Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

Intravascular lymphoma (IVL) is a rare and usually fatal disease that belongs to the class of high‐grade malignant lymphomas and which is characterized by proliferation of neoplastic lymphoid cells exclusively within the lumina of small blood vessels. Its polymorphic and nonspecific clinical manifes...

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Bibliographic Details
Published in:American journal of hematology 2005-05, Vol.79 (1), p.46-49
Main Authors: Aouba, Achille, Diop, Saliou, Saadoun, David, Trebbia, Grégoire, Vilde, Françoise, Patri, Blaise, Hermine, Olivier
Format: Article
Language:English
Subjects:
Aged
Biological and medical sciences
Fatal Outcome
Female
Hematologic and hematopoietic diseases
Humans
Hypertension, Pulmonary - etiology
Hypertension, Pulmonary - pathology
intravascular lymphoma
Lymphoma, B-Cell - pathology
Lymphoma, Large B-Cell, Diffuse - pathology
Medical sciences
Multiple Organ Failure
Pneumology
pulmonary arterial hypertension
pulmonary capillary‐wedge aspiration
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
right‐sided heart catheterization
tumor‐related thrombotic pulmonary microangiopathy
Vascular Neoplasms - pathology
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Summary:Intravascular lymphoma (IVL) is a rare and usually fatal disease that belongs to the class of high‐grade malignant lymphomas and which is characterized by proliferation of neoplastic lymphoid cells exclusively within the lumina of small blood vessels. Its polymorphic and nonspecific clinical manifestations make antemortem diagnosis very difficult. We report herein a case of IVL revealed by fatal, precapillary, pulmonary arterial hypertension and associated with long‐lasting fever. Extensive investigation of the usual causes of pulmonary arterial hypertension was negative. The diagnosis of intravascular lymphoma was made on postmortem analysis, revealing diffuse and sometimes complete obliterations of the lumina of small blood vessels by large B‐cell lymphoma, including pulmonary capillaries. Thus, we propose that IVL must be added to the spectrum of etiologies of subacute pulmonary arterial hypertension, notably in the context of associated fever, both entities requiring emergency diagnosis. For this purpose, blood collected via pulmonary capillary‐wedge aspiration for cytologic examination may be associated with right‐sided heart catheterization when this latter procedure is suitable. Am. J. Hematol. 79:46–49, 2005. © 2005 Wiley‐Liss, Inc.
ISSN:0361-8609
1096-8652
DOI:10.1002/ajh.20300