Monoclonal Gammapathy

Composite lymphoma (CL) is a rare disease defined by the occurrence of two distinct lymphomas within a single tissue at the same time. We present the case of an 89-year-old male with a clinical history of immunoglobulin M monoclonal gammopathy of undetermined significance. The patient presented cutaneous eruption of nodules on the right bottom and arm. An excisional biopsy revealed cutaneous infiltration composed of two components. The first one consisted of large B-cells with CD20+/MUM1+/BCL2+ phenotype whereas the second one involved the subcutaneous fat in a panniculitic manner, and was CD3+/CD8+/granzyme B+/TCRβF1+...

RATIONALE: Lymphoid interstitial pneumonia (LIP) is a rare disease with lymphocytic infiltration of the alveolar interstitial and air spaces, sometimes classified as a clonal lymphoproliferative disease (LPD) with high prevalence in patients with immunodysregulation. Although association of mucosa-associated lymphoid tissue (MALT) lymphoma development with infectious agents has been well described, it is not so in the case of LIP. Attempts to demonstrate an infective cause by direct microbe detection have failed, but association with atypical specific immune response to opportunistic infectious agent has not been studied...

There is currently no consensus for the quantification and identification of weak gammaglobulin monoclonal spike. We conducted a study of 12 months including 410 serum protein electrophoresis (SPE) with suspected weak monoclonal spike in gammaglobulin area without anteriority, which led to the realization of an immunofixation (IF); 276 SPE has a weak spike in gammaglobulins (defined with perpendicular drop method (orthogonal (O) quantification <5 g/L) but only 157 (57%) has monoclonal immunoglobulin by immunofixation of serum...

Multiple myeloma has varied manifestations which resemble common patient complaints and that is why this disease is typically not diagnosed until it reaches an advanced stage. Spinal pains can be an expression of deformative and discogenous changes, but also a symptom of multiple myeloma. Pains in the long bones may result from the pain radiating from an arthrotic joint, but also from a large myelomatic osteolytic lesion which makes the bone prone to a spontaneous fracture. Pathological weariness may have many causes, multiple myeloma being one of them...

Castleman's disease is a lymphoproliferative disorder characterized by angiofollicular lymph node hyperplasia. Recently, a new variant of multicentric Castleman's disease has been identified in Japan called TAFRO syndrome. It is characterized by a constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction and organomegaly (TAFRO). It is usually associated with polyclonal hyperimmunoglobulinemia. Here, we report the first and unique case of TAFRO syndrome with monoclonal gammapathy...

IgD multiple myeloma (MM) is a rare subtype of myeloma, it affects less than 2% of patients with MM. To evaluate the clinical and prognostic attributes of serum free light chains (sFLCs) analysis, we examined 17 cases of IgD MM. From 1998 to 2012, we obtained 1250 monoclonal gammapathies including 590 multiple myeloma and 17 patients had IgD MM. With preponderance of men patients with a mean age at diagnosis of: 59±12years. Patients with IgD MM have a short survival (Median survival=9months). The presenting features included: bone pain (75%), lymphadenopathy (16%), hepatomegaly (25%), splenomegaly (8%), associated AL amyloidosis (6%), renal impairment function (82%), infections (47%), hypercalcemia (37%) and anemia (93%)...

Skin manifestations associated with monoclonal gammapathy are common and can present with various clinical and pathological aspects. They can be the first events leading to the diagnosis of monoclonal gammapathy. They may be present either as specific lesions, including lymphoplasmacytic or pure plasma cell neoplastic infiltrates and monoclonal immunoglobulin deposits, or as non-specific dermatitis, such as leukocytoclastic vasculitis, neutrophilic dermatoses, mucinoses or xanthomatosis, giving little clues for the diagnosis of the underlying disease...

Liver disease is the major complication of chronic HCV infection. However, extrahepatic complications are common (50-75%), including mixed cryoglobulinaemia and B-cell lymphomas. Given that chronic hepatitis C has become curable using expensive oral direct-acting antivirals (DAAs), it seems worth revisiting the whole spectrum and burden of disease in HCV carriers.Herein, we update the most clinically significant medical complications associated with chronic hepatitis C and the evidence of benefits that would derive from a wide use of curative DAA therapies...

Acquired angioedema are rare. They are associated with monoclonal gammapathies of uncertain significance (MGUS) or lymphomas. They give the same symptoms as the hereditary form and the same laryngeal risk. They are characterized by a low level of C4, C1Inh and C1q. They are linked to the consumption of C1Inh by the lymphoid cells or to the presence of anti-C1Inh autoantibodies. They must be treated by symptomatic treatment when attack occur (C1Inh concentrate and icatibant). The use of rituximab needs to prove its efficiency...

OBJECTIVES: Biclonal gammopathies are characterized by the clonal proliferation of plasma cells or their B-lymphoid progenitors and are associated with the production of abnormal immunoglobulins (M proteins or paraproteins). There are no known studies that have analyzed this disease in Spain. We studied the underlying diseases, characteristics of paraproteins and the evolution of a series of patients with biclonal gammopathy. MATERIAL AND METHODS: We reviewed clonal gammopathies at the Department of Immunology of Hospital Puerta de Hierro in Madrid, between 1970 and 2011, selecting those patients with biclonal gammopathy in one reading...

