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Cutaneous involvement in lymphoblastic lymphoma
Author(s) -
Chimenti Sergio,
FinkPuches Regina,
Peris Ketty,
Pescarmona Edoardo,
Pütz Barbara,
Kerl Helmut,
Cerroni Lorenzo
Publication year - 1999
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.1999.tb01861.x
Subject(s) - pathology , cd5 , cd20 , cd15 , immunophenotyping , cd34 , lymphoblastic lymphoma , cd30 , lymphoma , cd99 , cd43 , not otherwise specified , medicine , biology , immunohistochemistry , antigen , t cell , stem cell , immunology , vimentin , immune system , genetics
Lymphoblastic leukemia/lymphoma (LBL) is a malignant neoplasm of precursor lymphocytes of B‐ or T‐cell phenotype. Involvement of the skin is relatively uncommon. We examined retrospectively the clinicopathologic, immunophenotypic, and molecular genetic features of six patients with cutaneous involvement of LBL (B‐LBL=5; T‐LBL=1). Patients presented clinically with solitary, large tumors located on the head (3 cases) or the back (1 case), or with generalized tumors (2 cases). Ulceration was uncommon. In two patients the onset of skin lesions was concomitant to the diagnosis of lymphoblastic leukemia. Histopathologic examination showed in all cases a dense, diffuse, monomorphous infiltrate located in the entire dermis and subcutaneous fat. A typical “starry sky” pattern was observed in the majority of the lesions. In some areas neoplastic cells were aligned in a “mosaic‐like” fashion. Cytomorphologically, medium sized lymphoid cells with round or convoluted nuclei, inconspicuous nucleoli and scant cytoplasm predominated. There were no significant differences in the histopathologic features of skin lesions in T‐ and B‐LBL. In B‐LBL, CD79a was more useful than CD20 in determining the phenotypc of neoplastic cells (4/5 cases positive for CD79a as compared to 2/5 cases positive For CD20). TdT, CD10 and CD43 were positive in 4 cases, CD34 in 2. The case of T‐LBL revealed positivity for CD 1a, CD3, CD43 and TdT, and negativity for CD34 and for B‐cell markers. All neoplasms were positive for CD99 and bcl‐2, and showed a high proliferation rate. Molecular genetic analysis of J H and T‐cell receptor (TCR) genes performed using a polymerase chain reaction technique revealed a monoclonal rearrangement of J H genes in all five B‐LBLs. One of these cases showed also a concomitant TCR‐γ gene rearrangement. A monoclonal rearrangement of the TCR‐γ gene was detected in the case of T‐LBL. Our study shows that skin lesions of LBL present characteristic clinicopathologic and molecular features allowing the differentiation from other cutaneous lymphomas, even in cases without clinical history of previous precursor lymphoblastic leukemia/lymphoma.