lymphoma prognosis | A rare cause of panniculite: Subcutaneous T Lymphoma

A rare cause of panniculite: Subcutaneous T Lymphoma




Adil Boudhas (adilanapath at yahoo dot fr) #, Mohamed Allaoui, Mohamed Reda El Ochi, Mohamed Oukabli, Abderrahmane Al bond
Pathological anatomy service, military hospital of education Mohammed V of Rabat, University Mohammed V, Faculty of medicine and pharmacy of Rabat, Morocco



Summary
Introduction: The lymphomas Subcutaneous T of type panniculite (SPTCL) is a rare entity of lymphomas, characterized by an infiltrate lobular of neoplastic T cytotoxic CD8 + cells with the TCR of phenotype αβ. Comment: We report the case of a young patient who presented a panniculite associated with an alteration of general State and at which SPTCL has been diagnosed on skin biopsy. He received a combination chemotherapy. Conclusion: The evolution of the SPTCL is chronic and their prognosis is good unless hemophagocytosis syndrome.

English Abstract
Introduction: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) are rare. They are characterized by a lobular infiltrates of CD8 + cytotoxic neoplastic T-cells with year α/β + T-cell phenotype. Box: We report a case of a young patient who presented with panniculitis associated to a poor general condition and who has diagnosed with SPTCL we Canada transition. He received polychemotherapy. Conclusion: The progression of SPTCL is chronic with a good prognosis, except in the case of haemophagocytic syndrome.

Introduction
Primitive skin lymphomas represent a heterogeneous group of tumours by their clinical presentation and prognosis. Type panniculite (SPTCL) Subcutaneous T lymphoma is an entity to share among the peripheral T lymphomas, indolent and slow. His diagnosis remains difficult and the problem of differential diagnosis with other lymphomas T close presentation, but to the more formidable prognosis, and with other causes of panniculite, especially the deep lupus.

A rare cause of panniculite: Subcutaneous T Lymphoma Figure 1
Figure 1. Infiltration of adipose tissue by atypical lymphoid elements small to medium in size (HE x 400).
Observation
A man of 32 years, no particular history, was hospitalized before the appearance of an erythematous lesion of the root of the left thigh of rapid expansion, with febrile seizures valued at 41 ° C. At the time of admission, the lesion located at the level of the inner side of the thigh, included an inflammatory area of 7 cm in length to 4 cm wide with a painful indurated area of 4 cm of centerline. There was no skin ulceration, of hypoesthesia of functional impotence at the market, adenopathy, or joint disorder. Biologically, a bicytopenie was found with 2290 leucocytes/mm3 and thrombocytopenia, moderate to 148000/mm3 without anemia (haemoglobinemia to 14.8 g/dL). The C-reactive protein was high (46 mg/L). In addition, the complement of the biological assessment objectivait a moderate increase in plasma triglycerides (2.19 mmol/L), a serum hyperferritinemia (20200 µg/L), an increase of bilirubin total and combined plasma-26 and 17 µmol/L, a plasma rate of KPC to 164 mg/L and a plasmatic LDH rate at 3983 IU/L. The scanner of the root of the lower limbs, abdominal and chest was an infiltration of the subcutaneous fat of the left thigh without abscess, as well as subcutaneous plans and mesenteric fat next to the inguinal port right. There is no abdominal-pelvic, Mediastinal, Lymphadenopathy hepatosplenomegaly or. After eight days of hospitalization, the persistence of the clinical signs (with a fever in plateau around 40 ° C) and organic, despite antibiotics by intravenous way combining oxacillin, gentamicin and metronidazole, led quickly to the realization of a deep skin biopsy. It allowed to observe cell infiltration of density to moderate the deep DermIS, and the hypodermis dissociating the adipocytes, and respecting the superficial DermIS and epidermis. It consisted of many macrophages including a hemophagocytose, and especially a proliferation of round cells of lymphoid nature, small to medium, irregular and hyperchromatic nuclei (figures 1 and 2). These lymphoid cells expressing markers T, as well as CD8, CD4 expressionless, CD56 and CD30 (figures 3 and 4). These morphological aspects, corroborated the Immunohistochemistry profile, could offer the subcutaneous T Lymphoma diagnosis of type panniculite.

Immediately after, there was distribution of skin lesions at the level of the flanks and chest, the exacerbation of the syndrome of macrophagique activation, but the absence of osteo-medullar invasion, leading to propose to this form disseminated, a combination chemotherapy (of type ACVB).

A rare cause of panniculite: Subcutaneous T Lymphoma Figure 2

Figure 2. Immunohistochemistry with anti-CD3 antibody.

Discussion

T of the hypodermis lymphomas are rare and relatively recent identification, described initially by Gonzalez in 1991 [1]. Currently, Subcutaneous T Lymphoma to panniculite type, has been recognized as an entity to share among others of peripheral T-cell lymphomas [2, 3]. They represent less than 1% of non-hodgkiniens lymphoma. They can be seen at any age, but preferably reach young adults around the fourth decade, with a close ratio of 1 [4].

