T-cell Lymphoma Presenting Research Articles

Angioimmunoblastic T-cell Lymphoma Presenting With a Neutrophilic Leukemoid Reaction: A Diagnostic Challenge

Angioimmunoblastic T-cell lymphoma (AITL) accounts for 1-2% of all non-Hodgkins lymphoma. Patients present with lymphadenopathy, hepatosplenomegaly, polyclonal hypergammaglobulinemia and skin lesions. The presence of rash and neutrophilia in the absence of circulating abnormal lymphocytes in the peripheral blood film leads to a misdiagnosis. A 33-year-old presented with cervical lymphadenopathy with WCC of 104.9 g/dl, maculopapular rash, loss of weight and appetite associated with B symptoms. His peripheral blood film revealed hyperleukocytosis with a neutrophilic leukemoid reaction. Neutrophil alkaline phosphatase (NAP) score was high. Bone marrow aspirate and trephine were suggestive of neutrophilic leukemoid reaction with features of granulocytic hyperplasia with no lymphoid aggregates suggestive of neutrophilic leukemoid reaction secondary to underlying malignancy with a differential of myeloproliferative neoplasm if no reactive causes of granulocytic hyperplasia could be found. A lymph node biopsy was done later and was consistent with AITL. This case illustrates the rare presentation of AITL with a neutrophilic leukemoid reaction in the absence of anaemia, eosinophilia and abnormal lymphoid cells.

Vitritis as ocular presentation of t-cell primary CNS lymphoma (PCNSL)

Vitritis as ocular presentation of t-cell primary CNS lymphoma (PCNSL)

Angioimmunoblastic T-cell lymphoma presenting as extensive pseudovesicular papuloplaques

Angioimmunoblastic T-cell lymphoma presenting as extensive pseudovesicular papuloplaques

Vacuolated lymphocytes as initial presentation of angioimmunoblastic T-cell lymphoma.

Vacuolated lymphocytes as initial presentation of angioimmunoblastic T-cell lymphoma.

Primary CNS T-Cell Lymphoma Presenting as a Brainstem Lesion in a Young Female

Primary CNS T-Cell Lymphoma Presenting as a Brainstem Lesion in a Young Female

18F-Labeled Fluoro-2-Deoxyglucose Positron Emission Tomography and Computed Tomography Findings in a Case of Atypical Presentation of Adult Cutaneous T-cell Lymphoma.

Adult cutaneous T-cell lymphoma is an uncommon malignancy with poor prognosis and is usually seen in association with human T-cell lymphotropic virus type 1. We present the case of a 25-year-old female who gave a history of extensive whole-body polypoidal cutaneous and bilateral orbital and breast swellings. Biopsy was suggestive of cutaneous T-cell lymphoma and the patient was evaluated with 18F-labeled fluoro-2-deoxyglucose positron emission tomography and computed tomography for initial staging of the disease.

Subcutaneous Panniculitic T-cell Lymphoma Presenting With Anasarca in a Patient With Known Chronic Lymphocytic Leukemia

Subcutaneous Panniculitic T-cell Lymphoma Presenting With Anasarca in a Patient With Known Chronic Lymphocytic Leukemia

Subcutaneous panniculitis-like T-cell lymphoma presenting as an ulcerated plaque: A case report

Subcutaneous panniculitis-like T-cell lymphoma presenting as an ulcerated plaque: A case report

F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in a Case of Subcutaneous T-cell Lymphoma Presenting as Breast Mass and Panniculitis-Like Features: A Rare Entity.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an unusual subtype of cutaneous lymphoma and clinically appears as erythematous, subcutaneous infiltrates, and recurrent papulonodules. It is defined as a rare cytotoxic a/b T-cell lymphoma characterized by primary involvement of subcutaneous tissue-mimicking panniculitis and a predominant CD3+/CD4/CD8+ phenotype in (2005) the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. On metabolic imaging, these lesions are invariably fluorodeoxyglucose (FDG) avid. SPTCL which presents as a breast mass is a rare entity. Here, we present a case of SPTCL presents as FDG avid breast mass and subcutaneous nodules.

Diagnostic testing of eczematous dermatitis with incomplete response to dupilumab

Diagnostic testing of eczematous dermatitis with incomplete response to dupilumab

Tre-P-19 - Autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement: follow-up

Mycosis fungoides (MF) is a presentation of primary cutaneous T-cell lymphoma with adverse prognosis for patients with advanced stages of the disease. Refractory disease and advanced-stage disease require systemic therapy. We reported a rare case of an atypical predominantly CD8+ folliculotropic MF, a subtype of MF with poorer prognosis, in a 59-year-old woman. She was initially diagnosed with MF restricted to the skin, of T3N0M0B0/stage IIB according to the current World Health Organization–European Organisation for Research and Treatment of Cancer classification. First-line treatment with local percutaneous radiotherapy in combination with systemic interferon alfa-2a resulted in complete remission. However, 21 months later the disease progressed to T3N0M1B0/stage IVB with development of cerebral manifestation and thus very poor prognosis. Allogeneic stem cell transplantation (SCT) was not a therapeutic option due to the lack of a suitable donor. We initiated methotrexate and cytarabine chemotherapy, followed by high-dose chemotherapy with thiotepa and carmustine with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Readministration of interferon alfa-2a as a maintenance treatment after the salvage autologous SCT resulted in a complete remission during a follow-up period of 32.5 months after autologous SCT. After that, the disease relapsed with simultaneous skin and cerebral lesions. Despite immediate intrathecal and subsequently intravenous administration of high-dose methotrexate and cytarabin, we observed further progressed and the patient deceased 3.5 months after this second fulminant recurrence and in total 36 months after autologous SCT transplantation.

