T-cell Lymphoma Presenting Research Articles

Peripheral T-cell lymphoma presenting with eosinophilia due to Interleukin-5 produced by lymphoma cells

Peripheral T-cell lymphoma presenting with eosinophilia due to Interleukin-5 produced by lymphoma cells

A rare case of enteropathy-associated T-cell lymphoma presenting as acute renal failure

Enteropathy-associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma usually with cytotoxic phenotypes. We describe a first case of patient with EATCL that is remarkable for its fulminant course and invasion of both kidneys manifested as acute renal failure. The patient was a 23 year old woman with a long history of celiac disease. She was presented with acute renal failure and enlarged mononuclear infiltrated kidneys. Diagnosis of tubulointerstitial nephritis and polyserositis was confirmed with consecutive pulse doses of steroid therapy. After recovery, she had disseminated disease two months later. Magnetic resonance imaging showed thickened intestine wall, extremely augmented kidneys, enlarged intra-abdominal lymph nodes with extra-luminal compression of common bile duct. Laparotomy with mesenterial adipous tissue and lymph glands biopsy was done. Consecutive pathophysiological and immunohistochemical analyses confirmed the diagnosis of EATCL: CD45RO+, CD43+, CD3+. The revision of renal pathophysiology substantiated the diagnosis. The patient received chemotherapy, but unfortunately she died manifesting signs of pulmonary embolism caused by tumor cells.

A Rapidly Fatal Case of T-Cell Lymphoma Presenting as Idiopathic Orbital Inflammation

A 41-year-old Caucasian woman presented with a painful, red right eye with minimal systemic symptomatology, and was initially diagnosed with right idiopathic orbital inflammation. Ten days later, she developed abdominal and respiratory symptoms; this led to her demise within a further week. Post-mortem examination demonstrated widespread extranodal NK/T- cell lymphoma (nasal type), involving the right posterior orbit, lungs, uterus, left adrenal gland, pericardium and meninges. Thorough physical examination with early orbital biopsy should be considered to exclude underlying treatable pathology in managing patients with presumed idiopathic orbital inflammation.

Alemtuzumab: effective monotherapy for simultaneous B‐cell chronic lymphocytic leukaemia and Sézary syndrome

The simultaneous presentation of chronic lymphocytic leukaemia (CLL) and cutaneous T-cell lymphoma (CTCL) is a very rare occurrence where optimal treatment is unknown. We present the case of a 65-yr-old man who was successfully treated with alemtuzumab monotherapy for both disorders, but at a cost of severe infectious morbidity and prolonged pancytopenia.

T-cell lymphoma presenting as acute mastoiditis with a facial palsy

Isolated T-cell lymphomas affecting only the mastoid are extremely rare. Presentation with oto-neurological signs prior to systemic involvement of a lymphoproliferative disease is also unusual. This is the youngest reported patient with a peripheral T-cell lymphoma with disease isolated only in the mastoid who presented with acute mastoiditis and a complete seventh cranial nerve palsy.

Epstein-barr virus-associated intravascular large t-cell lymphoma presenting as acute renal failure in a patient with acquired immune deficiency syndrome

Intravascular lymphoma (IVL) is a rare neoplasm, recently included as a specific entity in the World Health Organization classification of lymphoid tumors. Most cases are of B-cell lineage; however, rare cases of T-cell phenotype have been reported. We report a human immunodeficiency virus (HIV)-positive patient who died of acute renal failure in whom IVL was identified at autopsy, predominantly involving the renal interstitial vessels. Immunohistochemical stains revealed a T-cell phenotype, which was confirmed by T-cell receptor gamma gene rearrangement studies. The lymphoma cells showed nuclear Epstein-Barr virus (EBV)-encoded RNA transcripts by in situ hybridization, suggesting that EBV might be of etiologic importance in this tumor. The predominant involvement of kidney is unusual. With effective therapy, morbidity and mortality of HIV-1 infection has been substantially reduced, and survival times have been prolonged. However, the relative risk of secondary neoplasms, especially non-Hodgkin’s lymphoma (NHL), has increased. Consequently, we conclude that unique types of NHL, such as this case of IVL, may be encountered more frequently in this patient population, and that NHL should be added to the list of differential diagnostic considerations in HIV-1-positive patients who develop acute renal failure.

Blastic natural killer cell and extranodal natural killer cell-like T-cell lymphoma presenting in the skin: report of six cases from the UK.

