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You are watching: Subcutaneous panniculitis-like t-cell lymphoma

StatPearls . Treasure Island (FL): StatPearls Publishing; 2021 Jan-.


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Continuing education and learning Activity

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare major cutaneous lymphoma the mimics panniculitis and also is composed of cytotoxic alpha-beta T-cells. Distinction from the an ext aggressive primary cutaneous gamma delta T-cell lymphoma was made in the 2008 revision of the people Health organization (WHO) category of Tumors that Hematopoietic and also Lymphoid Tissues. Autoimmune conditions occur in approximately 20 percent of cases, and also lupus erythematosus panniculitis (LEP) is generally on the differential diagnosis because of its similar clinical and histologic features. This task reviews exactly how to effectively evaluate because that subcutaneous panniculitis-like T-cell lymphoma and further actions that need to be taken when this condition is present. This activity highlights the function of the interprofessional team in caring because that patients v this condition.

Objectives:
Review the presenting attributes of subcutaneous panniculitis-like T-cell lymphoma.
Describe the review of subcutaneous panniculitis-like T-cell lymphoma.
Explain exactly how to regulate subcutaneous panniculitis-like T-cell lymphoma.
Summarize the crucial role of the interprofessional team in the detection, evaluation, and management the patients through subcutaneous panniculitis-like T-cell lymphoma.
Access cost-free multiple selection questions on this topic.


Introduction

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare major cutaneous lymphoma composed of cytotoxic alpha-beta T-cells the mimics panniculitis<1>. Difference from the more aggressive main cutaneous gamma delta T-cell lymphoma was made in the 2008 review of the world Health company (WHO) classification of Tumours of Hematopoietic and Lymphoid Tissues<1>. Autoimmune diseases occur in around 20% the cases, and also lupus erythematosus panniculitis (LEP) is usually part of the differential diagnosis due to comparable clinical and histologic features<2>.  Histologically, the neoplastic CD8+, beta F1 expressing cytotoxic T-cells characteristically surround and disrupt individual adipocyte membranes. Most cases have a an excellent prognosis and follow one indolent clinical course; however, 15% to 20% of cases are complicated by hemophagocytic syndrome (HPS)<2><1><3><4>.


Etiology

SPTCL is linked with an autoimmune condition in around 20% that cases<5>. The incidence the SPTCL in patients through lupus erythematosus and of lupus erythematosus in patients with SPTCL is greater than the of the general population<4>. Considering this association, some investigators have proposed the 2 entities exist top top the same condition spectrum, and cases with overlapping attributes should it is in called atypical lymphocytic lobular panniculitis<6><4>. Various other explanations include a propensity for patients through one an illness to develop the other or misdiagnosis<7>. Further studies must be excellent to make any type of definitive conclusions. 


Epidemiology

SPTCL account for much less than 1% that non-Hodgkin lymphomas and has a slight predisposition for females.  It occurs in both children and adults v an average age of onset of 36 years. <1><2>


Pathophysiology

Migration that the neoplastic T-cells come the adipocyte membrane is postulated come be helped with by the expression that CCR5 on the neoplastic T-cells.  The ligands because that CCR5 (CCL3, CCL4, and CCL5) are situated on the adipocyte membrane<3>.


Histopathology

There is a pattern of lobular panniculitis that frequently spares the interlobular septa, epidermis, and dermis.  A variably dense infiltrate consists of lymphocytes(neoplastic T-cells and few reactive B-cells), and macrophages are present.  The neoplastic T-cells space atypical through hyperchromatic nuclei and irregular nuclear membranes.  by immunohistochemistry, these neoplastic T-cells refer CD8, beta F1 and also other cytotoxic T-cell markers consisting of granzyme B, perforin, and also TIA1.  these CD8+ T-cells might be sparse but tend to surround or in salt the adipocytes and distort the adipocyte membrane.  The neoplastic T-cells directly adjacent to the adipocytes have a higher Ki67 proliferation index compared to LEP, which has a fairly lower Ki67 proliferation index.  Karyorrhexis, mitoses, and fat necrosis are generally seen<1>.  Some instances have been recorded to have actually mucin deposition, plasma cell aggregates, vacuolar interface changes, follicular plugging, epidermal atrophy, and periadnexal lymphocytes, do the distinction between SPTCL and also LEP difficult at times<8><6><4>.


History and Physical

Patients present with lot of subcutaneous plaques and nodules typically on the lower extremities, top extremities, or trunk <1>. These lesions have actually overlying erythema, room usually painless, and range in size from 0.5-2.0 cm. There may be lesions in various stages of healing, saying a waxing and also waning clinical course <5>. In contrast to main cutaneous gamma delta T-cell lymphoma, ulceration is less usual <3>. 

Systemic symptoms are present in about 50% that cases, including fever, chills load loss, cytopenias, myalgias, and also elevated liver duty tests <1>. These symptoms space seen more commonly in patients through concurrent HPS. Lymph node and bone marrow involvement are not generally encountered <3>. 


Evaluation

The an illness is generally confined come the subcutaneous tissue, however rare instances have disseminated to involve the lymph nodes, blood, and also bone marrow.  Laboratory experimentation reveals one elevated erythrocyte sedimentation price or C-reactive protein. Cytopenias, elevated liver function tests, and also hepatosplenomegaly need to prompt close monitoring for the advance of HPS.  18F-fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) may aid assess the degree of condition upon presentation, solution to treatment, and also detection of relapses<9><5>.

