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The etiology of chronic lymphocytic leukemia (CLL) remains unknown, though a genetic susceptibility has been suggested. Results of complete DNA typing of HLA alleles in CLL patients are lacking. We compared HLA class I and class II frequencies in 101 German CLL patients and 157 healthy German controls as determined by PCR-SSP/SSO DNA analysis and serologic typing. The most striking difference was the increased frequency of HLA-DRB4*0103 [relative risk (RR) = 2.74, p < 0.0025] among patients. The presence of alleles HLA-DRB1*0401, HLA-DQB1*0302 and HLA-DPB1*0301 as well as of homozygosity for HLA-DQB1 was also associated with a higher risk for CLL, though none of these differences remained significant after correction for multiple comparisons. No association was found for any HLA class I allele. Haplotype analysis revealed a CLL-specific linkage disequilibrium for HLA-DRB1*0401:DRB4*0103 and HLA-DRB4*0103:DQB1*0302. Our results suggest that CLL could be associated with distinct class II alleles of the Caucasian haplotype HLA-DR4:DR53:DQ8, which has also been related to a susceptibility for several auto-immune diseases. The positive, though weak, association of CLL with HLA-DPB1*0301 might represent an independent susceptibility factor since no linkage disequilibrium existed with any of the other CLL-associated alleles. None of the previously reported associations with HLA class I antigens was confirmed. Our results suggest that factors within or close to the human MHC class II region confer susceptibility to CLL. © 2001 Wiley-Liss, Inc.

The etiology of chronic lymphocytic leukemia (CLL) remains unknown, though a genetic susceptibility has been suggested. Results of complete DNA typing of HLA alleles in CLL patients are lacking. We compared HLA class I and class II frequencies in 101 German CLL patients and 157 healthy German controls as determined by PCR-SSP/SSO DNA analysis and serologic typing. The most striking difference was the increased frequency of HLA-DRB4*0103 [relative risk (RR) = 2.74, p0.0025] among patients. The presence of alleles HLA-DRB1*0401, HLA-DQB1*0302 and HLA-DPB1*0301 as well as of homozygosity for HLA-DQB1 was also associated with a higher risk for CLL, though none of these differences remained significant after correction for multiple comparisons. No association was found for any HLA class I allele. Haplotype analysis revealed a CLL-specific linkage disequilibrium for HLA-DRB1*0401:DRB4*0103 and HLA-DRB4*0103:DQB1*0302. Our results suggest that CLL could be associated with distinct class II alleles of the Caucasian haplotype HLA-DR4:DR53:DQ8, which has also been related to a susceptibility for several auto-immune diseases. The positive, though weak, association of CLL with HLA-DPB1*0301 might represent an independent susceptibility factor since no linkage disequilibrium existed with any of the other CLL-associated alleles. None of the previously reported associations with HLA class I antigens was confirmed. Our results suggest that factors within or close to the human MHC class II region confer susceptibility to CLL.

A case of 75 year-old man is reported, who died of a tuberculous leptomeningomyeloencephalitis complicating a generalized primary lymph node plasmacytoma. The malignant lymphoma has been diagnosed in a lymph node biopsy from the right supraclavicular region seven years before the death and was treated with cytostatics over a period of four and a half months. Autopsy revealed the neoplastic involvement of supraclavicular and axillary lymph nodes, tumour infiltration of the sternum and diffuse neoplastic plasmacytosis of the bone marrow. The lymph node plasmacytoma has to be differentiated from reactive lymph node plasmacytosis, angioimmunoblastic lymphadenopathy with excessive plasmacytosis, lymphoplasmacytic immunocytoma, metastasis of an extramedullary plasmacytoma or of a multiple myeloma, and from a lymph node involved by plasma-cell leukemia.

Clinical data and courses of the disease of 56 patients with non-Hodgkin-lymphomas of high malignancy were demonstrated. The age-depending summit of the frequency was between the 51st and 60th year of age, the age median was about 48 years. The initial remission rate three months after the beginning of the therapy was 66%. Out of the responders in 44% relapsed, in which cases 70% of the relapses developed in the first year after the beginning of treatment. After twelve months the survival rate was 0.52 and after 48 months 0.32. Patients with initial remission, with localized stages I and II (Ann Arbor) as well as with primarily extranodal manifestation and with histology of centroblastoma had a clear prognostic advantage. Initial B-symptomas, an advanced stage of the disease and a histology of immunoblastoma and lymphoblastoma as well were negatively correlated to the prognosis.