Localized primary (AL) amyloid tumor of the breast. Cytologic, histologic, immunocytochemical and ultrastructural observations

Two examples of localized primary amyloid tumor of the breast are presented, including one patient with metachronous bilateral lesions. Our findings and review of the literature indicate that this rare lesion occurs predominantly in elderly females and can be mammographically and clinically confused with carcinoma. Fine-needle aspiration biopsy can be a useful procedure to make a preliminary diagnosis. Congo red staining with prior potassium permanganate incubation confirmed the AL type of amyloid in our two cases; this might be the predominant type in the localized form involving the breast. Immunofluorescence studies demonstrated IgA, with kappa and lambda light-chain deposition within the amyloid foci in one case, and intracytoplasmic IgG with both light chains within plasma cells and amyloid deposits of the second case. Ultrastructural examination of one of the cases showed characteristic findings of straight, nonbranching fibrils of 4-9 nm, diagnostic of amyloid. From our findings and a review of the literature, we conclude that amyloid tumors of the breast can occur in three separate settings: secondary amyloidosis, systemic or multiple myeloma associated amyloidosis, and as a localized primary type having a benign course.     

Sensitivity and specificity of clinical case definitions for pertussis

We evaluated the diagnostic performance of 15 clinical case definitions for pertussis in 233 patients who developed acute respiratory illness during community outbreaks in Wisconsin and Delaware. Using results from culture (Regan-Lowe media) and serology (Ig-class-specific ELISA) as diagnostic standards, cough for greater than or equal to 14 days was both sensitive (84 per cent-92 per cent) and specific (63 percent-90 per cent) in identifying patients with pertussis. This definition may be useful in monitoring pertussis outbreaks and for investigating contacts of culture-positive cases.     

Chronic lymphocytic leukemia presenting in the marrow of a phalanx

Systemic disease may present first in the foot. The authors present a case in which the marrow of resected bone tissue from a phalanx showed the changes of chronic lymphocytic leukemia (CLL). Subsequent blood examination confirmed this diagnosis.     

Head and neck malignancies associated with HIV infection.

Immunosuppressed persons are at greater risk of developing malignancies. In human immunodeficiency virus (HIV) immunosuppression the most common oral cancers are Kaposi's sarcoma and non-Hodgkin's lymphoma. Squamous cell carcinoma has also been reported to be associated with HIV disease. Kaposi's sarcoma is the most frequent neoplastic disease in acquired immunodeficiency syndrome and is by far the most common in the head and neck area. This article reviews the prevalence, clinical features, and management of these diseases in HIV infection.     

Multicenter Pilot Study of a Serpentine Balloon-Expandable Stent (beStentTM): Acute Angiographic and Clinical Results

The beStent is a new stainless steel, balloon-expandable mesh stent which has a unique serpentine design. Rotation of the unique low stress junctions upon expansion leads to orthogonal locking of the wires, maximizing radial strength and assuring zero shortening. The stent has delineating gold markers which assure precise positioning. We aim to present the initial acute results in a pilot registry for stent evaluation. Two hundred eighty-four stents were used in a total of 217 patients (age 57.9 ± 3.10 years; 178 males; 39 females) in seven centers, for variable indications. Stents of 15-, 25-, and 35-mm length were used. The arteries treated were the left anterior descending (n = 112, 42%), circumflex (n = 54, 20.2%), right coronary (n = 95, 35.5%), left main (n = 1, 0.4%), and vein graft (n = 5, 1.9%). Lesion types were: A in 42 patients (16.5%); B1 in 53 patients (20.7%); B2 in 81 patients (31.8%); and C in 79 patients (31%). One hundred fifty-nine patients required one stent, 40 patients required two stents, and 18 patients required three or more stents. Anticoagulation protocol included procedural heparin with aspirin with/without ticlopidine. Smooth angiographie results were obtained in all cases with no plaque herniation. Acute angiographic success was obtained in 97% of the patients, and acute clinical success in 95% of the patients. Complications within 30 days were: 3 deaths (1.4%) (2 noncardiac); 2 (0.9%) myocardial infarctions; and 2 (0.9%) stent thromboses. Therefore, the beStent is useful in treatment of complex lesions of variable length and complexity, providing excellent acute results with a low complication rate, in spite of unfavorable basic clinical and angiographie characteristics.     

The epidemiology of Alzheimer disease in intellectual disability: results and recommendations from an international conference

Among adults with intellectual disability, virtually everyone with Down's syndrome (DS) over the age of 40 years has neuropathology currently viewed to be consistent with a diagnosis of Alzheimer disease (AD), while other adults with intellectual disability without DS display an increased prevalence of Alzheimer-type neuropathology after they reach the age of 65. This paper presents the results of discussions by an epidemiology workgroup, formed at an international conference convened to discuss AD among people with intellectual disability, concerning: (I) the incidence and prevalence of clinical dementia in adults with intellectual disability; (2) risk factors for the development of AD in adults with intellectual disability; and (3) a minimum data set that would be of great utility for future research on AD in adults with intellectual disability.