Evidence Of Visceral Involvement Research Articles

Chagas disease in Latin American migrants: a Spanish challenge

Chagas’ disease affects millions in Latin America and is the leading cause of cardiomyopathy and death due to cardiovascular disease in patients aged 30–50 years. As a consequence of immigration it has settled in several European countries, where besides imported cases, autochthonous infections arise through vertical transmission and blood/organ donation. All Latin American immigrants who attended our Unit were screened for T. cruzi infection (ELISA and IFAT ± PCR). An ECG and echocardiogram were requested for all positive patients, and oesophageal manometry, barium swallow and barium enema were requested according to patient symptoms. All patients under 50 years without severe cardiac involvement and who had not received correct treatment previously were treated with benznidazole 5 mg/kg/day for 60 days. Patients were followed-up with serology and PCR 1 month after treatment ended and every 6 months thereafter. A total of 1146 Latin Americans were screened for T. cruzi (357 positive serology results). The typical patient profile was a Bolivian female, of rural origin, in her fourth decade of life, without evidence of visceral involvement. Treatment tolerance was poor, with 29.7% discontinuing treatment due to adverse reactions. Among those with adverse reactions (52%), the most frequent were cutaneous hypersensitivity (68.7%), gastrointestinal upset (20%) and nervous system disturbances (16.2%). T. cruzi infection is no longer limited to Latin America. Poor treatment tolerance can limit current treatment options. More epidemiological data are necessary to estimate the magnitude of a problem of great relevance for public health and health resource planning.

The Classification and Typing of Amyloid Deposits

The diagnosis of amyloidosis, unlike many other diagnoses that fall on the shoulders of the pathologist, is based on a simple tinctorial property. Binding of the cotton wool dye Congo red, demonstrating green birefringence when viewed under polarized light, fulfills all diagnostic criteria for amyloid. Reporting amyloidosis in a pathologic specimen is reminiscent of a histologic diagnosis of “lymphoma.” The demonstration of amyloid in and of itself is not particularly helpful to the clinician, and an accurate determination of the type of amyloid is just as important as recognizing the presence of amyloid. The clinician will have many questions for the pathologist. Is the amyloid systemic or localized? Is the amyloidosis primary, secondary, or inherited? The former question is important because the majority of patients with localized disease do not require systemic therapy and the long-term prognosis is excellent. The latter question is important because the prognoses for the 3 forms of systemic amyloid and the treatment of the 3 disorders are vastly different. One often involves chemotherapy and stem cell transplantation; another might lead to liver transplantation. Accurate classification becomes imperative. Some forms of localized amyloid are light chain–derived, but the light chain production is local and not part of a systemic plasma cell dyscrasia.1 Localized forms of amyloid might be suspected because of the organ involved. When deposits of amyloid are found in the bladder, urethra, and ureter, it is virtually always a localized process. Most cases of amyloid in the skin and conjunctivae are localized. Amyloidosis of the larynx and tracheobronchial tree is not a systemic disorder, although it may cause troublesome complications. Amyloid deposits in the atria of the heart, pleura, and the articular cartilage generally are localized, and the diagnosis of systemic amyloidosis should not be made unless there is other evidence of visceral involvement. The majority of patients seen in the United States with systemic amyloid have immunoglobulin light chain amyloid (AL) or primary type. The origin of the fibril is an immunoglobulin light chain or immunoglobulin heavy chain fragment. Virtually all patients have a demonstrable clonal plasma cell dyscrasia, and this fact can be used to advantage in classifying the amyloid. These patients will have a detectable monoclonal light chain in the serum or urine shown by immunofixation or an elevation of the immunoglobulin free light chain level revealed by nephelometry.2 Bone marrow examination will demonstrate clonal plasma cells as the source of the light chain production.3 The finding of a monoclonal protein is helpful, but it is not sufficient to diagnose AL. Occasionally a patient will have an incidental monoclonal gammopathy of undetermined significance and a form of amyloid that is not immunoglobulin light chain in origin.4 Finding amyloid deposits on biopsy in the presence of monoclonal immunoglobulins is strong but not conclusive evidence of AL. Clinically, it is difficult to distinguish primary, secondary, familial, and senile forms of amyloid from one another. In the Mayo Clinic (Rochester, MN) experience, half of the patients with inherited amyloidosis do not have a positive family history. Patients with secondary amyloidosis typically have nephrotic syndrome. Renal biopsies demonstrate amyloid deposits. These patients also might have significant gastrointestinal involvement. Patients with hereditary systemic amyloidosis can have renal involvement

AIDS‐Related Malignancies

OVERVIEW OF MALIGNANCY IN PATIENTS WITH AIDS: Despite the advent of highly active antiretroviral therapy, the incidence of human immunodeficiency virus (HIV)-associated malignancies has not decreased. The United States Centers for Disease Control (CDC) has determined that Kaposi's sarcoma, non-Hodgkin's lymphoma (including primary central nervous system lymphoma), and cervical carcinoma define the acquired immune deficiency syndrome (AIDS). Some literature reports include Hodgkin's disease, anal carcinoma, lung cancer, and non-melanomatous skin cancers as ones commonly found in people infected with HIV. The oncologist is further challenged treating a patient with malignancy whose malignancy is complicated by a concurrent compromised marrow function. This article will review the clinical presentation and current treatment for the HIV-associated malignancies and selected other tumors in the HIV-infected patient. Furthermore, HIV-associated myelosuppression is a common problem in antiviral-undertreated or antiviral-resistant patients.

