Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 65-year-old male with rapidly progressive Lennert's lymphoma terminating in fulminant hepatic failure is presented. Staging radiological studies revealed that he had cervical and mediastinal lymph node swellings and multiple nodular lesions in the spleen. Lymph node biopsy specimens showed the proliferation of epithelioid cells interspersed with large blastic lymphocytes. These lymphocytes were CD3+, CD45RO (UCHL-1) +, CD4-, CD8+, CD56-, CD30-, CD15-, T-cell intracellular antigen-1+, granzyme B+ and perforin+, suggestive of the cytotoxic T-cell lineage. Under the diagnosis of Lennert's lymphoma, he was treated with standard CHOP chemotherapy. After two courses of the chemotherapy, despite the decreased size of cervical lymph nodes, high-grade fever and constitutional symptoms appeared. As multiple low-density nodules were observed in the liver by computed tomography, needle biopsy was performed. The biopsy specimens showed the proliferation of CD3+, CD4- and CD8+ lymphoma cells. Thereafter, the liver function deteriorated rapidly, and disseminated intravascular coagulation emerged. He died of rapidly progressive hepatic failure. This case is another example demonstrating that at least some of the Lennert's lymphomas phenotypically correspond with cytotoxic T-cell lymphomas, as was previously suggested by us [Am. J. Surg. Pathol. 24 (2000) 1627]. It should be also emphasized that Lennert's lymphomas containing cytotoxic proteins may have a fulminant clinical course, which cannot be rescued by the conventional chemotherapy.
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PMID:Rapidly progressive Lennert's lymphoma terminating in fulminant hepatic failure. 1295 36

The present article describes three cases of Lennert's lymphoma exhibiting aggressive clinical courses. These cases were accompanied by disseminated intravascular coagulation (DIC) or hemophagocytic syndrome (HPS). These cases were compared to non-aggressive type of Lennert's lymphoma. Of the three cases, two demonstrated involvement of the liver and the other possessed bone marrow involvement. In one patient, while a lymph node biopsy revealed Lennert's lymphoma histologically, a liver biopsy obtained 2 months later revealed a high-grade large cell cytotoxic T-cell lymphoma. Two of these cases showed HPS and the other exhibited DIC. All patients died within 1 year of diagnosis, with the shortest survival period being 1.5 months. Immunohistochemically, lymphoma cells were CD8+, CD4-, granzyme B+, and T-cell intracellular antigen-1 (TIA-1)+, showing a cytotoxic T-cell phenotype. Two cases demonstrated positive reactivity for Epstein-Barr virus in lymphoma cells by in situ hybridization. These cases were compared with eight cases of non-aggressive Lennert's lymphoma. In comparison to non-aggressive disease, these three cases displayed a higher percentage of Ki-67-positive cells. In conclusion it was found that a subset of Lennert's lymphoma cases share common features with high-grade cytotoxic T-cell lymphoma, indicating that Lennert's lymphoma may be part of the spectrum of cytotoxic T-cell lymphoma.
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PMID:Aggressive Lennert's lymphoma: report of three cases in comparison to non-aggressive Lennert's lymphoma. 1618 92