A rare cause of panniculite: Subcutaneous T Lymphoma Figure 3

Figure 3. Immunohistochemistry with antibodies anti-CD8.

Clinically, it presents itself in the form of scattered plates or multiple subcutaneous nodules erythematosus, size varied, sometimes bleeding, or even ulcerated. The lesions are locating preferentially at the level of the ends, especially on the lower limbs and the trunk. There is in principle no ganglionic impairment, except in the late stages of scattered. A syndrome of activation macrophagique (SAM) is associated in almost half of cases, which can manifest as an alteration of the general State (with fever, sweats, and asthenia) and hepatosplenomegaly, peripheral lymphadenopathy, rash skin morbilliform, or even signs of he failure in fulminant forms [1]. Biological anomalies are numerous, non-specific, often majors, leading to evoke the diagnosis of SAM in front of their association (bi - or pancytopenia, disorders of hemostasis, or even real DIC, a cytolysis, or liver failure.)

SPTCL diagnosis is pathological, often difficult, requiring deep bioptic samples. At the histological level, achieving more or less massive subcutaneous tissue is evocative, separating the fat lobules. A less dense infiltration, with scattered cells between the fat lobules and realizing something "lace" should also draw attention. The hypodermis is invaded by an atypical lymphoid population, Pleomorphic, made up of cells Lymphoma of small to large, irregular nuclei, sometimes hidden by macrophagique infiltration, associated phenomena of hemophagocytose. Aspects of cytophagie (intra-BP nuclear debris) or caryorrhexie are constant, with in principle, absence of necrosis adipocytaire [5]. Immunohistochemistry analysis shows that the lymphoma cells express CD2, CD3, CD7, CD8, but not CD4, with a cytotoxic profile (usual expression of TIA - 1, the Perforine and Granzyme B), without expression of CD56. In difficult cases, the use of molecular biology highlights a clone T α/β. These features allow to eliminate Subcutaneous T Lymphoma, often extending to the dermis, type γ/, CD4-, CD8-, + CD56 Immunohistochemistry profile, and worse prognosis [2, 3]. This profile also allows to eliminate other types of lymphoma T that can be accompanied by a subcutaneous location, no exclusive or marginal [6]: "natural killer" (NK) Lymphoma blast; skin T/NK "of nasal type" Lymphoma, characterized by the proliferation of lymphocytes + CD56 expressing CD30 heterogeneously and the Epstein Barr virus; anaplastic Lymphoma; and finally the mycosis fungoides and Lymphoma epidermotrope CD8 + T. However the main diagnosis to eliminate outside a pathology polyposis is the panniculite continuous or deep lupus, where the distinction may appear impossible, so well that the relationship between these two entities remains controversial as their clinical presentation may be similar and accompanied by a SAM SPTCL may be associated dysimmunitaires diseases in nearly 20% of cases [4, 5].

A rare cause of panniculite: Subcutaneous T Lymphoma Figure 4

Figure 4. Infiltration of adipose tissue simulating a panniculite (HES x 200).

The evolution of this type of lymphoma T is special because it is attached to a low scalability, with cases of spontaneous regression, except in the presence of a SAM [2, 4, 6]. Overall prognosis is excellent, with rates of 5-year survival of the order of 82 to 91% [6].

These clinical and evolutionary considerations usually lead to a certain therapeutic restraint, ranging up to the therapeutic abstention in the indolent forms beyond not regular and careful clinical follow-up. In chronic forms, oral corticosteroid therapy may be enough to control the disease, with as an alternative, in the event of poor tolerance, local radiotherapy, the monochimiotherapie at base of methotrexate or Cyclosporine [4]. The combination chemotherapy (CHOP, ESHAP) is reserved for severe forms scattered, along with SAM, as in the reported case.

Conclusion

The SPTCL are rare lymphomas, characterized by a lobular neoplastic T cytotoxic CD8 cells infiltrate the TCR is phenotype αβ. Evolution is chronic and their prognosis is good unless hemophagocytosis syndrome.

At the initial stage, the clinical picture of self-limiting panniculite or percentage associated with an infi ltrat lobular lymphohistiocytaire to the small cell atypies make the diagnosis of LTSP diffi cult.

The careful monitoring of patients with an array of panniculite, the repetition of the deep skin biopsies, analysis complete clinic, scalable, histological, immunophenotypique and molecular is essential to assert the diagnosis of LTSP and differentiate it from the so-called "benign" used

Statement

Conflicts of interest

There is no conflict of interest regarding the data published in this article between the authors.

Contributions of the authors

All authors have contributed to the development of this work on all its steps since the acquisition and exploitation of data, up to the writing and revision and final approval of the version of this article. All the authors contributed to the conduct of this work. The authors also declare having read and approved the final version of the manuscript.