Linear nonpalpable purpura in a young male: a unique presentation of leukemia cutis in T‐cell prolymphocytic leukemia

Khullar, Geeti; Singh, Suvir; Agrawal, Saurabh; Sachdeva, Man Updesh Singh; Saikia, Uma Nahar

doi:10.1111/ijd.13457

Cited by 3 publications

(2 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Classic clinical features of skin manifestations include erythematous nodules and papules on the legs, asymptomatic reddish patches over the chest and upper back, and exfoliative dermatitis covering the entire body. [3][4][5] Other common associated findings include lymphocytosis, lymphadenopathy, and splenomegaly. 2 Diagnosis requires a thorough assessment of clinical presentation, immunophenotypic profiles, and serologic and cytologic studies.…”

Section: Introductionmentioning

confidence: 99%

Cutaneous Presentation of T-Cell Prolymphocytic Leukemia Mimicking Dermatomyositis

Ingrasci

Díaz-Pérez²,

Verne³

et al. 2021

The American Journal of Dermatopathology

View full text Add to dashboard Cite

T-cell prolymphocytic leukemia (TPLL) is a rare form of leukemia by T lymphocytes at a post-thymic intermediate stage of development with an a/b immunophenotype. Facial involvement is common in TPLL and displays significant heterogeneity of the lesions' description and location. TPLL also contains a wide array of histology findings, cell cytology, and molecular studies. Here, we describe a TPLL patient who presented with an ill-defined erythematous patch involving the right axilla progressing to the left axilla, upper back, and face that resembled dermatomyositis. The diagnosis of TPLL was established using flow cytometry of bone marrow and peripheral blood, and histopathology of the involved skin. Dermatologists should be aware of these unique features.

show abstract

Section: Introductionmentioning

confidence: 99%

Cutaneous Presentation of T-Cell Prolymphocytic Leukemia Mimicking Dermatomyositis

Ingrasci

Díaz-Pérez²,

Verne³

et al. 2021

The American Journal of Dermatopathology

View full text Add to dashboard Cite

show abstract

“…In T-PLL, the white cell count is usually > 100 × 10 9 /L at presentation, as in B-PLL. Otherwise, most patients present with splenomegaly, lymphadenopathy, hepatomegaly, and skin lesions which include skin nodules, a maculopapular rash, or more rarely, erythroderma (Table 2 ) [ 1 , 5 , 9 , 48 , 49 ]. Peripheral edema, particularly periorbital and/or conjunctival, occurs relatively frequently and seems to be particularly characteristic of T-PLL.…”

Section: Introductionmentioning

confidence: 99%

Advances in the understanding and management of T-cell prolymphocytic leukemia

et al. 2017

View full text Add to dashboard Cite

T-prolymphocytic leukemia (T-PLL) is a rare T-cell neoplasm with an aggressive clinical course. Leukemic T-cells exhibit a post-thymic T-cell phenotype (Tdt−, CD1a−, CD5+, CD2+ and CD7+) and are generally CD4+/CD8−, but CD4+/CD8+ or CD8+/CD4− T-PLL have also been reported. The hallmark of T-PLL is the rearrangement of chromosome 14 involving genes for the subunits of the T-cell receptor (TCR) complex, leading to overexpression of the proto-oncogene TCL1. In addition, molecular analysis shows that T-PLL exhibits substantial mutational activation of the IL2RG-JAK1-JAK3-, STAT5B axis. T-PLL patients have a poor prognosis, due to a poor response to conventional chemotherapy. Monoclonal antibody therapy with antiCD52-alemtuzumab has considerably improved outcomes, but the responses to treatment are transient; hence, patients who achieve a response to therapy are considered for stem cell transplantation (SCT). This combined approach has extended the median survival to four years or more. Nevertheless, new approaches using well-tolerated therapies that target growth and survival signals are needed for most patients unable to receive intensive chemotherapy.

show abstract

Current understandings on T-cell prolymphocytic leukemia and its association with TCL1 proto-oncogene

Sun

Fang

2020

Biomedicine & Pharmacotherapy

View full text Add to dashboard Cite

Linear nonpalpable purpura in a young male: a unique presentation of leukemia cutis in T‐cell prolymphocytic leukemia

Cited by 3 publications

References 10 publications

Cutaneous Presentation of T-Cell Prolymphocytic Leukemia Mimicking Dermatomyositis

Cutaneous Presentation of T-Cell Prolymphocytic Leukemia Mimicking Dermatomyositis

Advances in the understanding and management of T-cell prolymphocytic leukemia

Current understandings on T-cell prolymphocytic leukemia and its association with TCL1 proto-oncogene

Contact Info

Product

Resources

About