Pseudo-monoclonal gammopathy: a report of four cases

Jawad, Majd D.; Go, Ronald S.; Witzig, Thomas E.; Mıkhael, Joseph; Ravindran, Aishwarya; Murrray, David L.

doi:10.3324/haematol.2017.171694

Cited by 12 publications

(5 citation statements)

References 8 publications

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“…Monoclonal immunoglobulin is produced by malignant plasma cells, and it can be diagnosed by serum protein electrophoresis, serum immunofixation, and the free light chain assay. 1 – 3 In our case, we found that hyperglobulinemia was polyclonal, and several autoantibodies were positive, including antinuclear antibody, anti-Sjogren’s syndrome A, anti-Sjogren’s syndrome B, and Ro-52, which support the diagnosis of Sjogren’s syndrome. Further examinations showed cirrhosis and hypersplenism, resulting from AID.…”

Section: Discussionsupporting

confidence: 68%

“…Hyperglobulinemia is usually caused by monoclonal immunoglobulin, which is produced by malignant plasma cells, or by polyclonal immunoglobulin, caused by autoimmune diseases (AIDs). 1,2 We report a complex case with presentation of hyperglobulinemia, increased plasma cells in the bone marrow, pancytopenia, hematemesis, and an enlarged spleen.…”

Section: Introductionmentioning

confidence: 99%

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Pseudo-monoclonal gammopathy due to autoimmune disease: a case report

et al. 2019

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Hyperglobulinemia is a common manifestation of plasma cell disease, and it is sometimes caused by autoimmune diseases (AIDs). We report an uncommon presentation of hyperglobulinemia, with a high amount of plasma cells in bone marrow, pancytopenia, hematemesis, and splenomegaly in an 18-year-old woman. Some examinations were performed to determine the diagnosis, including serum protein electrophoresis, immunofixation electrophoresis, the free light chain assay, abdominal enhanced computed tomography (CT) and CT venography, and positron-emission tomography-CT. The patient was finally diagnosed with AID. Considerable improvement in her symptoms was observed after immunosuppressive therapy. Findings in this case highlight that not only differentiation of hyperglobulinemia caused by monoclonal or polyclonal immunoglobulin, but also AIDs, need to be considered to exclude non-Hodgkin’s lymphoma and plasma cell disease.

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Section: Discussionsupporting

confidence: 68%

Section: Introductionmentioning

confidence: 99%

Pseudo-monoclonal gammopathy due to autoimmune disease: a case report

et al. 2019

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“…The hypergammaglobulinemia can be mistaken for a polyclonal increase in IgA, monoclonal gammopathy of undetermined significance or “biclonal” IgG kappa and lambda gammopathy, as laboratory physicians may not be familiar with the dense bands of IgG-lambda and kappa which in fact represent polyclonal IgG4. 36,37 Some patients have even been treated for myeloma before subsequently being found to have IgG4-RD as the cause of their protein abnormalities, plasmacytosis and renal disease. 9,38 Laboratory physicians must, therefore, consider the differential diagnosis of beta-gamma bridging and order or suggest additional investigations to clarify clonality and heavy chain composition where necessary.…”

Section: Clinical Presentationmentioning

confidence: 99%

IgG4-related disease: what a hematologist needs to know

Mattman²,

et al. 2019

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IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematologic manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. The disease can easily be missed by unsuspecting hematologists, as patients may present with clinical problems that mimic disorders such as multicentric Castleman disease, lymphoma, plasma cell neoplasms and hypereosinophilic syndromes. When IgG4-related disease is suspected, serum protein electrophoresis and IgG subclasses are helpful as initial tests but a firm histological diagnosis is essential both to confirm the diagnosis and to rule out mimickers. The central histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells (with an IgG4/IgG ratio >40%), storiform fibrosis, and obliterative phlebitis. Importantly for hematologists, the latter two features are seen in all tissues except bone marrow and lymph nodes, making these two sites suboptimal for histological confirmation. Many patients follow an indolent course and respond well to treatment, but a significant proportion may have highly morbid or fatal complications such as periaortitis, severe retroperitoneal fibrosis or pachymeningitis. Corticosteroids are effective but cause new or worsening diabetes in about 40% of patients. Initial response rates to rituximab are high but durable remissions are rare. More intensive lymphoma chemotherapy regimens may be required in rare cases of severe, refractory disease, and targeted therapy against plasmablasts, IgE and other disease biomarkers warrant further exploration.

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“…However, flow cytometry of peripheral blood indicated polyclonal plasma cell proliferation, along with elevated levels of both kappa and lambda free light chains, ultimately leading to reactive plasmacytosis. Jawad et al [ 11 ] reported four cases of pseudo-monoclonal gammopathy in which both serum free light chains (kappa and lambda) were abnormally elevated, and reactive plasmacytosis was later confirmed via mass spectrometry. Jang et al [ 12 ] and Xu et al [ 13 ] reported two concurrent AITL and plasma cell disorder cases, which were confirmed using flow cytometry.…”

Section: Discussionmentioning

confidence: 99%

Plasmacytosis mimicking multiple myeloma in angioimmunoblastic T-cell lymphoma: A case report and review of literature

Lin,

Lee,

Chuo

et al. 2024

World J Clin Cases

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BACKGROUND Angioimmunoblastic T-cell lymphoma (AITL) is a common subtype of peripheral T-cell lymphoma. Approximately half of patients with AITL may concurrently present with hypergammaglobulinemia. Increased numbers of plasma cells in the bone marrow are commonly observed at diagnosis. These tumors mimic plasma cell myelomas, hindering a conundrum of clinical diagnoses and potentially delaying appropriate treatment. CASE SUMMARY A 78-year-old woman experienced poor appetite, weight loss of 5 kg, fatigue 2 months before presentation, and shortness of breath 2 d before presentation, but no fever or night sweats. Physical examination revealed splenomegaly and many palpable masses over the bilateral axillary regions, approximately > 2 cm in size, with rubbery consistency and no tenderness. Blood tests revealed anemia and thrombocytopenia, lactate dehydrogenase level of 153 U/L, total protein level of 10.9 g/dL, albumin to globulin ratio of 0.2, and immunoglobulin G level more than the upper limit of 3000 mg/dL. The free kappa and lambda light chain concentrations were 451 and 614 mg/L, respectively. A pathological examination confirmed the diagnosis of AITL. The initial treatment was the cyclophosphamide, epirubicin, vincristine, and prednisolone regimen. Following this treatment, pleural effusion was controlled, and the patient was discharged in a stable condition and followed up in our outpatient department. CONCLUSION This report highlights the importance of differentiating reactive plasmacytosis from plasma cell myeloma in patients with hypergammaglobulinemia. A precise diagnosis of AITL requires a comprehensive evaluation, involving clinical, immunophenotypic, and histological findings conducted by a multidisciplinary team to ensure appropriate treatment.

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Pseudo-monoclonal gammopathy: a report of four cases

Cited by 12 publications

References 8 publications

Pseudo-monoclonal gammopathy due to autoimmune disease: a case report

Pseudo-monoclonal gammopathy due to autoimmune disease: a case report

IgG4-related disease: what a hematologist needs to know

Plasmacytosis mimicking multiple myeloma in angioimmunoblastic T-cell lymphoma: A case report and review of literature

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