Elsevier

Mayo Clinic Proceedings

Volume 82, Issue 12, December 2007, Pages 1525-1527
Mayo Clinic Proceedings

BRIEF REPORT
Use of Random Skin Biopsy for Diagnosis of Intravascular Large B-Cell Lymphoma

https://doi.org/10.1016/S0025-6196(11)61097-5Get rights and content

Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma with an aggressive clinical course characterized by proliferation of large lymphoma cells within the lumina of the small vessels. Because of its varied clinical symptoms and the absence of lymphadenopathy, diagnosis of IVL is extremely difficult and requires histological confirmation. We report here 6 consecutive patients with IVL, admitted to Kameda General Hospital, Kamogawa-shi, Japan, from June 7, 2006, to February 28, 2007, whose IVL was diagnosed by random skin biopsy of healthy-appearing skin. Three patients presented with progressive neurological deterioration and 2 others with hypoxemia with interstitial infiltration on chest radiography. One patient presented with confusion and severe hypoxia without apparent interstitial infiltration. Two patients showed localized skin involvement. Irrespective of the presence of skin lesions, almost all skin biopsy specimens showed obliteration of small vessels of subcutaneous fat tissues by lymphoma cells, allowing a prompt diagnosis of IVL. Early institution of rituximab-based chemotherapy induced favorable responses in all patients treated. Because diagnosis based on tissue other than skin is usually difficult in patients with suspected IVL, random skin biopsy should be considered even in patients with no evident skin lesions.

Section snippets

PATIENTS AND METHODS

This study was based on 6 consecutive patients suspected to have IVL, all of whom were admitted to Kameda General Hospital, Kamogawa-shi, Japan, from June 7, 2006, to February 28, 2007. Skin biopsy was performed by dermatologists at our hospital (R.S. and A.T.) using standard methods. All specimens obtained from the patients were fixed in 10% buffered formalin, and paraffin-embedded tissues were stained with hematoxylin and eosin. Immunohistochemical studies were performed by the

Clinical and Laboratory Features

The clinical and laboratory features of 6 patients with IVL, 1 of whom had a history of extranodal diffuse large B-cell lymphoma, are summarized in Table 1. Presenting symptoms included fever of unknown origin, progressive neurological abnormalities (4 patients), and dyspnea (3 patients). In all patients, remarkable deterioration in Eastern Cooperative Oncology Group (ECOG) performance status (to more than grade 3) was observed. None of the patients had peripheral lymphadenopathy, whereas

DISCUSSION

In this study, we clearly showed the clinical usefulness of random skin biopsy for the histological diagnosis of IVL in patients whose clinical and laboratory data are suggestive of IVL. Because of the heterogeneity of its clinical symptoms and the lack of focal disease, the clinical diagnosis of IVL is challenging and often difficult to diagnose antemortem.10 The poor prognosis of these patients reflects delays in diagnosis and institution of chemotherapy. The most commonly affected organs are

CONCLUSION

During a 6-month period, we diagnosed IVL in 6 patients by random skin biopsy from healthy-appearing skin. Almost all specimens from healthy-appearing skin revealed obliteration of the small vessels of subcutaneous tissue by lymphoma cells. Because a diagnosis based on a tissue other than skin is usually difficult in patients with suspected IVL, random skin biopsy should be considered even in patients with no evident skin lesions. Further, on the basis of our observations and those of previous

Acknowledgments

We thank Drs Takeshi Kokubo and Kazuhide Hoshi, Department of Pathology, Kameda General Hospital, Kamogawa-shi, Japan, for their help in this study.

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