Leg ulcer and thigh telangiectasia associated with natural killer cell CD56– large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome

Duarte, Ana Filipa; Nogueira, Ana; Mota, Alberto; Baudrier, Teresa; Canelhas, Áurea; Cancela, Jorge; Lima, Margarida; Azevedo, Filomena

doi:10.1016/j.jaad.2009.03.001

Cited by 6 publications

(7 citation statements)

References 39 publications

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“…3 Frequently, LGL leukaemia is related to autoimmune diseases such as Felty's syndrome and has a chronic and indolent course, with a mean survival of 10 years. 4,5 Occasionally, the behaviour of this form of leukaemia can be more aggressive, especially in cases with a natural killer phenotype. 2 Approximately one-third of patients with LGL leukaemia are clinically asymptomatic, with neutropaenia observed in up to 85% of cases.…”

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“…3 The disease is sometimes associated with arthritis, cytopaenia and splenomegaly, but only exceptionally with lymphadenopathy. 4 Cutaneous infiltration is rare and usually manifests as papules, nodules or, even less frequently, leg ulcers. 4,6 Other reports have described LGL leukaemia patients presenting with generalised erythema and pruritus, petechiae and disseminated granuloma annulare.…”

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confidence: 99%

“…4 Cutaneous infiltration is rare and usually manifests as papules, nodules or, even less frequently, leg ulcers. 4,6 Other reports have described LGL leukaemia patients presenting with generalised erythema and pruritus, petechiae and disseminated granuloma annulare. [6][7][8] The indolent course of the disease usually permits a 'wait and see' approach, although indications for treatment include recurrent infections, anaemia, symptomatic splenomegaly or severe B symptoms.…”

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T Cell Large Granular Lymphocyte Leukaemia with Cutaneous Infiltration

Ruiz‐Villaverde

Bueno-Rodriguez

Aguayo-Carreras

et al. 2018

Sultan Qaboos Univ Med J

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A 63-year-old woman was referred to the Department of Dermatology of the Complejo Hospitalario de Granada, Granada, Spain, in 2016 with painful erythematous desquamative nodules and papules of three weeks' duration. She had a history of stage I infiltrating ductal carcinoma of the breast, unilinear myelodysplastic syndrome with granulocytic involvement, chronic neutropaenia and a diagnosis of T cell (cluster of differentiation [CD]8 + ) large granular lymphocyte (LGL) leukaemia. Upon examination, the papules were distributed mainly on the forehead and right upper and lower limbs . Some of the lesions were fluctuating and suppurative, while the others were of a solid consistency. The examination also revealed oral aphthae which were causing dysphagia [ Figure 1D]. At referral, the patient was receiving treatment with 50 mg of low-dose cyclosporine on alternate days and 300 μg of granulocyte-colony stimulating factor per week, to which a partial response was noted.Given the suspicion of leukaemia cutis, a biopsy of the lesions was taken. The findings were compatible with a diagnosis of folliculitis, with polymerase chain reaction and direct immunofluorescence tests negative for Mycobacterium tuberculosis and immunoglobulin (Ig) G, IgA, IgM and complement 3 deposits, respectively. Consequently, treatment with 500 mg of cloxacillin every six hours and 15 mg of prednisone per day was initiated; however, there was no evidence of clinical improvement. Successive biopsies indicated systemic vasculitis caused by granulomatosis with polyangiitis. Eventually, nine months after the onset of the symptoms, a final biopsy revealed that the papules represented the cutaneous infiltration of LGL leukaemia [ Figure 2], associated with secondary interstitial granulomatous dermatitis. At the time of writing, the patient was being treated with combined cyclophosphamide, vincristine, doxorubicin and prednisone (CHOP) therapy which resulted in the resolution of her cutaneous symptoms [ Figure 3]. There was no evidence on computed tomography of any systemic progression to other organs.LGL leukaemia is a low-grade lymphoproliferative disorder characterised by monoclonal expansion in the peripheral blood and bone marrow with either an activated cytotoxic T lymphocyte (i.e. CD8 or CD57 + cells) or, less frequently, a natural killer (CD3 -, CD8 -or CD56 + cells) phenotype. 1 Generally, LGLs represent 10-15% of circulating mononuclear cells and are identified morphologically by their large size and rounded or dentate nuclei and abundant cytoplasm with azurophilic granules.2 Clonality has been confirmed by T cell receptor gene letter to the editor Sultan Qaboos University Med J, November 2017, Vol. 17, Iss. 4, pp. e489-490,

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T Cell Large Granular Lymphocyte Leukaemia with Cutaneous Infiltration

Ruiz‐Villaverde

Bueno-Rodriguez

Aguayo-Carreras

et al. 2018

Sultan Qaboos Univ Med J

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“…The overwhelming majority of such cutaneous features can be classified schematically within three major clinico-pathological categories: (/) cutaneous small-or medium-sized vessel vasculitis, usually presenting as palpable purpura, necrotic pustules, urticaria vasculitis, or polyarteritis nodosa-like ulcers (5, 6); (//) vasculopathy without histological evidence of true vasculitis, manifesting as livedoid vasculopathy and/or eruptive telangiectatic lesions (5,7,8); (///) persistent ulcérations with histological demonstration of intravascular LGL, often localized to the lower limbs Letters to the Editor 289 (7,9). Interestingly, Mallo et al (10) reported a case of indolent T-LGL leukaemia with persistent generalized pruritus as the only alleged manifestation.…”

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confidence: 99%

Mycosis Fungoides in the Setting of T-cell Large Granular Lymphocyte Proliferative Disorder

Saggini¹,

Saraceno²,

Anemona³

et al. 2012

Acta Derm Venerol

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“…Typically, T-cell LGLL is indolent and non-progressive. Previous studies showed that patients had a median survival of 13 years [5,11,12].…”

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confidence: 99%

CD20-Positive T-Cell Large Granular Lymphocytic Leukemia: A Case Report and Literature Review

Ferreira¹,

Culler²,

Hencklain³

et al. 2017

J Clin Case Rep

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T-cell large granular lymphocytic leukemia (T-cell LGLL) is a rare disorder characterized by the monoclonal expansion of CD3-positive cytotoxic T cells. Cell morphology and immunophenotyping are the main tools for diagnosis. Typical phenotypic findings include the expression of markers CD3+, CD5+dim, CD8+, CD16+, CD57+, and T-cell receptor (TCR) αβ (+). However, a few cases may present with the CD3+/CD4+ phenotype or the double expression of CD4 and CD8. Similarly, in 20% of cases, TCRγ/δ is positive. In this study, we aimed to describe a rare, unusual case of T-cell LGLL that exhibited expression of the B-cell antigen, CD20. Additionally, we performed a literature review to compare the clinical characteristics of our patient to those of other patients with CD20-positive T-cell LGLL and to seek new therapeutic possibilities.

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Leg ulcer and thigh telangiectasia associated with natural killer cell CD56– large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome

Cited by 6 publications

References 39 publications

T Cell Large Granular Lymphocyte Leukaemia with Cutaneous Infiltration

T Cell Large Granular Lymphocyte Leukaemia with Cutaneous Infiltration

Mycosis Fungoides in the Setting of T-cell Large Granular Lymphocyte Proliferative Disorder

CD20-Positive T-Cell Large Granular Lymphocytic Leukemia: A Case Report and Literature Review

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