The pathophysiology and current treatments for the subcutaneous panniculitis‐like T cell lymphoma: An updated review

Abstract

Subcutaneous panniculitis‐like T cell lymphoma (SPTCL) is a rare cutaneous T cell lymphoma, which is indolent in nature but could claim life if not correctly diagnosed and promptly treated. SPTCL is usually presented clinically as painless subcutaneous and erythematous nodules over the trunk or extremities. Active clinical vigilance for these subcutaneous nodules or panniculitis‐like lesions is warranted. A biopsy must be performed in order to make a correct diagnosis. Positron emission tomography scan is utilized for disease staging and treatment follow‐up. Due to the rarity of this lymphoma, a standard treatment protocol is not established yet. However, most cases of SPTCL could be treated well under immunosuppressive or polychemotherapeutic drugs except in cases with hemophagocytic syndrome. Hematopoietic stem cell transplantation may be used in refractory or relapse cases. In this review, we presented a case of SPTCL with long‐term complete remission. Meanwhile, since most clinical evidences and experiences of SPTCL are based mostly on case reports or small case series, and the understanding of the SPTCL pathophysiology is limited, we reviewed and updated the pathophysiology and treatments of SPTCL.

A case report and literature review: subcutaneous panniculitis-like T-cell lymphoma with liver failure as the main manifestation

“…Although our patient had a fatty liver, it did not explain her severe liver failure, and SPTCL was considered more likely to be the cause of her liver failure. This phenomenon is inconsistent with the mild liver injury common to SPTCL, such as elevated liver enzymes (10,12,18). A trans-jugular vein hepatic puncture test can help us to identify the cause of liver failure.…”

“…The primary clinical manifestation is non-specific subcutaneous nodules, which are easily misdiagnosed. The cutaneous lesions are usually on the trunk and extremities, and can sometimes be observed on the face, neck, axilla, groin, and hip (4,(9)(10)(11)(12). SPTCL predominantly presents as melasma to reddish subcutaneous plaques and nodules without tenderness, which can vary in size (diameter, 0.5-0.9 cm) (4,10,13-16).…”

“…The cutaneous lesions are usually on the trunk and extremities, and can sometimes be observed on the face, neck, axilla, groin, and hip ( 4 , 9 - 12 ). SPTCL predominantly presents as melasma to reddish subcutaneous plaques and nodules without tenderness, which can vary in size (diameter, 0.5–0.9 cm) ( 4 , 10 , 13 - 16 ). Furthermore, there is no obvious lymph node involvement as well as little ulceration and skin disease ( 10 , 17 ).…”

“…SPTCL predominantly presents as melasma to reddish subcutaneous plaques and nodules without tenderness, which can vary in size (diameter, 0.5–0.9 cm) ( 4 , 10 , 13 - 16 ). Furthermore, there is no obvious lymph node involvement as well as little ulceration and skin disease ( 10 , 17 ).…”

Subcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report

“…SPTCL is associated in 25% of patients [ 2 ] with preceding autoimmune disorders, particularly lupus erythematosus, juvenile rheumatoid arthritis, and Kikuchi disease [ 10 ]. Hemophagocytic lymphohistiocytosis, being characterized by overt systemic inflammation, hypercytokinemia, and multiple organ failure, has been reported in 15–37% of SPTCL patients [ 18 , 19 ]. Wu et al [ 16 ] reported a case with SPTCL who was previously diagnosed with LEP.…”