Biomedical subjects
Publications and source records attributed to Mariana Munichor.
A 57-year-old white female developed a Gottron's papules (GP)-like eruption, without any of the other clinical or laboratory signs of dermatomyositis (DM). She was under hydroxyurea treatment for chronic myeloid leukemia at the time. Skin biopsy was compatible with seborrheic keratosis. Conditions presenting with GP but unrelated to DM are reviewed, with emphasis on hydroxyurea-induced skin lesions.
We report a rare tumor called low-grade renal collecting duct carcinoma. Grossly, the tumor consisted of multiple cysts and solid white nodules, measuring 10 cm in diameter and occupying most of the renal parenchyma. Histologically, the tumor was characterized by well-differentiated tubules lined by eosinophilic cells without papillary projections, abundant predominantly extracellular mucin, minimal cellular atypia, no desmoplasia, and rare mitoses. This tumor occurs in collecting ducts and the tumor cells were positive for epithelial membrane antigen, high-molecular-weight keratin, CD15, and mitochondrial antibody and negative for CD10. Few cells stained weakly positive for ulex europaeus. Ultrastructural study showed a large number of mitochondria according to the eosinophilic cells seen in light microscopy.
OBJECTIVES: Only a few reports of nonsyndromic paucity of interlobular bile ducts (NS-PILBD) have been published. The authors' aim was to outline the clinical and laboratory profile of patients with NS-PILBD diagnosed at a tertiary referral center. METHODS: The authors reviewed all the reports of pediatric liver biopsies performed between 1991 and 2000 at their institution. Upon diagnosis of NS-PILBD, patients' records were examined for clinical, laboratory, and histologic data, and liver biopsy specimens were re-evaluated. RESULTS: Three hundred biopsies were performed in children during the study period, of which 64 were in infants younger than 1 year. NS-PILBD was diagnosed in 10 of 64 (16%) biopsy specimens. Mean age at presentation was 10 days (range, 1 day-6 weeks), and mean follow-up was 4.5 years (range, 1-9 years). An underlying condition was identified in 70% of children with NS-PILBD: namely congenital cytomegalovirus (n = 2), progressive familial intrahepatic cholestasis (PFIC, n = 2), mitochondrial DNA depletion (n = 1), Niemann-Pick type C (n = 1), and arthrogryposis multiplex congenita, renal dysfunction, and cholestasis (ARC syndrome; n = 1). All children presented with jaundice. Four children had initially acholic stools. At their last follow-up visit, failure to thrive was present in five children, and cholestasis in six children. Mortality was noted only in children with metabolic diseases (n = 2). CONCLUSIONS: In the study, NS-PILBD was common in young children undergoing liver biopsy. Although NS-PILBD is nonspecific, a wide survey for inborn errors of metabolism should be included in the diagnostic work-up of NS-PILBD. In the authors' center, the association of certain metabolic diseases with NS-PILBD carries a grave prognosis.
BACKGROUND: AIDS-related body cavity-based lymphoma, or primary effusion lymphoma (PEL), is a distinct clinicopathologic entity that occurs predominantly in immunosuppressed patients infected with human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus. Although it rarely occurs in human immunodeficiency virus (HIV)-negative patients, we report such a case here. CASE: A 74-year-old male, who was HIV and Epstein-Barr virus (EBV) negative, was admitted to the hospital with dyspnea and chest pain. Chest radiography and computed tomography showed right pleural effusion. Cytologic analysis of the pleural effusion revealed a high grade lymphoma with round nuclei, prominent nucleoli and abundant cytoplasm. Polymerase chain reaction performed on the pleural effusion was positive for HHV-8 and negative for EBV. On molecular studies, the immunoglobulin heavy and kappa light chains were rearranged. Flow cytometry revealed a hyperploid fraction with DNA index of 1.29 expressing CD30. Immunostaining for HHV-8 from a cell block was positive. Electron microscopy revealed lymphomalike cells, many in various stages of apoptosis, with large nucleoli and clusters of viruslike particles in the nucleoplasm. CONCLUSION: A firm diagnosis of PEL can be established by the examination of cells from the lymphomatous effusion by a combination of cytology, molecular genetics, phenotypic features, immunostaining and electron microscopy. To our knowledge, this is the first case in which immunostaining for anti-HHV-8 monoclonal antibodies was used to support the diagnosis.
BACKGROUND: Prosthetic joint replacement is frequently used for the treatment of degenerative joint disease, rheumatoid arthritis, bone tumors and traumatic lesions. The prostheses contain such materials as titanium, cobalt and chromium. We describe a patient who, after total hip arthroplasty, developed an inguinal-pelvic mass. Fine needle aspiration revealed metallic particles, also seen on light microscopy in reactive pelvic lymph nodes. Ultrastructure was consistent with the presence of foreign particles, while energy dispersive x-ray microanalysis established the presence of chromium. To our knowledge, this is the first report of chromium-related lymph node metallosis diagnosed by fine needle aspiration. CASE: Eight years after total hip arthroplasty, a 78-year-old woman developed a right pelvic cystic mass. Aspiration drainage was performed. Smears from fine needle aspiration showed numerous macrophages with abundant, foamy cytoplasm and round nuclei without atypia. Small, birefringent particles were seen in the cytoplasm. Histopathology showed fibroconnective tissue with chronic inflammation and marked lymph node sinus histiocytosis. Within histiocytes, numerous particles were present, identical to those seen in the smears. Their nature as "foreign bodies" was confirmed by electron microscopy, and the presence of chromium was shown by energy dispersive x-ray analysis. CONCLUSION: Fine needle aspiration and polarized microscopy are excellent techniques to evaluate foreign materials in lymph nodes draining the sites of joint prostheses, thus precluding confusion with other conditions, such as metastatic carcinoma.