Abstract
Pulmonary hypertension (PH) associated with malignancy, especially adenocarcinoma, is a well-known entity and is included in group V of the WHO classification. Intravascular lymphoma is a rare type of diffuse large B cell lymphoma, characterized by selective intravascular growth of malignant lymphocytes, aggressive behavior, and often a fatal course. Most of the time, diagnosis is postmortem due to the rarity and the protean manifestations of the disease. We present a rare case of an elderly patient presenting with severe pulmonary hypertension, fever of unknown etiology (FUO), and lymphadenopathy. Extensive evaluation searching for the etiology of her FUO and PH was noncontributory. The diagnosis of intravascular lymphoma was finally reached by the performance of a random abdominal fat pad biopsy and the patient was started on immunochemotherapy. She continues the follow up after 6 cycles of R-CHOP with no further febrile episodes and steady improvement in exercise tolerance.
Highlights
The prevalence of all types of pulmonary hypertension (PH) is not well defined; it is estimated that 10%–20% of the general population has PH by echocardiography [1]
We present a rare case of intravascular lymphoma (IVL) presenting with Fever of unknown etiology (FUO), severe PH, and disseminated lymphadenopathy diagnosed by random fat pad biopsy
Described by Pflegrand and Tappeiner in 1959 as “angioendotheliomatosis proliferans systemisata” and in 2008 defined by the World Health Organization (WHO) as intravascular large B cell lymphomas (IVBCL), IVL is a type of extra-nodal large B cell lymphoma where growth is restricted to the lumina of the vessels, the capillaries [7]
Summary
The prevalence of all types of pulmonary hypertension (PH) is not well defined; it is estimated that 10%–20% of the general population has PH by echocardiography [1]. Group 1 PH is the most widely studied and accounts for approximately 15 cases per million, but it is the other etiologies of PH which are more common. While left heart failure accounts for >65% of all clinically detected PH, the incidence and prevalence of other groups, especially group V, are less clearly defined [1]. Fever of unknown etiology (FUO) is one of the most challenging clinical presentations for the clinician. It may be associated with infections, malignancy, drug or toxin intake, and environmental toxicity among others. Adenocarcinoma, mainly of gastrointestinal etiology, is the most common malignancy associated with PTTM and related to severe PH [3,4,5]. We present a rare case of intravascular lymphoma (IVL) presenting with FUO, severe PH, and disseminated lymphadenopathy diagnosed by random fat pad biopsy
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