Treatment of Primary Pulmonary Lymphoma in Pulmonary Intravascular Lymphoma

This protocol addresses primary pulmonary lymphoma presenting in the specific histological form of pulmonary intravascular lymphoma — a pattern defined by atypical lymphoid cell proliferation confined largely to the intraluminal space of the pulmonary vasculature.

Clinical scenario

Pulmonary intravascular lymphoma is characterised by the proliferation of atypical lymphoid cells within the lumen of capillaries, arterioles, venules and lymph ducts, with little or no invasion of the adjacent parenchyma.

Treatment approach

Management involves combination chemotherapy regimens used in the setting of high-grade non-Hodgkin lymphoma (NHL). The complete protocol specifies regimen selection and the evidence behind it.

Full regimen details, sequencing, and clinical decision points are available via the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

Pulmonary intravascular lymphoma is due to proliferation of atypical lymphoid cells within the lumen of capillaries, arterioles, venules and lymph ducts, with little or no invasion of the adjacent parenchyma.

Combination chemotherapy regimens used in high-grade NHL have some efficacy, giving a complete response rate of approximately 50%.

DOI: 10.1183/09031936.02.00404102

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