Unusual presentation of extranodal peripheral T-cell lymphomas with multiple paraneoplastic features

Article Abstract:

Most commonly, tumors and cancers of different sorts exert their insidious influence on the basis of sheer size. When the mass is large enough, it blocks normal function and consumes the body's resources. However, some tumors cause paraneoplastic syndromes. These are metabolic and hormonal changes which occur in response to chemical influences of the tumor rather than its size or location. Three cases are presented in which patients with T-cell lymphoma had distinctive paraneoplastic symptoms. Between 5 and 20 percent of all lymphomas derive from T cells; the determination of cell lineage in cases of lymphoma is now easier and more accurate due to the availability of specific antibodies. In the present cases, the patients were diagnosed after developing symptoms including fever, weight loss, liver failure, and a tendency toward bleeding. One patient developed rhabdomyolysis, which is the appearance in the blood of proteins from disintegrating muscle cells, and another patient developed an intestinal abnormality which prevented the digestion of wheat flour (gluten). A bleeding abnormality is not common among lymphoma patients in general. It almost certainly results from the liver failure, and yet the livers of these patients were not found to be crowded with T cells. Apparently, the mere presence of the T cells was sufficient to cause liver failure; this effect may be caused by some substance secreted by the cells. Rhabdomyolysis has not been previously reported in any patient with T-cell lymphoma, and in the present case it could be confirmed that the muscles were not directly affected by a mass of T cells. It should be noted that the present cases did not have the lymph node abnormalities which are common in lymphoma. When a patient is seen with liver failure, fever, weight loss and paraneoplastic symptoms, lymphoma should be considered as a possible diagnosis. If the diagnosis of lymphoma is delayed, the progressive liver failure and secondary complications such as kidney failure may make effective treatment of the lymphoma impossible. (Consumer Summary produced by Reliance Medical Information, Inc.)

Paraneoplastic syndromes, Physiological aspects, Lymphomas

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An atypical myeloproliferative disorder with high thrombotic risk and slow disease progression

Article Abstract:

The chronic myeloproliferative disorders (CMD) represent a group of diseases which have in common the abnormal proliferation of some cell contributing to the blood. All are thought to arise when a single cell in the bone marrow goes awry and multiplies without adherence to its original 'plan'. If the cell is a precursor of red blood cells, then polycythemia vera is the result. If the precursor cell is a megakaryocyte, which forms platelets (thrombocytes), then idiopathic thrombocythemia is the result. Other myeloproliferative syndromes include myelofibrosis with myeloid metaplasia and chronic myelogenous leukemia. While these classes represent many of the cases observed in the clinic, there are intermediate forms which share features of two typical classes. Furthermore, there seem to be variant forms which have features of chronic myeloproliferative disorders but are not immediately recognizable as such. The authors describe the important features of 18 cases that failed to satisfy the diagnostic criteria for the typical forms of CMD. All 18 cases occurred in patients younger than 46 years of age. In 11 cases, the patients had signs such as enlarged spleen, excessive bone marrow growth, and a mild increase of platelets or white blood cells in the blood. Rare immature bone marrow cells appeared in the blood of some of the 11 patients. In the other seven patients, a diagnosis of CMD was made only after an enlarged spleen was removed and blood clotting complications began. In two cases, these complications were fatal. Fifteen patients are alive and appear stable; in 12 chemotherapeutic treatment to kill the renegade cells has not proved necessary. These cases illustrate the features of a slowly progressing chronic myeloproliferative disorder affecting younger patients. They also illustrate the dangers of removing an enlarged spleen when the cause of the splenomegaly has not been determined. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Myeloproliferative disorders, Thrombosis, Splenomegaly

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