Pulmonary hypertension in a patient with intravascular lymphomatosis.

Intravascular lymphomatosis (IVL) is a rare subtype of lymphoma characterized by proliferation of lymphoma cells into small vessels. Clinical presentations of IVL are considerably varied among patients, and antemortem diagnosis is sometimes difficult. We report a 75-year-old Japanese female who presented with gradually worsening shortness of breath and pulmonary hypertension. After routine examinations, the patient was diagnosed with pulmonary artery hypertension (PH) combined with pulmonary hypertension owing to left-sided heart disease possibly due to chronic atrial fibrillation. However, a standard therapy of PH and diuretics was ineffective. In the clinical course, sustained increase in serum lactate dehydrogenase level, progressive bicytopenia, and appearance of atypical cells in her peripheral blood led to the diagnosis of IVL. The symptoms, laboratory findings and PH improved after initial chemotherapy. <Learning objective: Pulmonary artery hypertension (PH) has been focused in cardiovascular diseases and considerable progress in diagnosis and treatment was achieved. The targets of the treatment are PH and underlying causes of PH. The prevalence and frequency of the underlying causes extensively vary. However, considering that PH is a progressive and fatal disease if untreated, sufficient evaluation and optimal treatments are essential. Intravascular lymphomatosis might be considered to have a potential to cause PH.>.