1.Pathogenesis and management of hemorrhage and thrombosis in plasma cell dyscrasias.
Journal of Experimental Hematology 2010;18(3):809-815
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
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etiology
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pathology
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therapy
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Humans
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Paraproteinemias
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complications
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pathology
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Thrombosis
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etiology
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pathology
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prevention & control
2.A Case of Cardiac Dysfunction Associated with Monoclonal Gammopathy of Undetermined Significance.
Suk Tae JANG ; Il Suk SOHN ; Eun Sun JIN ; Jin Man CHO ; Chong Jin KIM ; Sung Jig LIM
Journal of Korean Medical Science 2009;24(2):354-356
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
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Cardiomyopathies/diagnosis/*etiology/ultrasonography
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Humans
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Immunoglobulin kappa-Chains/analysis
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Male
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Middle Aged
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Paraproteinemias/*complications/pathology
3.A Case of Light Chain Deposition Disease Involving Kidney and Bone Marrow with Microangiopathic Hemolytic Anemia.
Young Uk CHO ; Hyun Sook CHI ; Chan Jeoung PARK ; Seongsoo JANG ; Yong Mee CHO ; Jung Sik PARK
The Korean Journal of Laboratory Medicine 2009;29(5):384-389
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
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Bone Marrow/*pathology
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Female
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Glomerulonephritis/complications/*diagnosis/pathology
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Humans
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Immunoglobulin Light Chains/*analysis
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Kidney Glomerulus/*pathology/ultrastructure
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Middle Aged
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Paraproteinemias/complications/*diagnosis/immunology
4.Widespread Scleredema Accompanied with a Monoclonal Gammopathy in a Patient with Advanced Ankylosing Spondylitis.
Hyun Kyu CHANG ; You Chan KIM ; Bum Sun KWON
Journal of Korean Medical Science 2004;19(3):481-483
Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.
Adult
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Collagen/metabolism
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Human
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Immunoglobulins, kappa-Chain/chemistry
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Inflammation
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Lumbar Vertebrae/radiography
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Male
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Mucins/metabolism
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Paraproteinemias/*complications/diagnosis
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Scleredema Adultorum/*complications/diagnosis
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Skin/pathology
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Spondylitis, Ankylosing/*complications/diagnosis
5.Small Bowel Obstruction Caused by Peritoneal Immunoglobulin G4-Related Disease Mimicking Carcinomatosis: Case Report.
Bruno COULIER ; Luc MONTFORT ; Gabriela BENIUGA ; Frederic PIERARD ; Isabelle GIELEN
Korean Journal of Radiology 2014;15(1):66-71
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
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Female
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Humans
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*Immunoglobulin G
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Immunohistochemistry
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Intestinal Obstruction/*etiology
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*Intestine, Small
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Middle Aged
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Paraproteinemias/*complications/diagnosis/pathology
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Peritoneal Diseases/*complications/diagnosis/pathology
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Peritoneal Neoplasms/diagnosis
6.Simultaneous Occurrence of Angioimmunoblastic T-cell Lymphoma and Plasma Cell Leukemia.
Mi Ae JANG ; Seung Tae LEE ; Hee Jin KIM ; Seokjin KIM ; Sun Hee KIM
Annals of Laboratory Medicine 2015;35(1):149-151
No abstract available.
Aged
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Humans
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Leukemia, Plasma Cell/complications/*diagnosis/pathology
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Leukocytosis
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Lymph Nodes/pathology
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Lymphoma, T-Cell/complications/*diagnosis/pathology
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Male
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Paraproteinemias/complications
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Polymerase Chain Reaction
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Receptors, Antigen, T-Cell, gamma-delta/genetics/metabolism
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Tomography, X-Ray Computed
7.A Case of Acute Promyelocytic Leukemia Concomitant with Plasma Cell Myeloma.
Jinsook LIM ; Gye Cheol KWON ; Sun Hoe KOO ; Ik Chan SONG ; Jimyung KIM
Annals of Laboratory Medicine 2014;34(2):152-154
No abstract available.