S3015 Primary Gastrointestinal T-cell Lymphoma Presenting as Spontaneous Perforation Peritonitis: A Case Report

S3015 Primary Gastrointestinal T-cell Lymphoma Presenting as Spontaneous Perforation Peritonitis: A Case Report

The first case of methotrexate‐associated lymphoproliferative disorder presenting as follicular T‐cell lymphoma

This is the first case of follicular T-cell lymphoma (FTCL) presenting as methotrexate-associated lymphoproliferative disorders (MTX-LPDs). A 69-year-old man treated rheumatoid arthritis with methotrexate presented with cervical swelling, hoarseness and fever. Imaging studies revealed multiple lymphadenopathy and lymphoma was suspected. Lymph node biopsy was performed to confirm the diagnosis. Pathologically, the lymph node was composed of atypical lymphocytes with a follicular growth pattern and area of necrosis. Immunohistochemical examination showed the atypical lymphocytes were positive for CD3, CD4, programmed cell death protein 1, and inducible T-cell co-stimulator. These findings are consistent with FTCL. During hospitalization, the patient's fever subsided and cervical lymphadenopathy improved, probably due to discontinuation of MTX. Here we presented the first case of FTCL presenting as MTX-LPDs. The T-cell phenotype MTX-LPDs are relatively rare and accounts for only 3.4%-6.3% of all MTX-LPD cases. Therefore, detailed clinicopathological features have not been clarified sufficiently. It is hoped that similar cases should be accumulated and studied to better understand the clinical and pathological features of this condition.

TCL-089: Clinicopathological Presentation of Peripheral T-Cell Lymphoma and Their Prognostic Factors: A Single Center Experience

<h3>Background</h3> Peripheral T-cell lymphomas (PTCLs) represent an uncommon and heterogeneous group of diseases. They comprise 10-15% of NHL, with epidemiological majority in South American and Asian populations. Historically these groups of malignancies have presented a poor prognosis, however, with the incorporation of brentuximab and autologous stem cell transplantation (ASCT) as consolidation strategy, the overall survival has improved. <h3>Objectives</h3> Describe the clinicopathological presentation of patients with PTLCs and review their prognosis features. <h3>Design</h3> Retrospective, observational study that included patients over 18 years with diagnosis of PTLCs, between January 2010 and December 2019. All of them were followed up at the HIBA. <h3>Results</h3> We included 43 patients with PTLCs diagnosis. The median age was 58 years (range 20-90) and 65% were male. The most common histopathological subtype was PTLC-NOS in 42% (n=18) followed by ALCL ALK- and AITL with 23% (n=10) and 14% (n=6) respectively. The 81% presented III/IV stage at the diagnosis and 58% had evidence of extranodal disease. Considering IPI and PIT scores, high risk populations slightly differ from 53% in the first score to 44% in the other. Nearly 80% of the patients received protocols including anthracyclines such as CHOEP in 44% (n=19) or CHOP in 32% (n=14) as first line treatment. Another 7% used brentuximab + CHP and 41% (n=18) of the total underwent ASCT as consolidation therapy. Almost 58% achieved complete remission. The overall survival (OS) at 5 years was 37% (IC 95% 20-56) with a median of 36 months. Variables significantly associated with OS were age range (48% vs 26% in under 60 years; p=0,12) and IPI score (65% <i>vs</i> 25% in low-risk groups; p=0.0044). Moreover, ASCT was associated with better OS and PFS with 61% and 58% respectively; <i>versus</i> 26% and 28% in the group without transplant (p=0,0064). <h3>Conclusions</h3> In our experience, PTLCs represent an ominous diagnosis with high mortality in older people and high-risk groups. ASCT showed improvement in OS. Nevertheless, the implementation of brentuximab is still being studied because of the few patients receiving this therapy at the moment.

Poster: TCL-089: Clinicopathological Presentation of Peripheral T-Cell Lymphoma and Their Prognostic Factors: A Single Center Experience

Poster: TCL-089: Clinicopathological Presentation of Peripheral T-Cell Lymphoma and Their Prognostic Factors: A Single Center Experience

A clinician's guide to cutaneous T-cell lymphoma presenting as recalcitrant eczematous dermatitis in adults.

