OBJECTIVE: To study the clinical and laboratory characteristics of monoclonal gammopathy anemia and explore the risk factors associated with anemia in monoclonal gammopathy. METHODS: A retrospective analysis was conducted on 5 539 patients who underwent immunofixation electrophoresis at the First Affiliated Hospital of Chengdu Medical College from January 2016 to February 2024. A total of 351 newly diagnosed M protein positive patients were selected as the study subjects, including 270 in the anemia group and 81 in the non-anemia group. Laboratory test results were compared between the two groups, and logistic regression models were used to analyze the risk factors for anemia. ROC curve analysis was performed to evaluate the predictive value of risk factors for anemia in monoclonal gammopathy. RESULTS: The proportion of non-anemic patients was 23.1% (81/351), with a median age of 67(60-75) years; the proportion of anemic patients was 76.9% (270/351), with a median age of 70(63-75) years. The total protein, globulin, urea, creatinine, uric acid, β₂-microglobulin, and ceruloplasmin levels in the anemia group were higher than those in the non-anemia group ( P < 0.05), while albumin, neutrophil count, lymphocyte count, monocyte count, complement C3, complement C4, haptoglobin, and transferrin levels were lower in the non-anemia group ( P < 0.05). After adjustment, multivariate logistic regression analysis shows that elevated GLB, increased β₂-MG, decreased ANC, and reduced complement C3 were independent risk factors for anemia in monoclonal gammopathy ( P < 0.05). ROC curve analysis demonstrates that GLB, β₂-MG, ANC, and complement C3 had good predictive value for anemia associated with monoclonal gammopathy. CONCLUSION Elevated GLB, increased β₂-MG, decreased ANC, and reduced complement C3 are independent risk factors for anemia in monoclonal gammopathy (P < 0.05). The combined assessment of these four factors has good predictive value for anemia in monoclonal gammopathy.
Humans ; Retrospective Studies ; Anemia/complications* ; Aged ; Middle Aged ; Paraproteinemias/diagnosis* ; Risk Factors ; Male ; Female ; Logistic Models ; ROC Curve ; Complement C3

Monoclonal gammopathy of undetermined significance combined with renal damage is named monoclonal gammopathy of renal significance. There are few reports about IgA vasculitis in patients with monoclonal gammopathy of undetermined significance. Here, we report a case of monoclonal gammopathy of renal significance, who had manifestations of IgA vasculitis, including purpura, gastrointestinal bleeding and joint pain. The patient had elevated serum creatinine levels, prompting further investigation through immunofixation electrophoresis and bone marrow aspiration biopsy. Immunofixation electrophoresis showed IgA-λ-type monoclonal immunoglobulin, while the bone marrow aspiration biopsy suggested plasmacytosis. Kidney biopsy indicated membranous hyperplastic glomerulonephritis, light and heavy chain deposition, IgA-λ. The patient was diagnosed with monoclonal gammopathy of renal significance. In light of the elevated serum creatinine, the patient was treated with chemotherapy regimen (bortezomib +cyclophosphamide +dexamethasone). After chemotherapy, there was no significant improvement in the patient's renal function. Subsequently, the patient experienced abdominal pain, skin purpura, joint pain and severe gastrointestinal bleeding. Gastroenteroscopy did not find the exact bleeding position. Angiography revealed hyperplasia of left jejunal artery. Surgical operation found that the bleeding site was located between the jejunum and ileum, where scattered hemorrhagic spots and multiple ulcers were present on the surface of the small intestine, with the deepest ulcers reaching the serosal layer. And the damaged intestine was removed during the operation. Intestinal pathology showed multiple intestinal submucosal arteritis, rusulting in intestinal wall necrosis and multiple ulcers. Considering intestinal lesions as gastrointestinal involvement of IgA vasculitis, methylprednisolone was used continually after the operation, and the patient's condition was improved. However, after half a year, the patient suffered a severe respiratory infection and experienced a recurrence of serious gastrointestinal bleeding. It was considered that the infection triggered the activity of IgA vasculitis, accompanied by gastrointestinal involvement. Finally, the patient died from gastrointestinal bleeding. The present case represented a patient with monoclonal gammopathy of renal significance and IgA vasculitis, prominently presenting with renal insufficiency and severe gastrointestinal bleeding, making the diagnosis and treatment process complex. Patients with IgA monoclonal gammopathy who presented with abdominal pain, purpura, and arthralgia should be vigilant for the possibility of concomitant IgA vasculitis. The treatment of cases with IgA vasculitis combined with monoclonal gammopathy of renal significance was rather challenging. Plasma cell targeting therapy might be an effective regimen for IgA vasculitis with monoclonal gammopathy. However, patients with poor renal response to the treatment indicated poor prognosis.
Humans ; Cyclophosphamide/administration & dosage* ; Gastrointestinal Hemorrhage/etiology* ; IgA Vasculitis/complications* ; Immunoglobulin A ; Intestine, Small/pathology* ; Kidney/pathology* ; Kidney Diseases/pathology* ; Monoclonal Gammopathy of Undetermined Significance/complications* ; Necrosis ; Paraproteinemias/complications* ; Vasculitis/etiology*

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

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

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

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

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

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