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)

We studied 11 cases of nodal cytotoxic T-cell lymphoma, which express the CD8+ phenotype and cytotoxic molecules (T-cell intracellular antigen-1, granzyme B and perforin), to characterize the clinicopathologic spectrum of these neoplasms. The 11 cases consisted of four men and seven women, aged 5 to 82 years (mean, 53 years). All cases were nodal, and eight of 11 had extranodal involvement, the most common being in bone marrow (eight cases) and liver (six cases). The expression of these cytotoxic molecules has been reported in some T/natural killer cell lymphomas mostly involved in extranodal sites of skin, nasopharyngeal region, or gastrointestinal tracts, but these types of extranodal involvement were rare in our cases. Morphologically these lymphomas could be divided into two groups. One group (n = 6) showed a diffuse large cell type and massive necrosis or apoptosis that was accompanied by disseminated intravascular coagulation (DIC) or hemophagocytic syndrome (HPS) on the initial. The prognosis of this group was generally poor (survival = 1-19 months, median = 5 mo), and four of these six cases were fulminant. The other group (n = 5) showed a diffuse medium or mixed cell type, and the prognosis was not so poor (median survival = 17 mo). Our results suggest that these nodal cytotoxic T-cell lymphomas originated from activated cytotoxic T-cells and were highly accompanied with DIC or HPS.
 ...
PMID:Nodal CD8 positive cytotoxic T-cell lymphoma: a distinct clinicopathological entity. 1242 91

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.
 ...
PMID:Rapidly progressive Lennert's lymphoma terminating in fulminant hepatic failure. 1295 36