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Polymyositis associated with Ki-1 lymphoma
D Bouscary1, M Tulliez, F Dreyfus
1Department of Hematology Internal Medicine, Hospital Cochin, Paris, France.
Leukemia & Lymphoma
|March 1, 1995
Summary
This study details a rare case of acute polymyositis linked to Ki-1 non-Hodgkin's lymphoma (NHL). The patient, diagnosed with a T-cell lymphoma, did not improve with treatment and succumbed to respiratory failure.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Polymyositis is an inflammatory myopathy characterized by muscle weakness.
- Ki-1 non-Hodgkin's lymphoma (NHL), now known as anaplastic large cell lymphoma (ALCL), is a distinct subtype of T-cell lymphoma.
Observation:
- A patient presented with acute polymyositis.
- The polymyositis was associated with a primary T-cell bone marrow Ki-1 lymphoma (ALCL).
- Malignant cells expressed CD30 (Ki-1), CD3, CD4, CD25, and betaF1 antigens.
Findings:
- The patient received chemotherapy and high-dose methylprednisolone pulse therapy.
- No clinical improvement was observed following treatment.
- The patient rapidly developed acute respiratory distress syndrome and died.
Implications:
- This case represents the first documented association between Ki-1 lymphoma and polymyositis.
- Highlights the potential for rare paraneoplastic syndromes in hematologic malignancies.
- Underscores the aggressive nature and poor prognosis of this specific lymphoma subtype when presenting with polymyositis.