Limited Role of Random Skin Biopsy in the Diagnosis of Intravascular Lymphoma in Adult Patients with Hemophagocytic Lymphohistiocytosis

Abstract

Background/Aims: This study examined the role of random normal skin biopsy in the diagnosis of intravascular lymphoma (IVL) in adult Western patients with clinically diagnosed hemophagocytic lymphohistiocytosis (HLH). Methods: In a retrospective chart review study, we analyzed a total of 59 skin biopsies that were performed to diagnose IVL in 21 adult patients with HLH seen at Stanford Hospital between 2004 and 2016. Results: Out of the 59 skin biopsies, 42 were taken from clinically normal-appearing skin and 17 from clinically abnormal-appearing skin. None of the 59 biopsies revealed a diagnosis of primary or metastatic malignancy, regardless of the malignancy history, clinical presentation, and biopsy and histopathologic characteristics. A review of 8 positive IVL cases at Stanford Hospital including 1 case associated with HLH showed 1 positive diagnosis by a targeted skin biopsy and other positive diagnoses by bone marrow (n = 4), lung (n = 2), brain (n = 2), muscle (n = 1), and nerve (n = 1). Conclusion: Random skin biopsies have a limited role in diagnosing IVL in adult patients with HLH, in the setting of a single academic institution in the USA. A review of the literature emphasizes the role of a full body skin exam with a selective skin biopsy in these patients.

Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years

“…RSB is now considered as a standard diagnostic procedure for patients with suspected IVLBCL in Japan but not in Western countries. One important reason that RSB has not been widely used in Western countries might be its limited diagnostic capability for IVLBCL, probably because those samples are collected by punch biopsy (Gill et al , ; Cho et al , ), which, despite being easy to perform, might not contain sufficient amounts of hypodermal adipose tissue for diagnosis. We recently reported the low frequency of IVLBCL lesions in the dermis and showed that most IVLBCL lesions can be found mainly in the hypodermic adipose tissues (Enzan et al , ).…”

“…Since Ferreri et al () comprehensively described the clinical features of IVLBCL 15 years ago, the number of reported cases of IVLBCL has increased in both Asian (Pongpudpunth et al , ; Sitthinamsuwan et al , ) and Western (Cho et al , ) countries. However, due to the small sample size (Arai et al , ), heterogeneity of diagnostic modalities (Ha et al , ; Brunet et al , ), cross‐sectional design in the reported multi‐institutional studies (Ferreri et al , ; Murase et al , ; Shimada et al , ; Brunet et al , ) and early diagnosis by the use of RSB, we feel that the current studies reported in the literature do not depict an accurate clinical representation of IVLBCL.…”

Intravascular large B-cell lymphoma in Hispanics: a case series and literature review

“…In the largest series conducted by Matsue et al [ 12 ], among the forty-two studied patients, twenty-nine (69%) patients were diagnosed via random skin biopsy (RSB) and only eight (19%) patients were diagnosed by BMBx alone. Meanwhile, the use of RSB in healthy-appearing skin still remains controversial [ 16 ]. While abdominal FPB has been used to diagnose amyloidosis as a less invasive approach; it was also useful in diagnosis of IVLBCL [ 17 ].…”

Incisional random skin biopsy, not punch biopsy, is an appropriate method for diagnosis of intravascular large B‐cell lymphoma: a clinicopathological study of 25 patients

“…Cho et al reported the maximum depth of specimens achieved using a 4-mm punch was 4-5 mm. 6 Hence, punch biopsy may fail in detecting IVLBCL lesions because of insufficient specimen depth. Specimen width, too, may reduce the number of lesions detected.…”

“…Although punch biopsy is frequently used to diagnose IVLBCL in Western countries, punch biopsy specimens do not contain a sufficient amount of subcutaneous fat tissue. 6,7 As most IVLBCL lesions are found in subcutaneous fat tissue and lesions occur at low frequency in the dermis, RSB specimens should contain a sufficient amount of subcutaneous fat tissue for diagnosis.…”