The "dural tail sign": not always a meningioma.

Periorbital masses are often referred to oculoplastic surgeons. We report a 20-year-old patient presenting with a tender supertemporal mass that on gadolinium-enhanced magnetic resonance imaging (MRI) demonstrated a prominent dural enhancement adjacent to the mass, the so-called "dural tail sign." This sign has been reported to be highly specific for a meningioma; however recent literature challenges this view. In this case as well, the "dural tail sign" was not produced by a meningioma.

Leukemic thyroiditis as the initial relapsing sign in a patient with acute lymphocytic leukemia and blast expression of the neural cell adhesion molecule.

We report a patient with a history of T-cell ALL in remission who presented with symptoms and laboratory values consistent with subacute thyroiditis but was found to have leukemic thyroiditis as the first clinical manifestation of leukemic relapse. Bone marrow examination at this time demonstrated recurrent ALL. After successful re-induction with chemotherapy and an allogeneic bone marrow transplant this patient developed an isolated recurrence of her ALL manifested by symptomatic thyromegaly and a new mediastinal mass that was treated with irradiation. Despite no medullary recurrence of ALL, the patient developed pleuritic chest pain and shortness of breath and succumbed to pericardial extramedullary leukemia 9 months later. This to our knowledge is the third reported case of symptomatic ALL involvement of the thyroid gland and the first to be confirmed histologically. Furthermore, this patient had blast expression of the neural cell adhesion molecule (CD56), a cell surface marker that has not been studied in ALL but has previously been identified as a risk factor for extramedullary leukemia (EML) in acute non-lymphocytic leukemia. The authors hypothesize that CD56 expression in this patient might have contributed to her predisposition to EML.

Extramedullary myeloid cell tumors in myelodysplastic-syndromes: not a true indication of impending acute myeloid leukemia.

The purpose of this report is to record a patient with myelodysplastic syndrome (MDS) associated acute myelogenous leukemia (AML) and leukemia cutis who had blast expression of the neural cell adhesion molecule (NCAM) and to review the world literature on prognostic implications of extramedullary myeloid cell tumors (granulocytic sarcoma, myeloblastoma, chloroma and leukemia cutis) in MDS and MDS associated AML. Case report and world literature from January 1965-January 1994 for all cases of MDS-associated extramedullary myeloid cell tumors (EMT) is reviewed, and the first patient with EMT, MDS associated AML and blast expression of NCAM is described. There have been 46 cases of MDS associated EMT previously reported. 32 cases occurred in the absence of AML. AML developed in 47% of these patients at a mean of 38 weeks from initial EMT. Of the patients not developing AML, median survival from initial EMT was 11 weeks. Nine patients received chemotherapy at the time of EMT and had a median survival of 36 weeks. The median survival for patients receiving conservative therapy for EMT was 48 weeks. Patients (n = 15) with EMT and MDS associated AML had a poor outcome regardless of therapy with a median survival of 11 weeks. Unlike other forms of isolated EMT, MDS associated EMT is not always a forerunner of AML. Premature induction therapy for MDS associated EMT does not appear to prolong survival. EMT in the setting of MDS associated AML is associated with a poor prognosis despite aggressive chemotherapy. Blast expression of NCAM may prove to be a risk factor for EMT in MDS associated AML.

A flow cytometric method for phenotyping recipient red cells following transfusion.

BACKGROUND: Reticulocyte phenotyping is used for transfused patients, who have red cell antibodies, to match blood for subsequent transfusion. Current methods are labor-intensive and require a significant amount of sample. STUDY DESIGN AND METHODS: A simple dual-color flow cytometry method developed for antigen typing of reticulocytes in mixed red cell populations is reported. Antigens were labeled by an indirect immunofluorescence technique using undiluted reagent sera as the primary label, biotinylated goat anti-human IgG as the secondary label, and avidin-phycoerythrin as the fluorescent stain. Reticulocytes were labeled with a thiazole orange fluorescent stain. Reticulocyte identification and antigen typing were performed on 319 samples to establish the validity of the procedure. Mixed red cells were prepared in all possible c antigen combinations to simulate transfusion concentrations of 25, 50, and 75 percent. RESULTS: The anti-c flow cytometry profiles readily distinguished between antigen-positive and antigen-negative populations and allowed the detection of reticulocytes at all simulated transfusion concentrations. Similar results were obtained in experiments using C, K, s, Fya, Fyb, Jka, or Jkb sera against equal volumes of antigen-positive and -negative cells. Anti-S gave inconsistent results. The in vitro results were confirmed in 19 transfused patients who had received red cells antigenically different from their own as well as cells from 1 chimera blood donor. CONCLUSION: This method provides a simpler, safer, less labor-intensive, and less subjective technique requiring far less sample volume than current methods for antigen typing of reticulocytes in mixed red cell samples from recently transfused patients.

Respiratory failure in rapidly progressing pulmonary lymphoma. Role of immunophenotyping in diagnosis.

An asymptomatic man was found to have bilateral small pulmonary infiltrates on a preoperative chest roentgenogram. Over a 4-wk period there was rapid progression of the infiltrates with a clinical picture suggestive of adult respiratory distress syndrome. Open lung biopsy showed a high grade lymphoma filling and distorting the pulmonary parenchyma. Flow cytometry of pleural fluid showed an aberrant phenotype consistent with T cell lymphoma. The patient died of progressive respiratory failure 6 wk after the first radiographic abnormalities were detected. With further experience, immunophenotyping of pleural fluid may secure definitive diagnoses in certain clinical situations, obviating more invasive procedures.