Blastic plasmacytoid dendritic cell neoplasm / Neoplasia blastica de celulas dendriticas plasmocitoides

Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.

A neoplasia blástica de células dendríticas plasmocitóides é uma neoplasia hematodérmica rara, agressiva, com frequente envolvimento cutâneo e disseminação leucêmica. Relatamos o caso de um homem de 76 anos com quadro clínico com 2 meses de evolução caracterizado por nódulos e tumor de tonalidade violácea, de consistência pétrea, localizados na cabeça, e linfadenopatias mandibular, cervicais e supraclaviculares. Identificaram-se múltiplas adenopatias torácicas e abdominais em tomografia computorizada. A análise por citometria de fluxo de biópsias cutânea, ganglionar e óssea demonstrou a presença de precursores neoplásicos das células dendríticas plasmocitóides (fenótipo CD4+, CD45+, CD56+ e CD123+). Após remissão clínica e laboratorial completa inicial com quimioterapia, veio a falecer por recaída da doença associada ao aparecimento de massa cervical com compromisso medular.

The expression of CD56 antigen is associated with poor prognosis in patients with acute myeloid leukemia

BACKGROUND: The expression of CD56 is considered a bad prognostic factor for overall survival, lower rates or short complete remission and extramedullary invasion but the results are controversial. The importance of validating new prognostic parameters in acute leukemias was the reason to investigate the CD56 expression in blast cells of patients with acute myeloid leukemia. METHODS: A cohort of 48 patients treated at Hospital de Clinicas de Porto Alegre and diagnosed with acute myeloid leukemia as classified by the French-American-British group (FAB) criteria using cell morphology, cytochemistry and flow cytometry were evaluated. RESULTS: Eight cases (16.7 percent) were CD56 positive without correlation to age or gender. The highest incidence of CD56 positivity was in FAB subtypes M4 and M5. The death rate during induction was not significantly different between patients with and without CD56 expression (62.5 percent vs. 27.5 percent; p-value = 0.097). However, patients that expressed CD56 had significantly lower overall survival than those who did not (mean 4.0 months vs. 14.5 months; p-value = 0.03). CONCLUSIONS: The data suggest that expression of CD56 in acute myeloid leukemia may be indicative of poor prognosis because it is associated with a shorter overall survival. The death rate during induction was not significantly different despite an apparent difference in proportions between groups.

Two cases of CD56 positive cutaneous lymphoma / 中华皮肤科杂志

In the first case, a 15-year-old girl presented with recurrent multiple erythematous edema,bullae and ulceration on the face and extremities for 3 years, which had developed into plaques and nodules on the face and trunk for 6 months. Histology revealed angiocentric and angiodestructive infiltrates with medium-sized atypical lymphoid cells positive for LCA, CD45RO, CD56 and EBV staining throughout the dermis. The patient was diagnosed with extra NK/T-cell lymphoma, nasal-type. She subsequently had a rapid downhill clinical course with resistance to systemic chemotherapy, and died one month later. In the second case, a 44-year-old male was admitted to the hospital with progressive infiltrated mass on the right waist for 1 year, and a 4-month-history of lymphadenectasis. Histologically, there was a massive and dense infiltrate with middle and large-sized, CD4 and CD56-positive lymphoblastics throughout the dennis and subcutaneous tissue. A diagnosis of blastic NK-cell lymphoma was made. The patient was managed with surgical excision followed by systemic chemotherapy. He had been followed up and free of relapse till the time of this writing.CD56 positive cutaneous lymphoproliferative disorders appear to be highly invasive.