Some lymphomas express natural killer (NK)-cell markers such as the neural cell adhesion molecule, which is recognized by the CD56 antibody. These lymphomas may present in the skin, but do not represent a homogeneous group. The new World Health Organization classification of lymphoma/leukaemia recognizes several types of NK/T-cell neoplasm, including blastic NK-cell lymphoma, which characteristically presents with cutaneous lesions. To describe the clinical, pathological and molecular features in six cases of CD56+ lymphoma with cutaneous presentation. The clinical, histopathological and immunophenotypic features of six patients were reviewed. In addition, in situ hybridization (ISH) to identify Epstein-Barr virus (EBV) mRNA, and polymerase chain reaction analysis to identify the presence of a clonal population of T cells or B cells were performed on lesional skin. All patients presented with widespread nodules and plaques, which in five cases were a characteristic purple colour. Four patients developed disseminated disease, three with neurological involvement. These four patients died between 14 and 46 months following diagnosis (median 30 months). In four of six cases the histopathological and immunohistological features were in keeping with a blastic NK-cell lymphoma. No clonal immunoglobulin heavy chain (IgH) or T-cell receptor (TCR) gene rearrangement was detected in the four cases consistent with an origin from NK cells. A further case fitted the criteria for an extranodal NK/T-cell lymphoma of nasal type and was also the only case to show evidence of EBV mRNA by ISH. A clonal T-cell population was identified in the final case. This patient also exhibited molecular evidence of a clonal B-cell population and a t(14;18) translocation confirmed by sequence analysis. Our data confirm that NK-cell lymphomas presenting in the skin are a heterogeneous group, and that in the U.K., blastic NK-cell lymphoma is more common than extranodal NK/T-cell lymphoma of nasal type. These lymphomas pursue an aggressive course, with rapid development of disseminated disease, and resistance to chemotherapy. Detailed immunophenotyping is needed to distinguish the different types. Our molecular data indicate that blastic NK-cell lymphoma cases lack clonal TCR/IgH gene rearrangements consistent with an NK-cell origin. Our ISH findings indicate that EBV plays a pathogenetic role only in extranodal NK/T-cell lymphoma of nasal type.

Peripheral T-cell Lymphoma Presenting with Rapidly Progressing Myelofibrosis

Myelofibrosis following peripheral T-cell lymphoma has rarely been reported. Described here is a case of peripheral T-cell lymphoma with myelofibrosis and elevated transforming growth factor beta (TGF- β ). A 69 years old male was admitted due to anemia and thrombocytopenia. His bone marrow showed fibrosis and was infiltrated with small lymphoid cells and a few residual normal hematopoietic cells. He had presented with hepatosplenomegaly and left inguinal lymph node swelling. Biopsy of the left inguinal lymph node revealed diffuse mature small lymphoid cells with atypical nuclei. Immunophenotyping of the small lymphoid cells were positive for CD3, CD8, TCR α β and HLA-DR and were negative for CD4, CD19, CD20 and CD56. T-cell receptor β -chain gene was rearranged in bone marrow cells. He was diagnosed as having peripheral T-cell lymphoma complicated with myelofibrosis. Chemotherapy was administrated which improved his pancytopenia and symptoms. Two years later, anemia and thrombocytopenia developed rather quickly, he died because of progression of myelofibrosis with severe pancytopenia.

Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course.

To describe and identify the clinical and pathologic features of prognostic significance for natural killer (NK) and NK-like T-cell (NK/T-cell) lymphoma presenting in the skin. This study was a retrospective review of 30 patients with CD56+ lymphomas initially presenting with cutaneous lesions, with analysis of clinical and histopathologic parameters. The median survival for all patients was 15 months. Those with extracutaneous manifestations at presentation (11 patients) had a shorter median survival of 7.6 months as compared with those without extracutaneous involvement (17 patients), who had a more favorable median survival of 44.9 months (P =.0001). Age, gender, extent of cutaneous involvement, and initial response to therapy had no statistically significant effect on survival. Seven patients (24%) had detectable Epstein-Barr virus (EBV) within neoplastic cells. The patients with tumor cells that coexpress CD30 (seven patients) have not yet reached a median survival after 35 months of follow-up as compared with those with CD30- tumor cells (20 patients), who had a median survival of 9.6 months (P <.02). Routine histopathologic characteristics had no prognostic significance nor did the presence of CD3epsilon, EBV, or multidrug resistance. NK/T-cell lymphoma is an aggressive neoplasm; however, a subset with a more favorable outcome is identified in this study. The presence of extracutaneous disease at presentation is the most important clinical variable and portends a poor prognosis. The extent of initial skin involvement does not reliably predict outcome. Patients from the United States with NK/T-cell lymphoma presenting in the skin have a low incidence of demonstrable EBV in their tumor cells. Patients with coexpression of CD30 in CD56 lymphomas tend to have a more favorable outcome.

