Rarely a patient with intravascular large B-cell lymphoma (IVLBCL) may present with pulmonary arterial hypertension (PAH). The diagnosis can be easily missed if certain tests are not performed.
Clinical features:
(1) severe hypoxemia
(2) constitutional symptoms such as fever
(3) elevated pulmonary arterial systolic blood pressure
Laboratory findings may include:
(1) very high serum LDH level
(2) high serum levels of soluble IL-2 receptor
Key diagnostic studies:
(1) A 18-FDG-PET scan can demonstrate uptake within pulmonary blood vessels.
(2) A lung biopsy shows diffuse infiltration of small arteries and arterioles by the lymphoma cells.
To read more or access our algorithms and calculators, please log in or register.
Specialty: Hematology Oncology
