1/5. Agranular CD4 /CD56 cutaneous neoplasm.CD4 CD56 cutaneous neoplasm with hematological relapse is a rare malignant disease and has been described recently in the literature as blastic or agranular NK-cell leukemia/lymphoma. The origin of this neoplasm is uncertain. We describe a 75-year-old patient with a primary cutaneous neoplasm CD4 CD56 who evolved to leukemic phase despite standard lymphoma chemotherapy. Morphologically, the cells were undifferentiated without granules in the cytoplasm. The immunophenotype showed the expression of CD4, CD56, CD68, CD33, CD7, CD2, CD45RA, and CD38. Histological analysis revealed a cell infiltration mainly located in the dermis. T-cell receptor and immunoglobulin heavy chain genes were in germline configuration. Cytogenetic study showed complex structural abnormalities with a deletion of the chromosome 5 del(5q). The clinical course was aggressive with an early hematological relapse.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/5. Concurrent Sweet's syndrome and leukemia cutis in patients with myeloid disorders.A 74-year-old woman with chronic auricular fibrillation, arterial hypertension, hypercholesterolemia, ischemic cardiopathy, and peripheral arteriopathy presented with purpuric lesions on the lower limbs (Fig. 1) and, to a lesser extent, on the anterior area of the chest. The mucous membranes were not affected. In 1989, she was diagnosed with anemia that evolved until 1998, when a bone marrow biopsy revealed a myelodysplastic syndrome unclassified in French-American-British Group (FAB). The patient has required periodic transfusions since February 1999. A skin biopsy of the purpuric lesions revealed a leukocytoclastic vasculitis; the lesions cleared with topical corticosteroid treatment. In May 1999, the patient presented with inflammatory and painful lesions localized on the vulva (Fig. 2), which had evolved over several days, without fever. No lesions were observed in other locations. A cutaneous biopsy showed an intense dermal edema and a diffuse and polymorphous dermal infiltrate involving the follicular structures. exocytosis, spongiosis, and mucin deposits, demonstrated by alcian blue stain, were observed in the follicular epithelium. Mature neutrophils were predominant in the dermal infiltrate, but a small number of eosinophils and immature cells were also present (Fig. 3). The myelogenous origin of the immature lining cells was further confirmed by positive staining of intracytoplasmic granules with naphthol-ASD chloroacetate sterase (Leder's stain). vasculitis was not observed. Routine laboratory tests revealed 3030 leukocytes/mm(3) (60% neutrophils), a hemoglobin level of 8.4 g/dL, and 92,000 platelets/mm(3). Treatment with 30 mg/day of prednisone was started, and the lesions cleared slowly within 4 weeks. A new bone marrow biopsy in September 1999 showed a similar appearance to that taken in 1998. The patient died in January 2000 as a result of pneumonia with cardiac and respiratory failure. A 66-year-old man presented with a febrile syndrome that had evolved over 5 days, and painful and pruritic cutaneous lesions on the face and posterior neck (Fig. 4). Three months before, the patient was diagnosed with chronic myelogenous leukemia in acceleration phase. Examination revealed an edematous and erythematous face with pustular lesions on the surface, also involving the neck and the upper part of the back. The histopathologic examination revealed an intense edema and abscesses in the dermis. The infiltrate of these lesions was composed of mature neutrophils with the presence of abundant immature cells with a myelogenous aspect (Fig. 5). Analytical studies revealed 26,130 leukocytes/mm(3) (42% blasts). No specific treatment for Sweet's syndrome was administered and the lesions showed an improvement within 5 days. Eight days after admission, the patient died as a result of acute hemorrhage, before treatment for leukemia was initiated.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
3/5. CD3 CD8 T cell lymphocytosis masking B cell leukaemia.A patient with CD3, CD8 positive lymphocytosis presented with features consistent with T cell chronic lymphocytic leukaemia/proliferations of large granular lymphocytes. The marrow and blood lymphoid populations (19.4 x 10(9)/l) contained more than 80% CD3 and CD8 positive cells with no evidence of a monotypic B cell population. A biopsy specimen of a vasculitic rash showed a diffuse infiltrate of CD3, CD8 positive cells into the upper dermis, consistent with T cell lymphocytic disease. After follow up for two years without treatment the blood lymphocyte count was 53 x 10(9)/l and was composed of cytologically small lymphocytes. A monoclonal SIg M D k lymphoid population (more than 90%) was demonstrable in sample blood and marrow aspirate. gene rearrangement studies carried out on dna extracted from peripheral blood lymphocytes at presentation and at two year follow up exhibited JH and Ck immunoglobulin gene rearrangement but no rearrangement of T cell receptor TcR gamma and beta genes. It is thought that this is the first well documented case of an aggressive CD8 positive lymphocytosis preceding, or in response to, an underlying B cell neoplasm.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
4/5. Preferential epidermotropism in adult t-cell leukemia-lymphoma.Three patients with systemic T-cell leukemia-lymphoma presented with generalized pruritic skin rash which was clinically nondiagnostic. Biopsies revealed infiltration of the superficial dermis by atypical lymphoid cells with epidermal exocytosis (Pautrier abscesses) producing a pattern of histologic involvement indistinguishable from that of mycosis fungoides and sezary syndrome. This striking similarity suggests that the phenomenon of preferential epidermotropism is not confined to mycosis fungoides and sezary syndrome, but may be a property shared by other subpopulations of t-lymphocytes.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/5. Pseudoleukemia cutis: report of a case in association with molluscum contagiosum.Histologic sections from a solitary cystic cutaneous lesion that showed atypical mononuclear cells in the dermis and within blood vessels were diagnosed by several general pathologists and dermatopathologists as leukemia cutis. The patient, who had no other cutaneous lesions, was consequently submitted to an extensive investigation for leukemia, which proved negative. Additional and deeper sections from the original block revealed that the cellular infiltrate so suspicious of leukemia cutis was secondary to rupture of a lesion of molluscum contagiosum. The correct histopathologic diagnosis, therefore, was pseudoleukemia cutis. The lessons of the case are that 1) further study of the specimen, solitary as it was and asymptomatic as the patient was, would have obviated worry and the expense and inconvenience of an extensive systemic investigation, and that 2) the diagnosis of leukemia cutis should never be made solely on the basis of histologic sections of skin, but rather after examination of blood and bone marrow.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |