Atypical Presentation of T-Cell Large Granular Lymphocytic Leukemia Mimicking Pleural Malignancy on 18F-FDG PET/CT.

T-cell large granular lymphocytic leukemia is a rare form of leukemia, caused by clonal proliferation of cytotoxic T-cells, characterized by modest lymphocytosis and cytopenias of other lineage with hepatosplenomegaly and relatively rare lymph nodal involvement. Involvement of other organs is extremely rare. It is predominantly an indolent disease and most of patients remain asymptomatic for a long period. We present a rare case of aggressive form (CD56 positive) of large granular lymphocytic leukemia with atypical presentations mimicking pleural malignancy on F-FDG PET/CT.

CD4-Positive T-Cell Large Granular Lymphocytosis Mimicking Sezary Syndrome in a Patient With Mycosis Fungoides.

A white woman aged 65 years presented with a macular, nonscaly, nonpruritic, erythematous lesion on her right breast. Test results revealed histological features similar to lichenoid dermatitis and early-phase primary cutaneous T-cell lymphoma with a subtype of mycosis fungoides (MF). Despite topical therapy with steroids, her skin disease continued to progress, so she underwent polymerase chain reaction and gene mutation testing. Two missense mutations were detected. The overall findings supported a diagnosis of co-occurring, CD4-positive large granular lymphocytosis and stage IA MF. The patient continued to receive topical steroids and maintenance phototherapy, and her skin lesions completely resolved after 14 weeks of therapy. Approximately 5 years after her initial presentation, she was free of symptoms, cytopenia, and no skin lesions were present. CD4-positive, large granular lymphocytosis was persistent. This patient case - to our knowledge, the first of its kind - posed dilemmas of a diagnostic and therapeutic nature. Correctly staging the lymphoma helped to aid the diagnosis and can help prevent patients similar to the one in this case from receiving unnecessary therapy.

The aggressive peripheral T-cell lymphomas: 2015.

BACKGROUND: T-cell lymphomas make up approximately 10%-15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia. DIAGNOSIS: The diagnosis of aggressive peripheral T-cell lymphoma (PTCL) is usually made using the World Health Organization classification. The ability of hematopathologists to reproducibly diagnosis aggressive PTCL is lower than that for aggressive B-cell lymphomas, with a range of 72%-97% for the aggressive PTCLs. RISK STRATIFICATION: Patients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, positron emission tomography scans seem to be useful as they are in aggressive B-cell lymphomas. The most commonly used prognostic index is the International Prognostic Index. The specific subtype of aggressive PTCL is an important risk factor, with the best survival seen in anaplastic large-cell lymphoma-particularly young patients with the anaplastic lymphoma kinase positive subtype. RISK-ADAPTED THERAPY: Anaplastic large-cell lymphoma is the only subgroup to have a good response to a CHOP-like regimen. Angioimmunoblastic T-cell lymphoma has a prolonged disease-free survival in only ~20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL-not otherwise specified is not one disease. Anthracycline-containing regimens have disappointing results, and a new approach is needed. Natural killer/T-cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy-containing regimens. Enteropathy-associated PTCL and hepatosplenic PTCL are rare disorders with a generally poor response to therapy, although selected patients with enteropathy-associated PTCL seem to benefit from intensive therapy.

A case of CD4(+)T-cell large granular lymphocytic leukemia.

We report here a case of a 59-yr-old man with CD4(+) T-cell large granular lymphocytic leukemia (T-LGL). Peripheral blood examination indicated leukocytosis (45×10(9) cells/L) that consisted of 34% neoplastic lymphoid cells. Other laboratory results indicated no specific abnormalities except for serum antinuclear antibody titer (1:640), glucose (1.39 g/L), and hemoglobin A1c (7.7%) levels. Computed tomography indicated multiple small enlarged lymph nodes (<1 cm in diameter) in both the axillary and inguinal areas, a cutaneous nodule (1.5 cm in diameter) in the left suboccipital area, and mild hepatosplenomegaly. Bone marrow examination revealed hypercellular marrow that consisted of 2.4% neoplastic lymphoid cells. The neoplastic lymphoid cells exhibited a medium size, irregularly shaped nuclei, a moderate amount of cytoplasm, and large granules in the cytoplasm. Immunohistochemical analysis indicated CD3(+), CD4(+), T-cell receptor ßF1(+), granzyme B(+), and TIA1(+). Flow cytometric analysis of the neoplastic lymphoid cells revealed CD3+, cytoplasmic CD3(+), CD4(+), and CD7(+). Cytogenetic analysis indicated an abnormal karyotype of 46,XY,inv(3)(p21q27),t(12;17)(q24.1;q21),del(13)(q14q22)[2]/46,XY[28]. The patient was diagnosed with CD4(+) T-LGL and received chemotherapy (10.0 mg methotrexate). This is the second case of CD4(+) T-LGL that has been reported in Korea.

Myelofibrosis on 18F-FDG-PET/CT in a case suspicious for post-transplant lymphoproliferative disorder.

Recent studies have advocated the utility of fluorine-18 fluorodeoxyglucose-positron emission tomography (18)F-FDG-PET imaging in evaluation of various hematological disorders. We report a case of a 61-year-old man with clinical suspicion of post-transplant lymphoproliferative disorder (PTLD) where (18)F-FDG-PET/CT (computerized tomography) was helpful in identifying myelofibrosis. This paper aims to reveal the potential diagnostic value of PET/CT as an imaging modality in the evaluation of myelofibrosis.