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Published on: March 30, 2018
Plasmacytoid lymphocytic lymphoma presenting with IgG hyperviscosity syndrome and peritoneal lymphomatosis
1Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
Insights
This case study highlights a rare presentation of plasmacytoid lymphocytic lymphoma with IgG hyperviscosity syndrome and peritoneal involvement. Despite initial improvement with plasmapheresis, the patient succumbed to sepsis.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Plasmacytoid lymphocytic lymphoma is a rare B-cell malignancy.
- Hyperviscosity syndrome can complicate lymphoproliferative disorders.
- Peritoneal involvement in lymphoma can mimic other conditions.
Observation:
- A 74-year-old woman presented with dyspnea, altered consciousness, ascites, and skull lesions.
- Laboratory findings revealed monoclonal IgG-lambda gammopathy and suppressed immunoglobulins.
- Abdominal CT suggested cancerous peritonitis.
Findings:
- The patient exhibited IgG hyperviscosity syndrome and diffuse peritoneal disease as initial manifestations of plasmacytoid lymphocytic lymphoma.
- Lytic bone lesions and a high M-component with suppressed normal immunoglobulins were noted, atypical for lymphoma compared to multiple myeloma.
Implications:
- This case underscores the importance of considering lymphoma in patients with unexplained hyperviscosity and peritoneal findings.
- The co-occurrence of lytic bone lesions and suppressed immunity warrants careful differential diagnosis between lymphoma and multiple myeloma.
- Early recognition and management of hyperviscosity syndrome are crucial for patient outcomes.
Abstract:
We reported a 74-year-old woman with plasmacytoid lymphocytic lymphoma who initially presented with exertional dyspnea, conscious disturbance, ascites, and lytic skull lesions. Protein electrophoresis and immunoelectrophoresis showed monoclonal IgG-lambda gammopathy with IgG level of 13300 mg/dl and marked suppression of the residual normal immunoglobulins. Abdominal computed tomography (CT) revealed a pattern mimicked cancerous peritonitis. She responded to plasmapheresis with much clinical improvement of the hyperviscosity syndrome but died of H. influenza sepsis 3 weeks after diagnosis. The unusual and interesting features of this case included: (1) IgG hyperviscosity syndrome and diffuse peritoneal involvement as the initial manifestations in plasmacytoid lymphocytic lymphoma, and (2) presence of lytic bone lesions in conjunction with high level of M--component and marked suppression of normal residual immunoglobulins in a patient with lymphoma rather than multiple myeloma.
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