PMID- 10637493 OWN - NLM STAT- MEDLINE DCOM- 20000202 LR - 20190915 IS - 0887-6924 (Print) IS - 0887-6924 (Linking) VI - 14 IP - 1 DP - 2000 Jan TI - Ph-negative non-Hodgkin's lymphoma occurring in chronic phase of Ph-positive chronic myelogenous leukemia is defined as a genetically different neoplasm from extramedullary localized blast crisis: report of two cases and review of the literature. PG - 169-82 AB - This report describes two cases of Philadelphia chromosome-negative (Ph(-)) non-Hodgkin's lymphomas (NHLs) recognized in patients with chronic phase Ph-positive (Ph(+)) chronic myelogenous leukemia (CML). Lymph node biopsy of patient 1 was initially diagnosed as diffuse large B cell non-Hodgkin's lymphoma (NHL, T cell rich variant), but at relapse showed immunoblastic features with a marked decrease of admixed lymphocyte components. Patient 2 presented with thickened parietal pleura which revealed a CD30-positive anaplastic large cell lymphoma showing null cell phenotype and genotype with abundant admixed neutrophils and lymphocytes. At the time of lymphoma diagnosis, the patients had CML for 33 and 10 months, respectively. DNA obtained from bone marrow cells at the time of lymphoma diagnosis showed BCR/ABL gene rearrangements by both Southern blot analysis and reverse transcription polymerase chain reaction (RT-PCR), but lacked both immunoglobulin and T cell receptor gene rearrangements. BCR gene rearrangement and BCR/ABL fusion gene were also identified in lymph node and pleural biopsies by Southern blot and RT-PCR analysis, respectively. However, both biopsy specimens also contained reactive lymphocytes and neutrophils, and no fusion signals between BCR and ABL genes were identified in the hyperdiploid lymphoma cells of either case by fluorescence in situ hybridization (FISH). These data suggest the lymphoma cells in both cases were not genetically associated with BCR/ABL. Therefore, these cases were not diagnosed as an extramedullary localized blast crisis in CML, but as Ph(-) NHLs. This represents the first definitive demonstration of peripheral B cell lymphoma occurring by a separate genetic pathway, lacking BCR/ABL, in patients with Ph(+) CML. A review of the literature identified two different subtypes of malignant lymphomas arising in patients with an antecedent or concurrent diagnosis of CML. The most common are T cell lymphomas displaying an immature thymic phenotype, while peripheral B cell lymphomas are more rare. Our study shows, however, that 'Ph(+) NHL' occurring in CML or acute lymphocytic leukemia (ALL) may represent an unrelated neoplasm, even if standard cytogenetic analysis reveals a Ph(+) chromosome, and that FISH is required to confirm whether a localized lymphoid neoplasm is either a true extramedullary localized blast crisis or genetically distinct neoplasm. Leukemia(2000) 14, 169-182. FAU - Ichinohasama, R AU - Ichinohasama R AD - Department of Oral Pathology, Tohoku University School of Dentistry, Sendai, Japan. FAU - Miura, I AU - Miura I FAU - Takahashi, N AU - Takahashi N FAU - Sugawara, T AU - Sugawara T FAU - Tamate, E AU - Tamate E FAU - Endoh, K AU - Endoh K FAU - Endoh, F AU - Endoh F FAU - Naganuma, H AU - Naganuma H FAU - DeCoteau, J F AU - DeCoteau JF FAU - Griffin, J D AU - Griffin JD FAU - Kadin, M E AU - Kadin ME FAU - Ooya, K AU - Ooya K LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Leukemia JT - Leukemia JID - 8704895 RN - 0 (DNA Primers) SB - IM MH - Aged MH - Aged, 80 and over MH - Base Sequence MH - DNA Primers MH - Flow Cytometry MH - Humans MH - Immunohistochemistry MH - Immunophenotyping MH - In Situ Hybridization, Fluorescence MH - Karyotyping MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*genetics MH - Leukemia, Myeloid, Chronic-Phase/*genetics MH - Lymphoma, Non-Hodgkin/*genetics/immunology/pathology MH - Male MH - Middle Aged MH - *Philadelphia Chromosome MH - Reverse Transcriptase Polymerase Chain Reaction RF - 56 EDAT- 2000/01/19 00:00 MHDA- 2000/01/19 00:01 CRDT- 2000/01/19 00:00 PHST- 2000/01/19 00:00 [pubmed] PHST- 2000/01/19 00:01 [medline] PHST- 2000/01/19 00:00 [entrez] AID - 10.1038/sj.leu.2401606 [doi] PST - ppublish SO - Leukemia. 2000 Jan;14(1):169-82. doi: 10.1038/sj.leu.2401606.