Blood Cell Count
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Bone Marrow Cells/metabolism/pathology
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Humans
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Leukemia, Promyelocytic, Acute/complications/*diagnosis/pathology
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Magnetic Resonance Imaging
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Male
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Middle Aged
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Multiple Myeloma/complications/*diagnosis/pathology
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Paraproteinemias/diagnosis
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Syndecan-1/metabolism
8.Aspergillus fumigatus Scleritis Associated with Monoclonal Gammopathy of Undetermined Significance.
Dong Hyun JO ; Joo Youn OH ; Mee Kum KIM ; Jang Won HEO ; Jin Hak LEE ; Won Ryang WEE
Korean Journal of Ophthalmology 2010;24(3):175-178
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
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Amphotericin B/administration & dosage
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Antifungal Agents/administration & dosage
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*Aspergillosis/therapy
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*Aspergillus fumigatus
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Disease Progression
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Eye Enucleation
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Female
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Humans
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Injections, Intraocular
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Paraproteinemias/*complications
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Sclera/pathology/ultrasonography
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Scleritis/*complications/diagnosis/*microbiology/physiopathology
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Vitrectomy
9.Incidence and Type of Monoclonal or Biclonal Gammopathies in Scrub Typhus.
The Korean Journal of Laboratory Medicine 2009;29(2):116-121
BACKGROUND: Korea is an endemic area of scrub typhus and it is a common seasonal febrile illness. Although, various humoral immune responses to scrub typhus have been documented, no association between gammopathy and scrub typhus has ever been reported. We analyzed the incidences and types of monoclonal and biclonal gammopathies in scrub typhus for better coping with those gammopathies in scrub typhus. METHODS: Anti-Orientia tsutsugamushi antibody-positive sera identified by indirect immunofluorescence assay were acquired from 40 patients with confirmed scrub typhus. Monoclonal and biclonal gammopathies were screened by protein electrophoresis and were confirmed using immunofixation electrophoresis (IFE). Laboratory findings on admission of the patients with monoclonal or biclonal gammopathy were investigated retrospectively to characterize the gammopathies. RESULTS: Monoclonal or biclonal gammopathies were detected in 30% (12/40) of patients with scrub typhus (IgG-lambda, 40%; IgG-kappa, 30%; IgM-kappa, 10%; IgM-lambda, 10%; IgA-kappa, 5%; IgA-lambda, 5%). Concentrations of clonal immunoglobulin were less than 3 g/dL in all gammopathies, and hypercalcemia was not detected in any of the patients. CONCLUSIONS: Our results suggest possible association between gammopathies and scrub typhus. Further studies in larger series will be needed for exact incidence and clinical course of gammopathies in scrub typhus.
Adult
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Aged
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Humans
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Immunoglobulin A/blood
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Immunoglobulin G/blood
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Immunoglobulin Heavy Chains/blood
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Immunoglobulin Light Chains/blood
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Immunoglobulin M/blood
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Incidence
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Middle Aged
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Orientia tsutsugamushi/immunology
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Paraproteinemias/complications/*diagnosis/epidemiology/immunology
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Retrospective Studies
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Scrub Typhus/complications/*diagnosis/immunology
10.Elevated fructosamine concentrations caused by IgA paraproteinemia in two dogs.
Florian ZEUGSWETTER ; Miriam KLEITER ; Birgitt WOLFESBERGER ; Ilse SCHWENDENWEIN ; Ingrid MILLER
Journal of Veterinary Science 2010;11(4):359-361
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
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Blood Proteins/analysis
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Dog Diseases/*blood/drug therapy
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Dogs
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Fructosamine/*blood
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Immunoglobulin A/*metabolism
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Male
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Melphalan/therapeutic use
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Multiple Myeloma/complications/drug therapy/*veterinary
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Myeloablative Agonists/therapeutic use
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Paraproteinemias/blood/complications/drug therapy/*veterinary