Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS)
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BACKGROUND:Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS). Our case is the first case to show two coinciding conditions; one is the association of GIT AL amyloidosis with the incidental finding of a rare type of MGUS (LC-MGUS) and the other is the radiologic presentation of GIT amyloidosis with omental calcification mimicking the GIT malignancy. CASE REPORT:A 68-year-old female presented with symptoms of partial bowel obstruction, including intermittent diffuse abdominal pain and constipation. After computed tomography (CT) abdomen and pelvis, an exploratory laparotomy was needed because of suspicion of abdominal carcinomatosis due to diffuse omental calcification. The tissue sent for biopsy surprisingly showed AL amyloidosis. The patient did not report any systemic symptoms. Further workup was advised to inquire about the plasma cell dyscrasia which eventually turned into a very rare version of MGUS knows as light chain MGUS (LC-MGUS). Following adequate resection of the involved structures, the patient was then placed on chemotherapy and successfully went into remission. CONCLUSIONS:This case report illustrates that in an era of evidence based medicine, it is important to show through case reports the association of GIT AL amyloidosis with LC-MGUS, as the literature on this topic is lacking. It also points to the importance of timely intervention that can greatly enhance, not only the only the chances of remission but also prevention of further complications such as malignant transformation.Keywords:
Plasma cell dyscrasia
AL amyloidosis
Amyloid (mycology)
Gammopathy
Exploratory laparotomy
Plasma cell dyscrasias are a group of lymphoproliferative disorders characterized by proliferation of plasma cells and often by the production of a monoclonal protein. More common plasma cell dyscrasias such as multiple myeloma, amyloidosis, and monoclonal gammopathy of undetermined significance (MGUS) are beyond the scope of this chapter. Other unusual lymphoproliferative disorders will be reviewed under "Rare Lymphomas" (see Rare Lymphomas). The uncommon plasma cell dyscrasias such as plasma cell leukemia (PCL), nonsecretory multiple myeloma, immunoglobulin D (IgD) myeloma, Waldenström macroglobulinemia, heavy chain disease, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome (osteosclerotic myeloma), and solitary plasmacytomas will be discussed in this chapter.
Dyscrasia
Organomegaly
Plasma cell dyscrasia
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Plasma cell leukemia
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Myeloma protein
Plasma cell neoplasm
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Castleman disease
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A number of common disorders of the peripheral nervous system are closely linked to a monoclonal gammopathy. In a minority of patients, the neuropathy represents the sentinel feature of a malignant plasma cell dyscrasia, such as multiple myeloma or its osteosclerotic variant, Waldenstrom's disease, amyloidosis, cryoglobulinemia or lymphoma; the vast majority have so-called "monoclonal gammopathy of undetermined significance" (MGUS). Sensory symptoms predominate with paresthesias, numbness, imbalance, and gait ataxia. Electrodiagnostic studies show mixed demyelinating and axonal features and often may be indistinguishable from findings in chronic inflammatory demyelinating polyneuropathy. Some have a pure axonal polyneuropathy, and in these patients the relationship to the paraprotein is less certain. With limited success, correlations have been made between the immunoglobulin type (IgM, IgG, or IgA) and the clinical and electromyographic characteristics of the neuropathy. The treatment of MGUS neuropathies poses a considerable challenge. Patients with IgG/IgA-MGUS have improved with corticosteroids or intravenous immune globulin. Only the benefit of plasma exchange has been substantiated in a controlled trial. The IgM neuropathies tend to be more refractory but often improve with similar regimens, particularly if cytotoxic agents are added in doses sufficient to reduce the amount of the M-protein. In addition to plasma exchange, chlorambucil, and cyclophosphamide, interferon-alpha is a novel therapy that holds promise for patients with IgM neuropathies associated with anti-myelin associated antibodies. J. Clin. Apheresis 14:149–153, 1999. © 1999 Wiley-Liss, Inc.
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Plasma cell disorders are a group of diseases characterized by the over production of the abnormal plasma cells derived from the terminal differentiation of B lymphocytes. A monoclonal (M) serum and/or urine protein almost always accompanies malignant plasma cell diseases such as multiple myeloma, amyloidosis, Waldenström's macroglobulinemia, solitary bone and extra medullary plasmacytoma, POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome and heavy chain disease, as well as the preceding asymptomatic, premalignant disease stages such as monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM). Monoclonal gammopathy, plasma cell dyscrasia, dysproteinemias, and paraproteinemias are other terms used to describe this group of disorders.
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A series of spontaneous changes affecting the nature of the immunoglobulin secretion of plasma cells is described in a patient initially diagnosed as IgG lambda benign monoclonal gammopathy. After several years a slight increase in the amount of serum monoclonal immunoglobulin occurred; shortly thereafter an aggressive form of multiple myeloma was diagnosed. Unexpectedly a rapid spontaneous decrease of the monoclonal immunoglobulin, accompanied by the appearance in the serum of increasing quantities of a complex containing intact lambda light chains, then occurred. Concomitantly a fragment of the corresponding free light chain was detected in the urine. A parallel is drawn between the facts observed in this patient and in an animal model recently proposed to explain the different types of structural immunoglobulin abnormalities in multiple myeloma.
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Plasma cell dyscrasia
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AL amyloidosis
Daratumumab
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Plasma cell dyscrasias are a group of lymphoproliferative disorders characterized by proliferation of plasma cells and often by the production of a monoclonal protein. More common plasma cell dyscrasias (multiple myeloma, amyloidosis and monoclonal gammopathy of undetermined significance) are beyond the scope of this chapter. Other unusual lymphoproliferative disorders will be reviewed in Chapter 47. For the puposes of this chapter, uncommon plasma cell dyscrasias are defined as plasma cell leukemia, nonsecretory multiple myeloma, immunoglobulin D myeloma, immunoglobulin E myeloma, immunoglobulin M myeloma, Waldenström macroglobulinemia, heavy chain disease, POEMS syndrome (osteosclerotic myeloma), and solitary plasmacytomas.
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Plasma cell leukemia
Myeloma protein
Lymphoproliferative Disorders
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Plasma cell neoplasm
Lymphoplasmacytic Lymphoma
Castleman disease
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Sporadic reports in the medical literature concern the significant incidence of neutrophils with ring-shaped nuclei in myeloproliferative disorders. We report our first encounter with ring neutrophils in patients with lymphoproliferative disorders. A significant incidence of ring neutrophils was observed in all of 20 patients with multiple myeloma and in nine of 10 patients with monoclonal gammopathy of undetermined significance. The mean percent of ring neutrophils was 9.5% (range, 1.0% to 28.0%) in patients with multiple myeloma, which was significantly greater than in those with monoclonal gammopathy of undetermined significance or in healthy controls. In multiple myeloma, the incidence of ring neutrophils in the pretreatment phase was greater than that in the remission phase. A great deal of overlap was noted between multiple myeloma in the remission phase and monoclonal gammopathy of undetermined significance. The incidence of ring neutrophils seemed to vary according to disease stage. Ring neutrophils may reflect abnormal granulopoiesis in plasma cell dyscrasias.
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YAGO, K., KANOH, T. and UCHINO, H. Multicentric Giant Lymph Node Hyperplasia, Plasma Cell Type, with Monoclonal Gammopathy. Tohoku J. exp. Med., 1987, 153 (1), 49-54 - The association of multicentric giant lymph node hyperplasia (MGLH) of plasma cell type with monoclonal gammopathy was observed in a 37-year-old man. The present case provides an additional new piece of evidence that MGLH is by no means a benign disorder but can transform into plasma cell dyscrasia or B-cell lymphoma.
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