Primary cutaneous gamma-delta T-cell lymphoma masquerading as severe soft-tissue infection
Nilay G Yalcin MBBS,1 Rodrigo P Teixeira MBBS FRACS (Plas)1,2
Northern Hospital Epping
Faculty of Medicine, Dentistry and Health Sciences
Name: Dr Nilay Yalcin
Address: Department of Plastic Surgery
Northern Hospital Epping
185 Cooper Street
Epping, Victoria, 3076
Phone: +61 (0) 421 247 205
Citation: Yalcin NG, Teixeira RP. Primary cutaneous gamma-delta T-cell lymphoma masquerading as severe soft tissue infection. Aust J Plast Surg. 2019;2(2):76–78. https://doi.org/ 10.34239/ajops.v2n2.108
Accepted for publication: 23 July 2019
Copyright © 2019. Authors retain their copyright in the article. This is an open access article distributed under the Creative Commons Attribution Licence which permits unrestricted use, distribution and reproduction in any medium, provided the original work is properly cited.
Section: General reconstruction
Accurate diagnosis, and prompt treatment, of a severe necrotising soft-tissue infection is one of the few diagnoses in plastic surgery that, if managed correctly, can be life-saving. A high degree of awareness and medical knowledge of this condition is important for the assessing surgeon who will determine the course of treatment. Early clinical findings of necrotising fasciitis may be limited to pain, mild swelling and redness. However, serial examinations will show failure to respond to antibiotics and progressive systemic signs of sepsis including haemodynamic instability, acidosis, leucocytosis and hyperthermia. The progression of a necrotising soft-tissue infection is variable and multifactorial. Most commonly, it presents with rapid progression and is life-threatening.
Differentiating necrotising infections from other soft-tissue infections can be challenging, especially in the early stages. In addition, other differential diagnoses, including non-infectious conditions, should be considered. Described below is the diagnostic conundrum of a patient presenting with a rare type of cutaneous lymphoma, primary cutaneous gamma-delta T-cell lymphoma (PCGDTL) associated with haemophagocytic lymphohistiocytosis (HLH), mimicking a necrotising soft-tissue infection.
Keywords: lymphohistiocytosis, hemophagocytic, diagnosis, differential, sepsis
A 21-year-old male presented with one week of high-grade fevers (>39 ˚C) and right thigh swelling. He was a smoker with no other significant medical history. On admission, the right thigh was painful and indurated. Due to clinical deterioration, concerns were raised for a necrotising soft-tissue infection, for which the patient was urgently taken to theatre.
Intraoperatively, an incision was made over the swollen area on the right thigh. Macroscopic exploration revealed the normal appearance of fascia and skin layers. Multiple biopsies were taken and the wound was dressed with a negative-pressure dressing. Despite continuous medical therapy and the innocuous surgical and pathological findings, the patient became haemodynamically unstable, requiring intensive care and ionotropic support. A second exploration was warranted. This revealed large amounts of grey, non-viable areas of subcutaneous tissue. However, the underlying fascia remained pristine. The abnormal areas of subcutaneous tissue were resected and sent for histopathology, which was reported as unspecific fat necrosis. Tissue and blood cultures showed no evidence of a micro-organism. In intensive care, the patient continued to deteriorate with multi-organ failure and disseminated intravascular coagulation requiring mass blood transfusion. He then gradually stabilised and was transferred to the ward under the multidisciplinary care of plastic surgery, general medicine and infectious diseases.
Several weeks into his admission, with ongoing high-grade fevers and negative cultures, the patient developed multiple areas of non-tender plaque-like rash over his torso and limbs (Figure 1). Punch biopsies of the newly formed skin lesions revealed the diagnosis of primary cutaneous gamma-delta T-cell lymphoma (PCGDTL) associated with haemophagocytic lymphohistiocytosis (HLH). In light of this, the initial intraoperative biopsy samples were re-reviewed by expert dermatopathologists who confirmed that PCGDTL was the missed diagnosis (Figure 2). The patient’s symptoms and fever settled once chemotherapy commenced.