Paraprotein interferences assays are known but rather unusual in clinical chemistry.we here report a paraprotein interference with a bicarbonate assay in a IgA kappa-type myeloma patient. Serum bicarbonate level was assessed by the enzymatic method of a multiparametric chemistry analyzer (Advia 1800, Siemens) as well as a specific electrode assay on a blood gas analyzer (GEM 4000, IL). Paraprotein interference with the enzymatic assay was evidenced by an abnomally low bicarbonate level measured by the enzymatic method in contrast with usual levels obtained by using the gas analyzer and with the clinical status of the patient...

Multifocal motor neuropathy (MMN) and anti-myelin-associated glycoprotein (anti-MAG)-associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49-year-old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits. Nerve conduction studies demonstrated persistent motor conduction blocks outside compression sites, typical of MMN...

OBJECTIVES: To investigate in primary Sjögren's syndrome, the differences between patients with and without widespread pain (WSP) with respect to the cumulative prevalence of extra-glandular manifestations (EGMs) and systemic auto-antibodies. METHODS: All outpatients diagnosed with primary Sjögren's syndrome (2) were included in a prospective follow-up, with at least one check up each year, from June 1991 until November 2011. Patients who also fulfilled criteria for concomitant connective tissue disorders were excluded...

PURPOSE: To investigate the role of hypoxia-inducible factor-1α (HIF-1α) in angiogenesis and drug resistance of bone marrow endothelial cells of patients with multiple myeloma. EXPERIMENTAL DESIGN: HIF-1α mRNA and protein were evaluated in patients with multiple myeloma endothelial cells (MMEC) at diagnosis, at relapse after bortezomib- or lenalidomide-based therapies or on refractory phase to these drugs, at remission; in endothelial cells of patients with monoclonal gammapathies of undetermined significance (MGUS; MGECs), and of those with benign anemia (controls)...

CASE REPORT: A 48-year-old male was referred to our hospital for further evaluation of eyelid edema with bilateral yellowish ulcerated nodules. Suspecting a xanthogranulomatosis, imaging tests and biopsy were performed with diagnosis of necrobiotic xanthogranuloma. IgG monoclonal gammapathy was diagnosed in a systemic study. Systemic corticosteroids and cyclosporine were initiated unsuccessfully; therefore intra-lesional injections of triamcinolone were started, which controlled the orbital disease...

Pulmonary involvement in the course of systemic senile amyloidosis caused by non-mutated transthyretin is rarely described. We report on concomitant monoclonal gammapathy of undermined significance (MGUS) and amyloidosis with non-mutated transthyretin with diffuse lesions in lung parenchyma. A female patient, 67 years old, was admitted with dyspnoea, malaise, weight loss, and disseminated radiological lesions in the lungs. On lung HRCT, signs of pulmonary hypertension, alveolar and interstitial involvement, with thickening of septal lines were found...

Smoldering multiple myeloma (SMM) is an asymptomatic plasma cell neoplasia, characterized by monoclonal plasma cell proliferation in the absence of end-organ damage, but with a high risk of progression to multiple myeloma. It has therefore to be distinguished from monoclonal gammapathy of undetermined significance (MGUS), which has a much lower risk of progression, but also from multiple myeloma, which remains an incurable disease and requires a specific treatment. The critical question in the management of SMM is whether an early therapeutic strategy could help delaying the progression to multiple myeloma, in order to lower the risk of serious complications related to this progression, or even to cure the disease...

Proteinuria is an expected complication in transplant patients treated with mammalian target of rapamycin inhibitors (mTOR-i). However, clinical suspicion should always be supported by histological evidence in order to investigate potential alternate diagnoses such as acute or chronic rejection, interstitial fibrosis and tubular atrophy, or recurrent or de novo glomerulopathy. In this case we report the unexpected diagnosis of amyloidosis in a renal-transplant patient with pre-transplant monoclonal gammapathy of undetermined significance who developed proteinuria after conversion from tacrolimus to everolimus...

BACKGROUND: Transplant patients have an increased incidence of lymphoproliferative disorders. The development of a sensitive immunoassay for serum free light chain (sFLC) determination has improved the diagnosis of monoclonal gammapathy. However, metabolism of FLC largely depends on renal function which could lead to misinterpretation of results. The aim of this study was to assess polyclonal FLC in kidney transplant recipients in relation to markers of renal function. MATERIALS AND METHODS: 97 renal transplant patients (median transplantation follow-up of 2...

In the attempt to harmonize clinical practices between different French transplantation centers, the French Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC) set up the third annual series of workshops which brought together practitioners from all member centers and took place in October 2012 in Lille. Here we report our results and recommendations regarding the management of common issues related to the donor: pre-transplant pregnancy and monoclonal gammopathy.