Early B-cell chronic lymphocytic leukemia presenting as cutaneous lesions with a normal peripheral blood lymphocyte count

Gibbs, Simon; Westerman, David; Lade, Stephen; McCormack, Christopher; Seymour, John F.; Prince, H. Miles

doi:10.1016/j.jaad.2005.02.037

Cited by 14 publications

(9 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our patients with cutaneous RS had features similar to those found in other RS series with and without skin involvement [3,18,24], but the survival after RS diagnosis was much longer in our series than the 5 or 8 months previously reported [18]. Our patients with initial cutaneous infiltration by small lymphocytes (2/4) seemed to have a poorer prognosis than previously described [18,26,28], and also a more rapid progression to RS. The cutaneous presentation of RS may develop more slowly than lymph node RS, possibly explaining the longer survival, although 3 of our 4 patients died of explosive tumoral or multivisceral RS.…”

Section: Discussionsupporting

confidence: 84%

Cutaneous Richter’s Syndrome, Prognosis, and Clinical, Histological and Immunohistological Patterns: Report of Four Cases and Review of the Literature

Duong¹,

Grange

Auffret

et al. 2010

Dermatology

View full text Add to dashboard Cite

Background: Richter’s syndrome (RS) corresponds to the transformation of chronic lymphocytic leukemia (CLL) into high-grade lymphoma. RS can involve extranodal sites including the gastrointestinal tract, lungs and skin. Cutaneous RS is rare, we describe 4 cases with clinical manifestations, histological and immunohistological patterns, and outcome. Methods: Clinical data were analyzed and all patients’ skin biopsy samples stained with HE for the CD20, CD5, CD3 and CD30 antigens. Epstein-Barr-virus (EBV)-encoded early RNA and clonal rearrangements were also analyzed. Results: The patients’ mean age at CLL diagnosis was 57 years (53–62 years), with a male/female sex ratio of 3:1. The transformation to cutaneous RS occurred between 8 and 75 months after initial diagnosis and progressed to a fatal systemic disease in 3 cases, between 24 and 129 months. Cutaneous CLL was associated with earlier transformation in our series and could not be distinguished from RS on clinical grounds alone. All patients had a large-cell infiltrate and clonal rearrangements. Conclusions: The precise mechanism of RS is unclear, but a role of EBV has been suggested in fludarabine-treated CLL. For all our patients, the diagnosis of transformation was made on the basis of cutaneous localizations and led to intensified CLL treatment.

show abstract

Section: Discussionsupporting

confidence: 84%

Cutaneous Richter’s Syndrome, Prognosis, and Clinical, Histological and Immunohistological Patterns: Report of Four Cases and Review of the Literature

Duong¹,

Grange

Auffret

et al. 2010

Dermatology

View full text Add to dashboard Cite

show abstract

“…Although it was reported at presentation in up to 17% of the CLL patients in one study, all patients were advanced stage 2 . Early CLL presents seldom with skin manifestation: to our knowledge, only four such cases of subclinical CLL, stage Rai 0, were described in the literature to date 3–5 . From these, only two patients lacked absolute lymphocytosis and lymphadenopathy, and had no other associated tumor or cutaneous non‐lymphoid lesion that lead to CLL detection 5 as was the case in our patient.…”

Section: Discussionmentioning

confidence: 46%

Subclinical chronic lymphocytic leukemia with atypical cutaneous presentation

Cheney

Merzianu

2010

Journal of Cutaneous Pathology

View full text Add to dashboard Cite

Chronic lymphocytic leukemia (CLL) involving skin is a rare but well-documented occurrence, mainly reported in advanced disease. In contrast, CLL presenting with skin lesions is exceedingly rare, only few reports existing to date. We report a 70-year-old man who presented with two non-pruritic, papular lesions on the lower abdomen and proximal thigh. Biopsies showed dense lymphohistiocytic infiltrates involving the reticular dermis and subcutis without epidermotropism consisting mostly of small, CD20 and PAX-5-positive B-cells expressing CD5, CD23, CD43 and BCL2. Numerous large B-cells were present in a T-cell, histiocyte-rich background. A staging bone marrow biopsy showed a clonal B-cell proliferation with typical CLL flow cytometry immunophenotyping but neither lymphadenopathy nor absolute lymphocytosis was present. Numerous B and T-cell cutaneous lymphoproliferative disorders can be associated with increased numbers of histiocytes occasionally masquerading as benign disorders. This was the case with our patient's lesions, originally interpreted as cutaneous Rosai-Dorfman disease. A high index of suspicion from both the pathologist and the dermatologist is essential in identifying these rare but probably underrecognized occurrences of early systemic lymphoproliferative disorders presenting as cutaneous lesions with an unexpected cellular composition.

show abstract

“…15 CLL can be notoriously insidious, even presenting in the skin in patients with normal peripheral blood counts. 12 This case reiterates the potential for involvement by CLL/SLL in the cutaneous biopsies of otherwise asymptomatic patients and highlights the importance of vigilance in evaluating lymphocytic infiltrates.…”

Section: Discussionmentioning

confidence: 63%

“…Other cases were varied with regard to the presence of lymphadenopathy (which our patient lacked) and lesional extent, which ranged from a single discreet nodule related to a clear background lesion, to the acute onset of multiple plaques, patches and nodules over the back, shoulders and cheeks. 5,10,12 The histologic differential diagnosis of pan-dermal, nodular to diffuse lymphocytic infiltrates composed of a majority of small cells is relatively broad. A predominance of large atypical lymphocytes, in contrast, would suggest entities such as diffuse large B-cell lymphoma or CD30-positive lymphoproliferative disorders.…”

Section: Discussionmentioning

confidence: 99%

CLL/SLL with initial presentation as a longstanding cutaneous plaque: a rare case

et al. 2016

View full text Add to dashboard Cite

While chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is the most common leukemia in the western world, it uncommonly initially presents as a cutaneous lesion. When it does, it is often found incidentally in the context of another distinct lesion. We present a case of CLL/SLL that initially presented as a solitary nodular erythematous plaque on the left flank of a healthy 60-year-old man without lymphadenopathy or constitutional symptoms. The lesion had been present for many years, but had recently begun to grow and become pruritic and painful. Excisional biopsy revealed a large nodular aggregate of variably sized CD20(dim), CD5(+), CD23(+), BCL-2(+) atypical lymphocytes in the dermis without epidermotropism. The lymphocytes were negative for BCL-1/cyclinD1, BCL-6, and CD10, and no other background lesion was identified. This constellation of biopsy findings was consistent with CLL/SLL. This case emphasizes the potential for involvement by CLL/SLL in the cutaneous biopsies of otherwise asymptomatic patients.

show abstract

Early B-cell chronic lymphocytic leukemia presenting as cutaneous lesions with a normal peripheral blood lymphocyte count

Cited by 14 publications

References 3 publications

Cutaneous Richter’s Syndrome, Prognosis, and Clinical, Histological and Immunohistological Patterns: Report of Four Cases and Review of the Literature

Cutaneous Richter’s Syndrome, Prognosis, and Clinical, Histological and Immunohistological Patterns: Report of Four Cases and Review of the Literature

Subclinical chronic lymphocytic leukemia with atypical cutaneous presentation

CLL/SLL with initial presentation as a longstanding cutaneous plaque: a rare case

Contact Info

Product

Resources

About