Angiotropic large cell lymphoma presenting as interstitial lung disease.

Abstract

A case ofangiotropic large cell lymphoma is reported in which the patient presented with only pulmonary symptoms. It suggests that this rare and highly malignant lymphoma should be considered in the differential diagnosis of interstitial lung disease, and shows the value of open lung biopsy in unexplained interstitial lung disease. This patient responded well to treatment with cyclophosphamide, doxorubicin, vincristine, and prednisone.Angiotropic large cell lymphoma is a rare disease characterised histologically by proliferating mononuclear cells within the lumen of capillaries, venules, arterioles, and small arteries.' Originally described in cutaneous small vessels,4 later reports have shown that various organs may be affected, especially the nervous system and adrenal glands.5Previously angiotropic large cell lymphoma was known as "malignant angioendotheliomatosis" presuming an endothelial origin of the tumour cells; but recent studies using cell surface markers suggest a lymphoid origin,236 and the terms angiotropic large cell lymphoma3 and malignant intravascular immunoblastic lymphoma7 have been suggested. We report a case presenting with pulmonary symptoms. Case reportA 56 year old woman, an insulin dependent diabetic, was admitted to hospital because of persistent fever over the preceding week, progressive exertional dyspnoea, a nonproductive cough, and malaise. Two years earlier she had had pancreatitis during a period of alcohol abuse, but she had since abstained from alcohol. Until the onset of fever she had been well. There were no complaints of weight loss, skin lesions, disorientation, arthralgia, pruritis, or nocturnal sweats. Ske kept a parakeet and a cat and had no known allergies. Her only treatment was insulin.Physical examination showed that she had a fever of 39°C and bilateral fine inspiratory rales at the bases of both lungs. No lymphadenopathy or hepatosplenomegaly was detected.The erythrocyte sedimentation rate was 45 mm in the first hour and the white blood cell count 4-8 x 109/1, with 57% segmented neutrophils, 17% band neutrophils, 18% lymphocytes, 3% monocytes, 1% myelocytes, 2% eosinophils, and 1% basophils. Arterial blood gas analysis showed the

Intravascular lymphomatosis presenting as adult respiratory distress syndrome

“…Demirer et al described a case of IVL presenting with fever, cough, dyspnea, and hypoxemia, yet with normal chest films, com- puterized tomogram, and pulmonary angiogram [11]. Other cases presenting with respiratory symptoms were also published [5,17,31,[38][39][40]. A significant number of patients had serological evidence of autoimmunity [5,12].…”

Intravascular large B‐cell lymphoma with FDG accumulation in the lung lacking CT/67gallium scintigraphy abnormality

“…Although the lung is not a common diagnostic site of IVLBCL, autopsies have revealed changes in the lung in approximately 60% of reported cases [5,17]. It has been reported that the majority of cases with IVLBCL containing pulmonary involvement exhibit diffuse interstitial infiltrates on chest radiographs [12,18,19]. Despite the presence of dyspnea and hypoxia, chest radiography showed no abnormal findings in this case.…”

Intravascular Large B-cell Lymphoma Presenting Pulmonary Arterial Hypertension as an Initial Manifestation

“…The most common clinical manifestations involve skin and the nervous system, and some authors found that 68% of IVL patients had symptoms present in at least one of these organs (9). Although autopsy findings indicated that lung involvement in IVLBCL is relatively frequent (approximately 60%) (3,4), predominant or primary presentation in lung has been rare and only a few cases have been reported in the literature to date (4)(5)(6)(9)(10)(11)(12) (10,(14)(15)(16), three of the four cases with PAH lacked such lesions that could promote physicians to take account of lung biopsy. Thus, two cases could not be treated with chemotherapy due to lack of evidence of lymphoma, and they were diagnosed at autopsy (4,6).…”