Assuntos

RESUMO

A 57-year-old woman consulted for a severe xanthelasma followed by a severe and complex systemic disease and died ten years later with xanthomas extended to the face and trunk. Her lipid and cholesterol levels were normal but HDL was below normal values. Diabetes as well as amyloidosis, paraproteinemia and Bence Jones protein in urine were not found. Three years after her initial consultation monocytosis appeared in most but not all of her hemograms. Soon after her spleen was palpable and became very large. She had three bone marrow biopsies showing progressive white cell hypercellularity that was considered as possibly reactive. During her long illness she had recurrent conjunctivitis and systemic complications such as arthritis, pleural and pericardial effusions, vasculitis, sudden deafness with Ménière-like vertigo, erythema nodosum and myositis that responded, at least partially, to steroid treatments though she remained corticoid dependent in order to control her more severe symptoms. Those complications were considered to be probably due to vasculitis or immune phenomena. After a long follow up it was clear that her disease was better classified as diffuse plane xanthoma (DPX) related to a myeloproliferative syndrome and vasculitis. Her final hospitalization was due to a severe esophageal moniliasis and she died a few days later. An autopsy was performed and a chronic myelomonocytic leukemia was the final diagnosis. The ultimate cause of death was a perforated acute duodenal ulcer with peritonitis. Possible relations between DPX, immunologic reactions and myelomonocytic leukemia are discussed.

Assuntos

RESUMO

A 57-year-old woman consulted for a severe xanthelasma followed by a severe and complex systemic disease and died ten years later with xanthomas extended to the face and trunk. Her lipid and cholesterol levels were normal but HDL was below normal values. Diabetes as well as amyloidosis, paraproteinemia and Bence Jones protein in urine were not found. Three years after her initial consultation monocytosis appeared in most but not all of her hemograms. Soon after her spleen was palpable and became very large. She had three bone marrow biopsies showing progressive white cell hypercellularity that was considered as possibly reactive. During her long illness she had recurrent conjunctivitis and systemic complications such as arthritis, pleural and pericardial effusions, vasculitis, sudden deafness with MéniÞre-like vertigo, erythema nodosum and myositis that responded, at least partially, to steroid treatments though she remained corticoid dependent in order to control her more severe symptoms. Those complications were considered to be probably due to vasculitis or immune phenomena. After a long follow up it was clear that her disease was better classified as diffuse plane xanthoma (DPX) related to a myeloproliferative syndrome and vasculitis. Her final hospitalization was due to a severe esophageal moniliasis and she died a few days later. An autopsy was performed and a chronic myelomonocytic leukemia was the final diagnosis. The ultimate cause of death was a perforated acute duodenal ulcer with peritonitis. Possible relations between DPX, immunologic reactions and myelomonocytic leukemia are discussed.

Assuntos

RESUMO

A 73 year old retired truck driver and blacksmith was studied in June 1996 for thoracic pain and was diagnosed as acute pericarditis which responded well to steroid treatment. In January 1997, he noted swelling of the abdominal skin, genitalia and limbs, sparing the feet. He was euthyroid, did not have evidence of diabetes or a Raynaud's phenomenon. His proteinogram showed an IgG-Kappa monoclonal paraprotein M component, 1.31 g/oo. TSH and tetraiodotironine were normal; ESR 16 mm in the first hour. As he did not respond to treatment he was referred to our hospital in March 1997. On physical examination the most relevant findings were a non-pitting edema of the abdomen and lower limbs, sparing the feet. An echocardiogram was consistent with an infiltrative cardiomyopathy. Soon after his hospitalization his condition worsened suddenly with severe bradicardia (28/minute) due to a junctional rhythm and righ bundle branch block. He suffered a cardiac arrest and died. The autopsy findings favoured the diagnosis of systemic scleredema adultorum of Buschke. Amyloid deposits were also found although not abundant, with a similar distribution except in the skin. In this article the clinical and autopsy findings are presented in a patient showing coexistence of systemic Buschke's scleredema with an infiltrative cardiomyopathy, IgG Kappa gammopathy and amyloidosis.

Assuntos

Assuntos

RESUMO

A study of 80 consecutive cutaneous malignant melanomas in 78 patients older than 60 years, with an average of 73 (60-93), is presented. The following data were considered: age, sex, localization, stage, other synchronous or metachronous malignant tumors, excluded basal cell epitheliomas, histological type and depth of cutaneous invasion according to Clark's levels and Breslow's measure. The follow up lasted 1 to 156 months with an average of 39 months. All patients were treated by surgical removal of the cutaneous lesion or the regional lymph node metastases, if they appeared. No prophylactic lymph node dissections were performed. There were 45 women and 33 men. The histological type was superficial spreading (ES) in 17 women and 7 men; nodular (NOD) in 18 and 16; lentigo maligna melanoma (LM) in 4 and 4 and acral in 7 and 7. Relations between sex, localization, histological type and actuarial survival curves can be observed in Figs. 5, 6, 7, 8 and Tables 1, 2. Even considering the shortcomings of this retrospective analysis and a relatively short follow up for melanomas, this study points out that the thickness of the lesion became prognostically significant in relation to survival, beyond 3.7 mm (p < 0.001); but when the tumors were thinner, this group of patients showed other causes of death (MPOC) with a greater frequency than those related to tumoral progression (MPM) (p < 0.001) and that maybe this should be taken into account when planning treatment for melanomas in elderly patients. Other malignant synchronous or metachronous tumors were found in 19% of the patients (Table 2).

Assuntos

RESUMO

A study of 80 consecutive cutaneous malignant melanomas in 78 patients older than 60 years, with an average of 73 (60-93), is presented. The following data were considered: age, sex, localization, stage, other synchronous or metachronous malignant tumors, excluded basal cell epitheliomas, histological type and depth of cutaneous invasion according to Clarks levels and Breslows measure. The follow up lasted 1 to 156 months with an average of 39 months. All patients were treated by surgical removal of the cutaneous lesion or the regional lymph node metastases, if they appeared. No prophylactic lymph node dissections were performed. There were 45 women and 33 men. The histological type was superficial spreading (ES) in 17 women and 7 men; nodular (NOD) in 18 and 16; lentigo maligna melanoma (LM) in 4 and 4 and acral in 7 and 7. Relations between sex, localization, histological type and actuarial survival curves can be observed in Figs. 5, 6, 7, 8 and Tables 1, 2. Even considering the shortcomings of this retrospective analysis and a relatively short follow up for melanomas, this study points out that the thickness of the lesion became prognostically significant in relation to survival, beyond 3.7 mm (p < 0.001); but when the tumors were thinner, this group of patients showed other causes of death (MPOC) with a greater frequency than those related to tumoral progression (MPM) (p < 0.001) and that maybe this should be taken into account when planning treatment for melanomas in elderly patients. Other malignant synchronous or metachronous tumors were found in 19

of the patients (Table 2).