Cutaneous T-cell lymphoma (CTCL) encompasses a group of low-grade, non-Hodgkin lymphoma, including mycosis fungoides and Sézary syndrome. Diagnosis of CTCL can be challenging given the prolonged, gradual onset and shared characteristics with many benign inflammatory skin diseases. In this case series, we describe four unique cases of patients with chronic, recalcitrant eczematous dermatitis who presented for a patch-test consultation and were ultimately diagnosed with CTCL. In particular, we highlight clinical pearls to aid in distinguishing CTCL from inflammatory dermatoses and describe the diagnostic strategy to help dermatologists arrive at the diagnosis of CTCL at earlier stages of the disease.

ALK-positive anaplastic large T-cell lymphoma presenting primarily as a sinonasal mass with pseudoproptosis: A case report

We report a case of anaplastic lymphoma kinase-positive anaplastic large T-cell lymphoma (ALK+ALCL) presenting primarily as a sinonasal mass with pseudoproptosis in an 11-year-old boy. The diagnosis was based on histopathological and immunohistochemical (IHC) evaluation, which is indispensable for determining tumor type. On the basis of clinicoradiological findings, provisional differential diagnoses of angiofibroma and rhabdomyosarcoma were made. Upon histopathological examination of the biopsy sent, the diagnosis of lymphoma in the sinonasal region was considered. Upon IHC, the tumor cells showed immunoreactivity for vimentin, CD45, CD30, and ALK. The tumor cells showed focal immunoreactivity for CD3 and CD68. Ki-67 labeling index was 70%. They were nonimmunoreactive for PAN cytokeratin, epithelial membrane antigen, cluster of differentiation (CD) 20, CD15, CD56, S100, smooth muscle actin, and myogenin. The diagnosis of ALK+ALCL was rendered. The studied IHC markers confirmed the histopathological diagnosis and helped in further subtyping. To the best of our knowledge, this is the first case of ALCL presenting primarily as a sinonasal mass with pseudoproptosis.

Delayed diagnosis of Angioimmunoblast T-cell lymphoma presenting with type II Cryoglobulinemia and acute kidney injury: a case report and narrative review of the literature

BackgroundAngioimmunoblastic T cell lymphoma (AITL) is an infrequent hematological malignancy with variable and often atypical presentations. The presence of dysproteinemia, autoantibodies and systemic involvement in AITL has often led to a delay in diagnosis or even misdiagnosis in practice. We herewith present a case of AITL that primarily presented with acute kidney injury associated with type II Cryoglobulinemia, the underlying cause was only identified 8 months after the emergence of initial symptoms.Case presentationA 67-year old woman presented with 2-month history of intermittent joint pain and a 3-day history of bilateral lower limb edema and acute kidney injury. Initial laboratory investigations showed marked hypocomplementemia with positive autoantibodies of ANA, anti-cardiolipin-IgM and direct antiglobulin. The serum and urinary Immunofixation and serum cryoglobulin tests were negative, while the serum free κ to λ light chain ratio was 0.231. A renal biopsy showed a diffuse proliferative glomerulonephritis with intracapillary pseudothrombi formation. There were orderly arranged microtubular structures of 20–35 nm in diameter in the subendothelial and mesangial area on electron microscopy. Shortly afterwards, the patient developed tingling affecting her finger tips and weak hands and legs. A diagnosis of cryoglobulinemia complicated with cryoglobulinemic glomerulonephritis and polyneuropathy was made. She responded well to methylprednisolone, plasma exchange and rituximab. However, 3 months later, she presented with generalized pruritic rash, weight loss, and inguinal lymphadenopathy. A subsequent inguinal excisional lymph node biopsy at month 8 revealed AITL as the underlying disease.ConclusionsAITL and its associated B cell dysregulation can give rise to autoimmunity and cryoglobulinemia which may conceal itself as the underlying disorder. In various clinical scenarios of auto-immune diseases, it is advisable that the clinicians should take into consideration the multi-faceted lymphoma.

A Rare Case of T-Cell Lymphoma Presenting With Bilateral Nephrolithiasis and Acute Renal Failure

We present the rare case of a young boy who was found to have T-cell lymphoma after presenting with flank pain and bilateral nephrolithiasis. He initially underwent bilateral ureteral stent placement but returned with oliguria and acute renal failure. His subsequent workup revealed lymphoma involving both kidneys. He was started on chemotherapy for his lymphoma and dialysis for his renal failure. His stones ultimately dissolved with aggressive hydration and correction of serum uric acid levels. In this report, we discuss the identification and management of this rare condition.

Hepatosplenic Gamma Delta T-cell Lymphoma Presenting as Progressive Hepatosplenomegaly Highlighted during Pregnancy

Hepatosplenic T-cell lymphoma (HSGDTL) is a rare variant of T-cell lymphoma with few reported cases in literature. It usually presents with hepatosplenomegaly and constitutional symptoms, primarily in young to middle aged patients. The prognosis is generally poor, with maximum reported survival periods of 2-3 years. There is a link to immunodeficiency and some biological treatments. Liver biopsy with flow cytometry is required to confirm the diagnosis.