonal gammopathy occurs in one-third of the patients with mucosa-associated lymphoid tissue lymphoma (MALT lymphoma). However, monoclonal gammopathy has been rarely reported in Korea. Paraprotenemia accompanying MALT lymphoma is strongly correlated with involvement of the bone marrow, and this involvement leads to the progression of the disease. Here, we present a case of a 66-yr-old man diagnosed with IgM monoclonal gammopathy and stage IV extranodal marginal zone lymphoma of the small intestine, with the involvement of the bone marrow.
Aged ; Antineoplastic Agents/therapeutic use ; Bone Marrow/pathology ; Drug Therapy, Combination ; Electrophoresis, Polyacrylamide Gel ; Humans ; Immunoglobulin M/analysis ; Intestinal Neoplasms/complications/drug therapy/*pathology ; Lymphatic Metastasis ; Lymphoma, B-Cell, Marginal Zone/complications/drug therapy/*pathology ; Male ; Neoplasm Staging ; Paraproteinemias/blood/complications/*pathology ; Positron-Emission Tomography ; Tomography, X-Ray Computed

A 68-year-old woman presented with pain in her left eye. Necrosis with calcium plaques was observed on the medial part of the sclera. Aspergillus fumigatus was isolated from the culture of the necrotic area. On systemic work-up including serum and urine electrophoresis studies, the serum monoclonal protein of immunoglobulin G was detected. The patient was diagnosed with monoclonal gammopathy of undetermined significance and fungal scleritis. Despite intensive treatment with topical and oral antifungal agents, scleral inflammation and ulceration progressed, and scleral perforation and endophthalmitis developed. Debridement, antifungal irrigation, and tectonic scleral grafting were performed. The patient underwent a combined pars plana vitrectomy with an intravitreal injection of an antifungal agent. However, scleral and intraocular inflammation progressed, and the eye was enucleated. Aspergillus fumigatus was isolated from the cultures of the eviscerated materials. Giemsa staining of the excised sclera showed numerous fungal hyphae.
Aged ; Amphotericin B/administration & dosage ; Antifungal Agents/administration & dosage ; *Aspergillosis/therapy ; *Aspergillus fumigatus ; Disease Progression ; Eye Enucleation ; Female ; Humans ; Injections, Intraocular ; Paraproteinemias/*complications ; Sclera/pathology/ultrasonography ; Scleritis/*complications/diagnosis/*microbiology/physiopathology ; Vitrectomy

An 8-year-old male Austrian Pinscher and a 14-year-old male Golden Retriever were presented for evaluation due to unexplainable high fructosamine values despite euglycemia and epistaxis in combination with polydipsia/polyuria, respectively. Blood analysis revealed severe hyperglobulinemia, hypoalbuminemia and markedly elevated fructosamine concentrations in both dogs. Multiple myeloma with IgA-monoclonal gammopathy was diagnosed by serum and urine electrophoresis including immunodetection with an anti-dog IgA antibody and bone marrow aspirations. Diabetes mellitus was excluded by repeated plasma and urine glucose measurements. Fructosamine values were positively correlated with globulin, but negatively correlated with albumin concentrations. These cases suggest that, as in human patients, monoclonal IgA gammopathy should be considered as a possible differential diagnosis for dogs with high fructosamine concentrations.
Animals ; Blood Proteins/analysis ; Dog Diseases/*blood/drug therapy ; Dogs ; Fructosamine/*blood ; Immunoglobulin A/*metabolism ; Male ; Melphalan/therapeutic use ; Multiple Myeloma/complications/drug therapy/*veterinary ; Myeloablative Agonists/therapeutic use ; Paraproteinemias/blood/complications/drug therapy/*veterinary

Unexpectedly high rates of venous thromboembolic events (VTE) induced by highly effective immune modulating drugs thalidomide and lenalidomide for treatment of multiple myeloma have focused attention on the incidence and underlying pathophysiology of VTE in patients with plasma cell dyscrasias, and on thromboprophylaxis approaches. While bleeding complications are relatively uncommon in the patients with lymphoproliferative disorders, acquired von Willebrand syndrome, typically occurring in the patients with monoclonal gammopathy of unknown significance, and acquired coagulopathies associated with primary amyloidosis can present with haemorrhagic complications and both are challenges to the management. This review highlights these important haemostasis-related complications of plasma cell dyscrasias and provides an overview of other uncommon bleeding and thrombotic events that can affect diagnosis and therapeutic management of clonal plasma cell disorders. Due to the infrequency of most these haemostasis complications, available information is typically based on retrospective cases or series analysis.
Hemorrhage ; etiology ; pathology ; therapy ; Humans ; Paraproteinemias ; complications ; pathology ; Thrombosis ; etiology ; pathology ; prevention & control

We report a case of light chain deposition disease in a 59-yr-old female showing deposition of monoclonal light chain in the kidney and bone marrow accompanied with a schistocytosis, the morphologic finding of microangiopathic hemolytic anemia. The immunofluorescence examination of the kidney revealed strongly stained kappa-light chain deposits on the glomerular mesangium and capillary wall, tubules, and vessel wall. The electron microscopy demonstrated electron-dense deposits on the glomerular basement membrane and mesangium. Anemia was observed with schistocytosis and Howell-Jolly body in the peripheral blood smears. The immunohistochemical examination of the bone marrow showed the presence of kappa-light chain deposits in scattered plasma cells and thickened vessel wall in the absence of a prominent plasma cell proliferation. Although an immunofixation electrophoresis failed to detect a monoclonal gammopathy, the presence of monoclonal protein could be identified by an abnormal kappa/lambda ratio on the serum free light chain analysis.
Anemia, Hemolytic/complications/*diagnosis ; Bone Marrow/*pathology ; Female ; Glomerulonephritis/complications/*diagnosis/pathology ; Humans ; Immunoglobulin Light Chains/*analysis ; Kidney Glomerulus/*pathology/ultrastructure ; Middle Aged ; Paraproteinemias/complications/*diagnosis/immunology

The monoclonal gammopathies (MG) are monoclonal neoplasms related to each other by virtue of their development from common progenitors in the B lymphocyte lineage. Cardiac dysfunction in patients with MG is not well established. We experienced a case of cardiac dysfunction associated with MG identified by echocardiography and biopsy. Fifty nine year-old man was admitted because of dyspnea for several months. Echocardiography revealed diastolic dysfunction showing restrictive physiology with elevated left ventricular filling pressure. Bone marrow (BM) studies and immunoelectrophoresis were compatible with monoclonal gammopathy of undetermined significance. Endomyocardial, BM, and enteral biopsies for ruling out for amyloidosis (Congo-red stain) were negative. This is the case of non-amyloidotic light chain deposition cardiomyopathy.
Bone Marrow/pathology ; Cardiomyopathies/diagnosis/*etiology/ultrasonography ; Humans ; Immunoglobulin kappa-Chains/analysis ; Male ; Middle Aged ; Paraproteinemias/*complications/pathology