Cytotoxic T‐cell lymphoma presenting as secondary myelofibrosis with high levels of PDGF and TGF‐β

Cytotoxic T‐cell lymphoma presenting as secondary myelofibrosis with high levels of PDGF and TGF‐β

Cutaneous T‐cell lymphoma presenting as disseminated, pigmented, purpura‐like eruption

A 48-year-old man with a history of psoriasis, who had received no oral pharmacologic treatment, presented in March 2000 with a progressive, cutaneous, pruritic eruption that began on his arms and legs, and became generalized. Examination revealed discrete, purpuric elements disseminated over the entire cutaneous surface, and in some areas confluent, large, erythematous, purpuric patches (1, 2). The palmoplantar surfaces and mucous membranes were spared. No lesions suggesting parapsoriasis or the usual presentation of cutaneous T-cell lymphoma (CTCL) were present. Figure 1Open in figure viewerPowerPoint Confluent, large, erythematopurpuric patches with sharply pointed purpuric elements on the buttocks Figure 2Open in figure viewerPowerPoint Similar lesions on the lower limbs Histopathologic examination of a cutaneous specimen showed numerous atypical lymphocytes in the superficial dermis, slight changes at the dermo-epidermal junction, and an intense epidermotrophism, with atypical lymphocytes in the epidermis. These intraepidermal atypical lymphocytes were present both isolated and forming aggregates of 4-7 cells (Fig. 3). Immunohistochemical studies demonstrated that these lymphocytes were T-helper cells. Figure 3Open in figure viewerPowerPoint Atypical lymphocytes in the superficial dermis, slight changes at the dermo-epidermal junction, and intense epidermotrophism, with atypical lymphocytes. Intraepidermal lymphocytes were present both isolated and forming small cellular aggregates Routine laboratory tests were normal, except for slight hypercholesterolemia. Direct examination of peripheral blood, coagulation, antinuclear antibody (ANA) immunofluorescent test, body scanner, and bone marrow study were normal. CTCL stage IB was diagnosed and psoralen plus UVA (PUVA) therapy was initiated.

Cutaneous T-cell lymphoma presenting as disseminated, pigmented, purpura-like eruption

Cutaneous T-cell lymphoma presenting as disseminated, pigmented, purpura-like eruption

Treatment of Cutaneous T-Cell Lymphoma from a Dermatologist's Perspective

Mycosis fungoides, the most common form of cutaneous T-cell lymphoma, is a helper/memory epidermotrophic T-cell lymphoma presenting as skin lesions. At the current time, curative therapy does not exist, and many patients have chronic skin lesions for many years, with successful treatment limited to the skin. Mycosis fungoides appears to start as a human lymphocyte antigen–restricted immune response, which may be antigen or superantigen driven, in early stages. From a dermatologist's perspective, removing the stimulating antigen(s), treating infections, preserving the skin barrier, targeting the abnormal clone, preserving the cytotoxic response, and using skin-directed therapy early in the disease are sensible strategies. As the disease progresses to involve more of the skin surface, systemic therapies, especially biological response modifiers (interferon and retinoids), phototherapy, or photopheresis help to preserve the patients' innate immunity and are widely used. New agents including bexarotene (a rexinoid) and DAB389IL-2 (interleukin-2 diphtheria fusion protein) offer new therapeutic options that are advantageous for treatment of mycosis fungoides in later stages.

T-Cell Lymphoma Presenting in the Breast: A Histologic, Immunophenotypic and Molecular Genetic Study of Four Cases

Primary non-Hodgkin's lymphoma of the breast is uncommon. Most primary breast lymphomas are of B-cell phenotype, with only rare cases showing a T-cell phenotype. In this study, we report the clinicopathologic features of four cases of T-cell lymphoma in the breast. The patients all were female with a mean age of 48 years (range, 13 to 77 years). All cases showed immunoreactivity in paraffin-embedded tissue for T-cell markers CD3, CD45RO, and CD43. βF1 was positive in three of four cases. The four cases were further subclassified as anaplastic large cell lymphoma (CD30 positive) of T-immunophenotype; natural killer/T-cell lymphoma; peripheral T-cell (CD4 positive), large cell type; and peripheral T-cell (CD8 positive, T-cell intracellular antigen positive), medium cell type. Three of the four cases were monoclonal for T-cell receptor β and/or T-cell receptor γ. The one case of natural killer/T-cell lymphoma was negative for monoclonality with both T-cell receptor β and γ by molecular diagnostic studies. In all cases, IgH was negative. Follow-up was obtained in three cases. Two patients died within less than 1 year after the diagnosis. The third patient died within 18 months of the diagnosis. Our results suggest an aggressive clinical course for T-cell lymphomas that present in the breast.

