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

Humans ; Immunoglobulin Light Chains ; blood ; chemistry ; immunology ; Kidney ; immunology ; pathology ; Paraproteinemias ; 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

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

Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. LCDD is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD. We report a rare case of LCDD limited to a duodenal polyp. A 63-yr-old man visited our hospital for health screening without symptoms in 2009. On gastrofiberscopy, a duodenal polyp was observed. The biopsy showed diffuse infiltration by atypical plasma cells, which were positive for kappa-type light chains by immunohistochemistry. While the patient refused further management, we could find no evidence of recurrence until 2 yr after the initial diagnosis. It has been reported that isolated LCDD has relatively good prognosis compared to systemic LCDD. However, treatment for this disease has not been established yet.
Duodenum/pathology ; Endoscopy, Gastrointestinal ; Humans ; Immunoglobulin kappa-Chains/*immunology/metabolism ; Immunohistochemistry ; Intestinal Mucosa/*pathology ; Male ; Middle Aged ; Paraproteinemias/*diagnosis/pathology ; 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

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 ; Collagen/metabolism ; Human ; Immunoglobulins, kappa-Chain/chemistry ; Inflammation ; Lumbar Vertebrae/radiography ; Male ; Mucins/metabolism ; Paraproteinemias/*complications/diagnosis ; Scleredema Adultorum/*complications/diagnosis ; Skin/pathology ; Spondylitis, Ankylosing/*complications/diagnosis

We report a case of IgA kappa light chain deposition disease and combined adult Fanconi syndrome with Auer rod-like intracytoplasmic inclusions in plasma cells and proximal renal tubular cells in a 54-yr-old female. Cytochemical stainings revealed a strong acid phosphatase activity of the inclusions and weak periodic acid-Schiff positivity, whereas the reactions for peroxidase and alpha-naphthyl acetate esterase were negative. An immunostaining verified IgA-kappa inside the plasma cells. Kidney biopsy revealed Bence Jones cast nephropathy with kappa light chain positivity, and Congo red staining was negative. Electron microscopy showed needle-shaped crystals located in tubular epithelial cells.
Fanconi Syndrome/diagnosis/etiology/*pathology ; Female ; Humans ; *Immunoglobulin A/analysis ; Immunoglobulin kappa-Chains/analysis ; Inclusion Bodies/*ultrastructure ; Kidney Tubules, Proximal/pathology/*ultrastructure ; Middle Aged ; Paraproteinemias/*pathology ; Plasma Cells/pathology/*ultrastructure

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