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Published on: May 23, 2015
[Two cases of intravascular lymphomatosis diagnosed antemortem by transbronchial lung biopsy]
1Department of Internal Medicine, Kokura Memorial Hospital.
Insights
Transbronchial lung biopsy enabled early diagnosis of intravascular lymphomatosis (IVL), a rare condition. Chemotherapy achieved remission in one patient, highlighting potential for antemortem treatment of IVL.
Area of Science:
- Hematology
- Pulmonology
- Oncology
Background:
- Intravascular lymphomatosis (IVL) is a rare hematologic malignancy characterized by proliferation of lymphoid cells within small blood vessels.
- Diagnosis is often challenging and typically made post-mortem, complicating timely therapeutic intervention.
Observation:
- Two male patients, aged 71 and 65, presented with high-grade fever, hypoxemia, and elevated lactate dehydrogenase (LDH).
- Chest CT imaging revealed distinct patterns, including peripheral clear and central high-density areas in one patient, and diffuse mild densities in the upper lungs of the other.
- Transbronchial lung biopsy (TBLB) was crucial in confirming the diagnosis of IVL in both cases, despite initial suspicion of interstitial pneumonia.
Findings:
- TBLB provided definitive histopathological evidence of IVL, enabling an antemortem diagnosis in both patients.
- Patient 1, despite diagnosis, succumbed to the disease before completing chemotherapy.
- Patient 2 achieved complete clinical remission following CHOP multiagent chemotherapy, demonstrating treatment efficacy.
Implications:
- This case series underscores the diagnostic utility of TBLB for IVL, facilitating earlier detection than traditional autopsy findings.
- The successful treatment of Patient 2 suggests that multiagent chemotherapy, such as CHOP, can induce remission in IVL.
- Early diagnosis and prompt treatment initiation are critical for improving outcomes in patients with intravascular lymphomatosis.
Abstract:
Patient 1 was a 71-year-old man who had been admitted to our hospital with high-grade fever. Chest computed tomographic (CT) images revealed clear peripheral and high-density central areas. Abnormal laboratory findings included elevated LDH and hypoxemia. Interstitial pneumonia was suspected, and transbronchial lung biopsy (TBLB) confirmed the diagnosis of intravascular lymphomatosis (IVL). The patient succumbed before completion of chemotherapy. Patient 2 was a 65-year-old man admitted with high-grade fever. Abnormal laboratory findings included pancytopenia, hypoxemia, and elevated levels of LDH and soluble interleukin-2 receptor. Chest CT images revealed diffuse, mildly dense areas in the upper fields of both lungs. TBLB specimens yielded a diagnosis of IVL. Complete clinical remission was obtained with CHOP multiagent chemotherapy. Although IVL is usually diagnosed at autopsy, in these 2 cases an antemortem diagnosis was made on the basis of TBLB findings. Also, multiagent chemotherapy achieved a complete clinical remission in Patient 2.
