Track topics on Twitter Track topics that are important to you
Patients with PDGFRA-rearranged hematopoietic neoplasms typically present with chronic eosinophilic leukemia and rarely with acute myeloid leukemia or T-lymphoblastic lymphoma. However, mature T-cell lymphoma has not been previously associated with PDGFRA aberrations. We report a patient who presented with simultaneous T-lymphoblastic lymphoma, focal myeloid proliferation, and cutaneous cytotoxic T-cell lymphoma refractory to chemotherapy. The presence of myeloid and lymphoid lineages prompted genetic and molecular studies. A PDGFRA rearrangement was identified in all compartments: cutaneous, lymph node, and bone marrow. Treatment with imatinib resulted in an excellent response in cutaneous and systemic disease. We report the first case of a mature cutaneous T-cell lymphoma with PDGFRA rearrangement, expanding the spectrum of neoplasms associated with this genetic abnormality. Our case underscores the great importance of recognizing PDGFRA rearrangement in unusual cases of cutaneous T-cell lymphoma, systemic lymphoid, and myeloid neoplasms. These patients may respond well to tyrosine kinase inhibitors, despite resistance to conventional chemotherapy.
This article was published in the following journal.
Name: The American Journal of dermatopathology
T-cell lymphoma is a neoplasm that expresses markers of T-cell or natural killer cell (NK)-origin but not those of B-cell origin. Although B-cell lymphoma with abundant expression of T-cell markers ex...
The cytotoxic lymphomas of the skin constitute a heterogeneous group of rare lymphoproliferative diseases that are derived from mature T cells and natural killer (NK) cells that express cytotoxic mole...
Primary cutaneous gamma/delta T-cell lymphoma (PCγδTCL) is a rare form of cutaneous lymphoma characterized by abnormal clonal proliferation of mature, activated gamma-delta T cells expressing the γ...
Subcutaneous tissue as a manifestation of generalized disease is commonly involved in lymphomas. However, only skin involvement without other sites of extracutaneous disease was rarely presented, espe...
There is no consensus on the treatment of multifocal primary cutaneous anaplastic large cell lymphoma (C-ALCL). Radiotherapy (RT) and methotrexate (MTX) are the current treatment options, but their ef...
Registry of patients with Primary Cutaneous Lymphoma seen at participating centers in Spain. The registry will identify patients with this disease and includes information about stage, dia...
The purpose of this study is to provide an opportunity for patients who exhibit progressive disease while receiving placebo on the companion 93-04-11 study to receive ONTAK. It is also de...
This study will evaluate the safety and efficacy of LBH489B in adult patients with refractory Cutaneous T-Cell Lymphoma.
This is a pilot study of the safety and tolerability of photopheresis in combination with increasing doses of oral bexarotene in patients with cutaneous T-cell lymphoma.Our hypotheses are ...
In this study, all patients will get investigational drug. There will be no comparator drug. This study will evaluate three tumor types: T-cell lymphoma, Indolent B-cell lymphoma, and A...
Anaplastic lymphoma of the skin which develops as a primary neoplasm expressing the CD30 ANTIGEN. It is characterized by solitary nodules or ulcerated tumors.
Clinically benign, histologically malignant, recurrent cutaneous T-cell lymphoproliferative disorder characterized by an infiltration of large atypical cells surrounded by inflammatory cells. The atypical cells resemble REED-STERNBERG CELLS of HODGKIN DISEASE or the malignant cells of CUTANEOUS T-CELL LYMPHOMA. In some cases, lymphomatoid papulosis progresses to lymphomatous conditions including MYCOSIS FUNGOIDES; HODGKIN DISEASE; CUTANEOUS T-CELL LYMPHOMA; or ANAPLASTIC LARGE-CELL LYMPHOMA.
A group of lymphomas exhibiting clonal expansion of malignant T-lymphocytes arrested at varying stages of differentiation as well as malignant infiltration of the skin. MYCOSIS FUNGOIDES; SEZARY SYNDROME; LYMPHOMATOID PAPULOSIS; and PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA are the best characterized of these disorders.
An anti-CD52 ANTIGEN monoclonal antibody used for the treatment of certain types of CD52-positive lymphomas (e.g., CHRONIC LYMPHOCYTIC LEUKEMIA; CUTANEOUS T-CELL LYMPHOMA; and T-CELL LYMPHOMA). Its mode of actions include ANTIBODY-DEPENDENT CELL CYTOTOXICITY.
B-cell lymphoid tumors that occur in association with AIDS. Patients often present with an advanced stage of disease and highly malignant subtypes including BURKITT LYMPHOMA; IMMUNOBLASTIC LARGE-CELL LYMPHOMA; PRIMARY EFFUSION LYMPHOMA; and DIFFUSE, LARGE B-CELL, LYMPHOMA. The tumors are often disseminated in unusual extranodal sites and chromosomal abnormalities are frequently present. It is likely that polyclonal B-cell lymphoproliferation in AIDS is a complex result of EBV infection, HIV antigenic stimulation, and T-cell-dependent HIV activation.