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[A case of intravascular lymphoma with diffuse centrilobular opacities]
Hiromi Shinoda1, Arafumi Maejima, Kumi Shimizu
1Department of Respiratory Medicine, Tokyo Medical Center.
Insights
Intravascular lymphoma (IVL) is a rare cancer. Early diagnosis using transbronchial lung biopsy is crucial for patients with specific CT findings and elevated LDH levels.
Area of Science:
- Hematology
- Oncology
- Pulmonology
Background:
- Intravascular lymphoma (IVL) is a rare extranodal non-Hodgkin lymphoma characterized by the proliferation of malignant lymphocytes within small blood vessels.
- Diagnosis can be challenging due to nonspecific symptoms and the absence of a distinct tumor mass.
Observation:
- A 54-year-old woman presented with fatigue and dyspnea, exhibiting markedly elevated serum lactate dehydrogenase (LDH) and diffuse centrilobular opacities on chest CT.
- Bronchoalveolar lavage fluid showed elevated cell counts with 98% lymphocytes.
- Transbronchial lung biopsy revealed CD20-positive atypical cells within alveolar capillaries, leading to an IVL diagnosis.
Findings:
- The patient was diagnosed with intravascular lymphoma based on lung biopsy findings.
- Bone marrow examination confirmed lymphoma cells in sinusoids without hemophagocytosis.
- Combined chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (CHOP-R) achieved complete remission.
Implications:
- This case highlights the importance of considering IVL in the differential diagnosis of patients presenting with diffuse centrilobular opacities on chest CT and elevated serum LDH.
- Transbronchial lung biopsy (TBLB) is a key diagnostic tool for identifying intravascular lymphoma in such cases.
- Successful treatment with CHOP-R demonstrates the efficacy of combined chemotherapy in achieving long-term remission for IVL.
Abstract:
A 54-year-old woman was admitted with general fatigue and dyspnea on exertion. Her serum LDH level was markedly elevated to 2145 IU/L, and chest CT revealed diffuse centrilobular opacities. Total cell counts in bronchoalveolar lavage fluid were elevated, and lymphocytes accounted for 98% of the cells. A transbronchial lung biopsy demonstrated numerous CD20-positive atypical cells in the alveolar capillaries, so intravascular lymphoma (IVL) was diagnosed as having. Lymphoma cells were also present in the bone marrow sinusoids, while there was no sign of hemophagocytosis. Combined chemotherapy (CHOP with rituximab) successfully induced complete remission, and she has been free of recurrence for 40 months. In cases with diffuse centrilobular opacities on chest CT, accompanied by elevated serum LDH, it is important to rule out IVL by performing TBLB.
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