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[Intravascular lymphomatosis manifesting in the lung]
J M Sastre1, R Folgado, O Burges
1Servicio Medicina Interna, Hospital Clínico, Universidad de Valencia, Avda. Blasco Ibáñez, 17, 46010 Valencia.
Insights
Intravascular lymphomatosis is a rare condition where atypical B-cells infiltrate blood vessels, primarily affecting the lungs. Postmortem examination confirmed this diagnosis, highlighting the need for awareness of its pulmonary manifestations.
Area of Science:
- Hematology
- Pulmonology
- Pathology
Background:
- Intravascular lymphomatosis (IVL) is a rare, aggressive non-Hodgkin lymphoma characterized by the proliferation of lymphoid cells within small blood vessels.
- This entity often presents with non-specific symptoms, making early diagnosis challenging.
Observation:
- A case of IVL is presented with initial predominant symptoms localized to the lung, including cough and interstitial pulmonary infiltrates.
- Late-stage symptoms included fever and confusion, underscoring the systemic potential of this disease.
Findings:
- Postmortem examination revealed atypical cells, positive for CD45 and CD20 (leukocyte and pan-B cell markers) and negative for CD45-Ro and CD34 (pan-T and endothelial markers), occupying arterioles and capillaries within the lung alveolar walls.
- Immunohistochemical analysis confirmed the B-cell lineage of the atypical cells and their intravascular location.
Implications:
- This case highlights the importance of considering IVL in the differential diagnosis of unexplained pulmonary infiltrates and neurological symptoms.
- Understanding the specific immunophenotype (CD45+, CD20+, CD45-Ro-, CD34-) is crucial for accurate pathological diagnosis.
- Further research into earlier diagnostic markers and treatment strategies for IVL is warranted.
Abstract:
A case of intravascular lymphomatosis with predominant symptoms in the lung (cough and interstitial pulmonary infiltrates) is presented. Fever and confusion were lateness symptoms. Examination postmortem established the diagnosis. In the lung alveolar walls, small arterioles and the capillaries were occupied by atypical cells positives for CD45 and CD20, leukocyte and pan-B markers, and negatives for CD45-Ro and CD34, pan-T and endothelial markers. The main characteristics and the lung participation of this entity were reviewed.