Fig 1. Left forearm of patient showing the rash that was biopsied to diagnose PCGDTL
Fig 2. Arrow showing lymphocytic infiltrate in between fat cells in the subcutis suggestive of cutaneous T-cell lymphoma with evidence of haemophagocytosis
For this patient, the PCGDTL presented as disseminated subcutaneous deposits with swelling, pain and erythema but intact skin. Primary cutaneous gamma-delta T-cell lymphoma may, in other cases, affect skin integrity, leading to ulceration or necrosis. It is more commonly located on the extremities but can also appear on mucosa and other extra-nodal sites.1,2 The high-grade fevers that our patient experienced can be attributed to HLH, which is due to the uncontrolled activation of the immune system by abnormal proliferation of T-cells and activation of macrophages and histiocytes. The soft-tissue swelling, accompanied by high-grade fever, increasing inflammatory markers and progressive clinical deterioration, mimicked necrotising fasciitis. However, soft-tissue infection was ruled out for our patient as no microorganism was cultured and his symptoms did not improve with antibiotics or surgical debridement.
Repeating and reviewing previous investigations is crucial in the context of unexpected clinical progress with new or ongoing symptoms. Likewise, this case highlights the importance of a multidisciplinary approach in treating complex clinical presentations, which may help clinicians to diagnose rare conditions.
Until recently, PCGDTL was considered a subtype of subcutaneous panniculitis-like T-cell lymphoma (SPLTCL).3 However, due to the aggressive nature, and significantly worse prognosis of the gamma-delta subtype, in 2008 the World Health Organization drew a distinction between the more indolent SPLTCL and PCGDTL.4 The rare and significantly more aggressive PCGDTL represents less than 1 per cent of all primary cutaneous T-cell lymphomas.5 Although our patient was younger, the median age of onset is 60 years, with a range between 25 and 91 years in previous reports.6 As in our patient’s case, PCGDTL may be associated with HLH, especially in the presence of panniculitis-like tumours.1,2 This subtype of T-cell lymphoma has a poor prognosis with a median survival time of only 15 months.1,2 Treatment is with chemotherapy; however, PCGDTL is often resistant to multi-agent chemotherapy. The prognosis is worse when the condition is associated with HLH.
When treating a possible severe soft-tissue infection, clinical vigilance and a multidisciplinary approach are essential to provide the best clinical setting to manage complex cases and are both key when facing diagnostic dilemmas. In addition, another aetiology mimicking infectious conditions, such as a rare primary cutaneous lymphoma, should be considered among the differential diagnoses.
The corresponding author is not a recipient of a research scholarship. This paper is not based on previous communication to a society or meeting. The authors have no financial or commercial interests to disclose.
- Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, Canninga-van Dijk MR, Carlotti A, Geerts ML, Hahtola S, Hummel M, Jeskanen L, Kempf W, Massone C, Ortiz-Romero PL, Paulli M, Petrella T, Ranki A, Peralto JL, Robson A, Senff NJ, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ, EORTC Cutaneous Lymphoma Group. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC cutaneous lymphoma group study of 83 cases. Blood. 2008;11:838–45 https://doi.org/10.1182/blood-2007-04-087288
- Toro JR, Liewehr DJ, Pabby N, Sorbara L, Raffeld M, Steinberg SM, Jaffe ES. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood. 2003;101:3407–12 https://doi.org/10.1182/blood-2002-05-1597
- Gonzalez CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue. Am J Surg Pathol. 1991;15:17–27. https://doi.org/10.1097/00000478-199101000-00002
- Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pilera SA, Stein H, Thiele J, Vardiman JW. WHO classification of tumors of haematopoietic and lymphoid tissues, 4th ed. Lyons: IARC Press, 2008.
- Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768–85. https://doi.org/10.1182/blood-2004-09-3502
- Guitart J, Weisenburger DD, Subtil A, Kim E, Wood G, Duvic M, Olsen E, Junkins-Hopkins J, Rosen S, Sundram U, Ivan D, Selim MA, Pincus L, Deonizio JM, Kwasny M, Kim YH. Cutaneous gamma-delta T-cell lymphomas: a spectrum of presentations with overlap with other cytotoxic lymphomas. Am J Surg Pathol. 2012;36:1656–65. https://doi.org/10.1097/PAS.0b013e31826a5038