Angioimmunoblastic T-cell lymphoma presenting with rapidly increasing biclonal gammopathy

Angioimmunoblastic T-cell lymphoma presenting with rapidly increasing biclonal gammopathy

Intestinal T-cell Lymphoma: A Case Report

A case of intestinal T-cell lymphoma (enteropathy- associated T-cell lymphoma) presenting in a 77 year old female is reported, and the usual clinical presentation, pathologic findings, and differential diagnosis of this uncommon entity are discussed. Because the morphologic appearance on H&E sections can be mimicked by other disease processes, it is imperative to document the nature of the malignant cells before an accurate diagnosis can be sendered. Paraffin section immunohistochemistry is ideal for this purpose, and its usefulness in the diagnosis of this entity is illustrated. The lymphoid nature of the tumor is confirmed by positive immunostaining with CD45 and negative markers for carcinoma (cytokeratin) and melanoma (S 100 and HMB-45). The T-cell nature of the tumor is reflected in the reactivity with CD3, CD43, and CD45R0, and B-cell markers (CD20 and CD79a) are negative. CD30 and EMA are positive, a common finding in these tumors. (The J Histotechnol 22:129, 1999)

Adnexotropic T-cell lymphoma presenting with generalized anhidrosis, progressive alopecia, pruritus, and Sjögren’s syndrome

We describe a unique presentation of T-cell lymphoma associated with generalized anhidrosis, alopecia, pruritus, and Sjögren’s syndrome (SS). T-cell receptor (TCR) gene rearrangement studies implicated a clonal, malignant T-cell process as responsible for destruction of both cutaneous adnexae and the parotid gland.

Plantar granulomatous cutaneous T-cell lymphoma

We describe a distinctive case of granulomatous cutaneous T-cell lymphoma presenting as an eczematous eruption with livedoid ulcerations involving the soles and dorsa of the feet as the first and only manifestation. (J Am Acad Dermatol 1997;37:326-8.)

Plantar granulomatous cutaneous T-cell lymphoma

We describe a distinctive case of granulomatous cutaneous T-cell lymphoma presenting as an eczematous eruption with livedoid ulcerations involving the soles and dorsa of the feet as the first and only manifestation. (J Am Acad Dermatol 1997;37:326-8.)

Sinonasal T-cell Lymphoma and Wegener's Granulomatosis: Aspects in Early Differential Diagnosis

The clinical course of 12 patients with sinonasal T-cell lymphoma retrospectively diagnosed using in situ hybridization for Epstein-Barr virus RNA was compared with that of 10 recently treated patients with Wegener's granulomatosis (WG) in the upper airways. In particular, we studied the presenting signs and symptoms of both diseases, which commonly offer a problem in differential diagnosis at the clinical and pathological level. A bimodal age distribution was suggested in both T-cell lymphoma and WG; five patients with T-cell lymphoma developed disease prior to 40 years of age. Four of the 12 lymphoma patients had a history of “chronic rhinitis” for several years before developing mucosal ulcerations, which were initially unilateral, as opposed to the bilateral ulcerations in early sinonasal WG. Two lymphoma patients had swelling of the nasal dorsum and cheek. In contrast to the WG patients, cases of T-cell lymphoma did not exhibit associated clinical signs of arthritis, conjunctivitis, pulmonary lesions, or nephritis in the early stage of the disease. Nine of the patients with T-cell lymphoma presenting as a sinonasal lesion developed disseminated disease, variably including infiltrates in intestine, lung, CNS, and skin. Four of these patients died from gastrointestinal complications of their disease. We conclude that unilateral ulcerative or hemorrhagic polypoid mucosal lesions in the sinonasal area are suggestive of lymphoma rather than WG, and nonspecific symptoms, at least in Western patients, may be present as early as the second or third decade of life. A biopsy specimen containing T lymphocytes positive for the EBV ribonucleoprotein EBER1 on in situ hybridization offers reliable confirmation of T-cell lymphoma and is of differential diagnostic value against WG.