One hundred and twenty-two patients with multiple myeloma were classified as mature, intermediate, immature, or plasmablastic subtype according to Greipp’s criteria. Contrary to Greipp’s report, the survival time of plasmablastic myeloma was not significantly shorter than other subtypes, nor was the plasmablastic subtype identified as a poor prognostic factor. The survival time of mature plus intermediate myeloma was significantly longer than that of immature plus plasmablastic myeloma. Between the former and latter, significant differences were found for sex, clinical stage, thrombocytopenia, bone marrow plasmacytosis, renal insufficiency, bone destruction, and response rate to treatment. Therefore, it was suspected that the immature and plasmablastic subtypes were unfavorable prognostic factors in patients with multiple myeloma.
De novo acute basophilic leukemia (ABL) is a rare form of myeloid leukemia. The low prevalence of ABL makes it difficult to define its clinical characteristics and to establish an effective therapeutic protocol. We present here a case of de novo ABL in a 64-year-old Japanese man. The diagnosis of ABL depended on the following: (1) metachromasia with toluidine blue stain, (2) intracytoplasmic theta granules identified by electron microscopy, and (3) findings obtained from extensive immunophenotypic analysis. Although blast cells lacked basophil-specific antigens such as CDw17, CD88, and FcepsilonRI, an expression profile of cytokine receptors including CD116 (GM-CSF receptor), CD117 (c-kit), and CD123 (IL-3 receptor alpha) helped to define the cellular lineage in our case. The patient achieved complete remission with intensive chemotherapy composed of idarubicin and cytosine arabinoside and was disease free during the following 30 months. We propose that immunophenotyping, especially focusing on cytokine receptors, is useful in diagnosing ABL.
We report a patient with primary nonsecretory plasma cell leukemia. These cells resembled prolymphocytes and expressed only CD38 and CD56. Due to the atypical morphology and lack of monoclonal immunoglobulins in both serum and urine, it was difficult to make a correct diagnosis. Electron-microscopic and immunocytochemical cytoplasmic studies were useful.
Of 130 Japanese patients with symptomatic multiple myeloma who were treated between 1970 and 1989, nine (6.9%) patients survived for more than 10 years. Younger age, low and intermediate tumour mass, chemotherapy with cyclophosphamide, the disappearance of myeloma protein, and a positive response to retreatment were correlated with long-term survival.
Four patients with common acute lymphoblastic leukemia antigen (CALLA)-positive myeloma are presented. The subclasses of monoclonal protein were IgD kappa (1 case), IgA lambda (1 case), and IgA kappa (2 cases). Bence Jones proteinuria was seen in all cases. The clinical stages were determined as IIA (2 cases) and IIIA (2 cases). All patients died with a median survival time after diagnosis of 62 days due to rapid development of renal failure (3 cases), and renal insufficiency and pneumonia (1 case). According to light microscopic evaluation, these myelomas corresponded to plasmablastic (1 case), immature (2 cases), and intermediate (1 case) types. Both CALLA and a cytoplasmic immunoglobulin identical with the serum monoclonal protein were simultaneously detected in single cells from all cases using immunofluorescent double labeling. These findings suggest that CALLA-positive and plasma-blastic myelomas constitute clinically a subgroup characterized by extremely poor survival but they represent cytologically different subcategories.
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