losartan-potassium and Paraproteinemias

Heavy chain deposition disease (HCDD) is a comparatively recently described entity characterized by glomerular and tubular basement membrane deposition of monoclonal heavy chains without associated light chains. To our knowledge, review of the literature shows only 24 previously reported cases of HCDD with unequivocal evidence of monoclonal heavy chain deposition in the kidney using immunofluorescence microscopic and electron microscopic studies. The predominant heavy chain subtype was γ. There has been a single case of μ HCDD and 2 previously reported cases of α HCDD. In this report, we describe 3 additional cases of α HCDD, all with a crescentic pattern of injury and one of which was associated with cutis laxa. We compare their clinicopathologic features with all previously reported cases of HCDD.

Topics: Adult; Aged; Anemia; Antineoplastic Combined Chemotherapy Protocols; Boronic Acids; Bortezomib; Cutis Laxa; Dexamethasone; Diabetic Nephropathies; Erythropoietin; Fatal Outcome; Female; Heavy Chain Disease; Hematuria; Humans; Hypertension, Renal; Immunoglobulin alpha-Chains; Immunoglobulin gamma-Chains; Immunoglobulin mu-Chains; Kidney Glomerulus; Male; Multiple Myeloma; Paraproteinemias; Proteinuria; Pyrazines; Thalidomide; Urticaria; Vasculitis, Leukocytoclastic, Cutaneous

The TEMPI syndrome is a rare and acquired disorder characterized by 5 salient features, which compose its name: (1) telangiectasias; (2) elevated erythropoietin and erythrocytosis; (3) monoclonal gammopathy; (4) perinephric fluid collections; and (5) intrapulmonary shunting. Complete resolution of symptoms following treatment with plasma cell-directed therapy supports the hypothesis that the monoclonal antibody is causal and pathogenic. Understanding the basis of the TEMPI syndrome will depend on the identification of additional patients and a coordinated international effort.

Topics: Erythropoietin; Humans; Lung Diseases; Paraproteinemias; Polycythemia; Syndrome; Telangiectasis

Collection of hematopoietic progenitor cells by apheresis (HPC-A) requires separation of cells by density. Previous studies highlighted the challenges of HPC-A collection from patients with abnormal red blood cells (RBCs). TEMPI syndrome is a recently described condition defined by teleangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. Patients with TEMPI syndrome have responded to therapies used to treat plasma cell dyscrasias and may benefit from autologous HPC transplantation. We report HPC-A collection from a patient with TEMPI syndrome that was complicated by severe iron deficiency.. The patient received granulocyte-colony-stimulating factor (G-CSF) and plerixafor for HPC mobilization and underwent 3 days of HPC-A collection.. The patient presented for collection with a microcytic erythrocytosis. Over 3 days, approximately 50 L of whole blood was processed, and 2 × 10(8) CD34+ cells were collected (2.8 × 10(6) CD34+ cells/kg). The mean collection efficiency (CE), percentage of mononuclear cells, hematocrit (Hct), and RBC count were 18%, 90%, 14%, and 9 × 10(11) , respectively. Altering collection variables to avoid RBC contamination reduced CE. Ficoll preparations of the products after freeze-thaw showed RBC contamination and hemolysis. Postthaw viability exceeded 95%. The products were not RBC reduced or washed. There were no adverse reactions during or after infusion.. HPC-A collection from a patient with TEMPI syndrome was complicated by microcytic erythrocytosis, leading to RBC contamination and hemolysis in the product. Adequate HPCs were collected and the patient tolerated infusion without RBC depletion or washing. Our report highlights difficulties of HPC-A collection from iron-deficient patients.

Topics: Benzylamines; Cyclams; Cytapheresis; Erythrocytes, Abnormal; Erythropoietin; Granulocyte Colony-Stimulating Factor; Hematopoietic Stem Cell Mobilization; Hematopoietic Stem Cells; Heterocyclic Compounds; Humans; Kidney Diseases; Leukocyte Count; Male; Middle Aged; Paraproteinemias; Polycythemia; Syndrome

We report here the interesting case of a 76-year-old man with severe proteinuria who was diagnosed with systemic mastocytosis accompanied by a clonal non-mast-cell lineage haematological disorder (a non-secretory plasma cell dyscrasia). This is a unique report of systemic mastocytosis with a non-secretory plasma cell dyscrasia and nephrotic syndrome. The pathophysiological relevance between these entities along with the probability of occult amyloidosis is discussed.

Topics: Aged; Amyloidosis; Anemia; Biopsy; Bone Marrow; Clone Cells; Coloring Agents; Congo Red; Darbepoetin alfa; Drug Therapy, Combination; Erythropoietin; Factor X Deficiency; Gingiva; Hemorrhagic Disorders; Histamine Antagonists; Humans; Male; Mastocytosis, Systemic; Nephrotic Syndrome; Paraproteinemias; Prednisone; Proteinuria; Splenectomy; Splenomegaly; Subcutaneous Fat

Topics: Antineoplastic Agents; Boronic Acids; Bortezomib; Erythropoietin; Female; Humans; Lymphangiectasis; Middle Aged; Paraproteinemias; Polycythemia; Pyrazines; Syndrome; Telangiectasis

Topics: Antineoplastic Agents; Boronic Acids; Bortezomib; Erythropoietin; Female; Humans; Male; Middle Aged; Paraproteinemias; Polycythemia; Pyrazines; Syndrome; Telangiectasis

Topics: Adult; Boronic Acids; Bortezomib; Erythropoietin; Female; Humans; Hydronephrosis; Lymphangiectasis; Male; Middle Aged; Paraproteinemias; Polycythemia; Protease Inhibitors; Pyrazines; Syndrome; Telangiectasis

Topics: Anemia, Hypochromic; Antirheumatic Agents; Autoimmune Diseases; Blood Loss, Surgical; Christianity; Crohn Disease; Erythropoietin; Folic Acid Deficiency; Hepatitis C, Chronic; Hip Fractures; Humans; Iron; Male; Middle Aged; Paraproteinemias; Premedication; Proteus Infections; Recombinant Proteins; Spondylitis, Ankylosing; Sulfasalazine; Urinary Tract Infections; Vitamins

A case of adult pure red cell aplasia (PRCA) with a serum IgG inhibitor to erythropoiesis and an IgG lambda M component is presented. The study of lymphocyte populations revealed a slight but definite decrease of E and EA rosettes, with dissociation between E rosettes and PHA blastic transformation of blood lymphocytes and increase of membrane IgM-bearing lymphocytes. The relationship between PRCA and paraproteinemia is discussed: it is suggested that the serum M component may derive from an immunological unbalance between T and B lymphocytes. Since a survey of the literature reveals 5 similar cases, it is suggested that paraproteinemia may be the hallmark of a particular variety of chronic PRCA 'type 1'.

Topics: Aged; Anemia, Aplastic; Autoimmune Diseases; Erythropoietin; Female; Fluorescent Antibody Technique; Humans; Immunoglobulin G; Lymphocytes; Paraproteinemias; Receptors, Antigen, B-Cell; Rosette Formation