Primary Cutaneous Peripheral T-Cell Lymphoma Not Otherwise Specified: A Rapidly Progressive Variant of Cutaneous T-Cell Lymphoma

Abstract

Primary Cutaneous Peripheral T-Cell Lymphoma NOS (PTL-NOS) is a rare, progressive, fatal dermatologic disease that presents with features similar to many common benign plaque-like skin conditions, making recognition of its distinguishing features critical for early diagnosis and treatment (Bolognia et al., 2008). A 78-year-old woman presented to ambulatory care with a single 5 cm nodule on her shoulder that had developed rapidly over 1-2 weeks. Examination was suspicious for malignancy and a biopsy was performed. Biopsy results demonstrated CD4 positivity, consistent with Mycosis Fungoides with coexpression of CD5, CD47, and CD7. Within three months her cancer had progressed into diffuse lesions spanning her entire body. As rapid progression is usually uncharacteristic of Mycosis Fungoides, her diagnosis was amended to PTL-NOS. Cutaneous T-Cell Lymphoma (CTCL) should be suspected in patients with patches, plaques, erythroderma, or papules that persist or multiply despite conservative treatment. Singular biopsies are often nondiagnostic, requiring a high degree of suspicion if there is deviation from the anticipated clinical course. Multiple biopsies are often necessary to make the diagnosis. Physicians caring for patients with rapidly progressive, nonspecific dermatoses with features described above should keep more uncommon forms of CTCL in mind and refer for early biopsy.

A case of primary cutaneous peripheral T‐cell lymphoma, not otherwise specified, with cytotoxic phenotype showing multiple ulcers on the entire body

“…So far, six cases of pcPTCL‐NOS presenting cutaneous ulcer, including our case, have been reported in the literature (Table ) . All cases were presented as generalized multiple ulcers.…”

“…Tolkachjov et al observed that patients were diagnosed a median of 6 months from symptom onset, with a 53% increased risk of death for each year from symptom onset to diagnosis . Therefore, diagnosis requires a high degree of certainty and multiple rebiopsies were often necessary . Most patients were treated with single or multi‐agent chemotherapy and/or radiotherapy, but they were resistant to treatment.…”

“…In addition, new small red papules appeared on the face and trunk, which gradually became ulcerated ( So far, six cases of pcPTCL-NOS presenting cutaneous ulcer, including our case, have been reported in the literature (Table 1). [7][8][9][10][11] All cases were presented as generalized multiple ulcers. Infiltrating lymphoid cells expressed cytotoxic proteins in five of the six cases.…”

“…6 Therefore, diagnosis requires a high degree of certainty and multiple rebiopsies were often necessary. 11 Most patients were treated with single or multi-agent chemotherapy and/or radiotherapy, but they were resistant to treatment. In the short term, among these cases, fatal cases that rapidly progressed were seen.…”

Pegylated liposomal-encapsulated doxorubicin in cutaneous composite lymphoma

“…Our patient suffered from a combination of PTCL-NOS and FBCCL. PTCL-NOS is the most common subtype of PTCL that runs an aggressive course [12] . FBCCL is an indolent lymphoma with good prognosis.…”