A beat biopsy of the lesion must be submitted for pathologic evaluation, i m sorry will enable for assessment of the area of interest. No certain genetic profile has actually been identified, and the Epstein-Barr virus is commonly not present. Clonality the the neoplastic T-cells deserve to be demonstrated by the identification of T-cell receptor rearrangements utilizing polymerase chain reaction (PCR) or high throughput sequencing<3>. However, clonality can additionally be it was observed in reactive lymphoid infiltrates and specific B-cell lymphomas, so results have to be taken in the context of the clinical scenario<8><6>. One study discovered monoclonal T-cell receptor gene rearrangements in 50% that cases<10>. 


Treatment / Management

Chemotherapy with one or multiple agents to be the conventional of care when SPTCL and primary cutaneous gamma delta T-cell lymphoma were thought to be the same disease.  Disparities in prognosis triggered the distinction of these two entities in the 2008 review of the WHO. No traditional treatment strategy currently exists for SPTCL. Researches have shown that most cases are efficiently treated through systemic corticosteroids or immunosuppressive agents such together etoposide, cyclosporine A, methotrexate, chlorambucil, and bexarotene<3>. 

Conventional doxorubicin-based chemotherapy may be employed as soon as the condition is progressive<5>. Radiation therapy have the right to lead to long term remissions and may have a role in palliation in patients through localized disease. Stem cell transplants can be perform in refractory or disseminated cases<1><3>. In patients v concurrent HPS, high dose corticosteroids v cyclosporine A or a stem cabinet transplant v chemotherapy room options<5>.


Differential Diagnosis

Lupus erythematosus (LE):

Clinically, LE presents much more commonly on the face and proximal extremities, while SPTCL usually reflects on the lower extremities, upper extremities, and also trunk. Findings such as fever and hepatosplenomegaly may favor SPTCL. However, lock can additionally sometimes be it was observed in LE. 

Histologically, LEP and SPTCL might display plenty of of the same features, most notably authorized of the epidermis. More often, LEP shows interface change, a mixture of CD4 and also CD8 T-cells, lymphoid follicles or B-cell aggregates, aggregates the CD123+ plasmacytoid dendritic cells, plasma cabinet aggregates and also intradermal mucin. A low Ki67 proliferation table of contents in the T-cells rimming the adipocytes is another useful attribute for separating SPTCL native LEP, together is the absence of cytologically atypical T-cells<8>. Inflammatory cells, such together eosinophils, neutrophils, and also plasma cells, are more commonly observed in LE<3>. A synthetic of the clinical features, histology, immunophenotyping, molecular analysis, and possibly repeat biopsies may be needed to differentiate the 2 entities. Even so, some instances remain ambiguous<11>. 

Primary cutaneous gamma-delta T-cell lymphoma:

Primary cutaneous gamma delta T-cell lymphoma has actually a preference for middle-aged adults, and the lesions might be more superficial with surface ulceration<1>. Systemic symptoms and lymphadenopathy room common, with HPS emerging in 50% that cases. Histologically, three patterns have the right to be observed: epidermotropic, dermal, and also subcutaneous. The subcutaneous pattern can appear comparable to the lobular panniculitis seen in SPTCL. However, rimming that the adipocytes is less prominent. By immunohistochemistry, the neoplastic cell are positive for CD56, TCR gamma, TIA1, granzyme B, and also perforin and are typically an adverse for CD4, CD8, and beta F1. Clonal rearrangements of the TRG and also TRD genes room present. This entity has a bad prognosis with a five-year survive of 10%.

Extranodal herbal killer/T-cell lymphoma, sleep type: may sometimes current with subcutaneous involvement.  In comparison to SPTCL, this reality is hopeful for EBV<11>.


Prognosis

Most cases have fantastic prognosis and follow an indolent clinical course with a 5-year in its entirety survival rate of 85% come 91%<6><5>.  HPS occurs in 15% to 20% that cases and portends a worse prognosis. More recently, upper extremity involvement is linked with a worse prognosis<10>. 


Complications

HPS complicates around 20% the cases and also carries a 46% 5-year overall survival rate. These patients will certainly require much more aggressive treatment<6>. 


Deterrence and also Patient Education

Subcutaneous panniculitis-like T-cell lymphoma is a well-behaving lymphoid neoplasm of the subcutaneous tissue that can be often efficiently treated through systemic corticosteroids or immunosuppressive agents<3>. 


Neoplastic cells normally express CD8, beta F1, granzyme B, perforin, and TIA1.  CD56 and EBV room negative.

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Enhancing health care Team Outcomes

An interprofessional team-best manages this disease.  The major care, nurse practioner, or emergency room doctor is often the very first to encounter this patients. Notice referral come an oncologist/hematologist is recommended when lymphoma is suspected.  this patients may existing with subcutaneous lesions and sometimes symptoms of HPS.  A an extensive patient evaluation, laboratory tests, and punch biopsy aid clarify the diagnosis.  therapy most commonly includes immunosuppressive agents or systemic corticosteroids. The oncology nurse and also pharmacist have to educate the patient on the adverse impacts of the chemotherapeutic agents and also the term of therapy. Ongoing monitoring of blood occupational is necessary. In addition, patients have to be educated on aseptic techniques, hand washing, and also avoid crowds during treatment.  If HPS is present, therapy will be more aggressive.