Infantile Myofibromatosis: Report of Two Cases

Two cases of solitary type infantile myofibromatosis (IM) were presented. Case 1 was a 6-month-old male infant with a firm, dark red, fixed tumor on his right knee. A computerized tomographic scan revealed that the tumor was attached to the underlying muscle. Case 2 was a 1-month-old male infant with a tumor on his right knee, similar to that in case 1. In neither case was there any clinical evidence of visceral involvement. Histological and immunohistochemical findings were similar. The lesions appeared histologically as non-encapsulated nodules composed of whorled fascicles of spindle-shaped cells and a vascular element with a hemangiopericytoma-like appearance. The tumor cells were positively stained with PTAH. They were positive for alpha-smooth muscle actin and vimentin, but negative for desmin. These findings support the myofibroblastic nature of IM. In case 2, the tumor regressed spontaneously at the age of 12 months. Unlike the multicentric form, spontaneous regression of the solitary form of IM has not previously been reported.

Multicentric Malignant Fibrous Histiocytoma of Soft Tissue

We present an unusual case of multicentric malignant fibrous histiocytoma of soft tissue. Over the past 24 years, a 67-year-old woman developed malignant fibrous histiocytoma in four different soft-tissue sites. There were two local recurrences of one of the tumors. Currently, there is no evidence of visceral involvement. The evidence suggests that this patient has a predisposition of the connective tissue to develop malignant tumors of fibrohistiocytic differentiation.

Cutaneous cryptococcosis without evidence of systemic involvement

As many as 15% of cases of systemic infection with Cryptococcus neoformans have cutaneous involvement. The existence of primary cutaneous disease is controversial. We report a patient with cutaneous cryptococcosis without evidence of visceral involvement at the time of diagnosis. She has been followed up for 5 years and remains free of systemic involvement. Cutaneous cryptococcosis does not always signify systemic disease.

Infantile myofibromatosis.

The clinical and pathologic features of 61 cases of congenital and infantile myofibromatosis are presented. The tumor affected almost exclusively infants and young children; 88% of cases occurred before the age of two years, and 60% were noted at birth or shortly thereafter. Solitary (45 cases) and multicentric (16 cases) forms of the tumor could be distinguished; the solitary form was more common in males (69%) and affected chiefly the soft tissues of the head-neck region and the trunk. The multicentric form, on the other hand, predominated in females (63%) and was found not only in soft tissues but also in bones and viscera. Fourteen of the 16 cases with multicentric lesions were present at birth, and four of them had evidence of visceral involvement. Among the 16 cases were two sets of siblings, both brother and sister. Grossly, nearly all of the tumors were well demarcated; they measured from 0.5 to 7 cm in greatest diameter. Microscopically, they formed well-circumscribed nodules consisting of short bundles of plump, spindle-shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. Necrosis or a hemangiopericytoma-like pattern was often found in the center of the tumor nodules. Intravascular growth was encountered in several instances, but this feature did not seem to affect the clinical behavior. Of the 43 patients with follow-up data (mean-follow up 5.1 years), 36 were alive and well. Of the solitary lesions, three recurred. Of the multicentric lesions, five of the 15 patients showed evidence of spontaneous regression of both soft tissue and bone lesions. Four died, three of extensive involvement of multiple viscera, especially the lung, and one of "crib-death."

Sporotrichoid Cutaneous Infection due to Mycobacterium chelonei

Extensive cutaneous lesions that simulated sporotrichosis developed in a patient. Mycobacterium chelonei, a facultative pathogen that exists as a saprophyte in the environment and rarely produces clinical disease in humans, grew from culture of biopsy material. There was no evidence of visceral involvement, and the lesions healed spontaneously within six months.

Arteriographic appearances in rheumatoid arthritis and other disorders

1. Brachial arteriography has been performed on 67 patients; 42 with rheumatoid arthritis, ten with primary Raynaud's phenomenon and a number with other disorders, including three with polyarteritis nodosa. 2. In the patients with rheumatoid arthritis two types of vascular lesion were demonstrated: occlusion and hyperaemia. 3. Occlusive lesions were often associated with clinical evidence of digital ischaemia, subcutaneous nodules, a positive D.A.T., peripheral neuropathy and evidence of visceral involvement. They are more common in digits affected by erosive bone disease and were relatively commoner in men. No relation could be found between occlusive lesions, the age of the patient, the duration of rheumatoid arthritis or previous steroid therapy. 4. Hyperaemic lesions appear to represent vascular synovium in joint and tendon sheath or granulation tissue in bone erosions or cysts. 5. Many of the patients with Raynaud's phenomenon showed occlusive lesions, especially when associated with some other disease. 6. Of special interest was the arteriographic appearance in three patients with polyarteritis nodosa, showing, in addition to vascular occlusion, a network of irregular, tortuous, abnormal arteries, a pattern which may be characteristic of the condition.